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Modern System of Ophthalmology [MSO] Series

Theory and Practice of

Squint and
Orthoptics
Third Edition
 Modern System of Ophthalmology [MSO] Series

Theory and Practice of

Squint and
Orthoptics
Third Edition

AK Khurana MS, CTO (London), FAICO

Senior Professor and Head
Paediatric Ophthalmology, Squint and Oculoplasty Services
Regional Institute of Ophthalmology
Pt. BD Sharma Postgraduate Institute of Medical Sciences
Rohtak, Haryana

Assisted by
Aruj K Khurana DNB, FICO, FVR, FNN
Consultant Vitreo-Retina Services
Nirmal Eye Institute
Rishikesh, Uttarakhand

Bhawana Khurana MS, DNB, FICO, FNN

Consultant Orbit, Oculoplasty and Ocular Oncology,
Paediatric Ophthalmology and Squint
Nirmal Eye Institute
Rishikesh, Uttarakhand

CBS Publishers & Distributors Pvt Ltd

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 to
My parents
and
teachers for their blessings
My wife, Indu
for her understanding
My children Aruj, Bhawna, Arushi and Gurukripa for
their love and affection
&
My grandson Agastya for providing endless joy
This Page is Intentionally Left Blank
 Preface to the Third Edition

Modern System of Ophthalmology (MSO) series comprises separate volumes on different

subspecialties of ophthalmology. Each volume is planned with a very specific aim to cater to
the needs of postgraduate students in ophthalmology.

Salient Features of MSO Series

• Each volume is edited by different editors, yet the layout and organization has been kept similar.
• Editors of different volumes are masters in their subspecialty with an uncanny knack of picking
up the right perspectives.
• Text matter is designed to meet the needs of residents in ophthalmology with a comprehensive
coverage in a concise manner. Text is complete and up-to-date with recent advances incorporated.
• Text is organized in such a way that the students can easily understand, retain and reproduce it.
Various levels of headings, subheadings, bold face and italics given in the text will be helpful
for a quick revision of the subject.
Theory and Practice of Squint and Orthoptics, a volume of Modern System of Ophthalmology
[MSO] Series, in its third edition presents an updated and thoroughly revised text in a
simplified from, so that the complex and intricated physiology of neuromuscular co-ordination
of extraocular muscles can be easily understood. This flavour of the book makes the strabismus
a fascinating interesting entity.
This book, in its third edition, continues its aim of providing information on basic principles
of squint and orthoptics to the residents in ophthalmology as well as students of orthoptics
and optometry. However, it is hoped that the book would be equally useful for the practising
ophthalmologists, optometrists and orthopticians as well. To keep it a basic book, advanced
theories and many diverse opinions have been excluded. I hope, the experienced
strabismologists and researchers in this field will bear this with me.
The text has been organized into fifteen chapters. First four chapters have been devoted to
the anatomical and physiological aspects of extraocular muscles, faculty of vision, and
binocular vision. The remaining eleven chapters have been planned to provide a clear insight
of the various disorders of ocular motility.

Salient Features of the Book

• The text has been organized in such a way that the students can easily understand, retain
and reproduce the facts. Various levels of headings, subheadings, boldface and italics given
in the text should be helpful for a quick revision of the text.
• A brief list of the contents given in the beginning of each chapter provides a clear layout of
the text.
• The text has been illustrated with high quality clear line diagrams and photographs depicting
vivid and lucid details in colour.
• The text matter in the chapters ‘Evaluation of Strabismus and Orthoptic Instruments’ and
‘Principles of Non-surgical and Surgical Management of Strabismus’ has been discussed in
length.
• Operative steps of various surgical techniques have been elucidated in a self-explanatory
manner.
 viii Theory and Practice of Squint and Orthoptics

Key Features of Third Edition

• Thorough revision of each chapter has been done to keep abreast with the newer concepts
and principles of investigative modalities, treatment modalities and surgical procedures
evolved over the period.
• The concept of ‘muscle pulley’ and ‘active pulley hypothesis’ has been explained in detail.
• Chapter on ‘Visual Acuity’ has been expanded and reorganised.
• Amblyopia Treatment Studies (ATS) have been summarized in tabulated form and the
recomendations incorporated in the management of amblyopia.
• Chapter on Nystagmus and related Ocular Oscillations, written as per new system of
classification and newer terminologies evolved by National Eye Institute’s working group
called the ’Classification of Eye Movement Abnormalities and Strabismus’ (CEMAS), has
been further simplified.
• Chapters on ‘Vertical Strabismus’ and ‘Incomitant Strabismus’ have been uniquely, classified
and organised.
The thorough revision and updating of the third edition of the book has been possible as a
result of active assitance from Dr Aruj K Khurana and Dr Bhawna Khurana and the active
criticism, suggestions and generous help received from many teachers and students from all
over India. Surely, I owe sincere thanks to them all. I must thank Dr Kanwar Mohan from
Chandigarh, Dr Kalpana and Dr Sandra from Arvind Eye Hospital, Coimbatore, and
Dr Shubhangi Bhave from Nagpur for providing valuable photographs of their patients. I
acknowledge with gratitude the special help rendered by Dr Subhash Dadeya, Director Prof
GNEC, MAMC and Chief Editor DOS Times and Dr Sonam Yangzes and Dr Savleen Kaur
from AEC, PGIMER, Chandigarh, for allowing to reproduce the Ambyopia Treatment Study
Reports. The help received from faculty memberms of RIO, PGIMS, Rohtak in general and
Dr Reena Gupta Singh, Associate Professor from Paediatric Ophthalmology Unit in particular
is highly appreciated. I also acknowledge the help rendered by Dr Amit Matrey from HIMS,
Jolly Grant and the residents RIO, PGIMS, Rohtak, especially Dr Kamal Garg. I also appreciate
Dr CS Dhull, Head RIO, Dr MC Gupta, Director, PGIMS and Dr OP Kalra, Vice-Chancellor,
UHS, Rohtak for providing the conducive environment.
The enthusiastic co-operation received from Mr SK Jain, Managing Director, Mr YN Arjuna
Senior Vice-President Publishing, Editorial and Publicity , and Ms Ritu Chawla, AGM Production,
CBS Publishers and Distributors, New Delhi, need special acknowledgement. Ms Jyoti Kaur,
DTP Operator and Mr Sanju graphic artist, need special mention because of their efforts to
provide considerable beauty to this volume.
Finally, I feel proud in expressing my sincre gratitude to my son-in-law Dr Gurukripa and
daughter Dr Arushi, Virginia Commonwealth University (VCU), Virginia, USA; and my wife
Dr Indu Khurana, Professor of Physiology and Dean-cum-Principal, World College of Medical
Sciences and Research, Gurawar, Jhajhar for their ever available love and encouragement.
Sincere efforts have been made to verify the correctness of the text. However, in spite of
the best efforts, ventures of this kind are not likely to be free from human errors, some
inaccuracies, ambiguities and typographic mistakes. Therefore, the users are requested to
send their feedback and suggestions which will be highly appreciated and duly acknowledged.
The importance of such views in improving the future editions of the book cannot be
overemphasized.
AK Khurana
 Contents ix

Preface to the First Edition

S trabismus, being an interesting subject, has always fascinated most of the ophthalmologists.
However, the extremely complex physiology and pathology of binocular vision and an
intricated neuromuscular co-ordination of the extrinsic ocular muscles have made strabismus
an untouchable entity for majority of the residents in ophthalmology as well as the practising
general ophthalmologists. Therefore, an attempt has been made in this book to present the
subject in a more easily understood form. In a bid to simplify the text, at places the description
looks more dogmatic than is warranted by the facts.
This book is intended to provide information on basic principles of squint and orthoptics
to the residents in ophthalmology and, students of orthoptics and optometry. It is hoped, the
book would be equally useful for the practising ophthalmologists and orthopticians as well.
To keep the book a basic text, advanced theories and many diverse opinions have not been
included. I hope, the experienced strabismologists and researchers in this field will bear this
with me.
First four chapters of this book have been picked up as such from the book, 'Anatomy and
Physiology of Eye'—a joint venture of mine with my wife. This would definitely extend some
relief to the students as this new text, comprising of fifteen chapters would practically get
down to eleven chapters. This becomes particularly relevant when the uncopable advances
in the field of ophthalmic technology has beset the present day residents in ophthalmology
with the voluminous texts on different aspects of the discipline. Some of the salient features
of this book are as follows:
• The text has been organised in such a way that the students can easily understand, retain
and reproduce it. Various levels of headings, subheadings, boldface and italics given in the
text should be helpful for a quick revision of the text.
• A brief list of the contents given in the beginning of each chapter provides a clear layout of
the text.
• The text has been well illustrated with high quality clear line diagrams depicting vivid
and lucid details.
• The text matter in chapters on 'Evaluation of Strabismus and Orthoptic Instruments' and
'Treatment Modalities and Outlines of Strabismus Management' has been discussed in
length. Operative steps of various surgical techniques have been illucidated in a self
explanatory manner.
Volumes like this are impossible without the contribution of several people. The significant
efforts put in by Dr. Vinod Kumar Sharma are loudly acknowledged. He has rendered an
unprecedented help in completion of this book. I am thankful to Dr. R. C. Nagpal, Prof. and
Head, Deptt. of Ophthalmology and Prof. D. S. Dubey, Director, Pt. B.D. Sharma PGIMS,
Rohtak for affording me a working atmosphere.
I am deeply indebted to my dear friend and old batchmate and a well known
strabismologist, Dr. Kanwar Mohan, Assoc. Prof., Deptt. of Ophthalmology, PGI Chandigarh,
for providing the valuable photographs of patients with palsy of third and fourth cranial
nerves (Figs 12.42 and 12.19) and Duane's retraction syndrome (Figs 12.43 to 12.45).
 x Theory and Practice of Squint and Orthoptics

It is a special pleasure to acknowledge the most assured co-operation and skill of Mr.
Mahesh, Mr.Updesh and M/s CBS Publishers & Distributors in general, and Mr. S. K. Jain,
Managing Director and Mr. V. K. Jain, Production Director, in particular. Mr. Dharamvir
deserves a special appreciation for his sincere efforts in streamlining the text. Lastly, I feel
proud in admitting that the main force behind all the little I am able to do, is my wife Dr.
(Mrs.) Indu Khurana, Assoc. Prof. in the Deptt. of Physiology, PGIMS, Rohtak. I must also
register my thanks to her for allowing me to lift the first four chapters from the book 'Anatomy
and Physiology of Eye' of which she is a co-author.
It is unequivocal that in spite of the best efforts,ventures of this kind are not likely to be
free from human errors, some inaccuracies, ambiguities and typographic mistakes. However,
a feedback and active criticism from the users is always of invaluable help in rectifying such
pitfalls. I shall eagerly await the response from all the learned readers.

AK Khurana
 Contents xi

Contents

Preface to the Third Edition vii

Preface to the First Edition ix

1. Anatomy of Extraocular Muscles and Related Structures 1

Extraocular Muscles and Orbital Fascia 1
Anatomy of Third, Fourth and Sixth Cranial Nerves 12

2. Physiology of Ocular Motility 20

Basic Kinematics 20
Mechanics of Actions of Extraocular Muscles 22
Agonist, Synergists, Antagonists and Yoke Muscles 30
Fundamental Laws Governing Ocular Motility 31
Ocular Movements 33

3. Visual Acuity and Contrast Sensitivity 37

Components of Visual Acuity 38
Measurement of Visual Acuity 38
Contrast Sensitivity 51

4. Binocular Vision 57
Binocular Vision: Definition and Grades 57
Psychophysics and Sensory Aspects of Binocular Vision 58
Development of Binocular Vision 74
Binocular Vision Tests 83

5. Strabismus: Definition, Classification and Etiology 91

Definition and Classification 91
Etiology of Strabismus: An Overview 97

6. Evaluation of a Case of Strabismus and Orthoptic Instruments including 100

Computer-Based Orthoptic Programs
Evaluation of a Case of Strabismus 100
Orthoptic Instruments 135
Computer-Based Orthoptic Programs and Instruments 145
 xii Theory and Practice of Squint and Orthoptics

7. Anomalies of Convergence, Divergence and Accommodation 152

Convergence 152
Divergence 161
Accommodation 163

8. Adaptations to Strabismus and Amblyopia 167

Sensory Adaptations 168
Amblyopia 177
Motor Adaptations 206
Psychologic Adaptations 207

9. Heterophoria 209

10. Concomitant Esotropias and Exotropias 217

Concomitant Esotropias 217
Concomitant Exotropias 239

11. Vertical Strabismus and Cyclodeviations 255

Vertical Strabismus 255
Cyclodeviations 271

12. Incomitant Strabismus 278

Vertically Incomitant Horizontal Heterotropias 279
Paralytic Squint 285
Restrictive Ocular Motility Defects 333

13. Supranuclear Control and Disorders of Ocular Motility 355

Supranuclear Control of Eye Movements 355
Supranuclear Disorders of Eye Movements 369

14. Nystagmus and Related Oscillations 376

Nystagmus 376
Nystagmus-Like Ocular Motor Oscillations 398

15. Principles of Non-Surgical and Surgical Management of Strabismus 400

Non-Surgical Management 400
Surgical Management 406
Outlines of Strabismus Management 448

Index 459
 1
Anatomy of Extraocular
Muscles and Related Structures
EXTRAOCULAR MUSCLES • Sleeves (fascial sheaths) of extraocular muscles
AND ORBITAL FASCIA • Intermuscular septa (anterior pulley slings)
Extraocular muscles • System of muscle pulleys and related facial
expansions
• Rectus muscles
• Oblique muscles Applied Anatomical Aspects
• Nerve supply ANATOMY OF THIRD, FOURTH AND SIXTH
• Blood supply
CRANIAL NERVES
• Actions
• Oculomotor nerve
• Structural characteristics • Trochlear nerve
Orbital Fascia • Abducent nerve
• Fascia bulbi

EXTRAOCULAR MUSCLES lateral rectus (LR). The oblique muscles include

superior oblique (SO) and inferior oblique (IO).
AND ORBITAL FASCIA
A set of six extraocular muscles (4 recti and 2 EXTRAOCULAR MUSCLES
obliques) controls the movements of each eye
(Fig. 1.1). Rectus muscles are superior rectus RECTUS MUSCLES
(SR), inferior rectus (IR), medial rectus (MR) and
Origin
The four rectus muscles originate from a common
tendinous ring (the annulus of Zinn), which is
attached at the apex of the orbit encircling the
optic foramina and medial part of the superior
orbital fissure (Fig. 1.2). The annulus of Zinn
appears oval on cross-section. Medial rectus
arises from the medial part of the ring, superior
rectus from the superior part, inferior rectus from
the inferior part and lateral rectus from the lateral
part by two heads which join in a ‘V’ form. Due
to slope of the orbital roof, the origin of the
superior rectus and medial rectus is slightly at
the anterior plane to others. The superior rectus
and medial rectus are closely attached to the dural
sheath of the optic nerve at their origin. This
attachment accounts for the characteristic pain
in a case of retrobulbar neuritis, felt during
Fig. 1.1 Extraocular muscles. upward and inward movements of the globe.
1
 2 Theory and Practice of Squint and Orthoptics

Fig. 1.2 Origin of the rectus and superior oblique muscles.

Course slightly laterally and superiorly in the superior

All the four recti from their origin run forward orbit. In most of its course, it is separated from
around the eyeball. The medial and lateral recti the orbital roof by the levator palpebrae
follow the corresponding walls of the orbit in superioris (LPS) muscle. In primary position, SR
most of the part of their course, and the inferior muscle forms an angle of 23° with the visual axis.
rectus remains in contact with the orbital floor This angle determines the secondary and tertiary
for only about half its length. The superior rectus actions of the SR muscle in primary position.
muscle is separated from the orbital roof by the Anteriorly, prior to insertion, the SR muscle
levator palpebrae superioris muscle. The course courses between the tendon of superior oblique
of the four recti, starting from the apex of the and LPS muscles. Fascial attachments from the
orbit, is diverging, however, somewhat in front SR muscle extend to SO and LPS muscles.
of the equator they turn towards the eyeball in Inferior rectus (IR) muscle, after arising from
a gentle curve to get inserted on the sclera. the inferior part of the annulus of Zinn, runs
Medial rectus (MR) muscle follows the medial anteriorly, slightly inferiorly and laterally
wall of the orbit in most part of its course. This making an angle of 23° with the visual axis in
proximity to the medial orbital wall accounts for its primary position. In posterior half of its
its inadvertent damage during standard or course, the IR muscle remains in contact with
endoscopic ethmoid sinus surgery. Medial the orbital floor. Anteriorly, the IR muscle
rectus muscle is the only rectus muscle that does courses between the globe and inferior oblique
not have a facial attachment to an oblique (IO) muscle prior to its insertion (Fig. 1.1). Facial
muscle; and because of this fact, it is at the attachments exist between the IR, IO and lower
greatest risk of slippage or loss during surgery. lid retractors. These connections are useful in
retrieving, the inadvertently slipped IR during
Lateral rectus (LR) muscle, after arising from surgery, in the region of Lockwood’s ligament.
the lateral part of annulus of Zinn, follows the Further, failure to dissect these connections
lateral orbital wall in most of the part of its during IR recession or resection may lead to eye-
course. Its inferior border passes just superior lid fissure widening or narrowing, respectively.
to the insertion of inferior oblique (IO) muscle
(Fig. 1.1). The facial connections between LR and Insertion
IO muscles at this point, which is 8–9 mm
At the insertion, the striated muscle fibres of
posterior from the insertion of LR, allow the
recti muscle with minimal tendinous connective
surgeon to retrieve the, inadvertently lost LR
tissue, directly attach to the sclera. All the four
muscle during surgery, at this point.
rectus muscles are inserted into the sclera just
Suprior rectus (SR) muscle after origin from the anterior to the equator of globe at different
superior part of annulus of Zinn runs anteriorly, distances from the limbus as under (Fig. 1.3A):
 Anatomy of Extraocular Muscles and Related Structures 3

(Fuchs, 1884) (Apt, 1980)

Medial rectus : 5.5 mm 5.3 mm
Inferior rectus : 6.5 mm 6.8 mm
Lateral rectus : 6.9 mm 6.9 mm
Superior rectus : 7.7 mm 7.9 mm
Thus the insertions of the recti, being not
equidistant from the limbus, do not form a circle
concentric with the limbus; rather form a spiral
(the spiral of Tillaux).
The insertion lines (Fig. 1.3A) of medial and
lateral rectus muscles show slight convexity
facing the limbus. The lines of insertion of
superior and inferior rectus muscles show
marked convexity towards the limbus and are
obliquely placed in such a way that the lateral
end of their insertion line is posteriorly placed
as compared to the medial end.

OBLIQUE MUSCLES
Superior Oblique Muscle
Origin
The superior oblique muscle arises from the
bone (body of sphenoid) above and medial to
the optic foramen (Fig. 1.2) by a narrow tendon,
partially overlapping the origin of levator
palpebrae superioris.

Course
The muscle moves forward between the roof and
medial wall of the orbit to reach the trochlea of
the superior oblique muscle. The trochlea is a
thick fibrous cartilagenous pulley attached to
spina trochlearis on the under aspect of the frontal
bone, at the superomedial angle, between the
anterior most parts of the superior and medial
walls of the orbit. After passing the trochlea, the
superior oblique muscle turns posterolaterally.
At about distal third of the direct portion (about
10 mm behind the trochlea), the muscle becomes
tendinous and remains so in its post-trochlear or
reflected part also.
The reflected tendon of the superior oblique
(so) passes under the superior rectus muscle and
fans out to get inserted on to the sclera. The fibres
of this fan-shaped tendinous part of SO muscle
Fig. 1.3 Insertion lines of the extraocular muscles on the
make an angle of 51° with the visual axis in sclera as seen from (A) front; (B) above; and (C) lateral
primary position. Functionally, these fibres can side. (SR, superior rectus; MR, medial rectus; IR, inferior
be separated into two parts: rectus; LR, lateral rectus).
 4 Theory and Practice of Squint and Orthoptics

• Anterior one-third fibres of the tendon are direct part is about 40 mm and that of the
exclusively responsible for the incyclotorsion reflected tendon is about 19.5 mm (total being
of the globe. Therefore, only these fibres of 59.5 mm). From a physiologic and kinematic
SO muscle are manipulated to enhance standpoint, the trochlea is the origin of the
incyclotorsion action in Harada-Ito procedure. muscle.
• Posterior two-third fibres of the fan-shaped
tendon of SO function to depress and abduct Inferior Oblique Muscle
the globe. Origin

Insertion The inferior oblique muscle arises by a rounded

tendon from a shallow depression on the orbital
The fanned out reflected tendon of the superior
plate of maxilla just lateral to the orifice of the
oblique is inserted onto the upper and outer part
nasolacrimal duct (Fig. 1.4). Some fibres arise
of the sclera behind the equator (Fig. 1.3B).
from the lacrimal fascia. It is the only muscle
The insertion line is curved with its concavity which takes origin from front of the orbit.
facing the trochlea with following landmarks:
• Anterior end of insertion lies some 13.8 mm Course
behind the limbus and about 3 to 4.5 mm From its origin, the muscle passes laterally and
behind the lateral end of the insertion line of backward, between the inferior rectus muscle
the superior rectus muscle. and floor of the orbit (Fig. 1.1). It is almost wholly
• Posterior end of the insertion line lies about muscular with a short tendon at origin and
18.8 mm behind the limbus and some 13.6 mm insertion. It is the shortest of the eye muscles,
behind the medial end of the insertion line of being only 37 mm long. The anterior fibres of
superior rectus muscle. the muscle are primarily responsible for
• Medial or posterior end of the insertion line lies extorsion and posterior fibres for the elevation.
about 8 mm from the posterior pole.
• Width of the insertion line is about 11 mm, but Insertion
it varies greatly from 7 to 18 mm. It is inserted by a short tendon (1 to 2 mm long)
The superior oblique is the longest and thinnest in the lower and outer part of the sclera behind
of all the extraocular muscles. The length of its the equator (Fig. 1.3C). The insertion line is

Fig. 1.4 Origin of inferior oblique muscle and innervation of extraocular muscles.
 Anatomy of Extraocular Muscles and Related Structures 5

curved with a concavity facing the origin, with junction of the posterior and middle thirds of
following landmarks: the belly) and inferior oblique (enters just after
• Its average width is around 9 mm. However, the muscle passes lateral to the inferior rectus
it varies widely from 5 to 14 mm. muscle) muscles. The branches for the superior
• Its anterior end is about 10 mm behind the lower rectus muscle originate from the upper division
edge of the insertion of the lateral rectus muscle. of the oculomotor nerve and enter the muscle at
the junction of the posterior and middle thirds.
• Its posterior end is about 1 mm below and
1 to 2 mm in front of a point corresponding Fourth cranial nerve (trochlear) innervates the
with the foveal region. superior oblique muscle. Unlike other muscles,
• Near the insertion, the inferior vortex vein is the nerve to this muscle enters from its outer
in relation to its posterior border. (orbital) surface near the lateral border. The nerve
divides into three or four branches. The most
Comparative Dimensions anterior branch enters the belly at the junction of
The comparative dimensions of the extraocular posterior and middle thirds of the muscle and
muscles are shown in Table 1.1. The table depicts the most posterior at about 8 mm from its origin.
the following: Sixth cranial nerve (abducent) innervates the
• Superior oblique is the longest and thinnest lateral rectus muscle by entering the muscle on
muscle. the bulbar side 15 mm from its origin.
• Inferior oblique is the shortest muscle.
BLOOD SUPPLY OF
NERVE SUPPLY OF EXTRAOCULAR MUSCLES
EXTRAOCULAR MUSCLES Muscular arteries, usually two—medial and
The extraocular muscles are supplied by third, lateral (with a few fine twigs), are branches of
fourth and sixth cranial nerves (Fig. 1.4). the ophthalmic artery. The medial muscular branch,
larger of the two, supplies the medial rectus,
Third cranial nerve (oculomotor) supplies the inferior rectus and inferior oblique muscles. The
medial, superior and inferior recti and inferior medial rectus also receives a branch from the
oblique muscles. The branches enter their lacrimal artery and the inferior rectus and inferior
respective muscles from their bulbar surfaces. oblique muscles receive branch from the
The branches from the inferior division of third infraorbital artery. The lateral muscular branch
nerve supply the medial rectus (enters its belly supplies the lateral rectus, the superior rectus, the
15 mm from origin), inferior rectus (enters at the levator muscle and the superior oblique muscle.

Table 1.1 Comparative dimensions of various extraocular muscles in mm

Dimension Superior Inferior Lateral Medial Superior Inferior

rectus rectus rectus rectus oblique oblique
 Total length 42 40 48 40 60 37
 Tendon length 5.8 5.5 8.8 3.7 20 1 to 2
 Muscle breadth 9 9 9 9 9 9
 Tendon breadth at insertion 10.8 9.8 9.2 10.3 11 9.4
(insertion line)
 Distance of mid-point of 7.7 6.5 6.9 5.5 16.3 18.4
insertion line from the limbus
 Arc of contact 6.5 6.5 12 7 7–8 15
 Cross-sectional area in mm
– Volkmann 11.3 14.8 16.7 17.4 8.4 6.7
– Nakagama 11.4 15.0 17.5 16 7.9 9.5
Note: These are average figures and may vary greatly among individuals.
 6 Theory and Practice of Squint and Orthoptics

Anterior ciliary arteries arise from the muscular Table 1.2 Actions of extraocular muscles in primary
branches. These are usually seven—two each position of gaze
from the superior, inferior and medial recti and
Muscle Primary Secondary Tertiary
one from the lateral rectus muscle.
action action action
Veins from the extraocular muscles correspond
Medial Adduction — —
to the arteries and empty into the superior and
rectus
inferior ophthalmic veins.
Lateral Abduction — —
ACTIONS OF rectus
THE EXTRAOCULAR MUSCLES Superior Elevation Intorsion Adduction
rectus in abduction
Detailed actions of each extraocular muscle,
Inferior Depression Extorsion Adduction
along with the mechanics of the ocular rectus in abduction
movements, are described in the section on Superior Intorsion Depression Abduction
physiology of ocular movements (page 22–30). oblique
However, a summary of actions of each Inferior Extorsion Elevation Abduction
extraocular muscle in primary position of gaze oblique
(Fig. 1.5) is depicted in Table 1.2.
smooth avascular surface of the muscle capsule
STRUCTURAL CHARACTERISTICS OF
allows the muscle to slide easily over the globe.
EXTRAOCULAR MUSCLES
Muscle capsule, a thin connective tissue Extraocular muscles are voluntary striated
covering of the belly of each extraocular muscle muscles. However, the EOMs differ from the
(EOM), extends from its origin to insertion. The typical skeletal muscle with the following
characteristics:
• Diameter of these fibres is small.
• Contain an enormous amount of fibroelastic
tissue
• Contain both slow and fast fibres
• Richly supplied by vessels
• Richly innervated at a ratio of nerve fibre to
muscle fibre up to 10 times that of skeletal
muscle.
Note: The above characteristics of the EOMs
allow quick as well as smooth eye movements.
Further the EOMs have high contraction speed
and participate in motor acts that are among the
fastest (saccadic eye movements) in human body
and among the most sustained gaze fixation and
vergence movements.

Structural characteristics of EOM, which need

to be elaborated are:
• Global and orbital layers of EOMs
• Transverse compartmentalization of EOMs

GLOBAL AND ORBITAL LAYERS OF EOMs

The muscle belly of each EOM can be divided
into two distinct portions: the superficial part
Fig. 1.5 Actions of extraocular muscles. or the orbital layer (OL) and the deep part or
 Anatomy of Extraocular Muscles and Related Structures 7

the global layer (GL). Both zones are distinctly EOM force in primary and deviated position,
separated from each other. by allowing most sustained pulley tension.
• In rectus muscles, the GL is located adjacent to Of all muscle fibre types, this type is most
globe (eyeball) and OL on the orbital surface affected by dennervation from damage to the
of muscles. motor nerves or the end plates, as occurs after
• In oblique muscles, the GL is located in the botulinum toxin injection.
central core and OL forms the concentric outer • MIFs of OL are thought to play the same role
layer. as that of GL.

Global layer TRANSVERSE COMPARTMENTALIZATION OF

EXTRAOCULAR MUSCLES
Global layer of each rectus EOMs passes through
its muscle pulley (described on page 10–11) Concept of transverse compartmentalization of
becomes contigenuous with its small terminal EOM fibres and their innervation has been
tendon and is inserted onto the sclera (Fig. 1.6A). suggested in many studies to explain the
This global layer (GL) is responsible for eye different functions being performed by a group
movements. It is mainly (90%) composed of of fibres from each EOM.
singly innervated fibres (SIFs) with only 10% Anatomical compartmentalization of EOMs
multiply innervated fibres (MIFs) and their innervation is reported as below:
• Singly Innervated fibres (SIFs) are fast twitch • Lateral rectus (LR) muscle: The abducent nerve,
generating and fatigue resistant fibres, and supplying LR muscle, often divides into two
thus allow for large, rapid and precise eye or more trunks. Further, longitudinal LR
movements. SIFs can be subdivided into three splitting is evident in several congenital
groups (red, intermediate and white), based cranial deinnervation disorders (CCDDs).
on the mitochondrial content, with the red
• Medial rectus (MR) muscle: Motor nerve to MR
fibres being the most fatigue resistant and the
also bifurcates into superior and inferior
white fibres, the least.
divisions.
• Multiple innervated fibres (MIFs), which
• Inferior rectus (IR) muscle has a selective lateral
constitute only 10% of the GL, are thought
trunk overlapping arborization of another
to be involved in fine control of fixation and
trunk throughout the entire muscle length.
in smooth and fine graded eye movements,
• Superior rectus (SR) muscle is, however, reported
particularly the vergence control move-
to lack selective compartmental innervation.
ments.
• Superior oblique (SO) muscle is reported to be
Orbital layer innervated by two (medial and lateral)
divisions of trochlear nerve in non-overlapping
• In rectus muscles, the orbital layer (OL) is medial and lateral compartments. The medial
inserted into their respective muscle pulleys compartment of SO muscle is contiguous with
and not the sclera (Fig. 1.6A). tendon fibres which are inserted on the
• Orbital layer (OL) of IO muscle inserts on the IR equatorial sclera, and produce incycloduction.
and LR pulleys. While the lateral compartment of SO muscle
• Orbital layer (OL) of SO muscle inserts via the is contiguous with tendon fibres which are
SO sheath on the medial aspect of SR pulley. predominantly inserted posterior to equator
and produce infracycloduction.
Types of muscle fibres
• Inferior oblique (IO) muscle is also reported to
OL for each EOM contains 80% SIFs and 20%
be innervated in compartmental fashion by
MIFs.
separate motor nerve trunks.
• SIFs of OL are specialized for an intense
oxidative metabolism, have a large blood Functional anatomical compartmentalization
supply and are fatigue resistant. They are of EOMs has also been demonstrated on MRI
considered the major contributor to sustained studies. A few observations are as below:
 8 Theory and Practice of Squint and Orthoptics

Change in posterior partial volume (PPV) is the ORBITAL FASCIA

best contractility index, observed on MRI
studies. It robustly correlates with the ductions The orbital fascia is a thin connective tissue
for horizontal rectus EOMs. membrane lining the various intraorbital
structures. Recent studies have shown that this
Horizontal rectus EOMs (MR as well as LR)
have been divided into superior and inferior is a complex interwoven connective tissue
halves (compartments) on MRI studies. Inferior joining the various intraorbital contents. Though
compartments are slightly larger than the the orbital fascia is one tissue, for descriptive
superior compartments of both MR as well as convenience, it can be described under the heads
LR muscles. of (Fig. 1.6):
• Fascia bulbi,
In lateral rectus (LR) muscle, MRI studies, the
• Sleeves (fascial sheaths) of extraocular muscles,
contractility for convergence as well as
• Extraocular muscle pulleys,
adduction is reported to be similar in both
superior as well as inferior compartments. • Anterior pulley slings (intermuscular septa), and
• Fascial expansions of extraocular muscles.
In medial rectus (MR) muscle, however, the
superior compartment exhibited much greater FASCIA BULBI
contractility in adduction than in convergence.
Fascia bulbi, or Tenon's capsule, envelops the
During vertical duction on MRI studies, no globe from the limbus to the optic disc. Its inner
differential compartmental changes were noted surface is well defined and lies in close contact
in vertical recti (SR and IR). However, superior with sclera to which it is connected by fine
compartments of MR muscles showed changes trabeculae. The outer surface of the fascia bulbi
in maximum cross-section and PPV. But no lies in contact with orbital fat posteriorly and
change was noted in inferior MR compartments with subconjunctival tissue anteriorly with
and in LR muscle compartments. which it merges near the limbus. Tenon's
capsule is separated from the sclera by episcleral
During vertical fusional vergence (VFV),
space (Tenon's space), which can be readily
induced by a 2 PD base up prism, the LR and
injected. Tenon's capsule can be divided into
SO muscles are reported to exhibit differential
anterior and posterior portions:
compartmental contraction. These observations
• Anterior Tenon’s capsule is the subconjunctival
suggest that the EOM mechanisms that normally
membrane that extends from the limbus to the
compensate for the vertical heterophoria are
level of penetration of the rectus muscles
highly complex.
(About 10 mm posterior to the insertion of
Compartmental brainstem control for the rectus muscles, i.e. just posterior to the equator).
differential compartmental behaviour of the • Anteriorly, it fuses with the conjunctiva 2 to
EOMs has also been reported in the neuroanatomic 3 mm posterior to the limbus. When suturing
studies. a muscle during strabismus surgery, it is
Compartmental involvement of extraocular important to clear anterior Tenon’s capsule off
muscles has also been reported in nerve palsies the tendon insertion to avoid complication of
as below: a slipped muscle.
• Greater atrophy of superior than inferior • Posterior Tenon's capsule extends from the
compartment of LR muscle is reported in about penetration of the recti muscles to the optic
30% cases of abducens palsy. An ipsilesional nerve and thus separates the orbital fat from
hypotropia, suggesting preserved inferior the sclera.
compartment function, is also reported in Around the distal end of optic nerve, the
some cases. posterior Tenon's capsule is fused with the dural
• Hypertropia, that increases in abduction, reported sheath of the optic nerve. Schwalbe, however,
in many cases of isolated LR palsy, suggests considered it to be continuous as a membrane
relationship to the residual LR contraction. surrounding the dural sheath to form a
 Anatomy of Extraocular Muscles and Related Structures 9

Fig. 1.6 Schematic depiction of orbital connective tissues: A, Axial view; B, C and D are coronal sections at levels I, II and
III, respectively; E, schematic section of lower eyelid showing suspensory ligament of Lockwood and inferior suspensory
ligament of the fornix and upper lid showing superior suspensory ligament of fornix. GL, global layer; OL, orbital layer; ON,
optic nerve; SR, superior rectus; MR, medial rectus; LR, lateral rectus; SO, superior oblique; IR, inferior rectus; LPS, levator
palpebrae superioris; IO, inferior oblique.

supravaginal lymph space, a view which is now sends a tubular reflection, which clothes the
considered doubtful. muscles like a glove and are called sleeves or
sheaths of extraocular muscles.
Fascia bulbi is pierced posteriorly by the optic
nerve, ciliary nerves and vessels, just behind the Note. The anterior Tenon's capsule, posterior
equator by venae vorticosae, and near the Tenon’s capsule, and the muscle sleeves are very
equator by six extraocular muscles; where it important structures, since they form the barriers
becomes continuous with the fascial sheaths of between the orbital fat and globe and extraocular
these muscles. muscles. When posterior capsule or muscle sleeve
is traumatically violated, fat adherence can occur.
SLEEVES (FASCIAL SHEATHS) OF The fat adherence can occur as a complication of
EXTRAOCULAR MUSCLES almost any extraocular surgery, e.g. strabismus
At the points near the equator, where the fascia surgery, buckling procedure for retinal
bulbi is pierced by an extraocular muscle, it detachment or periocular trauma.
 10 Theory and Practice of Squint and Orthoptics

INTERMUSCULAR SEPTA (ANTERIOR PULLEY SLINGS) Pulleys of MR and LR muscles are well
The sleeves and pulleys of the four rectus developed. Fascial expansions from the pulleys
muscles are joined to each other by a fascial of lateral and medial rectus muscles are strong
membrane called the intermuscular septum and are attached to orbital tubercle on the
(anterior pulley sling) (Fig. 1.6A to D). This zygomatic bone and to the lacrimal bone,
membrane divides the orbital cavity and orbital respectively. These are also called lateral and
fat into a central (canal) and a peripheral medial check ligaments.
(extraconal) part. Anteriorly the intermuscular
septa fuse with the conjunctiva 3 mm posterior Superior rectus muscle pulley is attached to the
to the limbus. Posteriorly the intermuscular pulley of levator palpebrae superioris. This
septa do not extend up to the orbital apex and attachment ensures synergic action of the two
vanish a short distance behind the area of globe– muscles. Thus, when the superior rectus makes
optic nerve junction. This fact has been revealed the eye look up, the upper lid is also raised. In
on high-resolution magnetic resonance imaging maximal levator resection for ptosis, hypotropia
(MRI). Thus, the muscle cone does not extend can be induced, if these connections are not
up to the orbital apex and that the conal and severed. A dense band extends from the lateral
extraconal spaces become one continuous space border of conjoint SR-LPS pulley to the superior
near the apex of the orbit. border of LR pulley. This band contains dense
collagen and elastin throughout and divides the
SYSTEM OF MUSCLE PULLEYS AND orbital lobe of lacrimal gland.
RELATED FACIAL EXPANSIONS
Fascial expansions of extraocular muscles. The Pulleys of inferior rectus (IR) and inferior oblique
muscular sheath of each extraocular muscle (IO) muscles are intimately coupled with each
sends expansions to the surrounding structures other and have main contribution in forming the
which along with muscle pulleys form a suspensory ligament of Lockwood (Fig. 1.6E).
complex system of muscle pulley and related • This ligament is a thickened sling or hammock
facial expansions (Fig. 1.6). of fascial sheath extending from the posterior
lacrimal crest to the lateral orbital tubercle, on
Muscle pulleys refer to stiff concentric ring-like
which rests the eyeball. It is formed by fusion
structures present around the belly of EOMs.
of expansions from the muscular sheaths of
These consist of collagen, elastin and smooth
the medial rectus, inferior oblique, inferior
muscle fibres.
rectus and lateral rectus muscles joined with
Pulleys of rectus EOMs, about 2–3 mm in length, the thickened inferior part of Tenon's capsule.
are present close to the equator of globe, i.e.
• Expansion from the inferior rectus muscle is
beginning at the points where the rectus muscles
attached to the capsulopalpebral fascia, a
penetrate the Tenon’s capsule. Thus, the rectus
tissue analogous to levator aponeurosis in the
muscle pulleys are co-axial with the underlying
lower lid and the sheath of inferior oblique
collagenous sleeves around the EOMs.
muscle. This relationship is important because
• The rectus muscle pulleys are stiff and are
an inferior rectus muscle recession can result
stabilised by septa which are attached around
in lower lid retraction with lid fissure
the facial bulbi, intramuscular septa and
widening while resection results in lid
periorbita.
advancement with lid fissure narrowing.
• Dynamic MRI studies have shown that the
Another importance of this relationship is that
pulleys act mechanically as the functional origins
when the inferior rectus is inadvertently
of the muscles and thus effectively modify the
disinserted or lost during surgery, these
direction of pull of the rectus muscles.
connections will hold the inferior rectus to the
• Pulleys also serve to stabilise the muscle path,
inferior oblique and keep it from retracting
preventing the sides slipping or movement
posteriorly. Therefore, the lost inferior rectus
perpendicular to the muscle axis.
can usually be found lying between the
• Anteriorly, the pulleys merge with muscle
inferior oblique muscle and the sclera.
sleeves.
 Anatomy of Extraocular Muscles and Related Structures 11

Superior oblique (SO) pulley, also known as pupillae and ciliary muscle accompanies the
trochlea, is a thick fibrocartilaginous pulley nerve to the inferior oblique muscle, pupillary
present at the superomedial angle of the orbital abnormalities may also result from surgery in this
wall. Around it turns the SO tendon. Orbital area.
layer (OL) of the SO muscle is inserted, via the 3. Inferior rectus muscle is distinctly bound to
SO sheath, on the medial aspect of SR pulley. the lower eyelid by the fascial extension from
• Superior transverse ligament of the Whitnall. It is its sheath. Recession of the inferior rectus muscle
a thickened band of orbital fascia which extends tends to widen the palpebral fissure, and
from the trochlear pulley to the lacrimal gland resection of the inferior rectus muscle tends to
and its fossa. It is formed by a condensation of narrow the fissure. Therefore, any alteration of
the superior sheaths of the levator muscle the inferior rectus muscle may be associated
joined medially by the sheath of the reflected with palpebral fissure change.
tendon of superior oblique muscle. It forms a 4. Superior rectus muscle is loosely bound to the
true check ligament of the levator muscle. levator palpebrae superioris muscle. The eyelid
• Suspensory ligaments of the fornices are also well may be pulled forward following resection of
recognized. Superior suspensory ligament of the superior rectus muscle, thus narrowing the
the fornix is formed by the continuation palpebral fissure; also, in hypotropia, a
forward of the fibrous tissue between the pseudoptosis may be present.
superior rectus and levator muscles to the 5. Blood supply to the extraocular muscles
upper fornix. During ptosis surgery, if this provides almost all of the temporal half of the
ligament is cut, fornix conjunctiva can prolapse. anterior segment circulation; it provides the
Similarly, the inferior suspensory ligament of the majority of the nasal half of the anterior segment
fornix is formed by the continuation forward circulation, of which some blood is supplied by
up to the inferior fornix of the fibrous tissue of the long posterior ciliary artery. Therefore,
lower lid retractors (Fig. 1.6E). simultaneous surgery on three rectus muscles
• Orbital septa of elastic and collagenous tissue may induce anterior segment ischaemia,
are well developed in the adults. These septa particularly in older patients.
pass inward from the periorbita to the 6. Whenever muscle surgery is performed, special
intermuscular septa (membrane pulleys and care must be taken to avoid penetration of
muscle sleevs) (Fig. 1.6B). Such septa also pass Tenon's capsule; if the integrity of Tenon's
to and between the extraocular muscles and capsule 10 mm posterior to the limbus is
provide specific supportive channels for the violated, fatty tissue may prolapse through
ophthalmic veins. Tenon's capsule and may form a restrictive
adhesion and limit ocular motility.
APPLIED ANATOMICAL ASPECTS 7. During resection or transposition of extra-
1. Nerves to the rectus muscles and the superior ocular muscles, the intramuscular septal
oblique muscle enter the muscles about one- connections and check ligaments attached to the
third of the distance from the origin to the Tenon’s capsule should be carefully severed.
insertion (or trochlea, in the case of the superior This prevents the relocation of adjacent muscles
oblique muscle). It is difficult, but possible, to and fat compartments. For example, if the
damage these nerves during anterior surgery. attachments between LR on IO are not severed,
However, if an instrument is thrust more than the IO is moved anteriorly during resection of
26 mm posterior to the rectus muscle's insertion, the LR.
injury to the nerve may occur. 8. During rectus muscle recessions, the severing
2. Nerve supplying the inferior oblique muscle of the intramuscular septal connections is not
enters the lateral portion of the muscle where the necessary.
muscle crosses the inferior rectus muscle; it can 9. Posterior dissection during the rectus muscles
be damaged by surgery in this area. Since the surgery may result in pulley damage, so special
parasympathetic innervation to the sphincter care is required.
 12 Theory and Practice of Squint and Orthoptics

10. When surgery is performed in the domain of preganglionic parasympathetic fibres along the
the vortex veins, accidental severing of a vortex other oculomotor fibres.
vein is possible. The procedures that present the
greatest risk for damaging a vortex vein are Course and distribution
inferior rectus and superior rectus muscle For the purpose of description, the course of the
recession or resection, inferior oblique muscle oculomotor nerve can be divided into: Fascicular,
weakening and exposure of the superior oblique basilar, intracavernous and intraorbital parts.
muscle tendon.
11. The sclera is thinnest just posterior to the four Fascicular part
rectus muscle insertions. This is the site for most The fasciculus consists of efferent fibres which
muscle surgery, especially for recession pass from the third nerve nucleus through the
procedures. Therefore, the risk of scleral red nucleus and the medial aspect of the
perforation is always present during eye muscle cerebral peduncle. They then emerge from the
surgery. This risk can be best minimized by using midbrain and pass into the interpeduncular
spatulated needles with swedged sutures; space (Fig. 1.8).
working with a clean, dry, and blood-free surgical
field; using loupe magnification; and employing Basilar part
a head mounted fibreoptic light source in The basilar part starts as a series of 15 to 20
addition to the overhead operating lights. rootlets in the interpeduncular fossa. These
coalesce to form a large medial root and a small
lateral root, which unite to form a flattened nerve,
ANATOMY OF THIRD, FOURTH which get twisted bringing the inferior fibres
AND SIXTH CRANIAL NERVES superiorly and superior fibres inferiorly; and thus
OCULOMOTOR NERVE the nerve becomes a rounded cord. The nerve
then runs forwards to reach the cavernous sinus
The oculomotor (third cranial) nerve is entirely (Fig. 1.9).
motor in function. It supplies all the extraocular
muscles except lateral rectus and superior oblique. Intracavernous part
After entering the cavernous sinus, the nerve
Oculomotor nuclear complex
descends to the lateral wall of the sinus, where it
The oculomotor nucleus complex has two motor lies above the trochlear nerve (Fig. 1.10). In the
nuclei: (a) the main motor nucleus of large anterior part of the cavernous sinus, the nerve
multipolar neurons, and (b) the accessory divides into superior and inferior divisions which
parasympathetic nucleus (Edinger-Westphal enter the orbit through the middle part of the
nucleus) of small multipolar neurons. superior orbital fissure within the annulus of Zinn
a. The main motor nucleus is composed of the (Fig. 1.11). In the fissure, the nasociliary nerve
subnuclei (Fig. 1.7A and B) supplying individual lies in between the two divisions, while the
extraocular muscles as follows: abducent nerve lies inferolateral to them.
1. Dorsolateral nucleus: Ipsilateral inferior rectus.
Intraorbital part
2. Intermedial nucleus: Ipsilateral inferior oblique.
3. Ventromedial nucleus: Ipsilateral medial rectus. In the orbit (Fig. 1.9), the smaller superior division
ascends on the lateral side of optic nerve and
4. Paramedial (scattered) nucleus: Contralateral
supplies the superior rectus and the levator
superior rectus.
palpebrae superioris. The larger, inferior division
5. Caudal central nucleus: Bilateral levator
divides into three branches: (1) nerve to the medial
palpebrae superioris.
rectus passes inferior to the optic nerve, (2) nerve
b. The accessory motor nucleus (Edinger- to inferior rectus passes downward and enters
Westphal nucleus). It is situated posterior to the the muscle on its upper aspect and (3) nerve to
main oculomotor nucleus mass. It sends inferior oblique (longest of the three branches)
 Anatomy of Extraocular Muscles and Related Structures 13

B
Fig. 1.7 Scheme to show components of oculomotor nucleus complex. A: old outdated concept, B: modern concept
(Warwick, 1953) (EWN, Edinger-Westphal nucleus; DN, Dorsal nucleus VN, Ventral nucleus, IC, intermediate column;
CCN, caudal central nucleus).

passes in between the inferior rectus and lateral TROCHLEAR NERVE

rectus and supplies the inferior oblique from its The trochlear (fourth cranial) nerve is entirely
posterior border. It gives off the motor root to motor in function and supplies only the
the ciliary ganglion. superior oblique muscle of the eyeball.
 14 Theory and Practice of Squint and Orthoptics

Fig. 1.8 Oculomotor nerve nuclei, their central connections and course of fascicular and basilar parts of the nerve.

Nucleus Fascicular part

The trochlear nucleus is situated in the The fasciculus consists of efferent fibres which
ventromedial part of the central grey matter after leaving the nucleus, pass posteriorly
of the midbrain at the level of inferior around the aqueduct in the central grey matter
colliculus (Figs 1.12 and 1.13). It is caudal to and decussate completely in the anterior
and continuous with the third nerve nucleus medullary velum (Fig. 1.13).
complex.
Precavernous part
Course and distribution
The trochlear nerve trunk after emerging from
For the purpose of description, the course of the the dorsal aspect of midbrain winds round the
trochlear nerve can be divided into fascicular, superior cerebellar peduncle and the cerebral
precavernous, intracavernous and intraorbital peduncle just above the pons. It then runs
parts. forwards and enters into the cavernous sinus.
 Anatomy of Extraocular Muscles and Related Structures 15

Fig. 1.9 Showing the course of oculomotor nerve.

Fig. 1.10 Coronal section through the middle cranial fossa showing the relations of cranial nerves (3rd, 4th, three divisions
of 5th and 6th) with each other in the lateral wall of the cavernous sinus.

Fig. 1.11 Apical part of the orbit showing the origin of extraocular muscles, the common tendinous ring and the structures
passing through the superior orbital fissure.
 16 Theory and Practice of Squint and Orthoptics

Fig. 1.12 Scheme to show the cranial nerve nuclei as projected onto the posterior surface of the brainstem.

Intracavernous part ABDUCENT NERVE

In the cavernous sinus, the nerve runs forwards The abducent (sixth cranial) nerve is a small,
in its lateral wall lying below the oculomotor entirely motor nerve that supplies the lateral
nerve and above the first division of the fifth rectus muscle of the eyeball.
cranial nerve (Fig. 1.10). In the anterior part of
the cavernous sinus, it rises, crosses over the 3rd Nucleus
nerve and leaves the sinus to pass through the The abducent nucleus is situated in the lower
lateral part of the superior orbital fissure (where part of pons, closely related to the fasciculus of
it lies superolateral to annulus of Zinn and the facial nerve (Fig. 1.15).
medial to the frontal nerve) (Fig. 1.11).
Course and distribution
Intraorbital part
For the purpose of description, the course of the
After entering the orbit through the lateral part abducent nerve can be divided into: Fascicular,
of the superior orbital fissure, the nerve passes basilar, intracavernous and intraorbital parts.
medially above the origin of levator palpebrae
superioris (Fig. 1.14) and ends by supplying the Fascicular part
superior oblique muscle through its orbital The fasciculus consists of efferent fibres which
surface. start from the nucleus, pass forward and emerge
 Anatomy of Extraocular Muscles and Related Structures 17

Fig. 1.13 Trochlear nerve nucleus, its central connections and course of fascicular and basilar parts of the nerve.

Fig. 1.14 Showing the course of trochlear nerve.

 18 Theory and Practice of Squint and Orthoptics

Fig. 1.15 Abducent nerve nucleus and its central connections.

by some 7 to 8 rootlets from the junction of pons Intracavernous part

and medulla which join to form one nerve In the cavernous sinus, the nerve runs almost
(Fig. 1.15). horizontally forward, occupying a position
below and lateral to the internal carotid artery
Basilar part
(Fig. 1.10). The nerve then leaves the cavernous
The nerve then runs forwards, upwards on the sinus to enter the orbit through the middle part
back of the petrous temporal bone near its apex. of the superior orbital fissure within the annulus
At the sharp upper border of the petrous bone, of Zinn (Fig. 1.11). In the superior orbital fissure,
the nerve bends forward at right angle and the abducent nerve lies inferolateral to the
enters the cavernous sinus. oculomotor and nasociliary nerves.

Fig. 1.16 Course of sixth cranial nerve.

 Anatomy of Extraocular Muscles and Related Structures 19

Intraorbital part Anatomy”. Annals of the New York Academy

In the orbit, the nerve runs forwards and enters of Sciences. Neurobiology of eye movements: from
molecules to behavior. 956: 17–32. doi:10.1111/
the ocular surface of the lateral rectus muscle
j.1749-6632.2002.tb02805.x.
just behind its middle portion after dividing into
5. Mehta MP; Perry JD (2015). “Medial orbital
three or four branches (Fig. 1.16).
wall landmarks in three different North
American populations”. Orbit. 34 (2): 72–8. PMID
BIBLIOGRAPHY 25804299. doi:10.3109/01676830.2014. 997394.
6. Orbit at the US National Library of Medicine
1. Bisaria KK. Cavernous portion of the trochlear
Medical Subject Headings (MeSH).
nerve with special reference to its site of entrance.
J. Anat. 159:29–35, 1988. 7. Tenon JR, Naus J, Blanken R (March 2003).
2. Duane’s Ophthalmology, Chapter 32 Embryology “Anatomical observations on some parts of the
and Anatomy of the Orbit and Lacrimal System. eye and eyelids. 1805”. Strabismus. 11 (1): 63–
(eds Tasman W, Jaeger EA) Lippincott/Williams 8. PMID 12789585. doi:10.1076/stra.11.1.63.
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Palsy Following Minor Occipital Impact. Neurol Quian, Carlos (2015). The Clinical Anatomy of
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System: A Revolution in Concepts of Orbital ISBN 978-1-118-49201-7.
 2
Physiology of
Ocular Motility
BASIC KINEMETICS AGONIST, SYNERGISTS,
• Positions of gaze ANTAGONISTS AND YOKE MUSCLES
• Centre of rotation FUNDAMENTAL LAWS GOVERNING OCULAR MOTILITY
• Fick's axes • Donders’ law
• Translatory and rotatory movements • Listing’s law
• Position of rest • Hering’s law
• Sherrington’s law
MECHANICS OF ACTIONS OF
EXTRAOCULAR MUSCLES OCULAR MOVEMENTS
• Factors involved • Monocular movements (Ductions)
• Classical concept of actions • Binocular movements
• Kinematics of muscle pulleys – Versions
• Field of action – Vergences

Secondary positions of gaze

BASIC KINEMATICS
These are the positions assumed by the eyes
POSITIONS OF GAZE while looking straight up (supraversion),
To understand the ocular movements and their straight down (infraversion), to the right
mechanics, a frame of reference against which (dextroversion) and to the left (levoversion)
the movements may be quantitated is (Fig. 2.1B, D, F, H).
necessary. The primary position of the eye is
that position from which all other ocular Tertiary positions of gaze
movements are initiated, changing the position
These describe the positions assumed by the
of eyeball from primary to secondary or
tertiary. All the extraocular muscles have a eyes, when combination of vertical and
given tone for every position of gaze. A total horizontal movements occurs. These include
of 9 positions of gaze have been described. position of eyes in dextroelevation, dextro-
These include one primary, 4 secondary and 4 depression, levoelevation and levodepression
tertiary positions (Fig. 2.1). (Fig. 2.1A, C, G, I).

Primary position of gaze Cardinal positions of gaze

The primary position has been defined by These are the positions which allow examination
Scobee as that position of the eyes in binocular of each of the 12 extraocular muscles, of the two
vision when, with the head erect, the object of eyes, in their main field of action. There are six
regard is at infinity and lies at the intersection cardinal positions of gaze, viz., dextroversion,
of the sagittal plane of the head and a horizontal levoversion, dextroelevation, levoelevation,
plane passing through the centres of rotation of dextrodepression and levodepression (Fig. 2.1A,
the two eyeballs (Fig. 2.1E). C, D, F, G, I).
20
 Physiology of Ocular Motility 21

Fig. 2.1 Diagnostic positions of gaze. Primary position (E); secondary positions (B, D, F, H); tertiary positions (A, C, G, I);
cardinal positions (A, C, D, F, G, I).

CENTRE OF ROTATION to some extent (i.e. laterally, vertically, or in or

It refers to a hypothetical point around which the out), and that the centre of rotation of the eye does
eyeball performs rotatory movements. Earlier this not have zero velocity. It has been reported that
has been assumed that the centre of rotation is a the centre of rotation moves in a semicircle in the
fixed point. However, newer experiments plane of rotation.1 This locus has been called the
indicate that translatory movements do take place space centroid (Fig. 2.2).

Fig. 2.2 Position of space centroid as computed by Park and Park.1

 22 Theory and Practice of Squint and Orthoptics

However, for all practical purposes, the globe allow the eyeball to rotate obliquely up and
can be considered to rotate around a fixed point. in (Fig. 2.3B I), up and out (Fig. 2.3B III), down
In primary position, the centre of rotation lies and in (Fig. 2.3 B VII) and down and out
some 13.5 mm behind the apex of cornea, when (Fig. 2.3B IX).
measured on the line of sight. This is in reality a Note. The X, Z and O axes lie in the same plane.
little behind the actual geometrical centre of the This plane passing through the centre of rotation
globe; but from a practical standpoint, it will be of the eye and containing the X, Z and O axes is
considered to coincide with the centre of the called Listing's plane (Fig. 2.3A and 2.3B V). The
globe. In big myopic eyes, the centre of rotation eyeball can reach all positions of gaze by
is a bit farther posterior and in small hyperopic rotations around the axes that are on Listing's
eyes, it is a bit anterior to this ideal position.2 plane, i.e. Z, X and oblique axes (Fig. 2.3B).

FICK'S AXES
TRANSLATORY AND ROTATORY MOVEMENTS
Fick described three axes (co-ordinates) to
analyse all movements of the globe around the Translatory movements refer to the movements
hypothetical centre of rotation. The proposed of the eyeball as a whole in the orbit, with eye
three axes are perpendicular to each other and remaining in primary position of gaze. These
intersect at the centre of rotation of the eye. In include shift of the eyeball upwards or
Fick's system, these coordinates (Fig. 2.3A) are downwards, anteriorly or posteriorly and
as described below.3 Recently, oblique axis has sideways. Rotatory movements occur along the
also been described. three axes of rotation around a fixed centre of
rotation as discussed above.
X (horizontal) axis
It lies horizontally, when the head is in an POSITION OF REST
upright position. Rotation around the horizontal Position of rest, i.e. a position without actions
(X) axis results in elevation (sursumduction) or of extraocular muscles is very hard to document.
depression (deosursumduction) (Fig. 2.3B II and Even in death, rigor mortis may make the
VIII). extraocular muscles pull the eye away from the
true rest position. It has been reported that the
Y (anteroposterior) axis
eye positions of orthophoric normal individuals
The anteroposterior axes of the two eyes are under deep anaesthesia with curare paralysis
parallel to each other and perpendicular to the (measured by an accurate photographic
horizontal axis. Rotation of the globe around the technique) are probably the closest approxima-
anteroposterior (Y) axis (Fig. 2.3A)produces the tion of the position of rest. Under such
torsional movements named according to the experiments, an exotropia of 2.25° in each eye
movement of the 12 o'clock meridian of the which increases with age has been observed in
cornea as extorsion (excycloduction) and intorsion young adult eyes.4
(incycloduction).

Z (vertical) axis MECHANICS OF ACTIONS

It extends vertically (direction of gravity), when OF EXTRAOCULAR MUSCLES
the head is in an upright position, and is FACTORS INVOLVED IN MECHANICS OF
perpendicular to the X and Y axes. Rotations of EXTRAOCULAR MUSCLE ACTIONS
the globe around vertical (Z) axis are known as
1. Cross-sectional area of the muscle. Cross-
adduction and abduction (Fig. 2.3B IV and VI).
sectional areas of the extraocular muscles as
described by Volkmann5 and Nakagama6 are
Oblique axis
shown in Table 2.1. On the basis of the cross-
The Listing’s plane also contains two oblique sectional area of the muscles, following
axes 0–0 and 0’–0’ (Fig. 2.3B I and III) which comments have been made:
 Physiology of Ocular Motility 23

Fig. 2.3 A. Fick's axes and Listing's plane, B. Note that eyeball can reach all positions of gaze by rotations around axes that
are on Listing's plane, i.e. Z, X and O (oblique) axes.
 24 Theory and Practice of Squint and Orthoptics

• Cross-sectional area of the horizontal recti is muscle. The position of this point changes, when
maximum. This is sensible, since they alone the muscle contracts or relaxes and the globe
are horizontal movers. rotates (Fig. 2.4).
• The vertical recti average about 75% and the The arc of contact is the distance on the scleral
obliques about 50% the size of the horizontal circumference between the tangential point (T)
muscles. and the centre of anatomic insertion of the
• In general, antagonists such as medial and muscle (A) on the sclera. The lengths of the
lateral recti are similar in size, thereby contact area for various extraocular muscles
balancing opposing forces. in primary position of the gaze are given in
• Muscles exert force in proportion to their Table 2.1.
cross-sectional area. The arc of contact represents the lever arm in
2. Length of the muscle. The average length of the mechanical system. The arc of contact varies
various extraocular muscles is shown in with the position at tangential point, e.g. as the
Table 2.1. It has been reported that for the eye is abducted, the arc of contact of lateral
normal amplitude of rotation (45°–50° each way rectus is reduced (Fig. 2.4B), while in adduction,
from the primary position), approximately it is increased (Fig. 2.4C). However, the location
10 mm (about 25% of the normal resting length) of the tangential point with respect to the centre
change in muscle length is required in each of rotation of the eye (C) and the point of origin
direction. Therefore, a sacrifice of muscle length of the muscle (O) remains unchanged (Fig. 2.4A,
during resection of the muscles usually reduces B and C); so that the torque (i.e. the force of
the amplitude of eye rotation. rotation) also remains constant. This holds true
3. Arc of contact. The distal portion of each
extraocular muscle or muscle tendon lies flush Table 2.1 Lengths of the contact arc for various
extraocular muscles in primary position of the gaze
against the globe for a variable distance before
it blends into the sclera (anatomic insertion). The Muscles Lengths of contact arc
point at which the centre of the muscle or its Lateral rectus 15 mm
tendon first touches the globe is the tangential Medial rectus 6 mm
point. It is also referred to as the physiologic or Superior rectus 8.4 mm
Inferior rectus 9 mm
effective insertion of the muscle, since from a
Superior oblique 5 mm
mechanical point of view, a tangent to the globe
Inferior oblique 17 mm
at this point indicates the direction of pull of that

Fig. 2.4 The arc of contact of the lateral rectus muscle.

 Physiology of Ocular Motility 25

till arc of contact is practically zero that is, the

tangential point coincides with the anatomical
insertion.
Since the power of a muscle is proportionate
to its length and arc of contact, retroplacement
of the insertion of muscle (recession) weakens
the action of the muscle by shortening its
effective length and by reducing its arc of contact
in the various positions of gaze. Advancement
of an extraocular muscle has a strengthening
effect because of the increase in the effective
length as well as its arc of contact.
4. Muscle plane. It is an imaginary plane which
passes through the midpoints of origin of the
muscle (O), anatomical insertion (A), functional
insertion, i.e. tangential point (T) and the centre
of rotation of the globe (C) (Fig. 2.5). Thus each
muscle plane runs through the long axis of the
muscle. The angle formed by a muscle plane Fig. 2.6 Relation of muscle plane of superior and inferior
with the visual direction or line of fixation recti with the visual line in primary position.
depends on the position of the globe. When the
line of fixation lies in or parallel to the muscle
plane, the angle is zero.
The ocular muscles are paired, each pair
having a common muscle plane. These pairs are
the medial and lateral recti [their muscle plane
coincides with the horizontal plane of the globe],
superior and inferior recti [their muscle plane
makes an angle of 23° with the visual line in
primary position (Fig. 2.6)] and superior oblique
tendon from the trochlea to globe and the
inferior oblique [their muscle plane makes an
angle of about 51° with the visual line when the
eye is in the primary position (Fig. 2.7)].

Fig. 2.7 Relation of muscle plane of superior and inferior

obliques with the visual line in primary position.

5. Muscle axis of rotation. It is perpendicular to

the muscle plane erected in the centre of rotation.
The individual muscle pulling on the eye will
rotate the globe around this axis through the
Fig . 2.5 Schematic presentation of muscle plane (TACO). centre of rotation.
 26 Theory and Practice of Squint and Orthoptics

6. Role of muscle pulleys. Muscle pulleys, primary action. Thus lateral rectus causes
formed by stiff connective tissue have been abduction and medial rectus causes adduction.
described to exist for rectus muscles as part of
their sleeves at or just posterior to the equator Vertical rectus muscles
of globe. The muscle pulleys redirect the The superior and inferior rectus muscles have a
extraocular muscles and act as their functional common muscle plane which is in the same line
origins; and thus affect the ocular movements as the orbital axis and thus form an angle of 23°
in different positions of gaze. with the optical axis (Fig. 2.6).

CLASSICAL CONCEPT OF ACTIONS Actions of superior rectus

OF THE EXTRAOCULAR MUSCLES • In the primary position, the primary action of the
The basic and classical concept implies that the superior rectus is elevation (Fig. 2.8A). This
actions of the extraocular muscles depend upon movement occurs about the horizontal 'X' axis.
the position of the globe at the time of muscle The secondary action is intorsion (around 'Y' axis)
contraction. The primary action of a muscle is its and tertiary action is adduction (around 'Z' axis).
major effect, when the eye is in the primary • When the globe is abducted 23°, the axis of rotation
position. The additional effects (other than the of superior rectus muscle and optical axis coincide
primary action) in the primary position are so that the muscle has no subsidiary actions and
called subsidiary (secondary and tertiary) can only act as elevator (Fig. 2.8B). This is, therefore,
actions. Duane7–9 proposed that opposed vertical the best position of the globe for testing the
muscles act as synergists in certain gaze function of the superior rectus muscle.
positions and that their functions vary with • If the globe could be adducted 67°, the superior
change in direction of gaze. rectus would produce pure incycloduction
Based on the old classical concept by Duane,7–9 (Fig. 2.8C). Since the globe cannot adduct,
the muscle actions may be summarized as below. therefore, there is some elevating component
to the action of superior rectus even in
Horizontal rectus muscles adduction.
The horizontal recti have a common muscle
Actions of inferior rectus
plane which is horizontal in primary position
and their axis of rotation coincides with the 'Z' Actions of inferior rectus are analogous to the
axis of the globe. superior rectus, i.e.:
When the eye is in primary position, the • In primary position, its primary action is
horizontal recti are purely horizontal movers depression, secondary action is extorsion and
around the vertical 'Z' axis and have only a tertiary action is adduction.

Fig. 2.8 Actions of superior rectus muscle. A, in primary position; B, when the globe is abducted 23o; and C, when the globe
could be adducted 67o.
 Physiology of Ocular Motility 27

• When the globe is abducted 23°, its only action • When the globe is adducted 51°, its only action is
is depression. elevation.
• If the globe could be adducted 67°, inferior • When the globe is abducted 39°, its only action is
rectus will produce only extorsion. extorsion.

Oblique muscles Contribution of different extraocular muscles in

vertical eye movements
The oblique muscles are inserted behind the
equator and form an angle of 51° with the optical The vertical recti are primary elevators and
axis (Fig. 2.7) and thus have following actions. depressors, while the obliques are the primary
torsional muscles.
Actions of superior oblique Elevation in primary position and in
• In primary position, the primary action of abduction is contributed by superior rectus—
superior oblique is intorsion (Fig. 2.9A), which 60%, inferior oblique—30% and the rest 10% by
occurs about anteroposterior (Y) axis; the the medial and lateral recti. While in adduction
secondary action is depression and the tertiary between 10° and 30°, the contribution of
action is abduction. superior rectus, inferior oblique and horizontal
• When the globe is adducted 51°, the axis of muscle recti is about 40%, 30% and 30%, respectively.
rotation coincides with the optical axis so that Depression in primary position, in abduction
it can only act as a depressor (Fig. 2.9B). This is and up to 10° adduction is contributed by
the best position of the globe for clinically inferior rectus—80%, superior oblique—10%
testing the action of superior oblique muscle. and horizontal recti—10%. The corresponding
• When the eyeball is abducted by 39°, the optical figures in a position of adduction between 10°
axis and line of pull of the superior oblique and 30° are inferior rectus—50%, superior
make on angle of 90° with each other. In this oblique—20% and horizontal recti—30%.
position, the superior oblique can only cause
KINEMATICS OF MUSCLE PULLEYS
intorsion (Fig. 2.9C).
Rectus muscle pulleys are fundamental to ocular
Actions of inferior oblique kinematics. Mechanics of action of extraocular
These are analogous to the superior oblique as muscles is presently being considered on the
follows: basis of kinematics of muscle pulleys.9a
• In primary position, main action is extorsion, Anatomical aspects of system of muscle pulleys
secondary action is elevation and tertiary and their connections are described on page
action is abduction. 10–11.

Fig. 2.9 Actions of superior oblique muscle. A, in primary position; B, when the globe is adducted 51°; and C, when the
globe is abducted 39o.
 28 Theory and Practice of Squint and Orthoptics

Physiological aspects, i.e. kinematics of pulleys pulley and scleral insertion and thus vertical
need delibrations on the following: in central gaze (Fig. 2.10A).
• Relationship of the pulley to rotational axis of • Muscle pulley is pulled posteriorly during EOM
EOMs. contraction by the orbital layer (OL) of the
• Half-angle-rule muscle inserted over it. These muscle pulleys
• Pulley suspension forces move in coordination with the insertion and
• Kinematics of individual EOM pulley the sclera.

Relationship of pulley to Half-angle-rule

rotational axis of EOMs • Rotation axis of the eyeball shifts equivalent to half
• Pulleys of rectus EOM located just behind the of the angle by which eyeball moves during
equator, serve as functional origin of the duction movement from the primary position.
muscles (Fig. 2.10). This has been depicted on dynamic MRI
• Rotational axis of all rectus EOMs is studies. For example, if in supraduction the
perpendicular to the segment between the eyeball moves up by angle , the horizontal

Fig. 2.10 Schematic depiction of relationship of pulleys to the rotational axis of horizontal rectus muscles: A, Rotational axis
of medial rectus (MR) muscle is perpendicular to the segment between the pulley and scleral insertion, and is thus vertical
in central gaze; B, In supraduction by angle , the distance L1 from the pulley (ring) to globe center is equal to distance L2
from globe center to the insertion, this causes the rotational axis of MR to tilt posteriorly by approximately angle /2, the half-
angle rule to implement Listing's law; C, Axial view showing pulleys (depicted as spindles) of the horizontal rectus muscles
in central gaze; D, In adduction, the orbital layer of contracting MR shifts its pulley posteriorly, while the relaxing lateral rectus
(LR) orbital layer allows its pulleys to move anteriorly (modified from Demer II, Invest Ophthalmol Visc Sci 2004).
 Physiology of Ocular Motility 29

axis, around which supraduction occurs, insertion. The mechanical load of OL is due
moves posteriorly by half the angle (i.e. /2) to the elasticity of the pulley suspension. This
(Fig. 2.10B). load is independent of speed of eyeball
• Half-angle-rule is maintained due to appropriate movement but proportional to the gaze angle.
location of the rectus muscle pulleys in such a way • Neural command needed by OL and GL of EOMs
that the distance from pulley ring to centre of during contraction is different. The ratio of
globe rotation (L1) is equal to the distance from motor nerve fibre to muscle fibre in GL is low
center of rotation to the muscle insertion on (about 1:1) for rectus EOMs, reflecting high
the sclera (L2) (Fig. 2.10B). precision for ocular rotation.
• Half-angle-rule allows the prerequist for the However, for OL this ratio is higher (1:5 in
Listing’s law, which states that the eyeball can horizontal recti and 1:2.5 in vertical recti);
reach all positions of gaze by rotations around reflecting less percision for pulley control.
the axis that lie on the Listing’s plane (vertical
Inferior oblique (IO) muscle kinematics are as
plane passing through the centre of rotation
below:
of eyeball).
• Half-angle-kinematics is also observed by IO
Pulley suspension forces muscle.
As mentioned above, the muscle pulleys move • Orbital layer (OL) of IO muscle is inserted on
along the length of the EOMs by the action of the pulley of IR and LR, and so the pulley of
orbital layer of the muscles. However, the IO moves by half of the vertical ocular duction.
pulleys are located quite stably and stereo- • During oblique gaze shift from supraducted
typically in the transverse direction. Stability is adduction to infraducted abduction, the IO
provided by the suspensory forces due to pulley moves anteroposteriorly by half the
interconnections of the pulleys to muscle sleeves, movement of IR pulley.
intermuscular septa, pulleys of other muscles Superior oblique pulley and its kinematics is as
and the periorbita (see Fig. 1.6) below:
• Superior oblique pulley is fixed and so does not
Pulley kinematics of individual EOMs
move during ocular movements.
Active pulley hypothesis (APH) states that the • Half-angle-kinematics is also exhibited by SO,
pulley shifts during the eye movements are since the distance from trochlea to centre of
generated by the contractile activity of the orbital globe is approximately equal to the distance
layer of EOMs. from its insertion to centre of globe; so the
• Figure 2.10C is the diagrammatic depiction of rotational axis of SO muscle shifts by half the
the muscle pulleys, horizontal recti and the horizontal duction.
suspension forces in the central gaze (axial
view) FIELD OF ACTION
• During adduction, the contracting orbital This term is used in two ways to describe
layer of the MR muscle pulls its pully entirely separate and distinct concepts. Field
posteriorly, while the relaxing lateral rectus of action may be used: (1) to indicate the
orbital layer allows its pulley to move direction of rotation of the eye, when a muscle
anteriorly (Fig. 2.10D). contracts, and (2) to refer to the gaze position
Rectus EOMs activity during eye movements in which the effect of the muscle is most
is as below: readily observed. The field of action for lateral
• Global layer (GL) contraction leads to rectus muscle and medial rectus muscle is same
movement of eyeball. The mechanical load of by both the concepts, i.e. abduction and
GL is predominantely the viscosity of relaxing adduction, respectively. However, they are not
antagonist EOMs and a load proportional to the same for other muscles, for example, the
the speed of rotation. inferior oblique muscle creates some vertical,
• Orbital layer (OL) contraction moves the pulley torsional, and horizontal movements whenever
posteriorly by the same distance as the scleral it contracts. Furthermore, the amount of vertical,
 30 Theory and Practice of Squint and Orthoptics

horizontal, and torsional changes depends on inferior oblique from one eye act as synergistic
the position of the eye. Thus, a field of action is elevators; however, in respect to torsion
not a single unvarying movement for the inferior movement, they act as antagonists, as the
oblique muscle. Also, only attempted elevation superior rectus produces intorsion while the
of the eye increases inferior oblique muscle inferior oblique produces extorsion.
activity; it does not increase with attempted Each extraocular muscle has two synergists
abduction. (The vertical rectus muscles also tend and two antagonists with exception of medial
to remain at similar levels of innervation across and lateral recti which have two synergists and
the horizaontal plane.) "Field of activation" would three antagonists (Table 2.2).
perhaps be a better term for this innervation
sense of what a muscle does. The inferior oblique Yoke muscles (contralateral synergists)
muscle is usually tested by its contribution to It refers to a pair of muscles (one from each eye)
vertical eye movement in the adducted position; which contract simultaneously during version
however, this is only because this is its field of movements, e.g. right lateral rectus and left
greatest vertical action. medial rectus muscles act as yoke muscles for
Thus, one must keep in mind three separate dextroversion movement. The yoke muscle pairs
things: (1) the plane of the muscle action, (2) the for six cardinal positions of gaze are listed in
gaze direction, which increases or decreases the Table 2.3.
innervation to the muscle, and (3) the vector According to recent theories, a pair of muscles
distribution of the muscle's force (vertical, in one eye can be yoked with a pair in the other
horizontal, torsional) in various gaze positions. eye. 10 For example, the elevators of one eye
The importance of fields of action is that a (superior rectus and inferior oblique muscles),
deviation (strabismus) that increases with gaze are yoked as a unit to the elevators of the fellow
in some directions is possibly due to weakness eye. Similarly, a pair of depressors of one eye
of the muscle normally pulling the eye in that are yoked with a pair of depressor from the
direction. For example, esotropia increasing fellow eye. Further, yoking may change
with gaze to the right may be due to right lateral according to the different types of eye movement;
rectus weakness. e.g. left medial rectus is yoked with right lateral

AGONIST, SYNERGISTS, Table 2.2 Agonist, synergist and antagonist extraocular

muscles
ANTAGONISTS AND YOKE MUSCLES
Agonist Synergists Antagonists
Agonist
Medial rectus Superior rectus Lateral rectus
It refers to any particular extraocular muscle Inferior rectus Superior
producing a specific ocular movement; e.g. for oblique
producing abduction in right eye, the right Inferior oblique
lateral rectus muscle is agonist. Lateral rectus Superior oblique Medial rectus
Inferior oblique Superior rectus
Synergists Inferior rectus
Two muscles moving an eye in the same Superior rectus Inferior oblique Inferior rectus
direction are called synergists. For example, Medial rectus Superior
oblique
superior rectus and inferior oblique muscles of
the same eye act as synergists for elevation Inferior rectus Superior oblique Superior rectus
Medial rectus Inferior oblique
movement.
Superior oblique Inferior rectus Inferior oblique
Antagonists Lateral rectus Superior rectus
These are the muscles having opposite action in Inferior oblique Superior rectus Superior
oblique
the same eye, e.g. medial and lateral recti.
Lateral rectus Inferior rectus
Further, as we know the superior rectus and
 Physiology of Ocular Motility 31

Table 2.3 Yoke muscle pairs From the Donders' law, it can be inferred that
only one orientation of the retinal meridians is
Agonist Synergists Antagonists
permissible with each position of the eyes. This
Cardinal direction of gaze Yoke muscle pair one and only one orientation does not allow for
Dextroversion Right lateral rectus the infinite number of other orientations around
Left medial rectus the line of sight that could exist, if the freedom
Levoversion Left lateral rectus of cyclorotations was unrestricted.
Right medial rectus Listing’s law
Dextroelevation Right superior rectus
Listing12 did not add anything essentially new
Left inferior oblique to the Donders' law; but he elaborated in
Levoelevation Left superior rectus considerable details on the geometry and
Right inferior oblique mathematics of the ocular rotations. In the
Dextrodepression Right inferior rectus analysis of eye movements, the question arises
Left superior oblique whether the eye performs a torsional movement
Levodepression Left inferior rectus around its anteroposterior axis, when it turns
Right superior oblique from the primary position into a tertiary
position. Listing's law implies that this is not the
case. It states that every eye movement from the
rectus for dextroversion and with the right
primary into tertiary position can be described
medial rectus for convergence.
as a rotation around one axis. For a given
Contralateral antagonists movement, this axis would be perpendicular to
(antagonist of the yoke muscles) the plane that contains the line of sight in the
primary position and the line of sight in the
This refers to a pair of muscles (one from each
tertiary position into which the eye has moved.
eye) having opposite action; e.g. right lateral
Such an axis would always be in Listing's plane.
rectus and left lateral rectus muscles. The term
Compared to the objective vertical of space, the
contralateral antagonist is commonly used in
vertical meridian of the cornea is tilted, when
inhibitional palsy. For example, in paralysis of
the eye is in tertiary position, i.e. instead of true
right lateral rectus muscle, there occurs
torsion movement, there occurs pseudotorsion
inhibitional palsy of the left lateral rectus
(defined below). According to Listing's law, this
muscle. However, this term is contradictory and
can be expected, if the eye turns around an
is thus not much used in general.
oblique axis in Listing's plane and is not the
consequence of an active torsional movement
FUNDAMENTAL LAWS around the anteroposterior (Y) axis.
GOVERNING OCULAR MOTILITY True torsion is a true movement around the
anteroposterior (Y) axis. As said above, true
Donders’ law torsion does not enter into the usual ocular
Donders’ law (1848) states that for each tertiary movements. Pseudotorsion is the deviation of a
position, there is one and only one orientation plumb line or true vertical and the vertical
of the vertical and horizontal meridians of meridian of the eye. When we have two
retina.11 This orientation depends solely upon coordinate systems, one fixed (orbital) and one
the amount of elevation and horizontal movable (globe), there is an angle produced,
movement and is independent of the path by when the moved system is compared to the fixed
which this position was arrived. There is no system. Such motion can occur without any true
rotation around the anteroposterior (Y) axis, i.e. torsional movement having actually been made.
no torsion or twist occurs. The eye reaches all
tertiary positions without movement around the Hering’s law of equal innervation
AP axis. However, the eye always returns to the This law, also known as Hering’s law of motor
same orientation from which it started. correspondence, states that an equal and
 32 Theory and Practice of Squint and Orthoptics

simultaneous innervation flows from the brain the concomitant excess supply to the yoke
to a pair of muscles of both eyes (yoke muscles) muscle from the normal eye causes excess
which contract simultaneously in different contraction leading to more, the so-called,
binocular movements. For example, an equal secondary deviation.
and simultaneous innervation flows to: 2. Inhibitional palsy of contralateral antagonist
• Left lateral rectus and right medial rectus muscle developing in patient with paralytic
muscles during levoversion (Fig. 2.11); squint is also based on Hering's law. For
• Both medial recti during convergence; and example, when right lateral rectus muscle is
• Right superior rectus and left inferior oblique paralysed, left lateral rectus muscle develops
muscles during dextroelevation. inhibitional palsy.
Hering's law is also necessary to explain the
This law is the major physiologic principle condition in superior oblique muscle paresis in
involved in the understanding of binocular which there is said to be an inhibitional paresis
motor co-operation of the eyes. When this law of the contralateral antagonist, when the paretic
was formulated by Hering in 1868, reference was eye is fixating. The term contralateral antagonist,
made only to the voluntary eye movements.13 when used in conjunction with the concept of
Many authors still write that Hering's law is inhibitional paresis, is a contradiction in terms.
valid for voluntary movements. Actually, it A more accurate description would be an
applies to all normal binocular eye movements inhibitional paresis of the antagonist of the yoke
including vergences and other involuntary muscle of the paretic muscle.
movements. However, it is not must that both For example, if a patient has a right superior
eyes should make an observable equal movement. oblique muscle paresis and fixates with the right
Because, under certain circumstances in spite of eye an object that is located up and to the
equal innervation to the yoke muscles, the two patient's left, less innervation of the right inferior
eyes may make unequal movements. oblique muscle is required to move the eye into
this gaze position, because it does not have to
Clinical applications of Hering's law overcome the normal antagonistic effect of the
1. Secondary deviation (deviation of normal eye right superior oblique muscle. Therefore,
under cover, when patient fixates with the according to Hering's law, less innervation is
squinting eye) is more than the primary received by the right inferior oblique muscle's
deviation (deviation of squinting eye, when yoke muscle, namely the left superior rectus
patient fixates with the normal eye) in patients muscle. This could lead to the incorrect
with paralytic squint. This is based on the impression that the left superior rectus muscle
Hering's law; since when the patient fixates with is paretic. This is what is implied by the phrase
the squinting eye, an excess innervation is "inhibitional paresis of the antagonist (left
required to the paralysed muscle to fixate and superior rectus muscle) of the yoke muscle (left
inferior rectus muscle) of the paretic muscle
(right superior oblique muscle)".
Sherrington's law of reciprocal innervation
This law states that during ocular motility, an
increased flow of innervation to the contracting
agonist muscle is accompanied by a decreased
flow of innervation to the relaxing antagonist
muscle.14 For example, during abduction, an
increased innervational flow to the lateral rectus
is accompanied by a decreased flow to the
Fig. 2.11 Equal (+++) and simultaneous innervations flow medial rectus of the same eye (Fig. 2.12).
to left lateral rectus and right medial rectus (yoke muscles) With the help of electromyographic studies,
during levoversion. the validity of Sherrington's law of reciprocal
 Physiology of Ocular Motility 33

OCULAR MOVEMENTS
MONOCULAR EYE MOVEMENTS (Ductions)
These are called 'ductions' and include the
following:
1. Adduction. An inward movement (medial
rotation) along the vertical axis.
2. Abduction. An outward movement (lateral
rotation) along the vertical axis.
3. Supraduction (sursumduction). An upward
Fig. 2.12 During abduction, an increased innervational flow movement (elevation) along the horizontal axis.
(+++) to the lateral rectus muscle is accompanied by propor-
tionate decreased flow to the medial rectus muscle (– – –). 4. Infraduction (deosursumduction). A downward
movement (depression) along the horizontal
axis.
innervation has been established in intact 5. Incycloduction (intorsion). A rotatory movement
human eyes (Fig. 2.13). Co-contraction of along the anteroposterior axis in which superior
antagonistic muscles (instead of relaxation of the pole of the cornea (12 o'clock point) moves
antagonist muscle) occurs in certain pathologic medially.
conditions (e.g. Duane's retraction syndrome 6. Excycloduction (extorsion). A rotatory movement
and retraction nystagmus). These conditions along the anteroposterior axis in which superior
would seem to represent as exceptions to the pole of the cornea (12 o'clock point) moves
Sherrington's law. laterally.
Clinical applications of Sherrington's law BINOCULAR MOVEMENTS
• Occurrence of strabismus following paralysis These are of two types—versions and vergences.
of an extraocular muscle is explained by
Sherrington’s law of reciprocal innervation. VERSIONS
• Reciprocal innervation must be kept in mind Versions, also known as conjugate movements,
while performing surgery of extraocular are synchronous (simultaneous) symmetric
muscles. movements of both eyes in the same direction.

Fig. 2.13 Electromyographic (EMG) tracing during adduction movement depicts increased activity from the medial rectus
(MR) and decreased activity from the lateral rectus (LR).
 34 Theory and Practice of Squint and Orthoptics

Versions may be voluntary or involuntary Depending upon the specific features of the eye
movements. Involuntary versions are semireflex movements (irrespective of the direction of
movements occurring in response to optical, movement)
acoustic, or other stimuli. The versions include the following movements:
1. Saccadic movements. These are rapid
Classification of versions conjugate eye movements performed to bring
According to the direction of binocular movement the image of an object quickly on the fovea.
1. Dextroversion. In it, both eyes rotate to the right These movements may be voluntary or
(Fig. 2.1D). It results due to simultaneous involuntary which may occur following a
contraction of right lateral rectus and left medial number of stimuli which may be optical,
rectus muscles. acoustic or other. Examples of different types of
2. Levoversion. In it, both eyes rotate to the left saccadic (jumping) or rapid eye movements are
(Fig. 2.1F). It is produced by simultaneous as follows:15
contraction of left lateral rectus and right medial • Command random movements.
rectus muscles. • Voluntary refixation saccades.
3. Supraversion (sursumversion). In it, both eyes • Sensory evoked saccades (visual, auditory).
rotate straight upward (Fig. 2.1B). It results due
• Nystagmus fast phase: (a) pathological,
to simultaneous contraction of bilateral superior
(b) induced: optokinetic nystagmus (OKN),
recti and inferior oblique muscles.
vestibular.
4. Infraversion (deosursumversion). It is straight
• Rapid eye movement (REM) of sleep.
downward rotation of both eyes (Fig. 2.1H). It
results due to simultaneous contraction of • Rapid pursuits (above 45°/sec.).
bilateral inferior recti and superior obliques • Microsaccades.
muscles. Characteristic features of saccades are as follows:
5. Dextroelevation. In it, both eyes rotate up and • The purpose of saccades is to place the image
to the right (Fig. 2.1A). It results from on the fovea and to keep it there as long as it
simultaneous contraction of right superior attracts attention.
rectus and left inferior oblique muscles. • For production of saccades, alertness is
6. Dextrodepression. In it, both eyes rotate down required.
and to the right (Fig. 2.1G). It is brought about
• During saccadic movements, although the
by simultaneous contraction of right inferior
visual world is rapidly sweeping across the
rectus and left superior oblique muscles.
retina, there is no sense of blurring. This
7. Levoelevation. In it, both eyes rotate up and to
phenomenon is called 'saccadic omission'.
the left (Fig. 2.1E). It results from simultaneous
• Saccades are blastic movements and proceed
contraction of left superior rectus and right
according to the preprogrammed velocity.
inferior oblique muscles.
Once initiated, they cannot be stopped or
8. Levodepression. In it, both eyes rotate down and
modified during the course of movement.
to the left (Fig. 2.1I). It results from simultaneous
contraction of left inferior rectus and right • There is a long delay of about 200 msec from
superior oblique muscles. stimulus to execution.
9. Dextrocycloversion. It is rotational movement • Velocity of saccadic movement ranges from
around the anteroposterior axis, in which 100°/sec. to 700°/sec.
superior pole of both the cornea tilts towards 2. Smooth pursuits or following movements. These
right. It results from simultaneous contraction are made when tracking a moving object. Thus
of inferior rectus and inferior oblique muscles the function of the saccadic eye movements is to
of the right eye and superior rectus and superior correct the position error between the target and
oblique muscles of the left eye. fovea; and the function of the pursuit system is to
10. Levocycloversion. It is just the reverse of the match eye velocity to target velocity. For example,
dextrocycloversion. the pursuits are performed while watching a bird,
 Physiology of Ocular Motility 35

which is moving right and left, up and down. Thus, the head is tipped backward, the eyes will
the pursuits help to keep the image of a moving rotate downward (and vice versa). This
object on the fovea constantly. phenomenon of tonic eye movements is also
called as 'doll's eye' mechanism.
Characteristic features of pursuits are as follows:
• Images moving away from the fovea constitute Note: In monocular vision, the saccades,
the strongest stimuli for pursuit movements. pursuits, position maintenance movements and
the stabilization movements are a form of
• Pursuit movements are elicited after a latency ductions.
of 125 msec.
• Smooth following movements can keep up
with targets moving up to 30° to 40°/sec. VERGENCES
Beyond that point, the eyes tend to fall behind Vergences are disjugate, synchronous and
and saccades have to be made to catch up. symmetric movements of the two eyes in
• The system has a very limited ability to follow opposite direction. Vergences are tonic move-
targets moving back and forth; beyond 2 Hz, ments and are much slower than the versions
it breaks down. (with a velocity of 80 to 250 times per second).
A vergence may be a voluntary movement or
• Only one image can be tracked normally.
an optomotor reflex. The same muscle in one
• Usually, pursuits are performed to track an eye may be involved in both versions and
image of some real object in space. But it can vergences. However, the underlying neuro-
also be an after image placed on the retina of muscular mechanism may be quite different. For
some real object in space or a bright light. Also, example, in the pathologic condition of a lesion
a few people are able to track hallucinated interrupting the medial longitudinal fasciculus
targets, suppressing the saccadic system. in the midbrain between the left sixth nerve
• The effectiveness of pursuit system is nucleus on one side and the opposite right third
dependent on the degree of alertness. nerve nucleus, the right medial rectus may show
3. Position maintenance movements. These help impairment of adduction on a movement of
to maintain a specific gaze position by means of levoversion. However, it may demonstrate good
rapid micromovements called 'flicks' and slow adduction on convergence. This condition is
micromovements called 'drifts'. known as internuclear ophthalmoplegia.
4. Stabilization movements. These include
dynamic and tonic movements: Types of vergences
• Dynamic movements. Suppose one is sitting in Depending on the direction of movement,
a bus watching a bird out of the window. As vergences are of following types:
the bus bounces, the eyes of the observer do 1. Convergence. It is simultaneous and
not go off the target; since there is an synchronous inward rotation of both eyes which
immediate correction from the vestibular results from co-contraction of the two medial
system to the eye muscles to correct for rotation rectus muscles. For details, see page 152.
of the head. Thus if the head is turned to the
2. Divergence. It is simultaneous and asynchro-
right, the eyes turn to the left.
nous outward rotation of both eyes which
• Tonic movements. Gravity and other linear results from co-contraction of the two lateral
accelerations also influence eye position by rectus muscles. For details, see page 161.
labyrinthine reflexes from the otoliths. These
influences persist with head position and are 3. Vertical vergence. By definition, vertical
not transient inputs just during the head vergence refers to disjugate vertical movements
movements. Examples of tonic movements: of the two eyes in opposite direction, i.e. one
(1) when one is lying supine and the head is eye should rotate upward and the other
rotated to the right, the eyes will rotate to the downward. However, in practice, the vertical
left (and vice versa); (2) if while standing erect, vergence movements are classified as positive
 36 Theory and Practice of Squint and Orthoptics

vertical divergence (in which, the right eye 2. Donders FC: On the Anomalies of Accommo-
rotates upward in relation to the left one and dation and Refraction of the Eye. R/London,
Hatton, 1952, p 404.
so also called as right supravergence or right
3. Howard IP, Templeton WB: Human Spatial
sursumvergence) and negative vertical divergence Orientation, London, John Wiley, 1966.
(in which, the left eye rotates upward in 4. De Groot JA, Scott AB, Sindon A, Authier L: The
relation to the right one and so also called as human ocular anatomic position of rest: a
left supravergence or left sursumvergence). quantitative study. In Fells P (ed): Second
Congress International Strabismological
In practice, the vertical vergence movements
Association, 1974, Marseilles, Marseilles,
are said to occur in a bid to correct the vertical Diffusion Generale de Librairie, 1976, pp 408–414.
heterophoria. Hence, these are also reflexly 5. Volkmann AW: On the mechanics of the eye
controlled and stimulated by retinal image muscles. In Berichte der K sachs Ges d Wissensch
disparity. The amplitude of vertical fusional math-phys K1 21:28, 1869. Summarized by H von
vergences is much smaller than the amplitude Helmholtz in: Physiological Optics, 3rd ed,
Leipzig, 1910. Translated and edited by JPC
of horizontal fusional vergences. The average Southall. R/New York, Dover, 1962, III, p 128.
amount of vertical fusional vergence is reported 6. Nakagama T: Topographic anatomic studies on
to be 3D to 8D. the orbit and its contents. Acta Soc Ophthalmol
4. Cyclovergence. Cyclovergence is the Jap 69: 2155, 1965.
7. Duane, A: A new classification of the motor
disjugate torsional movement in which the anomalies of the eye, Ann. Ophthalmol. Oto-
vertical meridians of the two eyes move in laryngol. 5:969, 1896.
opposite directions to each other. Incyclover- 8. Duane, A: The basic principles of the diagnosis
gence is a torsional disjugate movement in in motor anomalies of the eye, Arch. Ophthalmol,
which the upper end of the vertical meridian 48:2, 1919.
9. Duane, A: Anomalies of ocular muscles, Arch.
(superior pole or 12 o'clock position) in each
Ophthalmol, 11:394, 1934.
eye tilts towards the nose. Excyclovergence is 9a. Demer II. Pivotal role of orbital connective tissues
opposite to incyclovergence, i.e. in it the upper in binocular alignment and strabismus. The
end of the vertical meridian in each eye tilts fundamental lecture. Invest Ophthalmol Vise Sci
away from the nose. Cyclovergences are also 2004, 45;72–38.
involuntary movements stimulated by retinal 10. Boeder, P: Cooperative action of extraocular
muscle, Br J Ophthalmol. 46:397, 1962.
image disparity due to cyclophoria and thus 11. Donders, FC Beitrag zur Lehre von den
occur in the interest of fusion to compensate Bewegungen des menschlichen Auges, Holiand
for the cyclophoria. Their amplitude is Beitr. Anat. Physiol. Wiss. 1:104, 384, 1848.
expressed in degrees with average values being 12. Listing, JB: Reported from Listing's original work.
6° to 10° for incyclovergence and 4° to 8° for In Ruete, C.g.Th., Lehrbuch der Ophthalmologie
fur Aerzte and Studirende, ed. 2, vol 1,
excyclovergence. Thus, these movements are
Braunschweig, 1855. Friedr Vieweg and Sohn
slow, of low amplitude, and their significance GHBH. 37ff.
in compensating for a manifest cyclodeviation 13. Hering, E: die Lehre vom Binocularen Sehen,
is not clear. These findings do not invalidate Leipzig, 1868, Wilhelm Engelmann.
the concept that cyclofusion occurs predo- 14. Sherrington, CS: Experimental note on two
minantly on a sensory basis.16 movements of the eyes, J Physiol (Lond.) 17:27, 1894.
15. Daroff, RB: Physiologic, anatomic and patho-
physiologic considerations of eye movements,
BIBLIOGRAPHY Trans. Ophthalmol. Soc. U.K. 90:409, 1970.
16. Noorden, GK von: Clinical and theoretical aspects
1. Park GS, Park GE: The center of ocular rotation of cyclotropia, J Pediatr Ophthalmol Strabismus
in a horizontal plane. Am J Physiol 104:542, 1933. 21:126 1984.
 3
Visual Acuity and
Contrast Sensitivity
VISUAL ACUITY • Measurement of visual acuity in infants
General Considerations • Assessment of visual acuity from 1 to 3 years
• Visual angle • Measurement of visual acuity in preschool children
Components of Visual Acuity (3–5 years)
• Minimum visible • Measurement of visual acuity in school children
• Resolution (above 5 years) and adults
• Recognition • Measurement of visual acuity for near
• Hyperacuity CONTRAST SENSITIVITY
Measurement of Visual Acuity • Introduction
• Milestones in development of vision • Types of contrast sensitivity
• Tests for visual acuity assessment • Measurement of contrast sensitivity

VISUAL ACUITY

GENERAL CONSIDERATIONS
The vision or visual perception is a complex
integration of light sense, form sense, contrast
sense and colour sense. Visual acuity is
considered a measure of form sense, so it refers Fig. 3.1 Visual angle (ANB) subtended at the nodal point by
to the spatial limit of visual discrimination. the physical dimensions (AB) of the object.
Technically speaking, visual acuity measure-
ment involves the determination of a threshold. It has been observed that the two adjacent
In terms of visual angle, the visual acuity is points can be seen clearly and discretely only
defined as the reciprocal of the minimum when these two points (say A and B in Fig. 3.1)
resolvable visual angle measured in minutes of produce a visual angle not less than 1 minute.
arc for a standard test pattern. Therefore, to The dimensions of the visual angle depend upon
understand visual acuity, the knowledge about the size of the object as well as its distance from
visual angle is essential. the eye. Therefore, to be seen clearly either the
object should be large enough or it should be
VISUAL ANGLE placed near the eye (at an appropriate distance).
Visual angle is the angle subtended at the nodal In terms of the length of the retinal image, it has
point of the eye by the physical dimensions of been seen that the two points (A and B) will be
an object in the visual field (Fig. 3.1). Visual seen clearly when their image size (A9B9) is
angle is a useful and convenient mode of more than 4.5 m. This is so, because the diameter
specifying the spatial extent of objects or of individual cone stimulated by the image
elements in the visual field. points A9 and B9 is 1.5 m each and at least one
37
 38 Theory and Practice of Squint and Orthoptics

cone in between (of 1.5 m diameter) must be RECOGNITION

unstimulated. The retinal image size for a given It is that faculty by virtue of which an individual
visual angle may vary slightly with changes in not only discriminates the spatial characteristics
viewing distance and associated changes in of the test pattern but also identifies the patterns
accommodation of the lens, but this effect is with which one has had some experience.
relatively small. Recognition is thus a task involving cognitive
components in addition to spatial resolution. For
COMPONENTS OF VISUAL ACUITY recognition, the individual should be familiar
In clinical practice, measurement of the with the set of test figures employed in addition
threshold of discrimination of two spatially to being able to resolve them. The most common
separated targets (a function of the fovea example of recognition phenomenon is
centralis) is termed visual acuity. However, in identification of faces. An average adult can
theory, visual acuity is a highly complex recognize thousands of faces.
function that consists of the following
components: MINIMUM DISCRIMINABLE OR HYPERACUITY
• Minimum visible, Minimum discriminable refers to spatial
• Resolution, distinction by an observer when the threshold is
• Recognition, and much lower than the ordinary acuity. The best
• Minimum discriminable. example of minimum discriminable is vernier
acuity, which refers to the ability to determine
MINIMUM VISIBLE whether or not two parallel and straight lines are
The ability to determine whether or not an object aligned in the frontal plane. The threshold values
is present in an otherwise empty visual field is of vernier acuity (Fig. 3.2) are in the range of only
termed visibility or detection. This kind of task is a few seconds (2–10) of arc. Hyperacuity should
referred to as the minimum visible or minimum not be confused with the threshold for the
detectable. minimum visible, where merely the presence or
absence of a target is being judged. The
RESOLUTION (ORDINARY VISUAL ACUITY) mechanism subserving hyperacuity is not clearly
known, but so much is clear; no contradiction is
Discrimination of two spatially separated targets
involved with the optical and receptor mosaic
is termed resolution. The minimum separation
factors that limit ordinary visual acuity.
between the two points, which can be
discriminated, is known as minimum resolvable.
Measurement of the threshold of discrimination
MEASUREMENT OF VISUAL ACUITY
is essentially an assessment of the function of the As discussed earlier, the visual acuity is a highly
fovea centralis and is termed ordinary visual acuity. complex function that consists of:
The distance between the two targets is specified
by the angle subtended at the nodal point of the
eye. The normal angular threshold of discrimina-
tion for resolution measures approximately 30–
60 seconds of an arc; it is usually called the
minimum angle of resolution (MAR). The clinical
tests determining visual acuity measure the form
sense or reading ability of the eye. Thus, broadly,
resolution refers to the ability to identify the
spatial characteristics of a test figure. The test
targets in these tests may either consist of letters
(Snellen’s chart) or broken circles (Landolt’s ring).
More complex targets include gratings and Fig. 3.2 Typical target configuration for detecting vernier
checkerboard patterns. acuity.
 Visual Acuity and Contrast Sensitivity 39

• Minimum visible, i.e. detection of presence or between 30 seconds and 1 minute of arc.
absence of stimulus, Therefore, all the clinical tests employed to
• Minimum separable, i.e. judgement of location measure the visual acuity are designed taking
of a visual target relative to another element into consideration the threshold of the one
of the same target, and minimum resolvable. Based on this basic
• Minimum resolvable (ordinary visual acuity), principle, many visual acuity charts have been
i.e. the ability to distinguish between more developed.
than one identifying feature in a visible
target. MILESTONES IN DEVELOPMENT OF VISION
In clinical practice, the measurement of visual Before discussing the various methods of visual
acuity is considered synonymous with the assessment in infants, children, and adults, it will
measurement of ‘minimum resolvable’. (However, be worthwhile to have a quick look on the visual
in theory, it is not so, as is clear from the above.) development. Important milestones in develop-
The threshold of the minimum resolvable is ment of vision are summarized in Table 3.1.

Table 3.1 Milestones in the development of vision

Age Visual milestone Visual acuity
Newborn • Pupillary reaction to light 6/360 to 6/120 (by OKN)
• Blinking to light stimulus
• Conjugate horizontal gaze developed
1 week • Vestibulo-ocular reflex
2 weeks • Small saccades develop
• Follows horizontal moving objects
1 month • Fixation developing 6/480–6/120 (by PL tests)
• Can watch mother’s face for prolonged time
2 months • Bifoveal fixation 6/120–6/60
• Large saccades
• Pursuits and convergence movements
• Conjugate vertical gaze developed
3 months • Watches movements of own hands and reaches
out towards interesting objects
• Prefers photographs to patterns
4 months • Foveal differentiation complete 6/120–6/30
• Sensory fusion and accommodation begins to develops
5 months • Blink response to visible threat (menace response) 6/90–6/24 6/12–6/6 (by VER)
• Grasps and explores objects
• Stereopsis begins to develop
6 months • Accommodation well developed 6/90–6/24 6/12–6/6 (by VER)
• Fusionalvergence well developed
9 months • Visual differentiation of objects
• Picks up small objects 6/48–6/12 6/6 (by VER) 18
18 months • Visual acuity at adult levels on paediatric acuity card
• Myelination of optic nerve completed 6/18–6/7.5
2–3 years • Best visual acuity approaches near adult levels, 6/12–6/6 (36 months)
but may not be 6/6
• Can play picture or letter recognition games
• Can respond to some binocular vision tests
• Contrast sensitivity well developed
5 years • Stereopsis fully developed 6/6–6/5
8–10 years • Critical period of monocular deprivation ends 6/6–6/5
 40 Theory and Practice of Squint and Orthoptics

TESTS FOR VISUAL ACUITY ASSESSMENT MEASURMENT OF VISUAL ACUITY IN INFANTS

Various visual acuity tests available can be
ASSESSMENT OF VISUAL ACUITY FROM
grouped as follows:
BIRTH TO 3 MONTHS
I. Detection acuity tests. These assess the ability At birth,visual acuity is 1/60 which improves
to detect the smallest stimulus without very fast to 2/60 at 1 month and 6/60 at 4 months.
recognizing correctly. Common detection acuity With 1/60 vision, the child is able to fix a face
tests are: moving within one metre. The fixation reflex and
1. Dot visual acuity test following reflexes take about 6–8 weeks to
2. Catford drum test develop before which an infant may fix for a few
3. Boek candy bead test seconds and give up. There are a few bizarre
4. STYCAR graded ball’s test movements which appear till the development
5. Schwarting metronome test of definite fixation reflex. Neonates have
sporadic jerky movements made up of saccadic
II. Recognition acuity tests. These are designed
eye movements without smooth pursuit. So,
to assess the ability to recognize the stimulus or
Visual acuity in a newborn and infant up to
to distinguish it from other competing stimuli.
3 months of age can be determined by the tests
These include:
given below.
A. Direction identification tests
1. Snellen’s E-chart test 1. Blink reflex test. Blink reflex is present since
2. Landolt’s C-chart test birth (after 30 weeks of gestational age). It is
occasionally present in decorticate infants as
3. Sjogren’s hand test
well. When bright light is shown, a normal infant
4. Arrows test
should respond by blinking.
B. Letter-identification tests
2. Pupillary light reflex test. Presence of
1. Snellen’s letter chart test pupillary light reflex indicates intact afferent
2. Sheridan’s letter test visual neurologic pathways to the level of the
3. Flook’s symbol test brachium of the superior colliculus and efferent
4. Lipman’s HOTV test pathways to the iris sphincter. This reflex is
C. Picture identification charts (miniature toy test) present in premature babies over 29–31 weeks
1. Allen’s picture cards test of gestational age. This is most reliable test to
2. Beale Collins picture charts test determine presence of vision except in cortical
blindness. The test is best performed in a semi-
3. Domino cards test
darkned room because the infant’s pupils are
4. Lighthouse test
smaller than that of a normal adult and constrict
5. Miniature toy test of Sheridan in the presence of bright light in the room. In
D. Tests based on picture identification on the semi-dark room, the pupil comes to a state
behavioural pattern of semi-dilatation that reacts briskly. The light
1. Cardiff acuity cards test used should be small, well focused and bright.
2. Bailey Hall cereal test Visualization in very young children sometimes
requires a magnifying glass, as their pupils are
III. Resolution acuity tests
smaller than those of the older children (because
1. Optokinetic nystagmus (OKN) test
of decreased sympathetic tone) and the light
2. Preferential looking test (PLT) responses are of small amplitude.
i. Two-alternative forced choice test
3. Vestibulo-ocular reflex. The vestibulo-ocular
ii. Operant variation looking test
reflex (VOR) is generally tested by turning the
iii. Teller acuity cards test newborn’s head on his/her long axis and
3. Visually evoked response (VER) observing for the doll’s eyes response (the eyes
Tests employed for visual acuity assessment deviate opposite to the direction of head
at various age groups are summarized in Table 3.2. rotation).
 Visual Acuity and Contrast Sensitivity 41

Table 3.2 Commonly used visual assessment tests at various age groups
Age Tests for assessment of vision Type of visual acuity
Birth–3 months • Blink reflex Resolution acuity
• Pupillary light reflex
• Vestibulo-ocular reflex test
• Eye popping test
• OKN
• VER
3–6 months • Fixation and following of objects or small toys Resolution acuity
• CSM (central, steady and maintained) fixation
• Response to occlusion
• OKN
• VER
6–12 months • Preferential looking tests (Teller acuity tests) Resolution acuity
• Catford drum test Detection acuity
1–3 years • Cardiff acuity tests Resolution + Recognition
• Marble game test acuity
• TYCAR ball test Detection acuity
• E game test
• Boeck candy test
3 –5 years • Broken wheel test
• Landolt C test
• Isolated hand figure test
• Pictorial vision chart tests Recognition acuity
• Tumbling E test
• HOTV test
• Snellen’s numbers
• Snellen’s letters
Above 5 years • Snellen’s numbers
• Snellen’s letters chart Recognition acuity
• LogMAR chart

4. Eye popping test. Another behaviour that is succession of black and white stripes by means
unique to babies is eye popping. Sometimes, for a of OKN drum of the size 10 × 8 inches diameter,
variety of reasons, very young infants don’t which is rotated at 8–10 rpm through the
show any distinguishable visual behaviour at patient’s field of vision (Fig. 3.3). Eyes respond
all. In this case, the eye popping reflex indicates with a slow movement in the direction of drum
at least the baby’s ability to detect changes in lasting about 0.2 sec and fast phase in the reverse
the room illumination. When the room lights are direction of 0.1 sec. The visual angle subtended
suddenly dimmed, the baby’s upper eyelids by the smallest strip width that still elicits an
should pop open wide for a moment. The baby eye movement (minimum separable) is a
will often close its eyes when the lights are measure of visual acuity. The only cooperation
brought up back, but will again pop its eyes open required in this test is that the infant be awake
when the lights are dimmed. This behaviour is and should hold both eyes open. It is reported
documented as “positive eye popping. that OKN acuity is at least 6/120 in the
5. Optokinetic nystagmus (OKN) test. It is an newborns and improves fairly rapidly during
objective method of visual assessment in infants the first few months of life, reaching to a level
and uncooperative children as well as adults. of 6/60 at 2 months, 6/30 at 6 months and 6/6
In this test, nystagmus is elicited by passing a by 20–30 months. OKN is asymmetric in
 42 Theory and Practice of Squint and Orthoptics

Fig. 3.3 Optokinectic nystagmus test for visual acuity

(Courtesy: Dr Elizabeth Joseph).

newborns and becomes symmetric by 4–6

months of age.
6. Visual evoked response (VER). It refers to
electroencephalographic (EEG) recording made
from the occipital lobe in response to visual
stimuli. VER is the only clinically objective
technique available to assess the functional state
of the visual system beyond the retinal ganglion
cells. It is quite useful in assessing visual
function in infants. It reflects acquity from the
central retina and thus forms a good macular
function test.
Flash VER is usually preformed in very young
children or those incapable of fixing on a target.
It just tells about the integrity of the macular and
visual pathway.
Pattern reversal VER is recorded using some Fig. 3.4 Technique of recording visually evoked response
(VER) and record of normal VER pattern.
patterned stimulus, as in the checkerboard
(Fig. 3.4). In it, the pattern of stimulus is changed
(e.g. black squares go white and white become Note: The discrepancy between estimated visual
black), but the overall illumination remains the acuity values with optokinetic nystagmus,
same. The pattern reversal VER depends on preferential looking test and visually evoked
form sense and thus gives a rough estimate of response at 6 months of age must be kept in
the visual acuity. VER studies have shown mind while performing these tests (Table 3.3).
visual acuity in infants to be 6/120 at the age of
1 month, which reaches to 6/60 at 2 months and ASSESSMENT OF VISUAL ACUITY
6/6–6/12 at the age of 6 months to 1 year. FROM 3 TO 6 MONTHS
Since the fixation develops to moving objects by
Drawbacks of VER include: 3-4 months of age, the visual acuity in this age
• Expensive group can be assessed, in addition to the above
• Time consuming mentioned tests, the help of following tests
• Limited availability based on fixation behaviour of the infant.
• Not standardized 1. Fixation behaviour test. Ability of the child to
• Little clinical relevance fix and follow the face of the examiner, toys or
 Visual Acuity and Contrast Sensitivity 43

Table 3.3 Estimated visual acuity at different ages attention to be fixed at a distance. Assess the red
Age Optokinetic Preferential Visually evoked
reflex both before and after dilatation to see how
(months) nystagmus looking test response
much of the pupillary space is obscured. An
overall whitening of the red reflex across the
1 6/120 6/120 6/120
entire pupil of one eye indicates strabismus or
2 6/60 6/60 6/60
anisometropic amblyopia. While the absence of
6 6/30 6/30 6/6–6/12
a Brückner reflex is not a good indication of
Age 20–30 24–36 6–12
alignment, the presence of a Brückner reflex is
(months)
considered a positive result, and is a good
at which
indication of strabismus, even of small amounts.
6/6 is
4. Menace reflex test. Menace reflex, i.e. reflex
achieved
closure of the eyes on the approach of an object,
is usually present after the age of 5 months, if
interesting object. The test is done first with both vision is normal.
eyes open followed by monocular testing by 5. Cover test. By 3–6 months, infants have
occluding the other eye by hand. If the child adequate refixation reflex to permit cover test.
habitually fixates with one eye, it indicates poor This test is needed, if there is a concern about
vision in the non-fixating eye and hence he or strabismus. In patients with normal vision, both
she will violently resist occlusion of the better eye. eyes look at an object at the same time.
2. Central, Steady, Maintained (CSM) method. Therefore, if one eye is occluded, the opposite
CSM method is a useful test in this age groups. eye should not move. In patients with strabismus,
It implies: one eye is deviated. If the straight eye is covered,
• Central. The infant is asked to fixate on the other eye will make a movement to line up
penlight and then the examiner looks at the the visual target. If a patient is exotropic, the eye
corneal light reflex from a fixation light which will make an inward movement. If an eye is
is falling at the centre of the pupil. The reflex esotropic, it will make an outward movement.
is considered central, if it falls in the same
location in both the eyes in monocular ASSESSMENT OF VISUAL ACUITY FROM
condition. 6 TO 12 MONTHS
• Steady. This is tested with a small target (thumb- In addition to the above mentioned tests, the
sized toy) which is coupled with light held in tests described below are more useful in 6–12
front of the child and moved slowly. months age group.
Nystagmus or oscillation results in unsteady 1. Preferential looking test (PLT). This test is
fixation. based on the observation that when presented
• Maintained. The ability to keep the eye fixed with two adjacent stimulus fields, one of which
when either eye is covered. is striped and the other is homogeneous, the
Results of this test can be interpreted as below: infant will tend to look at the striped pattern for
• CSM: 6/9 to 6/6 a greater portion of the time. Test procedures
• CSNM: 6/36 TO 6/60 have been developed in which an examiner is
• Unsteady central fixation: <6/60 hidden behind a screen on which one projects a
3. Bruckner’s red reflex test. Bruckner’s reflex homogeneous surface on one side and black and
is helpful in children uncooperative to the cover white stripes on the other side. These two stimuli
test when an assessment is being carried out for are alternated randomly. The observer is able
small angle strabismus. In this test, fixation and to look at the eyes of the infant through a hole
binocular comparison of the red reflex is done. in the screen but is unaware of which target,
The examiner should stay far enough to stripes or homogeneous field is presented on
illuminate both pupils by the same direct which side of the screen.
ophthalmoscope beam. The examination should Teller acuity cards (TAC) test. This is the most
be carried out in dim illumination and the child’s commonly used preferential looking test in
 44 Theory and Practice of Squint and Orthoptics

clinical practice. TAC is recommended to test pictures or Snellen’s letters. In normal children,
visual acuity in infants from 1 month to 1 year grating acuity is better than recognition acuity.
of age. This test is modification of preferential Further, it has been suggested that different
looking test. This is simple to perform and very neural processing mechanisms in the brain are
reliable and efficient test. The testing distance involved with spatial discrimination and
varies with age of the child, like the test being recognition tasks. Hence, it is not advisable to
performed at 36 cm in infants and toddlers, at equate grating acuity with recognition acuity
54 cm in children up to 3 years and at 84 cm in (Snellen’s).
adults. Estimates of visual acuity, using the TAC Limitations. TAC are relatively expensive and
grating targets, show a rapid increase in acuity less cost effective. Therefore “budget” versions
during the first six months of life from 1.0 cycle of FPL acuity testing have emerged in the form
per degree at one month of age to five cycles of spatial frequency paddles.
per degree by six months of age, then a gradual 2. Catford drum test. It is a detection acuity test,
increase to 40 cycles per degree. Adult like levels useful in infants and children less than 2 years
are reached at 5 years of age. The results are of age. In this test, the child is made to observe
obtained in cycles which can be converted to an oscillating drum with black dots of varying
Snellen’s equivalent. There are 17 cards, on one sizes ranging from 0.5 to 15 mm in diameter
half of each card is a set of vertical black-and- representing vision between 6/6 and 2/60
white bars of varying size which form the (Fig. 3.6). Rotation of disc at a distance of 60 cm
pattern stimulus and on the other half, a uniform evokes pendular movements. The smallest dot
gray background which is the blank target that evokes pendular eye movements (not an
(Fig. 3.5). In the centre of each card, there is a OKN) denotes the level of visual acuity. This test
small hole through which the examiner observes is unreliable since it overestimates the vision.
the infant’s fixation. In this by varying the spatial
frequency of the bars shown, the finest bar which ASSESSMENT OF VISUAL ACUITY
can no longer be resolved by the infant is used FROM 1 TO 3 YEARS
to determine the vision as the infant no longer
Above 1 year of age, the child is able to visually
shows the preference for patterned stimulus.
differentiate the small objects and is able to reach
This test can be used effectively on neuro-
out for toys. So, in addition to the above-
logically impaired children.
mentioned tests, the following detection acuity
Visual acuity determined with this method
tests are more useful in this age group.
has been reported to range from approximately
6/240 in the newborns to 6/60 at 3 months and
6/6 at 36 months of age. It must be well
understood that grating acuity testing cannot
automatically be equated with acuity testing
based on recognition task, such as naming

Fig. 3.5 Tellers acuity card test (a type of preferential

looking test). Fig. 3.6 Catford drum for visual acuity.
 Visual Acuity and Contrast Sensitivity 45

Limitations include:
• May miss some cases of visually significant
refractive errors.
• TAC is more dependable test to assess
amblyogenic conditions despite the use of
gratings.
1. Marble game test. In children of 6–12 months
of age, reaching or placing games can be used
Fig. 3.7 Cardiff acuity cards test.
to estimate visual function. One such game is
the ‘marble game’. In it, the child is asked to
1. Cardiff acuity cards test. Cardiff acuity cards place marbles in the holes of a card or in a box.
test or vanishing optotypes test (Fig. 3.7) is used This test is not intended to measure visual acuity
to measure visual acuity in this age group. The of each eye, but rather to compare the
principle is that as long as the child can see the functioning of the child’s eye when one or the
optotype (line drawings of pictures of fish, car, other is closed. The vision of an eye is then noted
etc.), the child will show a preference for the as being ‘useful’ or ‘less useful’.
picture as compared with the plain grey 2. Sheridan’s ball test. Mary Sheridan (1960)
background. The black and white lines forming used a series of styrofoam balls of progressively
the pictures become finer with each set of three smaller sizes. One records the smallest ball that
cards, until the picture cannot be seen (vanishing the infant can fixate and follow at a distance of
optotype) and the preference for fixation to the 10 ft. Rolling the ball on a white or grey
picture is lost. The pictures are presented on background and asking the child to pick it up,
cards with the optotype appearing either on the and noting the smallest size to which the child
top or the bottom of the card. The rest of the gives a good response is a rough way of
card is a homogenous grey that matches with estimating visual acuity.
the mean luminance of the picture. A total of 11 3. Worth’s ivory ball test. Ivory balls ranging
sets of cards are available, with acuity values in size from 0.5 to 2.5 inches in diameter are
ranging from 20/400 to 20/20, which have been rolled on the floor in front of the child who is
calibrated for two presentation distances—0.5 m asked to retrieve each ball. Acuity is estimated
and 1 m. The patient is presented with one sets on the basis of smallest size of the ball for the
of cards at a particular acuity equivalence, one test distance.
card at a time. By observing the child’s eye 4. Dot visual acuity test. Child is shown an
movements and fixations, the examiner must illuminated box with black dots of different sizes
decide if the optotype is on the top or bottom of printed on it. The smallest dot identified denotes
the card. the visual acuity of the child.
The acuity is determined by the narrowest 5. Coin test. In this test, the child is asked to
white band for which the target is visible to the identify the two faces of coins of different sizes
child and correct response is obtained at least held at different distances.
75% of the time when the particular finest line 6. Miniature toy test. In this test, the child is
drawing is shown to the child. shown a miniature toy from a distance of 10 ft
and is asked to name or pick the pair from the
Advantages include: assortment.
• It is an excellent way to determine minimum MEASUREMENT OF VISUAL ACUITY IN
separable acuity in a child 1–3 years of age, PRESCHOOL CHILDREN (3–5 YEARS)
unless the child can respond to recognition At this age, the child is able to verbalize and
acuity chart. recognize well, so in addition to the above
• The fixations of the child to the pictures on mentioned test, the following tests (based
the Cardiff cards are relatively easy to assess. mainly on recognition acuity) are more useful
• CAC is a child friendly test. for visual assessment.
 46 Theory and Practice of Squint and Orthoptics

1. Landolt C test. This test attempts to test • The broken wheel and Snellen tests are highly
minimum separable acuity in young children correlated and that acuities measured with
who can understand the concept of break in the this test is equivalent to Snellen chart with a
circle. Landolt Cs are presented with the certainty of 94%, if using four-of-four criterion.
opening of the optotype at 3, 6, 9 or 12 o’clock. 3. Illiterate E-cutout test. This test is useful in
The child has to tell where the opening is. The children between 2½ and 3 years of age. The
separation at the break in the C represents 1 child is given a cutout of an E and asked to match
minute of arc and the entire C subtends 5 this E with isolated Es of varying sizes. The first
minutes of arc at the eye for 20/20. (For further trial is not always successful. The mother may
details see page 48). be instructed to teach E-game at home. When
2. Broken wheel test. This test is another the child starts understanding the orientation of
subjective assessment of visual acuity in E, a visual acuity chart consisting of Es oriented
toddlers and preschoolers who are not able to in various directions may be used.
perform matching tasks. A pair of cars in 4. Tumbling E-pad test. It consists of different
progressively smaller sizes, one of which has a sizes of E in one of the four positions (right, left,
wheel cut across, like Landolt C (broken wheel), upward and downward) on a dice (Fig. 3.9).
is shown to the child and the child is asked to Basically, it is similar to E-cutout test.
identify the one with the broken wheel (Fig. 3.8). 5. Isolated hand-figure test. Sjogren has replaced
The car represents on seven pairs of cards the E with the isolated figure of a hand, and in
designed to use at 10 feet, providing Snellen’s some children it works better than Es.
equivalents from 20/20 to 20/100 (shown in 6. Sheridan–Gardiner HOTV test is another test
Fig. 3.8) presented in a forced choice paradigm similar to E-cutout test (Fig. 3.10). This is an
without the need for verbal responses. The initiative test, used to test vision in the age group
visual acuity tester holds up one pair of cards at of 2–5 years. The child is handed a card with
a time and asks the child to point towards the HOTV and is asked to match the letters on the
car with the broken wheels. The child should chart. Snellen’s equivalent of 6/6–6/60 can be
correctly score four out of four responses and estimated using this method.
then, the next smaller set of cards is used until
the child can no longer consistently identify the
car with broken wheels.
Advantages include:
• The child has to simply locate the broken
wheel and need not to identify the direction
of the opening.

Fig. 3.9 Tumbling E-pad test. Printed with large 20/200 E

on one side and a series of five 20/20 tumbling Es on the
Fig. 3.8 Broken wheel test. other—calibrated to a 20 ft. distance.
 Visual Acuity and Contrast Sensitivity 47

Fig. 3.10 Sheridan–Gardiner single letter optotypes.

Fig. 3.12 Kay picture test.

7. Pictorial vision charts. When the child is able
to verbalize, visual acuity chart showing
booklets are for 6 m and 3 m distance. The 3
pictures, rather than symbols, may be used.
m booklet used for younger children who will
Many such charts have been devised, and one
not be attentive at 6 m. Near point cards are
should be chosen that presents pictures of
also available to assess near visual acuity.
objects with which the child is likely to be
• Lae symbols test (Fig. 3.13) was developed by
familiar. Pictorial vision charts include Kay
Dr Lea Hyvärinen, a Finnish paediatric
picture test, Allen cards test, Lae symbols test
ophthalmologist, who developed a vast array
and BUST.
of testing devices that have been standardized
• Allen cards test (Fig. 3.11). In this test, seven
using four pictures—circle, square, house and
optotypes are presented to the child for
apple. Lea numbers were developed in 1993
recognition at a test distance of 15 feet (20/
and calibrated in 1994. These optotypes can
40) at 3 years of age and 20 feet (20/30) at
be presented as single characters, as a wall
4 years of age.
chart at a distance of 10 to 20 feet. They can be
• Kay pictures (Fig. 3.12) is another picture presented on a video display terminal screen
optotype developed to assess visual acuity in or in the form of a flip book. With the Landolt
young children at distance as well as at near. C type being the reference optotype since 1988,
The figures are child friendly with matching earlier to which Snellen E chart was the
cards for children who cannot speak. The reference optotype, the size of the 1.0 (20/
individual elements subtend a visual angle of 20,6/6) optotypes was reduced from 7.5 to 6.84
1 min of arc and the total figure subtends 10 minutes of arc.
minutes of arc at the eye. The available test
Lea symbols now have two important basic
features of good optotypes that they blur
equally and are calibrated against the
Landolt–C. This is a good way of testing

Fig. 3.11 Allen cards test. Fig. 3.13 Lae symbols test.
 48 Theory and Practice of Squint and Orthoptics

individuals who do not use the western 1. Snellen’s test types

alphabet. Hence it eliminates the problem with The distant central visual acuity is usually tested
language barriers. by Snellen’s test types. The fact that two distant
• BUST is another picture test designed to test points can be visible as separate only when they
visual acuity of children with vision subtend an angle of 1 minute at the nodal point
impairment and developmental handicaps. of the eye forms the basis of Snellen’s test types.
BUST is an acronym for the Swedish words It consists of a series of black capital letters on a
for “visual acuity and picture perception test”. white board, arranged in lines, each progressively
The range of visual acuity for distance acuity diminishing in size. The lines comprising the
measurement values goes from 0.02 to 1.6 (20/ letters have such a breadth that they will subtend
1000to 20/10). an angle of 1 minute at the nodal point. Each
7. Boek candy bead test. The child is asked to letter of the chart is so designed that it fits in a
match beads at 40 cm. Snellen’s visual acuity square, the sides of which are five times the
equivalent of 20/200 is estimated by this breadth of the constituent lines. Thus at the
method. given distance, each letter subtends an angle of
8. Light home picture cards. A chart containing 5 minutes at the nodal point of the eye (Fig. 3.15).
an apple, a house and an umbrella (Fig. 3.14), The letter of the top line of Snellen’s chart
arranged in Snellen’s equivalents of 20/200–20/ (Fig. 3.16) should be read clearly at a distance of
10 is used, and the child is asked to identify the 60 m. Similarly, the letters in the subsequent
pictures along the lines. The test is carried out lines should be read from a distance of 36, 24,
at 10 ft. 18, 12, 9, 6, 5 and 4 m.
Landolt’s test types It is similar to Snellen’s test
MEASUREMENT OF VISUAL ACUITY IN SCHOOL types except that in it instead of the letter the
CHILDREN (ABOVE 5 YEARS) AND ADULTS broken circles are used. Each broken ring
• Snellen’s visual acuity charts are most subtends an angle of 5 minutes at the nodal point
commonly employed in this age group. and is constructed similar to letter of Snellen’s
Illiterates E charts and Landolt’s C charts test types (Fig. 3.17).
are used as alternative to Snellen’s test With Snellen’s letters, the end point consists
types. of letter recognition; with Landolt’s rings, it
• LogMAR charts enable a more accurate consists of the detection of the orientation of the
estimate of acuity as compared to other charts. break in the circle. Each method has advantages
Because of high accuracy, these are the most and disadvantages. Letter targets represent a
commonly used charts in research settings/ practical visual test. However, the ability to
clinical trials. recognize the target is influenced by literacy and
past experience, even if the targets are somewhat
blurred. Landolt’s rings were designed to
eliminate these factors and present a more
objective test. However, since the gap can be
placed in only four positions (up, down, left and
right), guessing becomes an important factor.

Fig. 3.14 Light home picture cards. Fig. 3.15 Principle of Snellen’s test types.
 Visual Acuity and Contrast Sensitivity 49

from the Snellen’s chart, so that the rays of light

are practically parallel and the patient exerts
minimal accommodation. The chart should be
properly illuminated (not less than 20
footcandle). The patient is asked to read the chart
with each eye separately and the visual acuity
is recorded as a fraction, the numerator being
the distance of the patient from the letters and
the denominator being the smallest letters
accurately read.
When the patient is able to read up to 6-m line,
the visual acuity is recorded as 6/6, which is
normal. Similarly, depending upon the smallest
line that the patient can read from the distance
of 6 m, his or her vision is recorded as 6/9, 6/
12, 6/18, 6/24, 6/36 and 6/60. If one cannot see
the top line from 6 m, he or she is asked to slowly
walk towards the chart till one can read the top
line. Depending upon the distance at which one
can read the top line, the vision is recorded as
5/60, 4/60, 3/60, 2/60 and 1/60.
If the patient is unable to read the top line even
from 1 m, he or she is asked to count fingers
(CF) of the examiner. His or her vision is
recorded as CF-39, CF-29, CF-19 or CF close to
face, depending upon the distance at which the
patient is able to count fingers. When the patient
fails to count fingers, the examiner moves his or
Fig. 3.16 Snellen’s test types. her hand close to the patient’s face. If one can
appreciate the hand movements (HM), visual
acuity is recorded as HM positive. When the
patient cannot distinguish the hand movements,
the examiner notes whether the patient can
perceive light (PL) or not. If yes, vision is
recorded as PL positive and if not it is recorded
as PL negative.

2. LogMAR visual acuity charts

LogMAR stands for Logarithm of the Minimum
Angle of Resolution. A LogMAR chart comprises
rows of letters and has equal number of letters
Fig. 3.17 Construction of Landolt’s visual acuity target. in each line (Fig 3.18). It is used at a distance of
4 meters. It is designed to enable a more accurate
Also letter tests remain much less confusing for estimate of acuity as compared to other charts
the patient and the examiner, since the (e.g. the Snellen chart), for this reason, it is
identification of letters is both immediate and recommended in research settings.
unequivocal. An observer who can resolve details as small
Procedure of testing. For testing distant visual as 1 minute of visual angle scores LogMAR 0,
acuity, the patient is seated at a distance of 6 m since the base-10 logarithm of 1 is 0; an observer
 50 Theory and Practice of Squint and Orthoptics

MEASUREMENT OF
VISUAL ACUITY FOR NEAR
Near vision is tested by asking the patient to read
a near-vision chart which consists of a series of
different sizes of ‘printer types’ arranged in
decreasing order and marked accordingly.

Near-vision charts
Commonly used near-vision charts are as
follows.
1. Jaeger’s chart. Jaeger, in 1867, devised the
near-vision chart that consisted of the ordinary
printers’ fonts of varying sizes used at that time.
Fig. 3.18 LogMAR visual acuity chart. Printers’ fonts have changed considerably since
then; however, it is now a general custom to use
who can resolve details as small as 2 minutes of various sizes of modern fonts that approximate
visual angle (i.e. reduced acuity) scores Jaeger’s original choice. In this chart, prints are
LogMAR 0.3, since the base-10 logarithm of 2 is marked from 1 to 7 and accordingly patient’s
0.3; and so on. acuity is labelled as J1–J7, depending upon the
print one can read.
Visual acuity equivalents in different notations 2. Roman test types. The Jaeger’s charts made
Table 3.4 indicates different ways for specifying from the modern fonts deviate considerably
visual acuity levels, viz. Minimal angle of from the original standard, but they are
resolution (MAR), Snellen’s acuity, efficiency probably sufficiently accurate for all practical
rating, Snellen’s fraction (that is the reciprocal of purposes. However, to overcome this theoretical
the MAR) and the logarithm of Snellen’s fraction. problem, the Faculty of Ophthalmologists of

Table 3.4 Visual acuity equivalents in different notations

MAR or Snellen’s visual Snell-Sterling Loss of central Snellen’s LogMAR
minimum acuity visual efficiency (%) vision (%) fraction acuity acuity
angle of ft m relative relative to
resolution 20/20
(minutes of
arc)
0.5 20/10 6/3 109 0 2.0 0.3
0.75 20/15 6/4.5 104 0 1.33 0.1
1.00 20/20 6/6 100 0 1.0 0
1.25 20/25 6/7.5 96 4 0.8 –0.1
1.5 20/30 6/9 91 9 0.67 –0.18
2.0 20/40 6/12 84 16 0.5 –0.3
2.5 20/50 6/15 76 24 0.4 –0.4
3.0 20/60 6/18 70 30 0.33 –0.5
4.0 20/80 6/24 58 40 0.25 –0.6
5.0 20/100 6/30 49 50 0.2 –0.7
6.0 20/120 6/36 41 60 0.17 –0.78
7.5 20/150 6/45 31 70 0.133 –0.88
10.0 20/200 6/60 20 80 0.10 –1.0
20.0 20/400 6/120 3 90 0.05 –1.3
 Visual Acuity and Contrast Sensitivity 51

Great Britain in 1952 devised another near-vision The graded sizes of pleasing types of passages
chart. It consists of ‘Times Roman’ type fonts from literature, the reading of which helps in
with standard spacing (Fig. 3.19). According to the interpretation, are habitually employed.
this chart, the near vision is recorded as N5, N6, 4. Lea near-vision cards. This test assesses a
N8, N10, N12, N18, N36 and N48. child’s functional vision at near distances. It can
3. Snellen’s near-vision test types. Snellen also be used to familiarize child with testing
introduced the so-called ‘Snellen’s equivalent procedure before introducing a distance test. It
for near vision’ on the same principles as his consists of cards measuring 8" × 10" (20.3 cm ×
distant types. The graded thickness of the letters 25.4 cm) which contain proportionally spaced
of different lines is about 1/17th of the distant- (logMAR) lines on one side and more tightly-
vision chart letters. In this event, the letters spaced symbols on the opposite side. Line sizes
equivalent to 6/6 line subtend an angle of range from 20/400 to 20/10 (6/120 to 6/3)
5 minutes at an average reading distance (35 cm/ equivalent, 0.05 to 2.00. Response key is printed
14 in.). on test card. Testing distance is about 16 inches/
The unusual configuration of letters of this 40 cm.
chart, however, cannot be constructed from the
available printers’ fonts. It can only be Procedure of testing
reproduced by a photographic reduction of the For testing the near vision, the patient is seated
standard Snellen’s distant-vision test types to in a chair and asked to read the near-vision chart
approximately 1/17th of their normal size. kept at a distance of 25–35 cm, with a good
Further, such a test has never become popular. illumination thrown over his or her left
shoulder. Each eye should be tested separately.
The near vision is recorded as the smallest type
that can be read comfortably by the patient. A
note of the approximate distance at which the
near-vision chart is held should also be made.
Thus near vision (NV) is recorded as:
• NV 5 J1 at 30 cm (in Jaeger’s notation)
• NV 5 N5 at 30 cm (in Faculty’s notation)

Near-vision equivalents in different notations

These are shown in Table 3.5.

CONTRAST SENSITIVITY

INTRODUCTION
Contrast sensitivity is the ability to perceive
slight changes in luminance between regions
that are not separated by definite borders and
is just as important as the ability to perceive
sharp outlines of relatively small objects. It is
only the latter ability that is tested by means of
the Snellen’s test types. In many diseases, loss
of contrast sensitivity is more important and
disturbing for the patient than is the loss of
visual acuity. Further, contrast sensitivity may
be impaired even in the presence of normal
Fig. 3.19 Near-vision chart. visual acuity.
 52 Theory and Practice of Squint and Orthoptics

Table 3.5 Equivalent visual acuity notations for near

Visual Snellen American
angle equivalent Medical Decimal Jaeger Faculty’s Metre Central Vision
(minutes) Association notation notation Roman test notation (m) visual loss (%)
notation types nota- efficiency
tion for near (%)
5.00 20/20 14/14 1.00 J1 N5 0.37 100 0
6.25 20/25 14/17 0.80 J1 N6 0.43 100 0
7.50 20/30 14/21 0.66 J2 N8 0.50 95 5
10.00 20/40 14/28 0.50 J4 N10 0.75 90 10
12.50 20/50 14/35 0.40 J6 N12 0.87 50 50
15.00 20/60 14/42 0.33 J8 N14 1.00 40 60
20.00 20/80 14/56 0.25 J10 N18 1.50 20 80
25.00 20/100 14/70 0.20 J1 N24 1.75 15 85
50.00 20/200 14/140 0.10 J17 N36 3.50 2 98

TYPES OF CONTRAST SENSITIVITY Contrast sensitivity is measured as (Lmax 2

Lmin)/(Lmax 1 Lmin), where L is the luminance
1. Spatial contrast sensitivity
recorded by photocells scanning across the
Spatial contrast sensitivity refers to detection of gratings.
striped patterns at various levels of contrast and
spatial frequencies. In its measurement, patient VARIABLES IN THE MEASUREMENT
is presented with sine wave gratings of parallel
There are three variables in the measurement of
light and dark bands (Arden gratings) and is
contrast sensitivity:
asked to tell the minimum contrast at which the
• Average amount of light reflected depends on
bars can be seen at each frequency. The width
illumination of paper and darkness of ink.
of the bars is defined as spatial frequency which
expresses the number of pairs of dark and light • Degree of blackness in relation to the white
bars subtending an angle of 18 at the eye. A high background, i.e. contrast.
spatial frequency implies narrow bars, whereas • Distance between the grating periods or cycles per
a low spatial frequency indicates wide bars. degree of visual angle.

2. Temporal contrast sensitivity METHODS OF MEASUREMENT

Here the contrast sensitivity function is Various methods have been developed to
generated for time-related (temporal) processing measure contrast sensitivity. Bodis-Wollner,
in the visual system by presenting a uniform introducing contrast sensitivity measurement in
target field modulated sinusoidal in time, rather clinical practice, suggested the name visuogram,
than as a function of spatial position. analogue to an audiogram, to describe a patient’s
Both temporal and spatial contrast sensitivity ‘contrast sensitivity curve’. The deficits were
testing yield significantly more complete and expressed in terms of decibels, and three types
systematic data on the status of visual of deficits were described:
performance than the conventional tests. • High-frequency type characterized by
increasing loss at high frequency.
MEASUREMENT OF CONTRAST SENSITIVITY • A level-loss type characterized by a similar loss
When a subject is presented with the grating for all spatial frequencies.
frequencies and contrast below which resolution • A selective-loss type characterized by deficits
is impossible, it indicates the threshold level; and of spatial frequencies in a narrow band.
the reciprocal of this contrast threshold gives the In general, the methods recommended to
contrast sensitivity. measure contrast sensitivity include: simple
 Visual Acuity and Contrast Sensitivity 53

plates, cathode ray tube display on a screen,

letter acuity charts, laser interferometer (LI)
which produces grating on the retina, visual
field testing using low contrast rings on stimuli,
pattern discrimination test, prototype for forced
choice printed test, visually evoked cortical
potentials to checkerboard pattern reversal
dependent contrast threshold measurement,
two-alternative forced choice test and many
more.
Some of the simple, inexpensive but reliable
methods of measuring contrast sensitivity are
described in brief in the following text.
1. Arden gratings. Arden, in 1978, introduced a
booklet containing seven plates: one screening
plate (No. 1) and six diagnostic plates (No. 2–7).
The contrast changes from top to bottom and Fig. 3.20 Cambridge low-contrast gratings.
covers a range of approximately 1.76 log units.
The plates are studied at 57 cm, with spatial
frequency increasing from 0.2 cycles/degree to
6.4 cycles/degree, each being double the
frequency of the previous one. A score of 1–20
is assigned to each plate, depending upon the
amount of plate uncovered. Sum of six plates
with an upper limit of 82 was established for
normal subjects together with an interocular
difference of less than 12.
2. Cambridge low-contrast gratings. Cambridge
low-contrast gratings consist of a set of ten plates
containing gratings in a spiral bound booklet.
To perform the test, the booklet is hung on a
wall at a distance of 6 m. The pages are presented
in pairs, one above the other. One page in each
pair contains gratings and the other is blank
(Fig. 3.20), but the pages have the same mean
reflectance. The subject is simply required to
choose which page, top or bottom, contains the
gratings. The pages are shown in order of
descending contrast and are stopped when the
first error is made. Four descending series are
shown separately to each eye. When no error is Fig. 3.21 Cambridge low-contrast gratings score sheet and
made at plate 10, then a score of 11 is given. conversion table.
Depending upon the total score of the patient
from four series, the contrast sensitivity is noted sequence but are otherwise identical. The letters
from the conversion table (Fig. 3.21). on chart are organized as triplets, there being
3. Pelli-Robson contrast sensitivity chart. This two triplets in each line (Figs 3.22A and B). The
chart consists of letters that subtend an angle of contrast decreases from one triplet to the next.
38 at a distance of 1 m. The chart is printed on The log contrast sensitivity varies from 0.00 to
both the sides. The two sides have different letter 2.25.
 54 Theory and Practice of Squint and Orthoptics

Fig. 3.23 Measurement of contrast sensitivity with Pelli-

Robson chart.

While recording, the subject sits directly in

front of the chart at a distance of 1 m (with the
best distance correction) (Fig. 3.23). The subject
is made to name or outline each letter on the
chart, starting from the upper left corner and
reading horizontally across the line. Subject is
made to guess, even when he or she believes
that the letters are invisible. The test is
concluded when the subject guesses two of the
three letters of the triplet incorrectly. The
subject’s sensitivity is indicated by the finest
triplet for which two of the three letters are
named correctly.
4. The Vistech chart. This chart consists of sine
wave gratings and is used at a distance of 3 m
from the subject. In this test, contrast is assessed
at several spatial frequencies (distance of the
separation of the grating bars) and the subject
has to identify the orientation of the grating, i.e.
whether vertical or 158 clockwise, or anti-
Fig. 3.22 Pelli-Robson contrast sensitivity chart. A, clockwise.
Photograph; B, Log contrast sensitivity score of each triplet.
5. Vector vision chart. Vector vision CSV 1000
(USA) chart test frequency of 3,6,12 and 18 cpd.
To perform the test, the chart is hung on the
6. Fact CS chart. The fact CS chart tests for 1.5,
wall, so that its centre is approximately at the
3, 6, 12 and 18 cpd.
level of the subject’s eye. The chart is illuminated
as uniformly as possible, so that the luminance
of the white areas is between the acceptable BIBLIOGRAPHY
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 4
Binocular Vision

BINOCULAR VISION: DEFINITION AND DEVELOPMENT OF BINOCULAR VISION

GRADES • Prerequisites
• Definition • Eye at birth and normal postnatal
• Grades development
PSYCHOPHYSICS AND SENSORY ASPECTS • Maturation of binocular function
• Visual directions and the horopter • Neurophysiology of development
• Binocular fusion • Theories of binocular vision
• Dichoptic stimulation • Disturbances in the development
• Stereopsis BINOCULAR VISION TESTS
• Depth perception • Simultaneous perception
• Integration of the motor and sensory • Fusion
system into binocular vision • Stereopsis

Simultaneous perception
BINOCULAR VISION:
(first grade of binocular vision)
DEFINITION AND GRADES
Simultaneous perception exists when signals
DEFINITION transmitted from the two eyes to the visual
Romano and Romano1 defined binocular vision cortex are perceived at the same time. The term
as that state of simultaneous vision with two simultaneous perception does not imply that
seeing eyes (neither of which needs necessarily both eyes see the same object and transmit
be normal) that occurs when an individual fixes identical information to the visual centre, nor
his visual attention on an object of regard. By does it imply that the two pictures can be seen
and large binocular vision implies binocular superimposed. It consists of power to see two
single vision (fusion) and a high level stereo- dissimilar objects simultaneously. It can be
acuity. demonstrated by presenting separate stimuli to
Though, cursorily binocular vision may be the two eyes, such as a picture of a cage to one
defined as the coordinated use of the two eyes eye and a picture of bird to the other eye. If both
to produce a single mental impression, its full cage and the bird are seen at the same time, then
definition includes its full scope, i.e. grades of simultaneous perception is present (Fig. 4.1A).
binocular vision. Further, there is a fundamental difference
between seeing with two eyes alternately and
GRADES OF BINOCULAR VISION simultaneous binocular perception. An animal
Thus, binocular vision may be defined to consist whose eyes are situated laterally in the head so
of following: that the visual fields of the two eyes never overlap
57
 58 Theory and Practice of Squint and Orthoptics

or overlap in only a very small portion and thus and holding a bunch of flowers will be seen
can use one eye at a time. This is alternate use of (Fig. 4.1B). It should not be confused with the
the two eyes, rather than simultaneous use of the superimposition of two dissimilar (but not
two eyes. It can be further classified by the typical mutually antagonistic) pictures, such as a cage
example that, when a bird sees a worm on the and a bird. So, when a person sees the bird inside
ground and tilts its head so that it obtains a clear the cage (Fig. 4.1A), it is not fusion, but simply a
image of the worm with the right eye, the left is simultaneous perception in the same direction.
directed upward. Since the simultaneous image
of this eye would detract considerably from the Stereopsis (third grade of
binocular single vision)
perception of the worm, there is a reason to think
that one image is suppressed mentally. The bird Stereopsis implies the ability to obtain an
may direct its attention at will to the image of the impression of depth by the superimposition of
left eye and ignore the image of right eye two pictures of the same object which have been
temporarily. This would surely happen, if under taken from slightly different angles, such as a
bucket that is appreciated in three dimensions
these circumstances a hawk were to fly overhead.
(Fig. 4.1C). Stereopsis should not be considered
Under certain conditions, human beings suppress
synonymous with the depth perception, since,
the image from one eye with both eyes open. depth perception is the perception of distances
Simultaneous perception ceases to exist under of objects from each other or from the observer.
these circumstances. For example, when using a Even a monocular observer is quite capable of
monocular microscope, one suppresses the image judging distances and of obtaining an
of the other eye. impression of spatial order. Therefore, stereopsis
refers to the visual appreciation of three
Fusion (second grade of binocular vision) dimensions during binocular vision.
Fusion constitutes second grade of binocular
vision. It implies the ability of the two eyes to PSYCHOPHYSICS AND SENSORY
produce a composite picture from two similar ASPECTS OF BINOCULAR VISION
pictures, each of which is incomplete in one
small detail. For example, there are two rabbits The various facts about the psychophysics and
each lacking either a tail or a bunch of flowers. sensory aspects of the binocular vision (revealed
If fusion is present, one rabbit complete with tail by psychophysical and experimental physio-

Fig. 4.1. Grades of binocular single vision: A, simultaneous perception; B, Fusion; C, Stereopsis.
 Binocular Vision 59

logical studies), for the purpose of descriptive in the subjective visual space. The experiments
convenience, can be compiled as below: of Hering7,8 demonstrate that objects, which may
1. Visual direction and the horopter be widely separated in physical space, may have
• Visual space versus physical space a common direction in subjective space. It is
• Visual directions important to recognize that the anatomic
• Corresponding points and normal retinal distribution of retinal elements and the
correspondence physiologic distribution of spatial values do not
• Horopter coincide. There are many examples that
demonstrate the difference between physical
• Physiological diplopia
space and its subjective interpretation. For
2. Binocular fusion
example, if a vertical line is presented to a single
• Sensory fusion eye in the absence of other visual clues, it
• Concept of Panum’s area appears to be slanted or tilted. Disclination
• Fixation disparity (temporal shift) or conclination (nasal shift) of
• Theories of binocular fusion the vertical meridian in subjective space will
3. Dichoptic stimulation occur. This demonstrated that the spatial values
• Depth with fusion of the retinal receptors above and below the
• Depth with diplopia horizontal midline differ.
• Diplopia without depth Another example of the difference between
• Binocular rivalry and suppression subjective space and physical space is the
4. Stereopsis Kundt-Munsterberg illusion. In this illusion,
the temporal half of a horizontal line, when
• Physiological basis of stereopsis
viewed monocularly and kept at right angles
• Stereopsis and fusion
to the line of sight at the point of bisection,
• Stereoscopic acuity
will appear shorter than the nasal half of that
• Neurophysiology of stereopsis bisected line. Because of this illusion, when
5. Depth perception an attempt is made with one eye to bisect the
• Stereopsis horizontal line, the temporal segment will be
• Nonstereoptic binocular clues longer than the nasal. The distribution of the
• Monocular clues subjective retinal spatial values differs
• Influence of accommodation and convergence between the nasal and temporal halves of the
6. Integration of motor and sensory systems into retina.
binocular vision.
VISUAL DIRECTIONS

VISUAL DIRECTION AND THE HOROPTER Oculocentric visual direction (monocular vision)
When an object is viewed, its image falls on the
VISUAL SPACE VERSUS PHYSICAL SPACE foveola. The visual direction of the object can
Perception of space and spatial localization are be represented by a line joining the object to the
extremely intricate functions that are not fully centre of foveola—principal visual line or visual
understood. The perception of spatial order is a axis. The position of all other objects in the
mental phenomenon based on innate anatomic monocular field can be fixed by their oculo-
and physiologic systems, on visual clues, and centric visual directions with respect to the
on learning. The order in which objects are seen visual axis. Thus each point on the retina can be
in visual space is a subjective perception. thought of as having its own particular visual
Therefore, visual space is referred to as direction or visual line passing out through the
subjective space. The subjective space is distinct nodal point of the eye. A visual line is, therefore,
from the physical space of real objects. Location the locus of all points fixed relative to the eye
of an object is its position in physical space, whose images stimulated a given point on the
whereas localization is the position of an object retina.
 60 Theory and Practice of Squint and Orthoptics

For a given position of the eye, objects having

superimposed retinal images will be seen as
being in alignment in the visual field (law of
oculocentric visual direction), although at
different distance from the eye along the same
visual line.7, 8

Egocentric visual direction (binocular vision)

Retinal points in the two eyes are said to be
corresponding, if, when stimulated separately,
they appear to have the same common visual
direction.
Because each eye sees the world from different
view point, the oculocentric frame of reference
is necessarily different for the two eyes.
However, in binocular vision, a single system
of visual direction is needed whose frame of
reference is related to the head (egocentric)
rather than two eyes. When we use two eyes we
seem to see the visual space through some
imaginary single eye (cyclopean eye) situated
in the head midway between the two eyes
(Fig. 4.2).9
Foveae have a common subjective visual
direction (Hering's law of identical visual
direction). Hering8 described it by the following
classical experiment (Fig. 4.2): Let the observer
stand 1/2 metre from a window which affords
a view of outdoors, hold his head very steady,
close the right eye and direct the left eye to an
object located somewhat to the right, e.g. a
triangle. While fixing the triangle with the left
eye, a black mark (F) is made on the window
pane at a spot in line with the triangle. Now left
eye is closed and the right eye is opened and
Fig. 4.2. Concept of cyclopean eye. (diagrammatic
directed at the spot (F) on the window and representation of Hering's law of identical binocular direction)
beyond that to some object in line with it, e.g. a (Alter Howard and Templeton9).
hut. Then with both eyes open and directed at
the spot, the latter will appear to cover parts of relative, not absolute. They are related to the
the triangle and the hut both, which will be seen spatial value of the fovea in each eye. The fovea,
simultaneously.8 therefore, is the principal spatial value or
principal directional value of each eye.
CORRESPONDING POINTS AND In order to understand normal retinal
NORMAL RETINAL CORRESPONDENCE correspondence, it must be clearly understood
Each retinal element has a directional or spatial that correspondence refers only to the relative
value without which localization in space is localization of objects in space to each other
impossible. These are intrinsic, not learned, under binocular conditions. The relationships
values. The spatial value of retinal elements is of objects in space to ourselves—that is, to a
 Binocular Vision 61

coordinate system surrounding our person or vision. The mental image resulting from the
our "ego centre"—is called absolute localiza- fusion of the two retinal images can be conceived
tion. as the image or mental image received by a
The information gained from relative cyclopean eye. This is an eye, presumably
localization is only one of many clues used in located in the middle of the forehead, that sees
the process of absolute localization. Other in visual space the fused images of the two
information related to the vestibular mechanism, individual eyes (Fig. 4.3).12 Normal retinal
an ocular motor monitoring system, and correspondence is thus the basis for normal
possible proprioceptive feedback mechanisms binocular single vision. If, during fixation of an
is utilized by the brain to make the subjective object, one eye of the observer would be
interpretation of absolute localization. passively turned with forceps, the object would
Burian 10 states that corresponding retinal no longer be imaged on corresponding retinal
elements are those elements of the two retinae, points. While the images would fall on the fovea
the stimulation of which in binocular vision, in the one eye, it would be received somewhere
gives rise to the localization in one and the same on the peripheral retina in the deviated eye.
visual direction, no matter whether the stimulus Since these two areas are non-corresponding and
reaches the retinal elements in one eye alone, or have different spatial values, diplopia would be
its corresponding partner in the other eye alone, present (i.e. the object would be seen double).
or both simultaneously.
The fovea normally determines the principal HOROPTER
visual direction. The correspondence mechanism The term horopter, which literally means the
is based on the assumption that each retinal horizon of vision, was introduced by Aguilonius.13
receptor, when stimulated under monocular As we know, when, in normal binocular vision,
conditions, dictates a subjective visual direction both eyes fixate the same object point, this point
determined by the relationship of that receptor will be imaged on corresponding retinal
to the fovea. The retinal receptors in both eyes elements of the two eyes—the foveae. At the
that dictate a common visual direction under same time, there will be other object points in
binocular conditions are called corresponding space besides the fixation point that also will be
points or elements. Bagolini11 has shown that imaged on corresponding retinal elements of the
this is an area-to-area relationship rather than a
point-to-point relationship. Under binocular
conditions, the correspondence process analyses
the information relayed from each eye and may
modify it in making the determination of
absolute localization.
In normal retinal correspondence, both foveae
have the same space value—zero—the value of
the principal visual direction. The other receptor
elements correspond to each other in fair
approximation to their geometric locations so
that a receptor 5° temporal to the fovea in one
eye corresponds to a receptor 5° nasal to the
fovea in the other eye.
Images falling on corresponding points give
rise to a single mental impression to visual
Fig. 4.3. Normal retinal correspondence. The visual direction
direction that we call sensory fusion.
of f1 and f2 proceed from F the fovea of the imaginary
Corresponding retinal elements, therefore, have cyclopean eye. A and B represent the points from which the
a common visual direction and permit a single visual direction belonging to a and a´, b and b´ would
visual impression. They do not permit double proceed (From Bielschowsky12).
 62 Theory and Practice of Squint and Orthoptics

two eyes. If the position of these other points is The horopter can be determined with an
determined theoretically, a circle will be found instrument utilizing movable, vertically placed
passing through the fixation point and the wires set at a certain distance. While fixation is
entrance pupils of the two eyes. This circle is maintained upon a central wire, those on either
known as the theoretical or geometric horopter side in the periphery are adjusted forward or
(Vieth-Muller horopter).14,15 Theoretically, any backward by the subject until they appear to lie
point on it will stimulate corresponding retinal in the same visual plane. In determining the
elements in the two eyes, because every point horopter, the difference between subjective
on the horopter will form an angle with the two visual space and physical space is again
entrance pupils that is equal to the angle formed demonstrated. Subjectively, the movable wires
by the fixation point and the two entrance pupils appear to lie in the same visual plane; however,
(Fig. 4.4). It follows that there is a different they actually lie on a curved surface. Thus, the
horopter for each fixation distance. horopters found experimentally do not actually
In other words, the horopter can be defined coincide with the theoretical horopters. They are
as the sum total of points in physical space that called empirical horopters.
stimulate corresponding retinal elements of the
two eyes. It is a complex mathematical model PHYSIOLOGIC DIPLOPIA
having all three dimensions. The longitudinal Since only object points which lie on the
horopter is that surface that determines horopter will be imaged on corresponding
horizontally distributed object points in space.13 retinal elements, all objects that are either nearer
The longitudinal horopter curve is a line formed or farther away than the horopter will be imaged
by the intersection of the visual plane and the on disparate (i.e. non-corresponding) retinal
longitudinal horopter plane. The visual plane is elements and, consequently, will be seen double.
determined by the fixation point and the centres Point a in Fig. 4.5 A, located closer than the
of the two pupils.16, 17 horopter (point b), is imaged temporal to the
fovea in each eye. The right eye will see this
point as being on the left side of the fixation
point, while the left eye will see it to the right of
the fixation point. In other words, point a will
be seen double (a 1 and a 2 ). Since each eye
perceives it as being on the opposite side, this is
referred to as crossed diplopia. With respect to
the retina, point a shows temporal disparity.
Conversely point a, which is located beyond the
horopter (point b) in Fig. 4.5B; will be imaged
on the nasal retinae of both eyes and, therefore,
will also appear double. However, this time the
diplopia will be uncrossed—the right eye will
localize the object to the right of the fixation
point, while the left eye will place it to the left.
The retinal images will have nasal disparity.
Physiologic diplopia can be demonstrated by
holding two pencils vertically in front of the eyes
with one pencil about twice as far away as the
other. If either one of them is fixated, the other
will be seen double. If the more distant one is
Fig. 4.4. Diagrammatic representation of the theoretical
horopter (Vieth-Muller horopter circle). Points F, A and B fall
fixated, crossed diplopia will be experienced. If
on geometrically corresponding retinal points f1 and f2, a1 the closer one is fixated, the diplopia will be
and a2 and b1 and b2, respectively. uncrossed.
 Binocular Vision 63

Another contributing factor is the low visual

acuity of the peripheral retina, which frequently
does not yield sufficient resolution of peripherally
located objects for the recognition of diplopia.

BINOCULAR FUSION
When images of an object fall on corresponding
retinal points, in the normal subject, they seem
to be fused into a single mental impression. The
sensory fusion should be distinguished from
motor fusion, which refers to the ability to align
the eyes in such a manner that sensory fusion
can be maintained. The stimulus for these
fusional eye movements is retinal disparity.
Unlike sensory fusion, the motor fusion is
exclusive function of the extra-foveal retinal
periphery. Under normal conditions, sensory
fusion occurs when corresponding retinal
elements of the two eyes are stimulated by
images from the same object. However, fusion
will also take place when the stimuli in the two
eyes are not identical but only similar. The
dissimilarity may be one of form, size, colour,
luminosity, distinctness or contrast. To a limited
extent, it is also possible to fuse signals from non-
corresponding points (see discussion on fixation
disparity below).
Fig. 4.5. Diagram illustrating physiological diplopia. Points The anatomic basis which allows sensory
a and b which are not located on the horopter, are imaged fusion to occur is the course of the visual nerve
on non-corresponding retinal points and so physiological
fibres. By crossing in the chiasma, nerve fibres
diplopia either crossed (A) or uncrossed (B) is produced.
from the nasal retina are brought to the same side
This demonstration will not be immediately as their counterparts from corresponding points
successful in all subjects, since one of the images of the temporal retina of the other eye. While
may be suppressed from conscious perception, ascending in the visual pathway, the fibres from
a process which also is physiologic, as is corresponding retinal points converge until
explained later in this chapter. finally they are adjacent to each other. They
In view of what has been said about the terminate in the same cortical cell complexes.
horopter and physiologic diplopia, it should be But for our knowledge of the anatomic
expected that one would have constant diplopia arrangement, little definite information exists on
during casual daily vision, with the exception of the physiology of sensory fusion. It is probable
the object of fixation and the few objects that that fusion occurs in the cortex. It is not certain,
happen to be on the horopter for the momentary however, whether the two signals transmitted
fixation distance. Obviously, this is not the case; separately from two corresponding points are
and it is not primarily suppression that prevents synthesized so that they simultaneously
the occurrence of physiologic diplopia, but the contribute to the resulting single perception, or
exclusiveness with which our attention is usually whether the two signals are mutually exclusive
directed to the fixated object. Although diplopia and are utilized alternately to be combine into
is present, this rarely enters consciousness. the resulting single constant perception so that
 64 Theory and Practice of Squint and Orthoptics

first one eye and then the other contributes its it gains increasingly in depth, so that objects
stimulus to a given point of the whole. located peripherally may be farther from the
At birth, coordinated conjugate eye movements horopter without producing diplopia than
are absent, visual perception is poor, and fusion objects located more centrally. Any point not on
is not established as a stable binocular function. the horopter is not imaged on corresponding
If development proceeds normally, fusion retinal areas but will stimulate disparate retinal
evolves later as a conditioned reflex. If there is a areas. If, despite the disparity, no diplopia
significant abnormality in the optical occurs, fusion of signals from non-corresponding
performance of the eyes, in the function of the points must have taken place. Thus, a signal
nerve elements that are concerned with the from a given retinal point not only is fused with
transmission or perception of visual stimuli, or the signal from its corresponding point in the
in the motor cooperation of the two eyes, fusion other eye, but also may be fused with
may never develop normally. It is conceivable signals from a limited area surrounding that
that there may be individuals in whom a basic corresponding point. Such a retinal Panum’s
ability for fusion does not exist. area corresponds in size to the depth of Panum’s
fusional space. Since the latter is small in the
PANUM’S AREA vicinity of the fixation point, Panum’s areas in
As stated previously that only object points the fovea are also small, actually in the order of
which lie on the horopter and which stimulate 5’ of arc. Away from the fixation point, Panum’s
corresponding retinal points in the two eyes are fusional space enlarges (i.e. the size of Panum’s
seen as single is not quite exact and must be areas in the peripheral retina increases). Since
qualified. When a horopter is determined retinal disparity in the horizontal meridian can
experimentally, it is found that an object may be overcome to a larger extent than disparity in
be located a certain distance in front of or behind the vertical meridian, Panum’s areas have the
it without producing diplopia. The field in front shape of an oval with the longer axis horizontal.
of and behind the horopter, in which the If the fixation distance is more than 20 m, objects
expected diplopia does not occur is known as lying behind the horopter will always be seen
Panum’s fusional space. as single, since the disparity of their images is
Panum’s fusional space is smallest at the always smaller than Panum’s areas.
fixation point (Fig. 4.6). Toward the periphery,
FIXATION DISPARITY
Under laboratory conditions, it can be
demonstrated that, during binocular fixation,
the point of fixation is rarely ever imaged exactly
on corresponding points of the two foveae but
that the primary line of sight of one eye misses
the fixation point very slightly, being either
under-converged or over-converged (Fig. 4.7).
This phenomenon is called fixation disparity. It
does not give rise to diplopia, because the
disparity with which the fixation point is imaged
on the two retinae is less than the size of Panum’s
area.
Thus, in fixation disparity, one eye will fixate
the object directly with the central fovea, while
the other eye will fixate slightly eccentrically.
However, the horopter simply passes through
the actual point of fixation rather than through
Fig. 4.6. Diagram of Panum's fusional space. the fixation stimulus. Fixation disparity may
 Binocular Vision 65

DIPLOPIA WITHOUT DEPTH

When the degree of non-correspondence is such
that no fusion occurs and diplopia results
without depth perception. Depending upon the
location of non-corresponding points stimulated,
the diplopia may be homonymous or uncrossed
(Fig. 4.5B) and heteronymous or crossed
(Fig. 4.5A). Sensory adaptations which may
occur to compensate for the annoying diplopia
include: suppression, amblyopia, and abnormal
retinal correspondence. Motor mechanisms
which compensate for diplopia are an abnormal
head posture and certain permanent changes in
the extraocular muscles such as inhibitional
palsy, overaction and contractures.

RETINAL RIVALRY AND SUPPRESSION

When dissimilar contours are presented to
Fig. 4.7. Diagram illustrating fixation disparity. corresponding retinal areas (having same visual
arise from a small foveal scotoma or from an direction), fusion becomes impossible, since two
oculomotor imbalance which is tending to pull dissimilar objects localized in the same place
the eyes away from the appropriate angle of give rise to a conflict and confusion. The conflict
convergence. An example of a horopter showing which results when contradictory and
fixation disparity was obtained by Olge18 on his incompatible signals are transmitted from
own eyes. corresponding points to the visual cortex is
termed retinal rivalry. The phenomenon of
retinal rivalry, also termed as binocular rivalry,
DICHOPTIC STIMULATION
must be clearly distinguished from local adapta-
Dichoptic stimulation simply refers to a different tion, or Troxer’s phenomenon.24 Binocular
stimulation in the two eyes, which occurs when rivalry may also be produced by uniform
binocular stimuli fall on non-corresponding surfaces of different colour (colour rivalry) and
points on the two retinae. There are five classes unequal luminances of the two targets. Many
of percepts which are obtained, depending upon combinations of contours, colours and
the degree of non-correspondence between the luminances have been studied exhaustively
stimuli: since the days of Panum,25 Fechner,26 Helmholtz27
1. Depth with fusion; and Hering.28
2. Depth with diplopia;  Retinal rivalry is a very normal event, since
3. Diplopia without depth; corresponding areas are consistently exposed to
4. Binocular rivalry and suppression; and dissimilar images from objects located outside
5. Binocular lustre. Panum’s fusional space.
The visual system responds to retinal rivalry
DEPTH WITH FUSION AND DEPTH WITH DIPLOPIA by rejecting the signals transmitted from a given
Strictly speaking, these are not generally area of one retina in favour of those coming from
referred to as dichoptic stimuli, since the two the corresponding parts of the other retina. Thus,
retinal patterns are sufficiently similar to be only information from one retina becomes a
combined into a unified impression (particularly visual perception while the information
for fused stereopsis). Therefore, these will be simultaneously transmitted from corresponding
dealt in the section of stereopsis. parts of the other retina is not used, so that the
 66 Theory and Practice of Squint and Orthoptics

involved area may be considered temporarily normally corresponding retinal areas, suppre-
blind. This reaction to the influx of conflicting ssion is a frequent response to solve the
information is called suppression. problem of incompatible information
transmitted from corresponding areas of the
 Suppression is an innate, involuntary process.
two eyes. It has been shown experimentally
Under certain conditions, all the signals from
that retinal rivalry and the suppression
one retina may be suppressed so that no
resulting from it are influenced by certain
information from that eye reaches the threshold
conditions. If non-fusable patterns are
of conscious perception.29-33 Usually, however,
presented to the two eyes, it is found that
responding to the demand of the momentary
perception from corresponding areas alternates
conditions, suppression is restricted to limited
equally between the two eyes, if the objects are
areas in both retinae so that those portions of
of equal brightness, size and prominence. The
the subjective visual field in which fusion cannot
rate of alternation under such test conditions
occur may be composed, like a mosaic, of pieces
is influenced by the brightness of the field, the
of information from only one or the other retina
size of the target area, the distinctness of the
in constant variation (Fig. 4.8).
targets, and the location of the stimulated area
Although an eye may be suppressed for long
in the retina. If any of these factors is impaired
periods, suppression basically is a transient
equally for both eyes, the rate of alternation will
phenomenon, present only momentarily when
decrease. If the visual conditions are improved,
needed. Because it is a physiologic function that
it will increase. However, suppression will
does not have to be learned but is readily
always alternate equally between the two eyes.
available, it frequently becomes the firstline of
defence against pathologic interruption or In contrast, by changing the stimulus
embarrassment of bifoveal single vision. For conditions for only one eye, the rate of
example, a patient who has a marked refractive alternation can be changed markedly in its
error in only one eye may show suppression of favour or disfavour.
that eye to eliminate the disturbing effect of the Retinal or binocular rivalry fluctuations are
blurred image. In patients with strabismus, a similar in many respects to fluctuations of
condition in which objects are not imaged on attention, and are widely supposed to be under
voluntary control to some extent. Actually, a
number of studies have found that there is very
little voluntary control over which eye
dominates at any given time.34 The change of
dominance is not affected by eye blinks35 or by
variations in accommodation or pupil size.36 In
fact, the fluctuations in rivalry are well described
by a sequentially independent random variable
with no periodicities, as though the arrival of
each change in dominance had no effect on the
occurrence of subsequent changes.37
The phenomenon of retinal rivalry has been
explained in neurophysiologic terms by the
presumption that separate channels are present
for the right and left eyes which compete for
access to the visual cortex. A third binocular
channel is activated only by fusible input.38,39
Because of this competition and inhibition
Fig. 4.8. Retinal rivalry produced by dissimilar contours: A, elicited, only fragments of the image seen by
pattern viewed by left eye; B, Pattern viewed by right eye; C, each eye are transmitted to the visual cortex in
binocular impression (mosaic of pieces). After Panum.25 the case of non-fusible binocular output. This
 Binocular Vision 67

hypothesis looks logical but needs experimental

verification.

BINOCULAR LUSTRE
The lustrous appearance of surfaces like a waxed
surface, tabletop or a car body is essentially due
to binocular lustre. It results from the different
position of partially reflected objects in the surface
by virtue of the different position of the two eyes.
The partial reflection provides a fixation place at
which the partially reflected image usually has a
large disparity and hence areas of binocular
luminance difference. The lustrous region is not
localizable in depth, but it seems unitary and does
not fluctuate in the manner of binocular rivalry.
Binocular lustre was described by early authors
in visual science, such as Panum 19 and
Helmholtz,21 as a kind of lustrous or shimmering
surface of indeterminate depth.
The detection of binocular lustre during static
and dynamic random dot stereogram testing is
even more rapid than the detection of depth
changes.40-42 Fig. 4.9. Disparateness of retinal images producing stereopsis.

STEREOPSIS An object point which is located in front of

the fixation point but is still within the limits of
Stereopsis is the visual appreciation of three Panum’s fusional space will stimulate disparate
dimensions during binocular vision. It occurs retinal elements. The disparity will be temporal,
through fusion of signals from disparate retinal each image being temporal to the point that
elements. corresponds to the location of the image in the
As shown in Fig. 4.9, if one views two posts, other eye. Although both images are fused since
A and B, situated at slightly different distances, they are within the limits of Panum’s area, the
with both eyes open, their separation in depth fact that they are temporarily desparate is not
is readily apparent to a normal person. This lost in the resulting single perception but gives
perception of depth is maintained as long as both the perception of its additional quality of
eyes are used. If either eye is closed, the effect nearness relative to the fixation point.
of depth then vanishes. If the right eye is closed, Conversely, when an object is located behind the
the appearance of the two posts is that shown horopter but still within the Panum’s fusional
on the left hand side of Fig. 4.9, and, if the left space, nasal disparity will exist, adding the
eye is closed, the appearance is that shown on quality of farness to the perception. Thus, it can
the right side of the figure. The only difference be seen from Fig. 4.10 that, when the images do
between these two images is that the posts seem not fall on exactly corresponding retinal points,
to be farther apart in the frontoparallel plane an impression that the image is in front or
when seen with the left eye than when seen with behind a given frontal plane is produced. In the
the right. This difference in lateral separation is figure 4.10, F and F' are the foveae fixating a
due to the fact that the receptors stimulated in point C, straight ahead. This point determines
the left eye are more widely separated from each the frontal plane, XX’. An object A, whose image
other than those in the right retina (note distance falls on a in the left eye produces an image at b’
between a and b of two eyes in Fig. 4.9). in the right eye. b’ is not the corresponding point,
 68 Theory and Practice of Squint and Orthoptics

Worth classified fusion in three degrees.

First-degree fusion was the simultaneous
macular perception of two different non-
fusable objects. Second-degree fusion was
characterized as the ability of the motor system
to align the eyes in such a way that sensory
fusion was possible. Third-degree fusion was
stereopsis. Worth’s classification of third-
degree fusion, however, is not entirely correct
since stereopsis is a separate quality of the
visual system, a sensory responsiveness to
disparate stimuli that can occur, under certain
conditions, without second-degree fusion being
present.
In a two-dimensional representation, certain
colours seem to stick out and others do not.
Colour stereopsis is related to the differential
refraction for various wavelengths of light. The
Fig. 4.10. Scheme showing the effect produced by stimulation different blur circles can stimulate disparate
of slightly incongruous retinal points. a-a', corresponding retinal points within Panum’s area and thus give
points; b-b' corresponding points; F-F', the foveas
true stereopsis to a flat picture in which there is
no lateral or horizontal separation of the retinal
which is a’. Because of this, the impression is images.
that the point A lies nearer the eyes than the
frontal plane, XX’. Similarly, an object O, whose
image falls on b in the left eye produces its image PHYSIOLOGICAL BASIS OF STEREOPSIS
at a' in the right eye. The corresponding retinal Stereopsis a function of spatial disparity
point of b is b’. The image of object O lies to the Stereopsis arises when horizontally disparate
nasal side of this point at a’ in the right eye. The retinal elements are stimulated simultaneously.43
impression is gained, therefore, that point O lies The fusion of such disparate images results in a
behind frontal plane XX’. single visual impression perceived in depth,
Thus, it proves that in general nasal disparity provided the fused image lies within Panum's
gives the impression of remoteness of an object, area of single bionocular vision. Vertical
and temporal disparity gives the impression of disparities produce no stereoscopic effect. A
nearness of an object. solid object placed in the median plane of the
Stereopsis is a unique cognition, a distinct head produces unequal images in the two eyes
perceptional quality of its own. If one does not due to horizontal separation of the two eyes
have it, one cannot learn it even in the presence (Fig. 4.11). The sensory fusion of the two unequal
of all requirements such as bifoveal fixation, retinal images results in a three-dimensional
fusion, and good visual acuity. Although percept.
stereopsis is the most important factor in A stereoscopic effect can also be produced by
judging distances, especially at close range, two-dimensional pictures, some elements of
additional information about the absolute and which are imaged on corresponding retinal
relative distances of objects is gained in several points to give the frame of reference for the
other ways. While in bifoveal vision these other relative localization of other elements of figures
means are only an addition to stereopsis, they constructed to provide horizontally disparate
are the only source of information on which a imagery. Such figures must be viewed separately
monocular observer can base his distance but binocularly in a stereoscope or some
judgement. haploscopic device.
 Binocular Vision 69

Fig. 4.11. A solid object placed in the midline of the head creates slightly different or disparate retinal images, the fusion
of which results in a three-dimensional sensation.

Local and global stereopsis perception must obviously take account of both
Julesz 44,45
studied stereopsis perception utilizing local and global stereopsis.
random stereogram and put forward the concept
Fine versus coarse stereopsis
of local and global stereopsis. The term local
stereopsis is used to denote stereopsis elicited Fine stereopsis is a highly specific pattern
by the dot-by-dot or square-by-square matching matching process involving very local features
process that occurs between the right and left of the two retinal images and operating over a
stereogram. relatively narrow range of spatial disparities
probably no more than about 0.5 degrees.
When there is ambiguity as to which elements
Dissimilar images cannot be simultaneously
in the two retinal images correspond to each
perceived. If binocular vision is to occur, the two
other, a global process is needed that evaluates
retinal images must be closely similar, otherwise
different possible sets of corresponding pairs
there is retinal rivalry and suppression of one
and selects one set of matched pairs that, by their
or other of the antagonistic image features.
depth values, can provide the data for
recognition of a three-dimensional form. Julesz Coarse stereopsis is a much less specific process
refers to this additional mechanism as global that can operate on visual images quite
stereopsis, since the problem of ambiguities can dissimilar in form, luminance and contrast and
only be resolved on a global basis. Therefore, a separated by several degrees in spatial position-
satisfactory theory for binocular depth up to as much as 7 to 10 degrees of retinal image
 70 Theory and Practice of Squint and Orthoptics

disparity. Since coarse stereopsis acts beyond the

range of retinal rivalry, dissimilar images can
be simultaneously perceived. For coarse fusion
(single vision), however, the images must again
be similar, but the disparity limits over which
fusion can take place (Panum’s area of up to
about 7 degrees) are much larger than they are
for fine stereopsis. Coarse single vision may
occur in the absence of fine fusion, but fine single
vision is always accompanied by coarse fusion.

STEREOPSIS AND FUSION

Fusion is not absolutely required for stereopsis.
Stereopsis is independent of fusion, but it is
necessary that similar parts of stereograms be
fused (stimuli reaching corresponding elements),
since these form the place of reference.
The presence of sensory fusion, that is ability
to unify images falling on corresponding retinal
areas, in itself does not guarantee the presence
of stereopsis. A patient may fuse similar targets Fig. 4.12. The regions of obligatory and qualitative
but have no stereopsis. Such patients suppress stereoscopic perception of depth about the point of fixation.
selectively the disparately imaged elements of From Angle46
a stereogram seen by one eye.
A certain degree of stereopsis may occur in The degree of responsiveness to disparate
diplopia. Conversely, there may be fusion stimulation is also related to visual acuity. All
without stereopsis. Some people are capable of other things being equal, the better the visual
motor and sensory fusion but have no acuity, the better the stereoscopic acuity. Good
perceptual resonse to disparate stimuli and, visual acuity, however, does not guarantee
therefore, have no stereopsis. Peripheral stereoscopic acuity. Reduced visual acuity, such
stereopsis may exist where central stereopsis as that with monocular amblyopia, does not
does not. Our methods primarily test central preclude stereopsis. While fusion and acuity are
retinal areas. There are persons who show important factors in stereopsis, there is no linear
regional suppression but who have peripheral relationship between them. It is important to
stereopsis. realize that a shift of visual directions takes place
in stereopsis. In other words, objects viewed by
Experiments of Burian10 and others have shown
stereopsis produce a visual impression and a
that a sense of depth still occurs under certain
visual direction unlike the visual direction
conditions even though the disparity of the
initiated by either eye alone.
receptors stimulated is such that they lie outside
of Panum’s area and, therefore, are not seen
single, but double. Figure 4.12 shows the regions, STEREOSCOPIC ACUITY
as determined by Ogle,46 in which there is single Stereopsis is one of the finest spatial discrimina-
vision without depth (determined as the tions of which the human visual system is
horopter), single vision with stereoscopic depth capable. There is minimal disparity beyond
(determined as Panum’s area), and finally, which no stereoscopic effect is produced. This
outside the Panum’s area a crude sense of depth limiting disparity characterizes a person’s
and diplopia. As Burian10 points out, it is not stereoscopic acuity.
strictly correct to call stereopsis third-degree Stereoacuity of as low as 2 to 7 sec of arc have
fusion. It is more than that. It is a sense sui genesis. been found. But a threshold of 15 to 30 sec
 Binocular Vision 71

obtained in clinical tests may be regarded as I. STEREOPSIS

excellent. There are no standardized clinical As discussed earlier, stereopsis is the relative
stereoscopic acuity tests comparable to visual localization of visual objects in depth which can
acuity tests. occur only under conditions of binocular vision.
Under normal conditions, most observers with As described in detail (page 67–71), stereopsis
no ocular abnormalities can discriminate a depth is based on a physiologic process derived from
differ-ence between two images with a relative the organization of sensory visual system.
disparity of only about 10 arc sec (0.0028°). For Stereopsis is apparently innate, not acquired
the closest fixation of 10 inches away, the best through experience and is unequivocal and
stereoscopic threshold corresponds to the inescapable.
appreciation of a depth of one thousandth of an
inch.25 II. NON-STEREOSCOPIC CLUES TO THE PERCEPTION
Beyond 600 metres, there is no true stereopsis. OF DEPTH UNDER BINOCULAR CONDITIONS
At this distance, monocular clues take over for Under binocular conditions, retinal disparity
the perception of depth. This allows stereoscopic gives a clue to depth without stereopsis. When
discrimination of depth in some types of clouds. there is temporal retinal disparity, an awareness
These limits provide useful stereopsis over an of nearness occurs. When there is nasal retinal
extensive range of environmental conditions. disparity, an awareness of distance occurs.
Stereoacuity is excellent at the fovea, but
decreases from the centre to the periphery of III. MONOCULAR CLUES (NON-STEREOSCOPIC
retina. Stereopsis rapidly becomes very poor CLUES TO SPATIAL ORIENTATION)
beyond about 20° eccentricity, or outside the Stereopsis is restricted to relatively short visual
circle passing through the two blind spots in the distances and is not the only means we have for
binocular visual fields.47 spatial orientation. There is a set of monocular
or experimental clues that play an important role
in our estimation of the relative distance of
DEPTH PERCEPTION
visual objects. They are the result of experience
Depth perception is the perception of distances and are equivocal. Monocular clues include the
of objects from each other or from the observer. following:
It is somewhat ambiguous term since it is also
used to imply the visual perception of three- 1. Parallactic movements
dimensional space. Next to stereopsis, parallactic movements are
Using it in its first connotation, it is most important in the perception of depth. They
independent of the appreciation of three are the apparent movements made by objects
dimensions and does not require binocular when the observer moves his head. A slight shift
vision. A monocular observer is quite capable of the head in any direction while fixation is
of judging distances and of obtaining an maintained on the same point results in a change
impression of spatial order. Little is known of the relative position of all objects in the
about the quality and mechanism of absolute direction of gaze. Objects beyond the fixation
depth perception where a judgement cannot be point appear to move in the same direction as
based on any additional clues, as, for instance, the observer’s head, whereas objects closer than
when estimating the distance of a point light the fixation point appear to move in the opposite
source in an absolutely dark room. In a normal direction. Greater the displacement of an object
visual environment, several factors contribute relative to the fixation point the more distant
to the perception of depth: stereopsis, parallactic the object is from the fixation point.
movements, interposition of objects, size in
which an object is seen, distance of an object 2. Linear perspective
from the horizon, perspective, shadows, and Object points having a constant size appear to
aerial perspective. subtend smaller and smaller angles as they
 72 Theory and Practice of Squint and Orthoptics

recede from the subject, e.g. railroad tracks

which are in fact parallel seem to approach each
other in the distance. This is an example of the
perspective in which the three-dimensional
object space imaged geometrically in two
dimensions on the retina creates an impression
of depth. The geometric reproduction in two
dimensions is not only of theoretical interest
with respect to the translation of such two-
dimensional retinal stimuli into a three-
dimensional perception, but is also of great
importance in creating the feeling of depth in
drawings and paintings. Its most prominent
feature is the fact that lines which are parallel in
object space and at an angle with the picture
plane seem to be directed to one point, the
vanishing point (Fig. 4.13).

3. Overlay of contours
By superimposing or interposing the contour of
configuration, one can find distance clues.
Interposition of objects gives an absolute clue
as to their relative position, since the outline of
one object interrupts or hides the contour of
another object behind it (Fig. 4.14).

4. Size
The size in which an object is seen is another
Fig. 4.14. Effect of overlay of contours; A and D, depth is
important factor in the judgement of distances, achieved by interposing one object in front of the other; B,
provided the actual size is known to the C and E, the same forms are drawn to avoid a clue of depth.
observer. Objects encountered in daily life, such
as people and cars, allow a definite judgement of their distance. If the actual size of an object is
unknown, however, and a comparison with a
known object is impossible, marked errors
occur. This may be the case, for example, when
looking at a bird against the sky. If its real size
is unknown to the observer, then distance
judgement is merely a guess. The difficulty is
particularly obvious at night if an attempt is
made to estimate the distance of a light source
of unknown size.

5. Distance from horizon

The distance of an object from the horizon or its
relative height in the visual field gives an
important clue to distance. Since the eyes of an
observer are at a certain height above the
Fig. 4.13. Drawing demonstrating the affect of linear ground, it appears as if the ground is rising
perspective (vanishing point). toward the horizon. Thus, objects more distant
 Binocular Vision 73

the shadow is above, the object appears to curve

in.
A classic example of this is the picture of a pit
in a wall with sunlight falling from above at a
small angle against the wall. The upper portion
of the pit will be filled with a dark shadow while
the lower part is brightly illuminated. Turning
the picture upside down, one no longer sees a
pit but instead a distinct hump, the upper
portion of which stands out brightly while its
lower part casts a shadow (Fig. 4.16).

7. Aerial perspective
Fig. 4.15. Drawing showing effect of distance from horizon The influence of the atmosphere on contrast
in depth perception. Objects that are close to the horizon are
conditions and colours of more distant objects
perceived as being farther from the observer.
is referred to as aerial perspective. Objects
appear less clear and acquire a more bluish tinge
from the observer will be higher in the visual as the viewing distance increases. The colour
field (Fig. 4.15). change towards blue is particularly obvious, if
one views the shadows of a mountain chain from
6. Distribution of highlights, shadows, shades some distance.
and light
Highlights and shadows provide a very IV. INFLUENCE OF ACCOMMODATION AND
important monocular clue for perception of CONVERGENCE ON DEPTH PERCEPTION
depth. Since, sunlight comes from above, we A given object at a given distance is imagined
have learned that the position of shadow is in a certain size on the retina. It would appear
helpful in judging the raised and depressed area, natural that the size of the retinal image
that is the relative depth in objects. When there determines the size in which the object is seen
is a shadow on the lower portion of an object, subjectively. This, however, is not the case. There
the object appears to stick out in space. When is no doubt that the size in which an object is

Fig. 4.16. Drawing showing effect of highlight and shadows on depth perception: A, Upright picture; B, Same picture turned
upside down.
 74 Theory and Practice of Squint and Orthoptics

perceived can be changed mentally. The sensory system is the feedback loop, which
apparent increase in the size of an object that is controls this motor alignment.
brought closer to the eye does not correspond The processes of normal retinal corres-
to the increase that might be expected on the pondence allow the fusion of two physical
basis of the enlargement of its retinal image. This retinal images into a single mental impression.
effect has been attributed to the influence of When this fusion occurs, a new quality of the
accommodation and/or convergence, and in subjective visual process takes place and the
turn it was postulated that these functions perception of depth by parallax (stereopsis)
contribute a clue for depth perception. There is results. The correspondence mechanism only
no proof of this. However, experimental allows for the relative localization of one object
evidence points in the other direction that after to another in physical space, and stereopsis
a judgement of distance has been made, this then represents the latitude that retinal corres-
influences the apparent size of the object. pondence allows and in which diplopia does not
occur. The image perceived in depth is seen
CONCLUSION neither in the specific visual direction dictated
The impression of three-dimensionality by the right eye nor by the left eye but a visual
imparted by these clues is a judgement, an direction corresponding to non-stimulated
interpretation. So false judgements are possible. elements of the two eyes.
Also these depend on past experience. The mental process of absolute localization is
Monocular clues and binocular clues work essentially a compromise that not only requires
hand in hand, one enhancing the effect of the decoding and analysis of retinal information,
other, but this is not always true. If one supplied by the correspondence mechanism, but
introduces into a stereogram confusing clues, i.e. that is also modified by other information from
monocular clues that conflict with stereoscopic the efferent and afferent motor monitoring
clues, false observations can be made. systems. This is best demonstrated by the patient
Some people are more responsive to with paralytic strabismus with normal retinal
stereoscopic clues, whereas others respond more correspondence. The integration of the motor
readily to monocular clues. and sensory mechanisms in binocular vision
Thus humans have two sets of clues for their represents a complex process, as will be seen in
orientation in space (depth perception). pathologic conditions affecting either one or
Monocular clues depend upon past experience both processes. The child with strabismus may
and clues provided by fusion by disparate show sensory adaptation to a motor anomaly
retinal images afford the direct perception of this or, may adapt to a sensory anomaly by changing
relation on the basis of intrinsic physiologic motor response.
arrangements.
DEVELOPMENT OF BINOCULAR VISION
INTEGRATION OF THE MOTOR AND After having a workable knowledge about the
SENSORY SYSTEM INTO BINOCULAR psychophysical and sensory aspects of the
VISION binocular vision, it will be easier to understand
While it is convenient from a teaching point of development of this complex phenomenon. It is
view to separate the motor and sensory unequivocal that basic visual functions are
mechanisms of binocular vision, they are innate and, therefore, present at birth. But their
absolutely integrated in the visual process that coordination, maturation and refinement take
we call binocular vision. The motor system place during early postnatal period. Therefore,
aligns the foveae of the two eyes on the object any obstacle during this period may cause
of regard and maintains this fixation whether abnormalities in the development of normal
the subject or the object moves. It allows the field binocular vision. The subject matter on
of vision to become the field of fixation. The development of binocular vision includes:
 Binocular Vision 75

• Prerequisites for development of binocular in adulthood. Accordingly, the convergence

vision required to fixate an object at 33 cm distance
• Eye at birth and normal postnatal development increases from about 13 PD to about 19 PD.
• Maturation of binocular functions Angle lambda () which is positive and may be
• Neurophysiology of development as large as 10°, reduces to at least one-half of
• Theories of development that amount during postnatal development.
Eyeballs also continue to grow. Growth of the
PREREQUISITES FOR DEVELOPMENT OF globe is indicated by:
BINOCULAR VISION
• Change in length from 17.5 mm at birth to
In order that simultaneous binocular vision be 24 mm in adulthood.
obtained, following conditions must be • Meridional fibres of the ciliary muscle are
present:53 completely formed at birth, but the circular
1. Fixation. There must be proper fixation with portion continues to develop during the first
each eye. The muscles controlling the one or two years of life.
movements of each eye must function normally • Gross appearance of foveal cones changes during
and turn both eyes in such a manner that the the first a few months of life. Their fine
object of regard is fixated by corresponding structure, as revealed by electron microscopy,
retinae, i.e. the two foveae. seems to be well developed.
2. Visual fields of the two eyes must overlap to • Extraocular muscles, although not of ultimate
a large extent. size, are fully functioning at birth.
3. Image formed on each retina must be • Postural reflexes, which are innate and
approximately similar, i.e. should be of the same unconditioned, are also functioning fully.
size, shape, colour and intensity. This necessitates
approximate equality in the optical apparatus Bifoveal fixation does not exist at birth since
of each eye. binocular movements are not coordinated. The
4. Common visual direction must be there of two two eyes move more or less independent of each
eyes, i.e. the retinae must possess physio- other. Fixation, therefore, is at first only
logically corresponding points. monocular. The principal visual direction of the
5. Reflex activities which produce fusional central fovea and the visual directions of the
movements are very important. The eyes must other retinal points relative to the principal
be co-ordinated by this complex mechanism at direction are innate. Therefore, monocular
all times so that retinal receptors which have a fixation is present at birth, but it is poor.
common visual direction will receive the same Furthermore, conscious fixation requires
image at all times. awareness of the presence of an object and then
enough interest in the object to occupy an
EYE AT BIRTH AND NORMAL individual’s attention for a certain length of
POSTNATAL DEVELOPMENT time. The degree of mental activity necessary for
this exceeds the capability of the newborn infant,
The anatomic development of the orbits and
and this is another reason why fixation is only
eyes is not completed at birth.
rudimentary in the beginning.
Orbits continue to change their size and shape At about 2 to 3 weeks of age, the infant begins
throughout childhood into the teenage years, to make movements of regard, turning his eyes
and the angle which their axes form with one to fixate an object. At 4 to 5 weeks of age, he can
another decreases from 50° at birth to 45° in later sustain monocular fixation of large near objects.
life. Thus, in adults, the medial wall of the two Only after the age of 3 months the fixation
orbits are parallel to each other while the lateral become conscious rather than reflexive.
wall diverge at 90°. At the same time, the Although the two eyes move independently at
interpupillary distance increases from an birth, aimless conjugate movements may occur.
average of 45 mm at birth to about 58 to 66 mm At about 6 weeks of age, fixation alternates
 76 Theory and Practice of Squint and Orthoptics

rapidly between the two eyes, and a short time conscious rather than reflexive.56 The child starts
later the child begins to fixate binocularly and to focus on the parent’s face, toys and responds.
to perform conjugate pursuit movements, The infant responds better to high contrast
following persons or large near objects. Initially, images, bold colors and bright objects.
the 3e pursuit movements are saccadic in Therefore, the parents should be advised to
character but become smooth and gliding paint the baby’s room in bright colours, decorate
between 3 and 5 months of age. with contrasting shapes, handing brightly
coloured toys over the crib, adding new
Disjugate vergence movements develop after
furnishing to the room to retain interest,
conjugate movements. Convergence is demons-
changing the direction of the crib frequently so
trable after 3 months of age and is stable at about
that baby can see new furnishings. Even at night
6 months.
dim light should be kept on to help stimulating
Fusional movements occur at the same time, so the baby’s vision.
that following an interruption of fusion, such
as by introducing a prism before one eye, a
 At 6 months of age, vision improves to 6/12
corrective movement occurs to reestablish from 6/240 at birth (Table 4.1) as tested by
bifoveal fixation. Fusional movements are firmly visual evoked potential (VEP). However, on
established by the age of 1 year. testing with optokinetic nystagmus (OKN)
and preferential looking (PL) the visual acuity
Visual acuity and fixation has always been is reported about 6/30. 57,58 By the age of 6
considered to be very poor at birth. However, months the hand–eye coordination also
psychophysical and electrophysiologic research develops and the infant can now locate toys
during recent years has established that visual and tries to grasp them. So 6 months is the
acuity in infants develops much more rapidly time when a detailed ophthalmological
than once thought, and that an infant’s visual examination should be conducted for all
capacities are surprisingly rather well established infants to rule out gross refractive errors
shortly after birth, and that adult levels are amblyopia, strabismus and a definitive
reached at approximately 2 to 3 years of age. treatment is started.
This, of course, does not take into account the
state of development of the interpretative mental
 By 1 year of age, most children become
functions but refers strictly to the resolving emmetropic. At birth, all of the infants are born
power of the visual system. hypermetropic due to smaller axial length of
eyeball.59 Stimulation of retina by light results
Visual development and estimated visual acuity in growth of eyeball and resultant emmetropia
by the age of 1 year.60 The optic nerve is fully
 At birth, the fovea as well as lateral geniculate myelinated, the visual pathway is matured and
nucleus (LGN) is not developed, so an infant has the vision is about 6/6 to 6/9 by the age of
peripheral vision only and can see to the sides 1 year.
but cannot focus the eyes straight ahead or smile
back at the parents because of blurred central  Age by which 6/6 vision is achieved varies
vision.54,55 Visual acuity at birth is about 6/240 depending upon the test used as below:61
(Table 4.1). • Optokinetic nystagmus (OKN): 24–36 months
 At about 6 weeks, the child fixates peri-
• Preferential looking test (PLT): 24–36 months
pherally which alternates rapidly. Vision is
• Visual evoked potential (VEP): 6–12 months
about 6/60 (Table 4.1)
 By 2 months of age, LGN and its connections
to the visual cortex in occipital lobe develop and MATURATION OF BINOCULAR FUNCTIONS
the infant begins to follow moving objects.55 Development of fusion
 By 3 months of age, the fovea is fully Although basic visual functions are innate and,
developed. The fixation becomes central therefore, present at birth, other complex
 Binocular Vision 77

Table 4.1 Visual development and estimated visual acuity from birth to 6 years of age
Age Refractive Normal visual Estimated visual acuity
status development Optokinetic- Preferential Visual evoked
nystagmus (OKN) looking potential
(Keelar PL
cards)
At birth Hypermetropia Pupillary light reflex —————— 6/300 6/240
present, fovea and
lateral geniculate body
not developed, no
central fixation
1 month Hypermetropia 6/120 6/200–6/90 6/120
2 months Hypermetropia Follow moving objects 6/60 6/90–6/60 6/60
3 months Hypermetropia Fovea developed, 6/36 6/90–6/60 6/36
central fixation developed
6 months Hypermetropia Stereopsis developed 6/30 6/36–6/30 6/6–6/12
up to 600 sec of arc
(Firsby)
1 year Emmetropia Optic nerve myelination, 6/18 6/24 6/6–6/9
complete visual differen-
tiation of objects
developed, stereopsis
up to 210–170 sec of
arc (Frisby)
18 months Emmetropia Visual acuty at adult 6/12 6/18 6/6
level on paediatric acuity
card, stereopsis
developed up to 170–150
sec of arc (Frisby)
24 months Emmetropia Stereopsis developed 6/9 6/12 6/6
up to 100–85 sec of arc
(Frisby)
36 months Emmetropia Contrast sensitivity 6/6 6/6 6/6
fully developed,
stereopsis developed
up to 85–55 sec of
arc (Frisby)
Up to Emmetropia Stereopsis developed 6/6 6/6 6/6
5 years by 30–20 sec of
arc (Frisby)
Up to Emmetropia Stereopsis developed 6/6 6/6 6/6
6 years up to 10–5 sec of
arc (Frisby)

functions of binocular cooperation have to be At birth, the eyes are not associated with each
learned before normal bifoveal single vision other, but act as two independent sense organs.
eventually is firmly established. The mechanisms necessary for binocular single
 78 Theory and Practice of Squint and Orthoptics

vision are not completely developed. The  Stereopsis seems to be in a class by itself
foveas are not formed until the third month of because it is claimed by some that there are
life. As they develop, the stimulus to associate persons with entirely normal eyes and
these areas is provided. By trial and error, the neuromuscular apparatus who do not have
child learns that, when the image of an object depth perception by parallax when all other
is brought onto the two foveas simultaneously, clues to depth are eliminated. As Ogle46 has
the image is most detailed. For this reason stated, "Stereopsis is a sensory phenomenon in
alone, the visual axes are oriented in such a way its own right, with its own physiological
that each fovea is directed at the object of mechanisms. It seems to be an all-or-none
regard. phenomenon, in that in a given person it is either
present or not present. Training does not seem
Once this has become an established habit, the
to develop stereopsis as such, but training may
relative space perceptions of the child begin to
increase one’s ability to discriminate depth
take form. Objects to the right of fixation send
differences just as the visual acuity may be
images to retinal areas in the two eyes which
slightly improved by training".
have a common visual direction, i.e. to the right
of fixation. The crossfiring of various sensory  Age norms for stereoacuity development and
phenomena, such as touch with vision, the tests used are depicted in Table 4.2.
eventually leads to an accurate determination
of the child’s space. An object seen so many NEUROPHYSIOLOGY OF DEVELOPMENT
degrees to the right of fixation is eventually  The M and P cell neurophysiology. Neuro-
interpreted in its correct position, and this is physiological animal studies have identified two
rewarded by checking accurately with the specific pathways used to process visual
experiences of touch. Hence, by trial and error, information. These two pathways arise from
and with tactile sensations the eyes become different populations of retinal ganglion cells.
accurately associated with one another until Ganglion cell stimulation from a retinal image
gradually a normal child develops perception results in simultaneous parallel processing
of space. through these two different pathways. In the
If the eyes are never allowed to become lateral geniculate nucleus, the nuclei can be
associated, as for example by a failure of divided into parvocelluar (P cells, or small cells)
development of one fovea or because of and magnocellular (M cells, or large cells). In
paralysis of an ocular muscle so that the two the striate cortex, parvo- and magnorecipient
foveas cannot always be focussed together on
the object of regard, the child never acquires Table 4.2 Age norms for stereoacuity and the tests used
binocular single vision and never learns to fuse (measurements are all approximate)
the two images into one. Vision under these Age Stereo (secs of arc)
circumstances is always monocular and Birth ——
generally alternating; first one eye is used and One month ——
then the other. The image of the eye which is Three months ——
not fixating the object is suppressed. Six months 600 Frisby
Nine months 300 Frisby
Development of stereopsis One year 210 – 170 Frisby
 Anatomic and physiologic factors necessary 18 months 170 – 150 Frisby
for depth perception in the human being are Two years 100 – 85 Frisby
either present at birth or develop shortly after Three years 85–55 Frisby
birth so that, if the eyes are normal and the Four years 40–30 Frisby
neuromuscular mechanism for moving the eyes Five years 30–20 Frisby
is normal, depth perception will follow Six years 10–5 Frisby
automatically. (Adapted from A. Grounds by C. Rushen and L. Speedwell)
 Binocular Vision 79

lamellae are segregated; however, there are refinement of the neuronal connections in the
interconnecting pathways, so information visual cortex. A possible function of this
commingles. Parvocellular neurons are more plasticity in neuronal properties during early
sensitive to colour, high spatial frequencies, fine visual experience would be to allow the
two-point discrimination, and fine stereopsis, opportunity to match the properties of
and they project to areas of the central visual maximizing its capacity for analysis of the more
field and fovea. Magnocellular neurons, on the important components of its environment. Also,
other hand, are sensitive to direction, motion, the capacity to make modification in optimal
speed, flicker, gross binocular disparities, and disparity and preferred orientation would
gross stereopsis. Magnocellular neurons project ensure that binocular cortical cells adapt similar
to parafoveal and more peripheral retina. receptive field positions and preferred
Magnocellular neurons are used for determining orientations on the two retinae, which is a
where, whereas parvocellular neurons examine primary requirement for the probable role of
static objects and determine what. Even though these cells in binocular fusion and stereopsis.
the two pathways are distinct, they overlap; and Thus, any disruption of the normal develop-
both systems interact to process visual mental conditions, such as a congenital or early
information. From the striate cortex, information strabismus or significantly subnormal vision in
from M cells goes predominantly to parieto- one eye (e.f. due to congenital cataract), will
occipital areas, while information from P cells prevent the development of normal functional
goes to temporo-occipital areas. interrelationship and the loss of binocularity.
 Basic receptive field organisation of neurons  Maldevelopment of M versus P pathways
and cortical architecture are present since birth, secondary to strabismus or anisometropia and
although the retina and optic pathway are not a blurred retinal image is currrently being
completely developed.62 studied. Pattern deprivation amblyopia, which
is a failure to develop fine two-point discrimina-
 Properties of neurons in the visual cortex are
tion, is probably associated predominantly with
markedly influenced by visual experience during
abnormal P cell development. M cell development
the first a few postnatal months. Neuro-
is also affected, especially if the retinal image
physiologic studies have demonstrated there
disparity is quite large. M-neuron maldevelop-
specifically, these neural properties have been
ment occurs predominantly in case of
determined to involve binocularity, orientation
strabismus and may contribute to associated
specificity and disparity specificity.63,64
motor abnormalities such as latent nystagmus
 Excitatory connections of receptive fields and asymmetrical horizontal smooth pursuit
located in both retinae or retinotopic projections often seen in patients with congenital or infantile
are largely present at birth. However, simul- strabismus.
taneous occurrence of patterned visual input to
both eyes during the development period is
 Postnatal morphological changes in the retina
necessary to maintain their association.63,64 and retinal projections relative to the presence
or absence of visual stimuli are well-established.
 Plasticity during maturation stage (i.e. in the It has been observed that, in the lateral
neonatal visual system) is present to a great geniculate nucleus, neuronal cell growth is
deal.65, 66 This early plasticity seems to be vital markedly reduced in the laminae with
for the formation of cells with closely matched connection to visually deprived eye as compared
receptive field properties in the two eyes, which with a nondeprived eye.67, 68 The two types of
is a necessary condition to form the substrate retinal ganglion cells and geniculate cells seem
for stereoscopic vision. Patterned visual stimuli to be differentially affected by visual depriva-
during stage of plasticity of visual system seem tion. The large cells of the binocular segment are
to act not only as a catalyst but also as directional much more affected than either the large cells
stimuli in the consolidation, maintenance and of the monocular segment or the small cells
 80 Theory and Practice of Squint and Orthoptics

found mainly in the projection of the central • It has also been demonstrated that the
area.69 distribution of cortical neurons (as mentioned
above) is easily upset when animals are reared
 Orientation specificity and disparity specificity
with experimental strabismus, anisometropia,
of cortical neurons are also dependent on the
or from vision deprivation by lid suture. This
visual experience in the early postnatal period. It
observation corroborates the fact that the
has also been demonstrated experimentally.70, 71
properties of neurons in visual cortex are
THEORIES OF BINOCULAR VISION greatly influenced by the visual experience
during the first a few postnatal months.76,77
Theory of correspondence and disparity
At present, this is the most widely accepted Older theories of binocular vision
theory of binocular vision. Salient features of this All the older theories of binocular vision have
theory are as follows: been abandoned. However, these are mentioned
• Corresponding elements of retina form the in brief, just to become familiar with the old
framework or zero system of binocular vision. concepts.
Simultaneous stimulation of the corres- 1. Alternation theory of binocular vision. This
ponding points by one object transmits single theory states that sensory fusion is perceptual
visual impression with no depth quality. unification of images perceived in corresponding
• Simultaneous stimulation by two object points locations in the two retinae. It assumes that
that differ in character, results in binocular corresponding retinal units are represented
rivalry. separately in the brain but that each of every
• Diplopia occurs when disparate elements are pair is represented in consciousness by the same
stimulated by one object. single unit. This conscious unit would receive
the stimulus from only one retinal unit at a time,
• Binocular single vision with stereopsis results,
the other being excluded.78
when the horizontal disparity remains within
the limits of Panum’s area. This theory fails to explain many phenomena
of binocular vision, particularly stereopsis.
 Neurophysiologic basis of correspondence 2. Projection theory of binocular vision. This
theory: Psychophysical data collected from theory is based on the concept that the visual
human studies and neurophysiologic evidence stimuli are exteriorized (projected to physical
collected from animal experimental studies of space) along the lines of directions.79 This theory
various researchers, Hubel and Wiesel being the is not able to explain even the fundamental
pioneers,72-74 have corroborated the correspon- observation such as physiological diplopia and
dence theory. Till date following neuro- so abandoned.
physiological evidences are available:
3. Motor theory. This theory conceptualizes that
• Approximately, 80% neurons of striate cortex
the spatial orientation is obtained from the
are derived from each eye, 10% from the
sensation derived from the movement of the
contralateral eye and 10% from the ipsilateral
head, conjugate movements of eyes and
eye only. The two receptive fields of binocularly
convergence. The eyes are made aware of their
driven cortical cells are found to have
movements by muscle sense. It is this awareness
corresponding location in the two retinae.72-74
that produces spatial localization. The sensations
• Of the binocularily driven cortical neurons,
arising from the convergence effort determine
only 25% are stimulated equally well from
whether one object is nearer or farther away than
each eye, while the remaining 75% show
the others. This theory again fails to explain
graded degrees of influence from the right or
many sensory aspects of the binocular vision
left eye (disparity sensitive binocular cells).72-74
especially stereopsis.
• Stereopsis has been linked with horizontal
disparity sensitive binocularly driven cortical 4. Theory of isomorphism. This theory states that
neurons.75 there exists a strict point-to-point relationship
 Binocular Vision 81

between retina and cortex and strict comformity clearer than the nonfoveal image. The
or isomorphism between the distribution of symptomatology of diplopia depends on the age
objects in space and cortical events form the at onset, duration, and subjective awareness.
basis of spatial orientation. Subjective visual Visually immature children (less than about 6
directions as a property of the retinal-elements or 7 years) rarely complain of diplopia. The
do not exist and that retinal correspondence younger the child, the greater the ability to
cannot change.80 However, it is interesting to suppress.
point out that, there is no evidence for the
physiologic rigidity of the retinocortical
 Horror fusion is an intractable diplopia in
relationship or the convergence of the pathways which there is an absence of central suppression.
on which this theory is based. The angle of strabismus may be small or
variable. Horror fusionis may occur in a number
DISTURBANCES IN THE DEVELOPMENT OF of clinical settings: for example, after fusion has
BINOCULAR VISION been disrupted for a prolonged period, after
head trauma, and rarely in long-standing squint.
The time at which a lesion or defect in the visual The management of these patients can be
system occurs is a most important factor with frustrating.
respect to the effect it will have on the disruption
of existing functions and the prevention of  Confusion, like diplopia, is associated with
further development. During the formative ocular misalignment; however, confusion is very
years, a neural pathway or neural function is rare. Most adult patients with acquired ocular
maintained only through the stimulus of normal misalignment see double, i.e. two of the same
use. If this is disrupted, the involved structures image. Rarely, however, patients will describe
will lose their functional capability. Naturally, the simultaneous perception of two different
if the disruption occurs before a certain function images superimposed on each other. Because the
has become established, it will not be learned at eyes are misaligned, dissimilar images fall on
all. Thus, the age of the child at the onset of a each corresponding fovea, and this, in a rare
tropia is extremely significant in assessing the patient, will cause confusion rather than
prognosis and deciding on the management. The diplopia. In other words, objects that are
earlier the deviation occurs, the less function will physically separated in objective space are
have developed and the easier will be disruption imaged on corresponding areas of the two
of existing functions. If it occurs between 18 retinas and are, therefore, seen as having the
months and 2 years of age, the prognosis for same location in subjective space.
eventual bifoveal single vision is poor, whereas
if it occurs at a later age, normal function may Suppression
be regained with the adequate treatment. Suppression is an active cortical inhibition of the
Anomalies in the development of binocular vision of one eye. Generally, it is supposed that
vision may be in the form of suppression, the whole of one retinal function is extinguished
amblyopia, abnormal retinal correspondence. in consciousness, but this is not usually so, and
Burian10 believes it is not generally the rule.
Diplopia and confusion Instead of total extinction, he considers that
 Diplopia usually results from an acquired selective suppression in which only certain
misalignment of the visual axes that causes an regions of one retina are suppressed take place
image to fall on the fovea of one eye and more often. An example of this is the occurrence
simultaneously on a nonfoveal point in the other of suppression scotoma which can be
eye. The object that falls on these non- demonstrated in the foveal area of some children
corresponding points must be outside Panum’s with convergent strabismus. This scotoma
area to be seen double. In diplopia, the same disappears, however, in this eye when it is made
object is seen as having two different locations to take up fixation alone, showing that the
in subjective space; the foveal image is always scotoma is purely a functional one and not due
 82 Theory and Practice of Squint and Orthoptics

to any organic disease of the retina or visual the strabismus is monocular than those whose
pathways. Burian10 believes that suppression squint begins later. Further, it is said that, if
may be selective also with regard to a specific suppression stops the development of foveal
retinal function; that is, the ability to resolve function before it has matured, normal vision
contours may be defective momentarily. never can be expected. It will be possible to
The suppression which occurs under restore only the function of the retina to the level
conditions of strabismus, as just outlined, is to which it had developed before suppression
facultative, i.e. occurs only under certain set in.
conditions. If the strabismus is not alternating, Most of the evidence suggests that the site of
but is monocular with one eye remaining the interference in amblyopia is a block in the
fixation eye and the other constantly deviating, cortex and not a retinal activity. Most of the
the suppression may become so constant and functions of the macula are intact in the
so deep that it persists. Then when the usually presence of amblyopia, for example, dark
deviating eye is forced to take up fixation, the adaptation and colour vision, but that form
inhibition of its fovea remains, and the vision in vision alone is affected.82,83 In patients with
this eye is defective. The suppression under amblyopia, the absolute threshold was found
these conditions is no longer facultative, but to be normal, both foveally and peripherally
obligatory. 81 This obligatory suppression is in cones and rods and in light-adaptation and
called amblyopia. dark-adaptation. The entire apparatus of light
perception was found to be normal in these
Amblyopia patients. The capacity for fixating and
Amblyopia (functional amblyopia) by definition localizing illuminated points and areas on the
refers to a partial loss of sight in one or both central and peripheral retinae was also found
eyes, in the absence of ophthalmoscopic and/ to be normal. The capacity for discrimination
or other marked objective signs. It results from of pattern fell as low as 2/200 or 2/400, without
psychical suppression of the retinal image. It any loss of sensitivity to light. This shows that
may be anisometropic, strabismic or due to the apparatus for form vision is to some degree
stimulus deprivation-amblyopia exanopsia (e.g. distinct from that involved in simple light
in a child with congenital cataract, severe ptosis). perception.
Amblyopia follows through a stage of suppre- Therefore, strabismic amblyopia, according
ssion. to most authors, probably consists of cortical
As just stated, suppression is a process of inhibition of the higher cortical function of
active inhibition, and at first it is probably pattern vision, without notable impairment
always facultative, i.e. occurs only when both of the lower cortical functions of simple light
eyes whose visual axes are not in alignment are perception and spatial localization. Not all
being used simultaneously. However, the authors are satisfied with this concept that
degree to which facultative suppression can amblyopia is a selective inhibition of the form
produce obligatory suppression or amblyopia sense, as such, while all the other functions
probably depends upon the age of the child of the retina remain intact. The flicker fusion
when one recalls that an infant is not born with threshold of the foveal area of patients with
fully developed eyes, anatomically or functionally. amblyopia has been found to be considerably
If suppression is induced in a very young infant, depressed as compared with values obtained
it probably can become obligatory in a much from the surrounding retina and with the
shorter period of time than if it starts in an older values obtained from the nonamblyopic eye
child. The amblyopia is therefore deeper and less in the same patients. However, the true
easily broken up than when it begins later in nature of amblyopia is probably not known
life. This is in accord with clinical experience. entirely. In addition to decreased visual
Children whose strabismus begins early in life acuity, there is some evidence which points
generally have more deep-seated amblyopia, if to concomitant weakening of the power of
 Binocular Vision 83

central fixation. It is reported that only 20% BINOCULAR VISION TESTS

of the subjects with amblyopia fixate along
the central foveal axis of the poor eye when TESTS FOR SIMULTANEOUS MACULAR PERCEPTION
the good eye is occluded.84 The shift from the See page 57 and Fig. 4.1A.
true foveal axis to some outlying area
increases with the increased depth of the TESTS FOR FUSION
amblyopia. See page 58 and Fig. 4.1B.

Abnormal retinal correspondence TESTS FOR STEREOPSIS

Abnormal retinal correspondence (ARC) is an Stereopsis tests are available in a variety of
active cortical adjustment in the directional designs and produce a three-dimensional object
values of the two eyes which occurs in a child in a variety of different ways. Most tests use
with early onset of squint (especially esotropia, simple geometrical shapes as test objects
occurring before 2 years of age). In this presented against a random patterned
condition, the two foveae no longer have a background. It seems to be unavoidable that
common visual direction, and the fovea of one stereo tests produce monocular clues of depth
eye and a peripheral retinal element of the other to some degree; precautions may need to be
eye acquire a common visual direction. This taken in the application of the tests to ensure
adaptation is brought about by an inherent that these clues are minimised.
desire for some form of binocular vision and to • For young children, the Lang test and the Frisby
avoid diplopia and confusion that would screening test are designed to produce
otherwise take place. ARC is more common in behavioural response. With these, the child
esotropia than in exotropia. It is less common in attempts to reach out and grab the object.
vertical deviations and in true alternating These tests may provide a result in some
squints with equal vision. children as young as 6–12 months.
• In older children, a variety of tests are available.
Types: ARC is of two types:
Note. Various tests employed to test stereopsis
1. Harmonious ARC is present when the angle of
must incorporate two essential features:
anomaly (difference between the objective and
• The two eyes must be dissociated; that is, each
subjective angle of the squint) equals the
eye must be presented with a separate field of
objective angle of squint.
view, and
2. Unharmonious ARC is present when the angle • Each of the two fields or targets must contain
of anomaly is less than the objective angle of elements imaged on corresponding retinal
deviation. areas. The commonly employed tests are as
follows:
 Advantages of ARC include:
• It gives the patient a form of binocular single Tests employed to check stereopsis can be
vision. grouped as below:
• Synaptophore or stereoscope tests
• It tends to stabilize the angle of the deviation.
• Vectograph tests
• The patient has a better visual judgement • Random dot stereogram tests
because he may have some binocular • Simple motor task tests based on stereopsis
appreciation of depth.
I. Synoptophore or stereoscope tests
 Disadvantages of ARC are: See page 58, 138 and Fig. 4.1C.
• Once developed, it is extremely difficult to
establish normal correspondence. II. Vectograph tests
• Postoperatively, the angle of deviation may A vectograph consists of polaroid material on
increase sometimes. which the two targets are imprinted so that each
 84 Theory and Practice of Squint and Orthoptics

target is polarized at 90° with respect to the in each row, one of the animals correspondingly
other. The vectograph dissociates the eyes imaged in two eyes is printed heavily black
optically. With the use of properly oriented (serves as a misleading clue). The subject is
polaroid spectacles, each target is seen asked which one of the animals stands out. A
separately with the two eyes. subject without stereopsis will name the animal
printed heavily (misleading clue); while in the
Titmus stereo test presence of stereopsis he will name the
The Titmus stereo test utilizes the principle of disparately imaged animal.
vectograph. This is perheaps the most familiar 3. The circles test. It consists of nine squares,
stereo test. each containing four circles arranged in the form
The three-dimensional polaroid vectograph of a lozenge (Fig. 4.17). Only one of the circles
which constitutes the Titmus test is basically in each square is disparately imaged at random
made up of two plates in the form of a booklet with threshold ranging from 800 to 40 sec of arc.
(Fig. 4.17). To perform the test the plates are If the subject has passed other two tests, he is
reviewed with polaroid glasses. The Titmus asked to ‘push-down’ the circle that stands out,
stereo test consists of three parts: beginning with the first set. When he makes
1. The fly test. The right side of the test booklet mistakes or finds no circle to push down, the
contains a large housefly to test gross stereopsis limit of his stereopsis is presumably reached.
(threshold 3000 sec of arc). It is especially useful
Circle no. 5, equivalent to 100 sec of arc is
in young children. The subject is asked to pick
considered to be lowest limit of fine central
up one of the wings of the fly. If the subject sees
stereoacuity and is designated as the lowest limit
stereoscopically, he will reach above the plate.
of good stereoacuity.85
In the absence of gross stereopsis, the fly will
appear as an ordinary flat photogrpah (Fig. 4.18).  Advantages. The Titmus test is simple and
2. The animal test. It is performed, if the gross easy to perform and so is most widely used.
stereopsis is present. This test consists of three
rows of five animals each; one animal from each  Disadvantages
row is imaged disparately (thresholds 10, 200 1. Some of the circles of the Titmus test are
and 400 sec of arc, respectively) (Fig. 4.17). And, selected by even stereoblind observers, because

Fig. 4.17. The Titmus stereo test.

 Binocular Vision 85

Fig. 4.18. Titmus test using fly for gross stereopsis: A, No stereopsis; B, Stereopsis present.

they look ‘different’ and not because they are

seen stereoscopically.86
2. With the exception of the fine stereoacuity
circles 5 to 9, this test often is unreliable in
differentiating patients with amblyopia and
heterotropia from those with normal vision.87

III. Random dot stereogram tests

The random dot stereogram tests are devoid of
monocular clues and the patients cannot guess
what the stereo figure is and where it is located
on the test plate. So, this test provides truer
measurement of stereopsis than the Titmus
test.88
1. Random dot E-test (RDT). This test consists
of three cards to be viewed with polaroid
spectacles (Fig. 4.19).89 One card is a bas relief
model of the stereo test figure and is used to
show the patient for what he should look (A).
The second card contains the ‘E’ stereo figure
with a random dot background (B). The third
card is a stereoblank with an identical random
dot background (C).
To perform this test (after showing the bas
relief model), the two test cards are held 50 cm
in front of the patient, who is asked to indicate Fig. 4.19. Random dot E-test: A, Bas relief model of stereo
which card contains the letter ‘E’. The patient test figure; B, Random dot stereo figure seen without polaroid
glasses; C, Random dot stereo figure as seen in the polaroid
gives a ‘pass’ or ‘fail’ response. The stereoacuity, glasses—with stereopsis present; D and E, Stereo-blank
when present, can be quantitated by increasing card with random dot background as seen with (E) and
the testing distance from the patient. without (D) polaroid glasses.
 86 Theory and Practice of Squint and Orthoptics

2. TNO random dot test. The TNO random dot level of stereopsis. TNO test is available in two
stereo test is graded to provide retinal disparities versions one for adults and another for children.
ranging from 15 to 480 sec of arc. It is based on 3. Lang-test. This test consists of random dot
the same principle as ‘Random dot E-test’, but stereogram with panographic presentation.90,91
has the advantage of eliciting quantitative The stereoscopic images of a car, star and a cat
responses without changing the testing distance. (Fig. 4.21) embedded in random dots on the test
It consists of a booklet containing seven plates. card are seen disparately by each eye through
Each test plate consists of a stereogram in which the cylindrical lenses imprinted on the surface
various shapes (squares, dots, crosses) have lamination of the test (Fig. 4.22). Therefore,
been created by random dots in complementary polaroid glasses or red green spectacles are not
colours. The plates contain two types of figures, required in this test; so especially useful in
the one which can be perceived when viewed young children who refuse to wear glasses.
binocularly with red green spectacles by normal Lang test is available in two forms:
subject having stereopsis (Fig. 4.20B). The • Lang I test, which measures stereopsis at 550,
second set of figures can be seen with and 600 and 1200 seconds of arc and;
without the spectacles (Fig. 4.20A) even in the • Lang II test, which is finer and measures at 200,
absence of stereopsis. The first three stereograms 400 600 seconds of arc.
of the test booklet are used to establish the
To perform this test, the test card is held at a
presence of gross stereopsis quickly, while the
distance of 40 cm in front of the subject, who is
remaining four plates allow to quantitate the
asked to name or point to the shapes on the test
card. The disparity of the car and star is 600 sec
and of the cat 1200 sec of arc.92
4. Frisby test. In this test, stereogram consists of
three plastic cards each containing four squares
of small random shapes (Fig. 4.23). One of the
squares in each plate contains a hidden circle
which is seen disparately. The disparity is
created by displacement of random shapes by
the thickness of the plate. So, this test also does
not require use of glasses. Thus, it is especially
useful for young children who refuse to accept
glasses.

Fig. 4.20. Figures in TNO test: A, Cross is seen with and

without red-green spectacles; B, Four 'hidden shapes' which
can be seen with red-green spectacles in the presence of Fig. 4.21. Stereoscopic images embedded in random dots
stereopsis. of the Lang test stereogram.
 Binocular Vision 87

looking responses can be observed to establish

that stereopsis is present.
5. Stereoscopic contours induced optokinetic
nystagmus test87-88 and Television random dot
stereo test95 have recently been suggested to test
stereopsis in infants. Such electronically
generated stereopsis tests may become more
relevant in view of the current emphasis on early
diagnosis and treatment of strabismus in infants.

Fig. 4.22. Cylindrical gratings provide separate images for

each eye (From Lang).89

Fig. 4.23. The Frisby test.

Care must be taken to avoid monocular clues

through parallax movements when using the
test. The test can measure stereoacuity in the
range 600–15 seconds of arc. The Frisby test is
available in a screening version designed for
younger children and infants. It presents a three-
dimensional object field, together with a flat
image side by side in a preferential looking Fig. 4.24. Two-pencil test for stereopsis. For description see
format. In this case, a spontaneous pointing or text.
 88 Theory and Practice of Squint and Orthoptics

IV. Simple motor task test based on stereopsis 10. Burin H. Sensorial retinal relationship in concomi-
The two-pencil test tant strabismus. Tr Am. Ophth Soc. 43: 373, 1945.
11. Bagolini B. Anomalous correspondence:
It is very simple primitive but an effective test
definition and diagnostic methods, Doc.
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arc).92 Though known to even earlier ophthalmo- Hanover, 1940, Dartmauth college Publication.
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To perform this test, examiner holds a pencil Plantin, 1613.
vertically in front of the patient, who is asked to 14. Vieth GAU. Ueber die Richtung der Augen.
touch its upper tip with the tip of the pencil held Ann Phys 48:233–251, 1818.
in his hand by one swift movement from above 15. Muller J. vom Gesichtsinn. In Handbuch der
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eyes are open but stereopsis is absent (Fig. 4.24C). Engelman, 1864.
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 5
Strabismus: Definition,
Classification and Etiology

DEFINITION AND CLASSIFICATION • Vertical deviations

Definition • Cyclodeviations
• Orthoposition Symbols used for phorias and tropias
• Orthophoria
• Strabismus ETIOLOGY OF STRABISMUS
• Obstacles in the development of normal
Classification binocular vision and coordination
•Apparent squint – Sensory
•Latent squint – Motor
•Manifest squint – Central
• Role of heredity
Clinical types of ocular • Influence of age on the
deviations development of strabismus
• Esodeviations • Development of strabismus in a blind eye
• Exodeviations

DEFINITION AND CLASSIFICATION

DEFINITION
Normally visual axes of the two eyes are parallel
to each other in the primary positions of gaze
and this alignment is maintained in all positions
of gaze, except in convergence and divergence.
Perfect alignment of the two eyes is denoted by
the terms orthoposition and orthophoria.
Orthoposition may be defined as the position
of fusional demand in which both primary lines
of sight intersect at the fixation point (Fig. 5.1).
When fusion is interrupted by means such as
covering one eye, the eyes in a small number of Fig. 5.1 Diagram illustrating orthoposition—the position of
persons will remain in the orthoposition. Such fusional demand in which both primary lines of sight intersect
binocular co-operation in the absence of fusion at the fixation point.
is called orthophoria.
Orthophoria, thus, may be defined as a position other distances, an adjustment of the relative
of the eyes in which the primary lines of sight position of the eyes by the fusion reflex is
intersect at the fixation point, without any fusion necessary to obtain bifoveal fixation.
adjustment being necessary to attain or maintain Strabismus or squint or simply deviation of the
this position. A person may be orthophoric only eye refers to a misalignment of the visual axes
for one particular fixation distance, whereas at of the two eyes. The term strabismus is derived
91
 92 Theory and Practice of Squint and Orthoptics

from the Greek word strabismos (to look Apparent squint or pseudostrabismus
obliquely or askance). The term squint comes It refers to certain conditions in which visual
from the fact that strabismic patients often close axes of the two eyes are in fact parallel in all
one eye or squint to block out one image. positions of gaze and actually there exists a
Practically, the term eye deviation describes a normal bifoveal vision; but the eyes apparently
deviation from the orthoposition. Thus strabismus seem to have a squint.
refers to misalignment of eyes in which the fovea
1. Pseudoesotropia or apparent convergent
of one eye is aligned with the fixation target and
squint may be associated with:
the fovea of the fellow eye is off the target and the
• A prominent epicanthal fold, which covers the
image of fixation target is formed at a point nasal
normally visible nasal aspect of the globe and
to fovea in esotropia (Fig. 5.2A) and temporal to
gives a false impression of esotropia (Fig. 5.3).
fovea in exotropia (Fig. 5.2B).
• A negative angle kappa
CLASSIFICATION • A too small interpupillary distance.
Strabismus has been variously classified. • An excessively broad nasal bridge
However, no classification is perfect or all 2. Pseudoexotropia or an apparent divergent
inclusive. Broadly, strabismus can be classified squint may be associated with:
into latent and menifest strabismus.
• Hypertelorism, a condition of wide separation
of two eyes.
• A positive angle kappa.
• An excessively narrow nasal bridge.
• A too large interpupillary distance.
• Narrowing of lateral canthi (Fig. 5.4)
3. Pseudohypertropia. Pseudohypertropia or
apparent hypertropia may be associated with:

Fig. 5.3 Pseudoesotropia in a child with prominent epican-

thal fold (A), Corrected by pinching the skin fold (B)
(Courtesy: Dr Kalpna and Dr. Sandra).

Fig. 5.2 Diagram illustrating formation of image in tropia:

A, nasal to fovea in esotropia and; B, temporal to fovea in Fig. 5.4 Pseudoexotropia in a child with marked narrowing
exotropia. of lateral canthus.
 Strabismus: Definition, Classification and Etiology 93

• Orbital dystopia eye used to fixate. Incomitance may be caused

• Anterior segment anomalies by innervational, mechanical or restrictive factors.
• Vertical angle kappa (e.g. due to a displaced It includes the following conditions:
fovea secondary to retinopathy of prematurity • 'A' and 'V' pattern heterotropias
or other causes of retinal dragging). • Special ocular motility defects such as Duane's
Other conditions in which an eye may look to retraction syndrome.
have an apparent strabismus are: • Paralytic squint
• Marked facial asymmetry
(B) Depending upon the direction of deviation
• Enophthalmos
(Fig. 5.5)
• Exophthalmos
1. Esotropia or convergent squint. It denotes
Latent squint (heterophoria) inward deviation of the globe (Fig. 5.5A).
Heterophoria, the term introduced by Stevens, 2. Exotropia or divergent squint. It implies
refers to a condition in which the tendency of outward deviation of the eyeball (Fig. 5.5B).
the eyes to deviate is overcome (kept latent) by 3. Right hypertropia. The right eye is elevated
the fusion reflex during binocular vision. relative to the left (any of the eye may be fixing
Therefore, when the influence of fusion is eye) (Fig. 5.5C).
interrupted, the visual axis of the non-fixing eye 4. Left hypertropia. The left eye is elevated
deviates from the orthoposition. relative to right (any of the eye may be fixing)
Depending upon the direction of deviation, (Fig. 5.5D).
heterophoria may be classified into: 5. Incyclotropia. The deviated eye is intorted,
• Esophoria i.e. 12 O'clock meridian of the cornea is rotated
inward (Fig. 5.5E).
• Exophoria
6. Excyclotropia . The deviated eye is extorted,
• Right hyperphoria
i.e. 12 O'clock meridian of the cornea is rotated
• Left hyperphoria outward (Fig. 5.5F).
• Incyclophoria
• Excyclophoria (C) Depending upon the constancy of deviation
1. Constant squint. When the deviation remains
Manifest squint (heterotropia) constant in all directions of gaze, at all times and
Heterotropia is a manifest deviation of the eyes at all distances of fixation.
from the orthoposition that cannot be overcome 2. Intermittent squint. When the deviation may
by the fusion reflex. be present sometimes and absent at other times,
Heterotropia can be variously classified as under similar conditions of vision and fixation
follows. distances. It may develop in conditions of stress
and strain and also in patients with decom-
(A) Depending upon concomitance of deviation pensated heterophoria.
1. Comitant strabismus. It is a type of manifest 3. Periodic squint. It appears repeatedly under
squint in which the angle of squint remains the same set of conditions, e.g. a squint may
constant (unaltered) in all directions of gaze with always be present for a particular distance of
either eye fixating. fixation and absent for others. This term is not
The term concomitant is considered syno- commonly used and such patients are also
nymous with the comitant. However, the recent labelled as having intermittent squint.
literature prefers the term comitant over the
concomitant. (D) Depending upon fixation behaviour
2. Incomitant strabismus. It is a type of 1. Unilateral squint. The same eye always
heterotropia in which the angle of deviation deviates and the second normal eye fixates
varies with the direction of gaze and/or with the (Fig. 5.6A).
 94 Theory and Practice of Squint and Orthoptics

Fig. 5.6 A patient with primary alternate divergent squint

(ADS): A, Right exotropia while fixing with left eye; and B, left
exotropia while fixing with right eye.

(F) Depending upon the etiology

Depending upon the etiology/causative
mechanism, following terms are in vogue:
A. Paralytic strabismus, in which one or more
of the extraocular muscles are paralysed.
B. Non-paralytic strabismus. Depending upon
the etiology, non-paralytic strabismus may be
of following types.
1. Accommodative heterotropia. In it, act of
accommodation has a major influence on the
Fig. 5.5 Diagrammatic depiction of types of strabismus deviation.
depending upon the direction of deviation. A, left exotropia 2. Non-accommodative heterotropia. In it,
(LXT); B, left esotropia (LET); C, left hypertropia (LHT); accommodation has not much role to play.
D, left hypotropia or right hypertropia (RHT); E, left
incyclotropia and; F, left excyclotropia. 3. Primary heterotropia. Wherein no obvious
cause is found. Duane5 suggested following
2. Alternating squint. Either of the two eyes can terms:
deviate and the other eye fixates (Fig. 5.6B). • Convergence excess type of esodeviation
3. Bilateral squint. In it, both eyes are squinting which is more for near than distance fixation.
simultaneously. It is seen very rarely. • Divergence insufficiency type of
(E) Depending upon the time of onset
esodeviation which is more for distance
fixation than the near.
1. Congenital strabismus. A deviation that is
• Convergence insufficient type of
present at birth or appears in first a few months
esodeviation which is more for near fixation
of life. In the recent literature, the term congenital
than the distance.
strabismus has been almost replaced or is used
synonymously with the infantile strabismus, which • Divergence excess type of exodeviation
includes all forms of deviations with an onset which is more for distance fixation than near.
during first 6 months of life. 4. Secondary/sensory heterotropia is the term
2. Acquired strabismus. A deviation that used to describe a deviation which results
develops after 6 months of life. from some known cause of visual deprivation/
 Strabismus: Definition, Classification and Etiology 95

sight impairing disease of one eye, e.g. 2. A- and V-pattern esotropias

anisometropia, central chorioretinitis, etc. 3. Special types of restrictive esotropia
5. Consecutive heterotropia is the term used to i. Duane's retraction syndrome (with
describe the deviation resulting from surgical esotropia)
overcorrection or spontaneous conversion of ii. Thyroid myopathy
an esotropic eye into an exodeviation. iii. Medial orbital wall fracture
iv. Strabismus fixsus
CLINICAL TYPES OF OCULAR DEVIATIONS v. Excessively resected medial rectus muscle
In day-to-day clinical practice, the ocular
EXODEVIATIONS
deviations are classified as below.
A. Exophoria (X)
ESODEVIATIONS B. Intermittent exotropia (X [T])
C. Exotropia (XT)
A. Esophoria (E or S)
B. Intermittent esotropia (E [T] or S [T]) I. Concomitant exotropias
C. Esotropia (ET or ST) 1. Primary exotropias
i. Divergence excess type
I. Concomitant esotropia ii. Convergence insufficiency type
1. Infantile (congenital) esotropia iii. Basic exotropia
2. Accommodative esotropia iv. Pseudodivergence excess type
i. Refractive (normal AC/A ratio) 2. Sensory exotropia
ii. Non-refractive accommodative 3. Consecutive exotropia
– Hyperaccommodative (high AC/A ratio) II. Incomitant exotropias
– Hypoaccommodative 1. Paralytic exotropias
(weak accommodation) i. Isolated medial rectus paresis
iii. Mixed or partially accommodative ii. Complete third nerve paralysis
3. Non-accommodative esotropia iii. Paralysis of convergence
i. Acquired or late onset non-accommo- 2. A-, V- and X-pattern exotropias
dative esotropia 3. Special types of restrictive exotropias
– Basic esotropia i. Duane's retraction syndrome (with exotropia)
– Convergence excess type ii. Craniofacial anomalies with divergent orbit.
iii. Thyroid myopathy
– Divergence insufficiency type
iv. Restriction due to trauma or excessive surgery
ii. Acute concomitant esotropia
iii. Microtropia VERTICAL DEVIATIONS
iv. Nystagmus blockage syndrome A. Hyperphoria (H)
v. Cyclic esotropia B. Intermittent hypertropia [H (T)]
vi. Stress-induced esotropia C. Hypertropia (HT)
vii. Esotropia in myopia
I. Comitant hypertropia
viii. Esotropia due to spasm of near reflex 1. Induced (refractive)
4. Sensory esotropias 2. End result of long-standing paralytic
5. Consecutive esotropia deviation.
II. Incomitant esotropia II. Incomitant vertical deviations
1. Paralytic esotropia 1. Apparent oblique muscle dysfunction
i. Lateral rectus paralysis i. Inferior oblique overaction (strabismus
ii. Divergence paralysis sursoadductorious) now termed as over
iii. Mobius syndrome elevation in adduction (OEA).
96 Theory and Practice of Squint and Orthoptics

• Primary OEA, or ii. Iatrogenic displacement of inferior oblique

• Secondary OEA. muscle after its anteriorization.
ii. Inferior oblique underaction, now termed b. Restrictive vertical deviations due to
as under-elevation in adduction (UEA). mechanical restrictions
• Primary UEA, or i. Tight extraocular muscles
• Seconday UEA ii. Restrictive vertical deviation due to
iii. Superior oblique overaction (strabismus structural adhesions (induced adhesive
deorsadductorious), now termed as over syndromes)
depression in adduction (ODA). iii. Restrictive vertical deviation due to orbital
• Primary ODA, or mass lesions.
• Seconday ODA. III. Dissociated vertical deviations (DVD)
iv. Superior oblique underaction is now 1. Monocular DVD
termed as under-depresson in adduction 2. Binocular or alternating DVD
(UDA).
• Primary UDA, or CYCLODEVIATIONS
• Secondary UDA. A. Cyclophorias
2. Paretic vertical deviations 1. Incyclophoria
• Congenital unilateral superior oblique 2. Excyclophoria
paresis.
B. Intermittent cyclotropias
• Non-congenital superior oblique paresis. 1. Intermittent incyclotropia
• Bilateral superior oblique paresis. 2. Intermittent excyclotropia
• Monocular elevation deficiency (MED),
C. Cyclotropias
(old name: Double elevator palsy).
1. Incyclotropia
• Monocular depression deficiency (MDD);
i. Inferior oblique paralysis
(old name: Double depressor palsy).
ii. Inferior rectus paralysis
• Superior rectus paresis (isolated).
2. Excyclotropias
• Inferior rectus paresis (isolated).
i. Superior oblique paralysis
• Skew deviation.
ii. Superior rectus paralysis
3. Restrictive vertical deviations
a. Restrictive vertical deviations due to SYMBOLS USED FOR PHORIAS AND TROPIAS
misdirected muscle force In medical charts and orthoptic record, the
i. Congenital cranial dysinnervation deviations are usually indicated by the symbols.
disorders (CCDDs) primarily affecting Though, variations may exist between different
vertical ocular motility: squint clinics, the symbols listed in Table 5.1 are
• Congenital fibrosis of extraocular fairly uniformly used as abbreviations for
muscles (CFEOMs) different types of deviations.

Table 5.1 Symbols used for ocular deviations

Phoria Tropia
Deviation Distance Near Distance Near
Constant Intermittent Constant Intermittent
1 1
Eso E E ET E (T) ET E(T)1
Exo X X1 XT X(T) XT1 X(T)1
1 1
Right hyper RH RH RHT RH(T) RHT RH(T)1
Left hyper LH LH1 LHT LH(T) LHT1 LH(T)1
 Strabismus: Definition, Classification and Etiology 97

ETIOLOGY OF STRABISMUS: • Binocular similar images (in size and sharpness)

should be formed so that fusion can occur.
AN OVERVIEW
• Normal visual pathway from retinal receptors
Etiology of strabismus varies in different to visual centres in the brain is required for a
clinical varities and has been described there normal visual perception.
and then. Further, etiology of strabismus is still • Brain centres must fuse the input from both eyes
illusive or only presumptive in many cases. As into a unified single perception.
a general concept, causes of acquired paralytic • The neural pathway from the various oculo-
strabismus (which is usually acute in onset) are motor centres to the extraocular muscles must
comparatively better defined and different function properly.
from the causes of non-paralytic types of • The extraocular muscle function should be
strabismus, and are described (page 285). normal and symmetrical in two eyes.
In general terms, the development of a non- • The binocular co-ordination of eye movement must
paralytic strabismus is the result of an abnormality be such that the fixation object is imaged in
of one or more of the many factors which are the centre of each fovea.
concerned in the establishment of normal • Retinal correspondence must be normal.
binocular vision. As we know, the binocular vision Thus, malfunction of any of the above factors
and coordination of ocular movements are not can be an obstacle for the development of normal
present since birth but are acquired in the early binocular vision and binocular co-ordination
childhood. The process starts after birth and is, by resulting in strabismus. These obstacles can be
and large, completed by the age of 5–6 years. arranged into three groups namely: Sensory,
Therefore, any obstacle to the development of motor and central.
these processes may result in concomitant
strabismus. Basically, there is no difference 1. Sensory obstacles
between the causes leading to a heterophoria and
These are the factors which hinder the formation
those resulting in a heterotropia. Rather, it is a
of a clear image in one eye and include the
matter of degree to which the interference disrupts
following:
a given function. If the interference is minor, the
• Uncorrected refractive errors
resulting defect may be compensated during
binocular vision so that only a latent deviation • Prolonged use of incorrect spectacles
(heterophoria) results. If it is more severe, a • Anisometropia
manifest strabismus may be the result. • Opacities in the media such as corneal
The general etiological factors which play role opacities, lenticular opacities and vitreous
in the development of strabismus can be opacities
discussed as under: • Prolonged covering of one eye with a bandage
• Obstacles in the development of normal or eye shade
binocular vision and co-ordination. • Severe unilateral congenital ptosis
• Role of heredity. • Prolonged use of one eye as with watch-
• Influence of age on the development of maker's eye-glass
strabismus. • Diseases of macula, e.g. central chorioretinitis.
• Development of squint in a blind eye. • Optic atrophy

OBSTACLES IN THE DEVELOPMENT OF NORMAL 2. Motor obstacles

BINOCULAR VISION AND CO-ORDINATION
These factors hinder the maintenance of the two
Prerequisites for development of normal eyes in the correct positional relationship in
binocular vision and binocular coordination primary gaze and/or during different ocular
include the following: movements. A few such factors are:
• Normal optical media to produce an image on • Congenital abnormalities of the size and shape
the retina of each eye. of orbit.
 98 Theory and Practice of Squint and Orthoptics

• Abnormalities of extraocular muscles such as even small obstacles which mitigate against the
faulty insertion, faulty innervation, mild fulfilment of full binocular function, whereas in
paresis, underdevelopment, overdevelop- an older patient, the more mature binocular
ment and muscle slips. reflexes may be able to overcome quite
• Abnormalities of fascial structures such as formidable obstacles. Chavasse stated that a
check ligaments, intermuscular membranes, very slight and transient paresis in adult patients
connections between the fascial muscle sleeves with well-developed binocular vision may cause
where two muscles cross, etc. may also lead only heterophoria but in an infant it may be
to deviation. sufficient to severe the slighter bonds which
• Abnormalities of accommodation, conver- associate the two eyes.
gence and AC/A ratio play an important role
in ocular deviations. DEVELOPMENT OF STRABISMUS IN A BLIND EYE
It has been reported that if the loss of vision in
3. Central obstacles one eye occurs in the first a few months of life,
These may be in the form of: there may occur, sensory esotropia or exotropia,
• Deficient development of fusion faculty. and after this till childhood, usually there occurs
• Abnormalities of cortical control of ocular sensory esotropia. Occurrence of poor vision in
movements as occur in mental trauma, and adulthood due to any reason tends to cause a
hyperexcitability of the central nervous sensory exotropia. However, Sidkaro and von
system during teething. Noorden have reported that:
• Purposive use of the convergence reflex • Up to 5 years of age, there are almost equal
initiated in the motor area of frontal cortex chances of getting sensory esotropia and
may also play a role in the development of exotropia.
strabismus. This occurs classically in the • After 5 years, definitely there are more chances
voluntary squint which may be produced as of developing sensory exotropia.
a temporary phenomenon in most normal Note. The exact mechanism of development of
individuals; but in children it may be more sensory heterotropia is not known, however, some
sustained often as a result of some psycho- views have been put forward (see page 238).
logical upset.

ROLE OF HEREDITY BIBLIOGRAPHY

It is unequivocal that the incidence of strabismus 1. Bielschowsky A: Lectures on motor anomalies.
is higher in certain families than in general Hanover NH, 1943 (reprinted 1956). Dartmouth
population and that heredity plays a definite College Publications.
role in the occurrence of this disorder. However, 2. Bredemeyer, HG, and Bullock K: Orthoptics:
theory and practice, St Louis. 1968. Mosby - Year
this role cannot be defined as a distinct genetic
Book Inc, p.86.
pattern of strabismus; but certain defects such
3. Broendstrup, P: The squinting position of weak-
as a refractive error, usually a high degree of sighted eyes, Acta Ophthalmol 20:386, 1944.
hypermetropia, which in turn may cause 4. Chavasse, FB: Worth's squint or the binocular
strabismus are genetically transmitted. reflexes and the treatment of strabismus, ed. 7,
Philadelphia, 1939, P Blakiston's Sons & Co., Inc.
INFLUENCE OF AGE ON THE 5. Duane, A: A new classification of the motor
DEVELOPMENT OF STRABISMUS anomalies of the eyes based upon physiological
principles together with their symptoms,
The age of the patient, at the time, when the
diagnosis and treatment, reprinted from Ann.
strabismogenic factors become operative, plays Ophthalmol Otolaryngol, October, 1896, and
an important role in the determination of the edited by White, JW, nd.
strabismus. In a younger patient, the immature 6. Keiner, GBJ: New viewpoints on the origin of
binocular reflex may be unable to withstand squint. The Hague, 1951, Martinus Nijoff's ND.
 Strabismus: Definition, Classification and Etiology 99

7. Lancaster, WB: "Terminology," with extended 9. Paul, TO, and Hardage, LK: The heritability of
comments on the position of rest and fixation. In strabismus, Ophthalmic Genet. 15:1, 1994.
Allen, JH, editor: Strabismus ophthalmic symposium
10. Scobee, RG: Anatomic factors in the etiology of
I, St. Louis, 1950, Mosby -Year Book, Inc.
heterotropia, Am. J. Ophthalmol. 31:781, 1948.
8. Lyle, TK, and Wybar, KC: Lyle and Jackson's
practical orthoptics in the treatment of squint (and 11. Worth, C: Squint, its causes, pathology and
other anomalies of binocular vision), ed. 5, treatment, ed. 6, London, 1929, Bailliere, Tindall
Springfield, III, 1967, Charles C Thomas, Publisher. and Cox.
 6
Evaluation of a Case of Strabismus
and Orthoptic Instruments including
Computer-Based Orthoptic Programs
EVALUATION OF A CASE OF STRABISMUS • Fixation disparity method
History, vision evaluation and preliminary • Haploscopic methods
examination Assessment for extraocular muscle paresis
History • Diplopia charting
Vision evaluation • Quantitative measurement of action of
Prelimary examination extraocular muscles
• Inspection – Lancaster red-green test
• Pupillary reactions – Hess screen test
• Media and fundus examination – Lees screen test
• Refraction • Field of binocular fixation
Motor evaluation • Bielschowsky phenomenon test
Cover tests • Bielschowsky head tilt test
• Direct cover test Sensory evaluation: Assessment for
• Cover uncover test binocular cooperation and sensory
• Alternate cover test anomalies
Quantitative measurement of angle of deviation • Tests for fixation behaviour
• Bruckner pupillary red reflex test • Tests for the state of retinal correspondence
• Hirschberg corneal reflex test • Tests for binocular vision
• Prism and alternate cover test • Tests for suppression and amblyopia
• Prism reflex test (Krimsky's test) ORTHOPTIC INSTRUMENTS
• Perimeter method Conventional Orthoptic Instruments
• Maddox rod test • Synoptophore
• Synoptophore method • Livingston binocular gauge
• Double prism test • Visuscope
• Haploscopic test
• Euthyscope
Assessment of ocular movements Computer Based Orthoptic Programs
• Ductions • Computerised orthoptic diagnostic
• Versions programs
• Vergences • Computer based vision therapy and
Assessment of accommodation and neurovision therapy programs
AC/A ratio • Computer based combined diagnostic and
• Heterophoria method therapeutic programs
• Gradient method

EVALUATION OF of tests to be performed in a particular

patient.
A CASE OF STRABISMUS
Main components of evaluation of a case of
To avoid repetition, the subject of evaluation strabismus are:
is discussed even before the clinical I. History, vision evaluation and prelimary
description of different varieties of the examination
squint. Though the list of tests is exhaustive, • History
all of these may not be required in every case. • Vision evaluation
An experienced strabismologist chooses a set • Prelimary examination
100
 Evaluation of a Case of Strabismus and Orthoptic Instruments 101

II. Motor evaluation • Abnormal head posture (ask to demonstrate).

• Head posture evaluation • Associated symptoms such as diplopia,
• Evaluation for ocualr deviation blurred vision, headache, asthenopia.
– Cover tests • Any histroy of closing or covering one eye
– Corneal reflex tests in bright light indicates intermittent squint.
– Subjective tests for measurement of deviation 3. Birth history. It is specially important in cases
– Measurement for cyclodeviation of childhood onset deviations. Enquiry should
• Assessment for ocular movements be made regarding:
– Evaluation of duction • Problems during pregnancy and
– Evaluation of version • Problems during delivery
– Evaluation of vergence
4. Family history. History regarding any of the
• Assessment of accommodation, and AC/A
following disorders in grandparents, parents,
ratio
siblings or even in uncles, aunts and cousins is
• Assessment for extraocular muscle paresis
important;
III. Sensory evaluation
• Tests for fusion, • Strabismus
• Tests for binocularity and diplopia • Refractive errors
• Tests for fixation • Lazy eye
• Tests for status of retinal correspondance • Other ocular defects
• Tests for suppression and amblyopia 5. Past history
• Tests for stereopsis. • General ill health
In general, for a systematic evaluation, • Any systemic disease
following approach may be adopted. • Any ocular defect
6. History of previous treatment
HISTORY, VISION EVALUATION AND a. Optical treatment
PRELIMINARY EXAMINATION
• When and how long?
HISTORY • Were glasses worn constantly?
• Effect of glasses on the deviation.
A detailed and meticulous history taking before
beginning the examination is very important in • Date of last refraction.
the management of a case of squint. A complete b. Orthoptic treatment.
history taken should contain the following • Occlusion. Type, when, how long, effect
information. on vision.
1. Age of the patient • Exercises. When, type, how long, effect
2. History of present illness. Information about on deviation.
deviation of eye should include: c. Surgical treatment
• Time when first noticed. It is desirable to • When performed?
document the age of onset of deviation or • Eye operated: Right, left or both.
symptoms. All available old photographs of • Muscle touched. Medial, lateral or both
the child are invaluable for this purpose. (if known).
• Patient's/parents' opinion about the result
• Onset, sudden or gradual.
of surgery.
• Constant or intermittent deviation.
• Unilateral or alternating deviation. VISION EVALUATION
• If alternating, which eye more frequently Testing of visual acuity of each eye separately
fixates (dominating eye). is critical in evaluation of any patient with
• Alleged cause, if any, such as trauma, illness, strabismus. Visual acuity should be tested
psychologic disturbance, change in occupation, without glasses and with glasses (if worn); and
increase in close work, etc. for near and distance vision.
 102 Theory and Practice of Squint and Orthoptics

Methods of testing visual acuity in infants, observation should be made for all the three
preschool age children and in schoolage children components of head posture, i.e.
as well as adults have been described in • Chin elevation or depression (vertical
Chapter 3 (page 40–51). However, for a ready component),
reference, these are enumerated below. • Face turn to right or left (horizontal
Methods of estimating visual acuity in infants component), and
1. Fixation behaviour test • Head tilt to right or left shoulder (torsional
2. Preferential looking test component)
3. Optokinetic nystagmus Interpupillary distance (IPD) should also be
4. Visually evoked response inspected and measured. Unusually, narrow
5. CSM method IPD may be the cause of pseudoesotropia and
Methods of estimating visual acuity in an exceptionally wide IPD may be a cause of
preschool-age children pseudoexotropia.
1. Marble game test Inspection during pen light examination may
2. Hand chart sometimes erroneously reveal strabismus owing
3. Illiterate E-game test to presence of a large angle kappa. A large
4. Allen's preschool vision test positive angle kappa may be a cause of
5. Sheridan-Gardiner test pseudoexotropia and a large negative angle
Methods of estimating visual acuity in kappa may be a cause of pseudoesotropia. For
schoolage children and adults details of angle kappa and its measurement, see
1. Snellen's test types page 188.
2. E-chart
Pupillary Reactions
3. Landolt's broken-C chart
Light reflexes may be abnormal in patients with
Visual acuity in patients with nystagmus. In
sensory deviations due to diseases of retina and
patients with nystagmus, visual acuity may be
optic nerve.
better with both eyes open than with one eye
occluded. In order to assess this situation, it may
be helpful to place a +5.00D sphere lens in front Media and Fundus Examination
of the eye not being tested, and then reverse this It may reveal associated diseases of the ocular
in order to test the other eye. media, retina or optic nerve.

PRELIMINARY EXAMINATION Refraction

Inspection It is most important, because a refractive error
may be responsible for the symptoms of the
Large degree squint (convergent or divergent)
patient or for the deviation itself. Though,
is obvious on inspection.
mentioned in the beginning, in practice, refraction
Epicanthus, when observed in infants, may be is performed after complete squint check-up.
the cause of pseudoesotropia. Preferably, refraction should be performed under
Facial asymmetries may also create the full cycloplegia especially in children. The
impression of pseudostrabismus especially commonly used cycloplegics are as follows:
hypertropia. 1. Atropine is indicated in children below the
Abnormal head posture (AHP) when present, age of 7 years. It is used as 1% ointment thrice
becomes obvious during initial inspection of the daily for 3 consecutive days before performing
patient. Infact observation for AHP should retinoscopy. Its effect lasts for 10–20 days.
always be made without any instructions to the 2. Homatropine is used as 2% drops. One drop
patient, otherwise a lot of information may be is often instilled every 10 minutes for 6 times
lost after the patient becomes concious of being and the retinoscopy is performed after 1 to
examined. As described in detail on page 288, 2 hours. Its effect lasts for 24–28 hours. It is used
 Evaluation of a Case of Strabismus and Orthoptic Instruments 103

for most of the hypermetropic individuals

between 7 and 35 years of age.
3. Cyclopentolate is a short-acting cycloplegic.
Its effects last for 6 to 18 hours. It is used as 1%
eyedrops in patients between 7 and 35 years.
One drop of cyclopentolate is instilled every 10
minutes for 3 times (Havener's recommended
dose) and the retinoscopy is performed 1 to 1½
hours later after estimating the residual
accommodation, which should not exceed one
dioptre.

MOTOR EVALUATION Fig. 6.1 Direct cover test depicting left exotropia.

EVALUATION FOR OCULAR DEVIATION

No movement of the uncovered eye on covering the
COVER TESTS seemingly fixating eye indicates any of the
Prerequisites for cover tests following:
• Patient should be co-operative enough to • No squint (pseudostrabismus)
fixate a target. • Gross eccentric fixation (there may be very
small or no movement of redress in the
• Should have sufficient vision to see the target.
uncovered deviated eye)
• Should have central fixation in both eyes.
• No vision in the deviated uncovered eye.
• Latent nystagmus should not be present.
Cover-uncover test
Methods of cover tests Aim. To establish the presence and type of heterophoria
The cover tests should be performed with and (latent deviation).
without glasses at distance (6 m) and at near Procedure (Fig. 6.2). It is performed, when direct
(33 cm). The patient is asked to fixate a 6/12 cover test has established that no manifest
visual acuity symbol or any object that keeps deviation is present. To perform it, one eye is
patient's attention, such as a small picture or a covered with an occluder and the other is made
toy (especially in small children), with one eye.
This is to prevent the use of accommodation,
when torch light is used (which is a common
practice). The other eye is then covered with the
help of an occluder or palm of the hand. The
interpretations are made as described below.

Direct cover test

Aim. To confirm the presence of a manifest squint.
Procedure. To perform this test, patient is first
asked to fixate (Fig. 6.1) a point with both eyes
open. The normal looking eye is covered while
observing the movement of the uncovered eye.
In the presence of manifest squint, the
uncovered eye will move in the opposite
direction to take fixation. For example, when an
exotropia is present, the eye taking up fixation Fig. 6.2 Cover-uncover test depicting exophoria. Note or-
will move towards nose and in the presence of thophoria in primary position (A) and immediate inward
esotropia, it will move towards the temple. movement of right eye on removal of the cover (B and C).
 104 Theory and Practice of Squint and Orthoptics

to fixate an object. In the presence of hetero- In the presence of an alternate squint, either
phoria, the eye under cover will deviate (since eye fixates and the opposite eye under cover
covering one eye of a patient with normal deviates and maintains the position of deviation
binocular vision interrupts fusion). After a few on removing the cover; but in the presence of a
seconds, the cover is quickly removed and the unilateral squint, after removal of the cover,
movement of the eye (which was under cover) always the normal looking eye takes up fixation
is observed. Direction of the movement of the and the squinting eye deviates.
eyeball tells the type of heterophoria (e.g. the To differentiate concomitant squint from
eyeball will move towards the nose in the paralytic squint, observation about the degree
presence of exophoria and towards the temple of deviation in the eye under cover is made while
in the presence of esophoria) and the speed of performing the alternate cover test. In concomi-
movement tells whether recovery is slow or tant squint, primary deviation is equal to
rapid. secondary deviation (deviation of the normal
eye under cover); while in paralytic squint,
Alternate cover test secondary deviation is much more than the
Aim. To establish whether the squint is unilateral primary deviation.
or alternate and also to differentiate concomitant
squint from paralytic squint (where secondary Limitations of cover tests
deviation is greater than primary deviation). Following deviations may be either overlooked
Procedure (Fig. 6.3). To perform this test, patient or cannot possibly be diagnosed with cover tests:
is asked to fixate an object alternately with each • A small heterophoria
eye. It is important to place occluder alternately • A small angle esotropia (of less than 5Δ)
in front of each eye several times to dissociate • A microtropia
the eyes and to maximize the deviation. Further, • A monofixation syndrome
the occluder should be quickly transferred from • A cyclodeviation
one eye to other to prevent fusion from
occurring. Observations made are as follows: QUANTITATIVE MEASUREMENT
OF ANGLE OF DEVIATION
Various methods for quantitative measurement
of angle of deviation can be grouped as under:
Objective tests for heterotropia
• Hirschberg corneal reflex test
• Prism and cover test (prism bar cover test)
• Prism reflex test (Krimsky's corneal reflex test)
• Synoptophore test
• Perimeter method
Subjective tests for heterotropia
• Maddox rod test
• Synoptophore test
• Diplopia test
• Hess/Lees screen test
Objective tests for heterophoria
• Prism and cover test
Subjective tests for heterophoria
• Maddox rod test
• Maddox wing test
Fig. 6.3 Alternate cover test depicting alternate exotropia. • Double prism test
 Evaluation of a Case of Strabismus and Orthoptic Instruments 105

Tests based on corneal reflex

• Hirschberg corneal reflex test
• Prism and cover test (prism bar cover test)
• Prism reflex test (Krimsky's corneal reflex test)
• Perimeter method
• Synoptophore method
• Bruckner test
Dissimilar image tests
• Maddox rod test for heterophoria or hetero-
tropia
• Double Maddox rod test for cyclodeviations
• Red glass test
Dissimilar target tests
• Major amblyoscope test
• Lancaster red-green projection test
• Hess screen test

Bruckner pupillary red reflex test

This is a screening test and does not measure
the size of deviation. It is performed with the
help of a direct ophthalmoscope. Patient is made
to look into the light and examiner compares
the brightness of pupillary red reflex of both eyes
obtained simultaneously,
• In orthophoria, the Bruckner red reflex is
symmetric in the two eyes.
• In strabismus, the reflex is brighter in the
deviated eye.
• In ocular pathologic conditions such as large
anisometropia, gross retinal pathology, large
retinal detachment, media opacities (corneal,
lenticular or vitreal), the red reflex is altered
in the affected eye. Fig. 6.4 Hirschberg corneal reflex test depicting ortho-
position (A), right exotropia (B:15°, C:30°, D:45°) and right
esotropia (E:15°, F:30°, G:45°).
Hirschberg corneal reflex test
It is a rough but handy method to estimate the Prism and alternate cover test
angle of manifest squint. In this test, patient is (prism bar cover test, i.e. PBCT)
asked to fixate at a point light held at a distance
In practice, this test is most popular and a simple
of 33 cm and the deviation of corneal reflex from
method of measuring the angle of deviation
the centre of pupil is noted in the squinting eye.
objectively in various diagnostic positions of
Hirschberg reported that each 1 mm decent-
gaze.
ration of corneal reflex corresponds to 7° (14Δ)
of deviation of the visual axis. Thus, roughly the Prisms of increasing strength with apex towards
angle of squint is 15° and 45°, when the corneal the deviation are placed in front of one eye and
light reflex falls on the border of pupil and the patient is asked to fixate a target with the
limbus, respectively (Fig. 6.4). other eye. For large deviations, a loose prism of
 106 Theory and Practice of Squint and Orthoptics

Fig. 6.5 Prism and cover test with its optical principle. A, right esotropia; B, image of the object fixated by left eye is projected
on major half of retina of the right eye; C and D, when left eye is covered, right eye moves outwards to take over fixation
and under the cover left eye performs an inward movement of equal amplitude following Hering's law of equal innervation;
E and F, when cover is transferred to right eye, the left eye moves outwards to takeover fixation and under the cover right
eye performs an inward movement; G and H, a prism base-out is held before the right eye and cover is transferred to the
left eye. There is still outward movement of the right eye when taking over fixation; I and J, cover is again transferred and
a prism of greater power is held before the right eye; K and L, transfer of cover to the left eye does not show any outward
movement of the right eye indicating that it is the end point of the prism and cover test. At this juncture, prism of sufficient
power offsets the nasal displacement and the right eye will no longer change its position when left eye is covered. The power
of this prism equals the deviation.

30 or 45 prism dioptre is placed in front of one • Alternate cover test is then performed till there
eye and the prism bar is used in front of the other is no recovery movement of the eye under
eye. cover (Fig. 6.5). This will tell the amount of
• When horizontal and vertical deviations co-exist, deviation in prism dioptres.
the prisms are placed horizontally in front of Both heterophoria as well as heterotropia can
one eye and vertically in front of the other eye. be measured objectively by this method.
 Evaluation of a Case of Strabismus and Orthoptic Instruments 107

Test should be performed for distance and near Uses of prism and cover test
fixation. An accommodative target should be A carefully performed PBCT can provide
used for near fixation distances. following useful information:
Test should be performed, preferably in all the 1. Nature of deviation of esotropia (basic or
nine diagnostic positions of gaze. In Boyce- convergence excess or divergence insufficiency
Smith deviometer (Fig. 6.6), nine prefixed type) and exotropia (basic or convergence
retroilluminated slides (accommodative targets) insufficiency or divergence excess type) can be
positioned 35° from the primary position are known from the results of near and distance
used to perform cover and prism test in measurements.
diagnostic positions of gaze. 2. Accommodative element of deviation can be
One great advantage of using a deviometer is known from measuring deviation for far and
that the prism and cover test can be performed near with and without glasses (including
in diagnostic positions under exactly the same bifocals, if any).
conditions on different occasions and thus 3. Incomitance, if any, can be detected by
permit meaningful comparison of test results (e.g. performing the test in 9 cardinal positions of gaze.
preoperative and postoperative). 4. A-V patterns can be detected by measuring the
Comparison of prism and cover test performed deviation in upgaze of 25° and downgaze of 35°.
in primary position, straight up position and 5. Primary versus secondary deviation can be
straight down position is very useful in detecting detected by measuring the deviation with right
the 'A', 'V' and 'X' pattern heterotropias. and left eye fixating alternately.
Test should be repeated by holding the prism 6. Divergence excess versus simulated divergence
and occluder before the other eye to detect any excess exotropia can be differentiated by
difference between right and left fixation, which measuring the deviation before and after
would indicate incomitance. prolonged occlusion.

Limitations of prism and cover test

• Since, this test requires an accurate fixation,
so it cannot be performed, if the deviating eye
is blind or has gross eccentric fixation.
• The optical qualities of the prisms also limit
the test accuracy. The stronger the prism, the
greater the error.
• The spectacle lenses with power more than
±5D introduce a significant artifact in
measurement of deviation. Plus lenses
decrease and minus lenses increase the
measured deviation.
• The presence of manifest as well as latent
nystagmus hinders the accurate measure-
ments by prism and cover test.
• Test accuracy is also limited by the minimum
movement of redress that the examiner can
detect with naked eye.

Prism reflex test (Krimsky's corneal reflex test)

Prism reflex test first described by Krimsky has
become universally accepted as a practical
Fig. 6.6 The Boyce-Smith deviometer. method of estimating the size of the angle of
 108 Theory and Practice of Squint and Orthoptics

squint in patients with a blind or deeply reflexion through prisms is difficult, therefore,
amblyopic eye with or without eccentric fixation. the method described above is preferred.
To perform the test, patient is asked to fixate
Advantages of Krimsky's test
on a point light and prisms of increasing
strength (with apex towards the direction of • Since the test requires only that the patient
manifest squint) are placed in front of the normal fixate the light, being entirely objective
fixating eye till the corneal reflex is centered in otherwise, it is useful in testing small children.
the squinting eye (Fig. 6.7). The power of prism • It is quicker to perform than the prism cover test.
required to centre the light reflex in the squinting • It can be used in patients in whom the
eye equals the amount of squint in prism deviating eye has a low visual acuity or has
dioptres. To avoid errors from parallax, the lost central fixation.
examiner must observe the corneal reflex with Limitations of the Krimsky's test
one eye by sitting directly in front of the • Since the angle kappa is included in the
deviating eye while keeping his other eye closed. measurement, the test is inaccurate.
In an alternative method, prism of increasing • It is impossible to perform the test for distance
power can be placed in front of the deviating fixation, since the position of the examiner's
eye until the corneal reflexion is centred. head required to obtain an accurate obser-
However, since the observation of the corneal vation prevents the patient from seeing the
fixation light.

Simultaneous prism cover test

Aim. The aim of this test is to measure only the tropia
without dissociating the phoria.
This special cover test is used to measure the
tropia component of patient with small tropia
<10 PD and a larger phoria (i.e. monofixation
syndrome). It is not useful for larger tropia
(>10 PD), which does not permit fusion and,
therefore, does not have an associated phoria.
Procedure. This test is performed as below:
• Hirschberg corneal reflex test is performed to
estimate the approximate size of tropia
(Fig 6.8A).
• Simultaneous prism cover test is then performed
by placing the prism of the size of tropia in
front of deviating eye and an occluder in front
of the fixating eye (Fig. 6.8B). The power of
prism with which the deviated eye does not
show refixation shift equals the tropia.
Note. For patients with monofixation syndrome,
the amount of tropia is measured with simul-
taneous prism cover test and the total angle
(tropia plus phoria) is measured with the
alternate prism cover test.

Perimeter method
Fig. 6.7 The Krimsky's corneal reflex test. A, right exotropia;
B and C, prism base-in of increasing powers are placed in Patient is asked to fixate at O-mark on the arc
front of the fixing left eye till the corneal reflex centres in the perimeter with normal eye and a flash light is
right eye (C); D, optical principle of the prism reflex test. moved along its arc till the corneal reflexion is
 Evaluation of a Case of Strabismus and Orthoptic Instruments 109

Maddox rod consists of a series of parallel glass

cylinders of higher power (usually of red
colour) set together in a metallic disc (Fig. 6.9A).
The Maddox rod produces a linear image of a
point light. When viewed through the rod, the
line image is formed perpendicular to the axis
of the cylinders.

Measurement of heterotropia
The patient is asked to fix on a point light in the
centre of a Maddox tangent scale (Fig. 6.9B) or
any point light at a distance of 6 metres. The
Maddox rod is placed before one eye with axis
of the rod parallel to the axis of deviation
(Fig. 6.10). Thus, for measuring a horizontal
deviation, the rod is placed in such a way that
the patient sees a vertical line of light
Fig. 6.8 Simultaneous cover test: Hirschberg test depicting
small esotropia (A). Simultaneous placement of prism on (Fig. 6.10A). Depending upon the type of
the esotropic eye and occluder on the fixing eye will show deviation, the red vertical line will be seen either
fixing movement in the esotropic eye when the power of to the right or to the left of fixation light. The
prism is less (B) and no movement when the power of prism number on Maddox tangent scale where the red
is equal to the degree of tropia (C).
line falls will be the amount of deviation in
degrees. Alternatively, prisms of successively
centred in the pupil of the squinting eye. This increasing power (with apex towards the
point on perimeter gives the angle of manifest deviation) are placed in front of the rod until
squint in degrees. the patient sees the line passing through the
This method, used in the past, is not popular fixation light. This gives the amount of
nowadays. heterotropia in prism dioptres. The test should
always be repeated with the Maddox rod in front
Maddox rod test of the other eye, so that deviation during right
It is a subjective test, based on the principle of fixation and left fixation can be compared, and
diplopia, which can be employed to measure any discrepancy, if there, can be noted. Such an
both heterophoria as well as heterotropia. The endeavour, specially gives information about:

Fig. 6.9 A, Maddox rod; B, Maddox tangent scale.

110 Theory and Practice of Squint and Orthoptics

Fig. 6.10 Maddox rod test for horizontal (A) and vertical (B) heterotropia.

• Primary and secondary deviations in the Measurement of heterophoria

presence of paralytic element. To measure heterophoria, the Maddox rod test
• Any change in retinal correspondence with the is performed exactly in a similar manner as
change in fixation. performed to measure the heterotropia with the
• Presence of dissociated vertical deviations following one exception:
may be discovered. In heterophoria, an occluder is placed before
Maddox rod test in conjunction with the the Maddox rod and while the patient fixates
Maddox tangent scale can be performed the light source with his/her other eye, the
successfully in co-operative children as young occluder is removed only for a second and the
as 3 to 4 years of age. Such children should be necessary enquiries are made. After making
asked to go to the scale and put their finger on necessary adjustments of the prism, the occluder
the place where they saw the line rather than is again removed for a second. The procedure is
asked to tell the number from a distance. repeated till the patient sees red line and point
In case of vertical deviation, the Maddox rod light as superimposed.
is rotated so that the line is seen horizontally
The use of a cover is necessary in a phoria,
(Fig. 6.10B) and the deviation is measured
because, if both the fixation light and red line
directly from the tangent scale or by using prism
are seen continuously, there will be a constant
base-up or base-down depending upon the
change in the degree of deviation and the red
direction of deviation (apex of prism is kept
line will never achieve a steady position relative
towards deviation). To measure a cyclotropia,
to the fixation light.
the patient is asked to turn the Maddox rod
around the anteroposterior axis, until he/she
has the impression that the line is horizontal. Limitations of the maddox rod test
The amount of cyclotropia in degrees can be read 1. It can be performed, only if there is no
from the trial frame. suppression under the test conditions.
 Evaluation of a Case of Strabismus and Orthoptic Instruments 111

2. The true angle of deviation is measured, only

if the patient has normal retinal corres-
pondence.
3. It is useful only to measure small deviations,
since when large prisms are used, it is
difficult for the patient to see both the red
line and the fixation light, simultaneously.

Maddox wing test

Maddox wing is an instrument (Fig. 6.11) by
which the amount of heterophoria for near (at a
distance of 33 cm) can be measured subjectively.
Like the Maddox rod test, the Maddox wing test
is also based on the basic principle of
Fig. 6.12 Maddox wing test.
dissociation of fusion by dissimilar objects.
The instrument is designed in such a way that 2. Horizontal, vertical and cyclophorias can be
when the patient looks through the eyepiece of measured simultaneously.
the instrument, the right eye sees a vertical white
arrow and a horizontal red arrow, while the left Disadvantages
eye sees a vertical and horizontal line of 1. The interpupillary distance is not adjustable.
numbers. After a few seconds have elapsed to 2. The test is purely subjective, so the examiner
allow the eyes to assume the fusion free position, has no objective check.
the patient is asked to tell the number on the
horizontal line to which the vertical white arrow Synoptophore method
is pointing (this will give the amount of All types of heterophorias and heterotropias can
horizontal phoria) and the number on the be measured accurately both objectively and
vertical line at which the red arrow is pointing subjectively with the help of synoptophore. The
(this will measure the vertical phoria). The synptophore has been described on page 106.
cyclophoria is measured by asking the patient Techniques of measuring heterophorias and
to align the red arrow with the horizontal line heterotropias using this instrument are as
of number (Fig. 6.12). follows.
Advantages Measurement of objective angle of deviation
1. Since the numbers can be read, only when with synoptophore
the patient accommodates sufficiently, this The synoptophore is set for the patient's height
makes the test much more reliable than one and interpupillary distance. Simultaneous
in which the patient fixates a light. perception slides (e.g. lion and cage) are used.
The patient is asked to look at the pictures, and
the arm controlling the picture in front of the
deviating eye is moved by the examiner until
there is no movement of either eye on a cover
test performed by alternately turning off the
light (Fig. 6.13). The reading on the horizontal
scale in front of the deviating eye as well as the
one of the vertical scale, represents the objective
angle of deviation. For example, if the arm of
synoptophore in front of the deviating eye is at
20Δ base out and has to be raised 2Δ, the objective
angle is recorded as 20 Δ esotropia and 2 Δ
Fig. 6.11 Maddox wing. hypertropia.
 112 Theory and Practice of Squint and Orthoptics

Disadvantages
The disadvantages of measuring deviation
objectively with synoptophore are as follows.
1. Small children may not co-operate and might
be frightened.
2. Though the instrument is optically arranged
for distance, there is tendency for the patient
to converge as he/she thinks the pictures are
close to him/her. Consequently, esotropias
usually increase and exotropias decrease in
Fig. 6.13 Measurement of objective angle of deviation with size. Therefore, synoptophore is not being
synoptophore.
considered a very reliable instrument to
measure horizontal deviations.
Measurement of the objective angle for near fixation.
It can also be made as follows: Measurement of subjective angle of deviation
To measure the objective angle of deviation with synoptophore
for near, a –3.0D lens is inserted in the lens After measuring the deviation objectively (as
holder situated in front of the eyepiece lenses. above), the patient is asked to comment on the
In this way, the patient has to exert 3D position of the pictures used. If the patient claims
accommodation in order to get a clear image of superimposition (i.e. the lion seen in the cage)
the slides. In doing so, each eye exerts 3Δ of at his objective angle, this angle is also his
convergence for each dioptre of accommodation. subjective one. If this is not the case, the arms
In other words, 9Δ of convergence in one eye or are moved back to zero and the patient is asked
18Δ of convergence in both eyes—considering to move the handle controlling the picture in
the interpupillary distance (IPD) as being front of the non-fixating eye until he/she sees
60 mm. For a smaller IPD, the convergence the two pictures superimposed (Fig. 6.14).
requirement is less and for a bigger IPD, it is Adjustments can be made for vertical or
more (provided the AC/A ratio is normal). torsional separation, if necessary. This is the
Thus, when recording the angle of deviation, one subjective angle. At this point, one should by
must keep this in mind and either subtract 18Δ means of rapid alternate flashing, check whether
(for esodeviations) from or add 18Δ (in case of or not the eyes move, when the patient is asked
exodeviations) to the synoptophore readings. In to fixate on each picture in turn. This is done
other words, a synoptophore reading of 22Δ mainly to make sure that an actual change in
base-out should be recorded as 4Δ esotropia and the angle between the visual axis has not
a reading of 22Δ base-in should be recorded as occurred, as happens frequently through
40Δ exotropia.
Advantages
Advantages of measuring objective angle of the
deviation with synoptophore are as follows:
1. The objective angle can be measured with
either eye fixating and in all cardinal
directions of gaze.
2. It is possible to measure horizontal, vertical,
and torsional deviations fairly accurately.
3. Deviation of any size can be measured since
prisms can be used in the lens holder, when
necessary.
4. Measurement of objective angle for near Fig. 6.14 Measurement of subjective angle of deviation
fixation can also be made. with synoptophore.
 Evaluation of a Case of Strabismus and Orthoptic Instruments 113

relaxing or increasing the accommodative effort cyclophorias. To perform this test the, double
or in cases of a variable angle of deviation. prism is placed before one eye in such a manner
Problems which may come across while that the junction of the two bases intersects the
performing this test are as follows: pupil and is horizontal. Then the patient is asked
1. Suppression may prevent the patient from to look at a horizontal line against an empty
superimposing the pictures. In such cases, background which does not offer any fusional
simultaneous macular perception or simul- stimuli and inferences drawn are as follows:
taneous paramacular perception slides can be • Patient will see two parallel lines with the eye
used. The larger the image formed on the having double prism in front of it, i.e. one line
retina, the less likely it is to be suppressed. displaced above and the other displaced
2. The patient may never succeed in putting the below with respect to the single line seen by
lion in the cage, and it may suddenly be seen the other eye.
on the other side of the cage (in an uncrossed • In the absence of any cyclophoria, all three
or homonymous position in divergent lines will be parallel.
deviations and in a crossed or heteronymous • If a cyclophoria is present, the single line will
position in convergent deviations). In such have an angle relative to the other two lines
cases, the crossing point is considered to be as follows:
the subjective angle. – In incyclophoria, the line or lines seen by the
3. It must be realized that the measurement right eye will be tilted towards right and
obtained by the subjective method is only the those seen by left eye will be tilted towards
true angle of deviation, if normal retinal left.
correspondence is present. – In excyclophoria, the line or lines seen by the
right eye will be tilted towards left and those
Measurement of cyclodeviation with
synoptophore
seen by left eye will be tilted towards right.
There is no way to carry out an objective Haploscopic tests
measurement of a cyclodeviation. The subjective
Tests based on the haploscopic principle to
measurement can be performed as follows.
measure the deviation include Lancaster red-
Simultaneous perception slides are used. The
green test, Hess and Lees screen tests. These tests
slide with lion is kept in front of the right eye
are very useful for measuring incomitant
and that with cage is kept in front of the left eye.
strabismus in patients with diplopia (see page
The patient is asked to look at each one in turn
121–126).
and is asked whether the cage appears level. In
the presence of cyclodeviation, the cage appears ASSESSMENT OF OCULAR MOVEMENTS
tilted. In incyclotropia, the cage's left-hand side
is seen lower than the right-hand side. This is ASSESSMENT OF DUCTIONS
corrected by wheel rotating the slide towards 1. Duction test. Ductions are monocular
the patient. In the presence of excyclotropia, the movements and are measured at near distance.
cage's right-hand side appears lower than the When examining ductions, one eye is covered
left-hand side. This can be corrected by wheel- and the fellow eye fixates a spotlight which is
rotating the slide away from the patient (towards moved to bring the fixating eye to the farthest
the examiner). The amount of deviation is read possible position, in all the cardinal directions
in degrees from the scale located on the of gaze. For interpretation of the observations,
slideholder of the instrument. It should be following methods are in vogue:
remembered that the tilt of the image is in the i. In most frequent practice, the examiner
direction opposite to the tilt of the eye. observes whether movement lags or is excessive
in any direction. If no lags are noticed, the
Double prism test ductions are recorded as full; if lags are
Double prism test consists of two prisms which noticed, the muscle and the eye involved are
are mounted base to base. It is used to elicit indicated. Usually, a subjective assessment
114 Theory and Practice of Squint and Orthoptics

is made on scale of 7 points (+3 to –3) or

9 points (+4 to –4). Further, a note is also
made of the occurrence of any nystagmoid
movements in the presence of full ductions.
ii. Judging the normalcy of adduction and
abduction in relation to fixed points. Following
useful guidelines have been suggested:
• In maximal adduction, an imaginary
vertical line through the lower lacrimal
punctum should coincide with a boundary line
between the inner one-third and the outer two-
thirds of the cornea (Fig. 6.15A).
– In excessive adduction, more cornea is
hidden (Fig. 6.15B).
– In defective adduction, more cornea is
visible. Some of the sclera may also be
visible (Fig. 6.15C).
• In maximal abduction, the lateral limbus
touches the outer canthus (Fig. 6.15D).
– In excessive abduction, some of the cornea is
hidden under the outer canthus (Fig. 6.15E).
– In defective abduction, some of the sclera is
visible between the outer canthus and the
limbus (Fig. 6.15F).
2. Kestenbaum's limbus test of motility. The
duction movements are measured with the help
of a transparent ruler as follows:
• Adduction is measured by noting a difference
between the position of the temporal limbus
in primary position and maximum adduction.
• Abduction is measured by noting a difference
between the position of the nasal limbus in
primary position and maximum abduction.
• Similarly, elevation and depression are
measured with respect to inferior limbus and
superior limbus, respectively.
• Normal values reported are:
– Adduction : 10 mm
– Abduction : 10 mm
– Elevation : 5–7 mm
– Depression : 10 mm
3. Subjective perimeter method of measuring
ductions. In this method, to measure the Fig. 6.15 Judgement of adduction (A, B, C) in relation to
amplitude of duction movements, the patient's lower punctum and abduction (D, E, F) in relation to lateral
canthus. For explanation see text.
head is placed into the chin rest of a perimeter
in such a way that the eye to be examined is in patient is asked to fixate and follow the
the centre of the perimeter arc or perimeter perimeter target that is moved from the centre
hemisphere. The other eye is occluded and the of the field to periphery. He/she is instructed
 Evaluation of a Case of Strabismus and Orthoptic Instruments 115

to indicate, when he/she can no longer see the kept at such a distance that one can always
target. This point indicates the limit of the observe the corneal reflections in both eyes. The
duction movement in that particular direction. following observations should be made on
Normal values reported by this method are: version test.
– Adduction : 50° • For excessive or defective movements in any
– Abduction : 50° direction.
– Depression : 50° • To detect underaction of one muscle and
– Elevation : 40° overaction of its contralateral synergist.
• To detect overaction of one muscle without
4. Objective perimeter method or corneal
underaction of its contralateral synergist.
reflex method of measuring ductions. The
• To note any retraction of the globe and
amplitude of duction movements can be
narrowing of palpebral fissure in certain
checked somewhat more objectively by using
direction of gaze (as seen in Duane's retraction
corneal light reflex. In this method, after closing
syndrome).
one eye, patient is asked to turn his/her eye
• To detect the overaction of inferior and
maximally in a given direction. Then the
superior obliques.
examiner moves a small flash light along the arc
of the perimeter until the reflex from the Clinically, the overaction of oblique muscles
patient's cornea appears to be centred in the can be graded by following methods:
pupil. The examiner views it with one eye from i. Depending upon the vertical deviations, the
the position of flash light. This point gives the overactions of obliques is graded as:
limit of the particular duction movement.
a. Mild overaction—when vertical deviation
Note: It is important to be aware of the fact (e.g. hypertropia in inferior oblique
that, in practice, the measurement of ductions overaction) is appreciated only in
is not of much value in the investigation of sursumadduction.
strabismus, since only a small fraction of the b. Moderate overaction—when vertical
fibres of a muscle need to function in order to deviation is appreciable on adduction
rotate the eye to the limits of its field of duction. itself.
A defect in the amplitude of duction occurs, only c. Severe overaction—when hypertropia is
when almost complete paresis of a muscle seen in primary position.
occurs. Therefore, a partial paresis usually
cannot be diagnosed on testing ductions. ii. Depending on the angle, the adducting eye
makes with the horizontal line as it elevates
ASSESSMENT OF VERSIONS and abducts (if overacting) on lateral version
In general, study of versions is more important to the opposite side, the overaction of inferior
factor than the study of ductions, when deciding oblique is graded as shown in Fig. 6.16.
on which muscle or muscles to operate. Similarly, the overaction of superior oblique
Further, the investigation of versions is of also can be graded by observing the angle the
greatest importance in patients with non- adducting eye makes with the horizontal line
comitant strabismus, because comparison of the as it depresses and abducts.
extent of movement of the two eyes relative to
2. Perimeteric method of measuring versions
each other during a version is the most sensitive
test to detect underfunction of a muscle. The amplitude of versions can be measured on
the perimeter in the same way as ductions except
1. Version test that the patient fixates and follows the test object
It is performed at approximately 15 inches. The with both eyes until he/she sees it double or
patient is asked to hold his head straight and until it moves too far out for him/her to follow.
still and to make eye movements on command In general, such a measurement of the
or to follow a fixation light in all the cardinal absolute amplitude of versions is of little
directions of gaze. The fixation light should be practical value.
 116 Theory and Practice of Squint and Orthoptics

Fig. 6.17 Prism bars, horizontal (A) and vertical (B).

A prism bar consists of a series of prisms of

increasing strength (Fig. 6.17). It is held by the
examiner in front of one of the patient's eyes and
merely needs to be moved higher or lower to
bring a stronger or weaker prism into the line
of sight.
Amplitudes of divergence are measured first
and those of convergence second.

Measurement of amplitude of divergence

To perform the test for distance, patient is asked
to fixate the 6/12 symbol at 6 metres and the
prism bar is used with the prism base directed in
BI in front of the one eye (preferably the non-
dominant one). By progressively increasing the
amount of base in prism power, the eyes are
diverged to the limit of bifoveal single vision, i.e.
up to the point, when the patient just appreciates
Fig. 6.16 Grading of inferior oblique overaction depending diplopia. This point is the end point of the test
on the angle adducting eye makes with horizontal line. and is called the break point. Its reading is
recorded. At this point, the power of the prism is
MEASUREMENT OF VERGENCES decreased slowly, until he/she again fuses. This
The status of motor fusion is assessed by point called as the recovery point is also noted.
measuring the vergences, i.e. the fusional To measure the amplitude of divergence for
amplitudes. In the presence of heterophoria or near, the above test is repeated at 33 cm. The
an intermittent heterotropia, the fusional end point and the recovery point are recorded.
amplitudes can be measured both by the prism But unlike the test for distance, the end point
method or synoptophore method. While, in a for the near test is blur point, i.e. the maximum
patient with heterotropia, only the synopto- amount of base-in (BI) prism power after which
phore method is useful, since fusion in casual the patient's vision is blurred. The mechanism
gaze is necessary for testing with prism method. of blurring of vision is as follows:
Fusional divergence is measured from the The retinal disparity produced by the use of
subject's phoria position, whereas relative base-in prism evokes fusional divergence that
divergence is measured from the position of maintains bifoveal single vision until its
fusional demand, i.e. the orthoposition. amplitude is exhausted. At this point, the
patient, who is accommodating during near
Testing of fusional amplitudes with prism method vision, can produce further divergence and
The test can be performed using a prism bar or maintain single vision longer, if he/she relaxes
a rotatory prism (Risley prism) or single prism. his/her accommodation, because this simul-
 Evaluation of a Case of Strabismus and Orthoptic Instruments 117

taneously decreases the amount of accommo- these targets and sees them as one with both
dative, convergence present. But, due to control marks, the examiner blocks the arms at
relaxation of accommodation, the near object the objective angle. Then, first the amplitude of
becomes blurred. divergence and second the amplitude of
convergence are measured as below.
Measurement of amplitude of convergence
To measure the divergence, the arms of the
To perform the test for near, patient is asked to synoptophore are slowly diverged and the
fixate 6/12 symbol at 33 cm and the bar is used patient is instructed to report occurrence of
with prism base directed out (BO). By diplopia or the disappearance of one or the other
progressively increasing the amount of BO control mark of the picture (suppression). This
prism power, the eyes are converged to the limit point—the break point—is recorded and the arms
of bifoveal single vision, i.e. up to the point, of the synoptophore are slowly converged (i.e.
when the patient just appreciates diplopia. This brought to less divergent position) and the
point is the end point, of the test and is called recovery point, where fusion occurs, is noted.
the break point. Its reading is recorded. At this To measure the convergence, the arms of the
point, the power of the prism is decreased slowly synoptophore are further converged slowly till
until he/she again fuses. This point, called the the fusion breaks and the break point is noted.
recovery point, is also recorded. Theoretically, Then, the arms are moved back into a less
before the break point, there will be a blur point convergent position until fusion is regained and
because after the exhaustion of the fusional the recovery point is noted.
convergence patient starts using his/her
accommodative convergence to avoid diplopia. To measure the amplitude of vergences for near with
This, however, can only be done by accommo- synoptophore, a –3.0 DS lens is placed before each
dating in excess of the requirements for the given eye. In order to see clearly with –3.0DS lens, the
distance (pseudomyopia) and consequently the subject has to overcome these lenses by
image is blurred. Therefore, it is important to accommodating as if he/she was fixating an
record the blur point in order to know what kind object at a distance of 33 cm. To simulate the
of fusional amplitudes are measured. orthoposition for near fixation, the synoptophore
tubes have to be set according to the convergence
To perform the test for distance, the same
requirement for a point 33 cm distant which, in
procedure is repeated at 6 m and the blur point,
prism dioptres, is three times the patient's
break point and recovery point are recorded.
interpupillary distance in centimetres.
An example of a recording of fusional
amplitudes as tested with the prism bar: The procedure of testing for near is the same
as for distance.
Distance : Diverged to 12Δ BI/recovered at 9Δ BI Normal values of vergences are as follows:
Converged to 32Δ BO/recovered at 21Δ
BO Vergence Distance (6 m) Near (33 cm)
Blurred at 12Δ BO Convergence 14–20 Δ
35–40Δ
Near : Diverged to 14Δ BI/recovered at 9Δ BI Divergence 5–8Δ 15–20Δ
Converged to 36Δ BO/recovered at 24Δ Vertical vergence 2–4Δ 2–4Δ
BO Incyclovergence 10–12° 10–12°
Blurred at 18Δ BO Excyclovergence 10–12° 10–12°

An example of recording of fusional amplitudes as

Synoptophore method of measuring
tested with the synoptophore:
fusional amplitudes
Distance:
To begin with, the objective angle of deviation
is determined using simultaneous macular • 30Δ ET, objectively and subjectively.
perception slides. Then, the second-grade fusion • First- and second-grade fusion at angle.
slides (similar targets with control marks for • Convergence to 42Δ BO/recovery at 32Δ BO.
each eye) are introduced and if the patient fuses • Divergence to 12Δ BO/recovery at 20Δ BO.
 118 Theory and Practice of Squint and Orthoptics

Near (with –3.0 D): NPA in dioptres), and the age is indicated in
• 44Δ ET objectively and subjectively years on the fourth side. The sliding target
• First- and second-grade fusion at angle. contains targets for measuring NPA and NPC.
• Convergence to 56Δ BO/recovery at 44Δ BO. Procedure. For measurement of convergence, a
• No divergence past angle, suppression OD. dot or a vertical line may be used as the target.
It is advanced towards the patient at, or slightly
Measurement of near point of convergence below the eye level, until the patient has
The near point of convergence (NPC) is the converged maximally and cannot sustain single
closest point at which an object can be seen bifoveal fixation as the target is brought closer.
single during bifoveal vision. In other words, it At this break point, the subject's non-dominant
is the point at which the two foveal lines of sight eye will diverge (objective test) and patient may
intersect, when maximum convergence is appreciate diplopia (subjective test). The distance
exerted. from the canthus to this point is read on the rule
The NPC practically measures all types of and the NPC is recorded in mm or cm. Some of
convergence; since an object actually approaches the near point rules have the zero point of their
the eyes during testing. That is, the test for NPC scales at the so-called spectacle point (i.e. 27 mm
simultaneously stimulates fusional, accommo- in front of the baseline). Therefore, with such
dative and proximal convergence and during instruments, 27 mm must be added to the
the last phase, if the patient is co-operative, there distance that is read off the scale.
will be a strong voluntary effort to converge. Normal values. The normal values of NPC vary
Instruments. Near point of convergence can be considerably among different persons and even
measured simply with the help of a graded in different examinations of the same person. In
plastic rule placed at the outer canthus and a normal adults, its average value is 70 mm (7 cm)
fixation target (e.g. tip of a sharp pencil) moved with a range between 50 and 100 mm (5 to 10
towards the eye; or by use of specially designed cm). A distance closer than 5 cm is excessive,
rule such as RAF rule (Fig. 6.18), Livingstone however, in children it may be as close as tip of
binocular gauge (described on page 139) and the nose. NPC further away than 10 cm is
Prince rule. These specially designed defective or remote. In patients with conver-
instruments basically consist of a bar or rule gence insufficiency (CI), it may be as remote as
made from plastic, metal or wood on which a 25 or 30 cm or more.
rider with the test chart can be moved back and
forth (fixation target). At one end of the bar is a Measurement of maintenance of convergence
wing-like support that fits over the nose and The ability of the eyes to maintain convergence
rests against the lower orbital margins during after the patient has been able to converge his/
the measurement. In Prince rule, the bar is 24 her eyes to a near vision can be tested by the
inches long and 1/2 inch square that has inappropriately named test—the drop conver-
different markings on each of its four sides. One gence test. In this test, after bringing the fixation
side is divided into centimetres (to be used for target into reading distance, patient is asked to
measurement of NPC and NPA), the second one maintain convergence at this point and the
into inches, and the third one into dioptres (for fixation object is dropped suddenly. Some
patients are better able than others to keep their
eyes converged in the absence of a fixation
object.

ASSESSMENT OF ACCOMMODATION
AND AC/A RATIO
ASSESSMENT OF ACCOMMODATION
As we know, accommodation is a unique
Fig. 6.18 RAF rule. mechanism, by which our eyes can even focus
 Evaluation of a Case of Strabismus and Orthoptic Instruments 119

If, while measuring the NPA, the patient's

amplitude of accommodation is found so low that
his/her near point is beyond the length of the
instrument, plus lenses are added to his/her
correction until the near point is brought within
range. The dioptric power of these additional
Fig. 6.19 Effect of accommodation on divergent rays entering lenses is then deducted from the measured values
the eye. of amplitude of accommo-dation. Conversely, in
young patients with very high accommodative
the diverging rays coming from a near object power, minus lenses may be added to his/her
on the retina in a bid to see clearly (Fig. 6.19). distance correction to move his/her near point
Assessment of accommodation is of great away from his/her eyes. The dioptric power of
diagnostic value in cases of incomitant those minus lenses is then added to the measured
strabismus of non-paralytic origin. Assessment value of amplitude of accommodation.
of amplitude of accommodation (the difference
between the dioptric power needed to focus at Measurement of amplitude of
near point 'P' and far point 'R', i.e. A = P – R) in accommodation using minus lenses
practice can be made either by measurement of
This test is also performed first for each eye
near point of accommodation (NPA) or by use
separately and then for both eyes together and
of minus lenses as below.
during examination patient has to wear his/her
full refractive correction. The patient is asked to
Measurement of near point fixate 6/60 symbol at a distance of 6 metres and
of accommodation minus lenses of progressively increasing power
The near point of accommodation (NPA) is the are added before the eye till he/she can see the
closest point at which small objects can be seen target clearly. The power of this minus lens is
clearly. It is also called 'near point' or 'punctum equivalent to the amplitude of accommodation
proximum'. in dioptres.
NPA is measured using a near point rule such
as RAF rule (Fig. 6.18) or Prince rule. The ASSESSMENT OF ACCOMMODATIVE
description of such a rule has been given in the CONVERGENCE/ACCOMMODATION
discussion on the measurement of the near point (AC/A) RATIO
of convergence. The AC/A ratio is the relationship between
To determine the NPA, a sliding target with accommodative convergence (AC), expressed in
6/9 letters, numbers or fine lines is moved from prism dioptres ( Δ) and accommodation (A),
or towards the eye until closest point is found expressed in lens dioptres (D). This relationship
at which it still can be seen clearly. During the is linear one and is thought to be relatively stable
examination, the patient has to wear his/her full throughout life. The normal AC/A ratio is about
optical refractive correction. The NPA is 3 to 5 prism dioptres for one dioptre of
determined first for each eye separately and then accommodation. The concept of AC/A ratio was
for both eyes together. The NPA is measured in first clearly defined by Fry who later with
centimetres marked on one side of the Haines introduced the abbreviation AC/A ratio.
instrument bar. The side of bar marked in
dioptres will indicate the amplitude of Methods of measurement of AC/A ratio
accommodation in dioptres. The third side of 1. Heterophoria method. To measure AC/A
the bar shows the age corresponding the ratio, in this method, the deviation is measured
accommodation. For example, if the patient with full optical correction at 6 metres distance
reports that the point appears blurred at 25 cm, and at 33 cm distance in prism dioptres, and IPD
the dioptric markings will show +4.0 D and the is measured in centimetres. Then the AC/A ratio
age 40 years. is calculated from the following formula:
 120 Theory and Practice of Squint and Orthoptics

placed before his/her eyes and the distance

AC/A = IPD + , where; deviation ( ΔL) is measured once more. The
IPD = Interpupillary distance in AC/A is calculated as above.
centimetres The gradient method is inaccurate because it
Δ
n = Deviation at 33 cm or 3 dioptres does not take into account the patient's
distance in prism dioptres interpupillary distance (IPD).
Δ
d = Deviation at 6 metres distance in 3. Clinical distance-near-relationship method.
prism dioptres This is a very simple method in which AC/A
d = The fixation distance at near in ratio is known by substracting distance
dioptres deviation (D) at 6 metres from the near deviation
(N) measured at 33 cm; i.e: AC/A = N – D. For
Note: Esodeviations are denoted by positive examples:
(+) and exodeviations by negative (–) sign.
i. In a patient with esotropia (ET) of 40 PD at
For example, if IPD = 6 cm, Δn = 9Δ
near and 20 PD at distance, the AC/A ratio
exophoria and Δd = 3Δ exophoria, then
= 40 – 20 = 20D PD.
AC/A = 6 + ii. In a patient with distance orthophoria and
= 4 Δ/D near exotropia (XT) 15 PD the AC/A =
–15 – 0 = –15PD
2. Gradient method. This method is based on
The results are interpreted as below:
the fact that for a given fixation distance, minus
• Up to 10 PD of N – D is normal.
lenses placed before the eyes increase the
requirement for accommodation and plus lenses • >10 PD of N – D is high AC/A ratio and
relax accommodation. Further, it is assumed that • <10 PD of N – D difference is less AC/A ratio.
–1.0D lens produces an equivalent of 1.0D of 4. Fixation disparity method. In this method,
accommodation, whereas +1.0D lens relaxis AC/A ratio is indirectly derived from the fixation
accommodation by 1.0D. disparity induced either by forced convergence
In practice, original deviation is found at near by use of prism or by altering the accommo-
while the patient wears his/her optical dative stimulus by use of optical lenses. Because
correction and then with additional +3.0D lens of its complexity, this test is not performed in
and the calculations for AC/A ratio are made as routine clinical practice.
follows: 5. Haploscopic methods. In haploscopy, the
visual fields of the two eyes are differentiated
AC/A = where and a separate target is presented to each eye.
Δ Hering's original instrument was designed
L = Deviation with additional
primarily for studying the AC/A ratio. In
lenses.
Δ practice, this method is no more used. However,
O = Original deviation without
the haploscopic devices, such as the major
additional lenses.
amblyoscope, are of fundamental importance for
D = Dioptric power of the the study of the sensorimotor co-operation of
additional lenses. the eyes.
For example, if original deviation (ΔO) = 2Δ
esophoria, deviation with additional lenses (ΔL) ASSESSMENT FOR
= 10Δ exophoria and the power of additional EXTRAOCULAR MUSCLE PARESIS
lenses (D) used is +3D, then: When paresis of one or more extraocular
AC/A = = 4Δ/D muscles is suspected as cause of squint, in
addition to the duction test and version test,
Alternatively, the patient's original distance following tests should also be performed:
phoria (ΔO) is determined while he/she wears • Abnormal head posture examination
full optical correction. A –3.0D lens is then • Diplopia charting
 Evaluation of a Case of Strabismus and Orthoptic Instruments 121

• Quantitative measurement of extraocular the improvement or deterioration from the

muscle actions records of different dates in the same patient.
• Field of binocular fixation • The test requires intelligent patient, especially
• Bielschowsky phenomenon test to comment where the separation is maximum.
• Bielschowsky three-step test • It is not possible to perform the test in colour
blind patients.
I. ABNORMAL HEAD POSTURE EXAMINATION
• This test is not of use in congenital palsies and
Note the abnormal head posture, if any and those of long-standing onset, because due to
examine its components in detail (see page deep suppression diplopia cannot be elicited.
288–291).
III. QUANTITATIVE MEASUREMENT OF ACTIONS OF
II. DIPLOPIA CHARTING EXTRAOCULAR MUSCLES
Plotting of diplopia fields is indicated in patients The quantitative measurement of actions of
complaining of confusion or double vision. The extraocular muscles is essential to comment
test is easy to perform provided the patient is about the paretic muscles and the pathological
co-operative. To perform the diplopia charting, sequelae of the paralysis, viz. overaction,
patient is asked to wear red-green diplopia contracture and secondary inhibitional palsy.
charting goggles; red glass being in front of the The tests employed for quantitative measure-
right eye and green in front of the left eye. The ments of ocular movements are based on
patient is made to sit with his/her head straight haploscopy. The haploscopic tests are based on
in a semidark room and is shown a fine linear the principle described by Burian that in the
light from a distance of 4 ft. The light is moved presence of normal retinal correspondence, the
from primary position into all of the other eight two test objects presented to the two eyes will
directions of gaze. For each direction, patient is be superimposed, if they stimulate the foveae
asked to comment on the position, brightness of the two eyes, irrespective of the position of
and separation between the red and green the two eyes (Fig. 6.20).
images. From the patient's comments, the
Commonly used haploscopic tests to have a
examiner notes the following points:
graphic record of the relative power of
• Whether horizontal diplopia is homonymous extraocular muscles in all directions of gaze
or heteronymous. include:
• Whether the image seen by right eye (red • Lancaster red-green test,
image) is higher or lower than the image seen • Hess screen test, and
by the left eye (green image) or vice versa. • Lees screen test.
• In which direction of gaze, separation between
red and green images is greatest. 1. Lancaster red-green test
• Whether there are any directions in which The Lancaster red-green test is a haploscopic
fusion is present. test. It utilizes a Lancaster red-green screen
In a modified test of Franchchetti, instead of which is window-shade type of screen that can
red green goggles, a red Maddox rod is placed be rolled up when not in use. The screen contains
in front of right eye and white Maddox rod in horizontal and vertical lines forming squares of
front of the left eye and the patient fixates on a 7 cm (Fig. 6.21). All the squares are of the same
spotlight which is seen as vertical red line with size and the tangential error is not taken into
right eye and vertical white line with left eye. account. While performing the test, the patient's
Diplopia charts of patients with paresis of eyes should be in level with the centre zero
different extraocular muscles are shown in mark, and he/she can be seated at either 1 or
Figs 12.13 and 12.14. 2 metres. At 2 metres, each square subtends an
Disadvantages of diplopia plotting test angle of 2o = 3.5Δ; at 1 metre it subtends an angle
• This test is only qualitative, therefore, it is not of 4 o = 7 Δ. The patient is given a red-green
possible to comment on the minor changes of reversible goggles (e.g. red glass in front of right
 122 Theory and Practice of Squint and Orthoptics

Fig. 6.21 Lancaster red-green test.

cardinal directions of gaze. The distance

between the streaks of light represents the
measurement of the objective deviation provided
retinal correspondence is normal. The results are
plotted on a chart that is an exact replica of the
Fig. 6.20 Burian's principle of haploscopic tests. Note, the screen. Since the projected image is a line, the
right eye is esotropic and two different objects (red and patient's response may indicate the presence of
green) presented to the two eyes (A) are stimulating the cyclotropia, when his/her streak is tilted. This
foveas and are thus subjectively localized as superim-
posed over each other (B).
test is most useful in patients with ocular
paralysis and least useful in patients with
eye and green glass in front of left eye) and green heterophoria or intermittent heterotropias.
flashlight that projects a linear image. The
examiner has a similar red flash light and 2. Hess screen test
projects the red streak of light on the zero mark Principle
on the screen. The patient is asked to The Hess screen test is based on the haploscopic
superimpose his/her green light on the principle. It utilizes the Hering's law of equal
examiner's red light. This is then repeated in all innervation, which states that in all voluntary
 Evaluation of a Case of Strabismus and Orthoptic Instruments 123

movements of the eye, equal and simultaneous of gaze and the outer square the extreme
innervation flows from the brain to the muscles directions of gaze. In the original Hess screen,
of both eyes concerned in the respective indicator consists of a knot tying three green
direction of gaze (yoke muscles). cords together to form the letter Y. The end of
central vertical green cord is fastened to a
Prerequisites
movable black rod 50 cm long. The ends of the
Patient should have:
other two green cords, forming upper two limbs
1. Full understanding about what he/she is of the letter Y, are kept taut by black threads
supposed to do, since the test is purely that pass through loops to small weights at
subjective. corresponding upper corners of the screen. This
2. Good vision in both eyes. arrangement enables the patient to move the
3. Central fixation. indicator freely and smoothly over the whole
4. Normal retinal correspondence. surface of the screen in all directions.
Discription of conventional Hess screen Modified wooden Hess screen. One of the
modifications of the original Hess screen is a
Original Hess screen consisted of a single
wooden screen with small red lights forming the
tangent screen made up of a black cloth 3 ft wide
fixation points (Fig. 6.23) and a green dot light
× 3½ ft long, marked by a series of horizontal
projecter as the indicator. Presently, it is more
and vertical red lines (Fig. 6.22). The distance
commonly in use.
between each line subtends a visual angle of 5°.
Fixation points are indicated at the centre of the Procedure Hess screen test
screen and at the intersections of the 15° and 30°
The patient wears red-green goggles and sits
lines by red dots. Thus, the red dots form an
50 cm from the commonly used modified
inner square of 8 dots along the 15° lines and an
wooden Hess screen. The patient now sees the
outer square of 16 dots along the 30° lines. The
fixation points (red light) with one eye and the
inner square represents the 8 cardinal directions
indicator (green light of projector) with the other
eye. The patient is asked to superimpose the
indicator successively on each of the fixation
points, and the relative position of the eyes is
plotted for each of these directions of gaze on a
chart which is replica of the Hess screen.

Fig. 6.22 The Hess screen. Fig. 6.23 Modified Hess screen.
 124 Theory and Practice of Squint and Orthoptics

Digital Hess screen of the circles (Fig. 6.24B). This is repeated for
Digital or the PC Hess screen provides the either 9 or 25 directions of gaze (depending
clinician, a new computer-based tool for on the option selected). The colour of the
assessing patients suffering from paralytic circles is then reversed and measurements
strabismus, using image manipulation repeated with the left eye fixating. The nine
technology and software technology. It is point test takes approximately 4 minutes to
designed to run on any computer operating complete.
under Windows, with a 19’’ (or larger) monitor. • Results are then displayed in the conventional
When the program is run for the first time, the format on the screen.
user is required to calibrate the size of the screen • Multiple plots can be superimposed to assess
(by measuring the dimensions of a box longitudinal changes and the exact amplitude
displayed on the screen) and to enter the of any deviation can be displayed at any point
preferred viewing distance (usually 25–50 cm). on the chart (Fig. 6.25).
It provides integration function of diagnosis of
strabismus, data record and analyze.

Key features
• Rapid and accurate assessment of the size and
direction of phoria/tropia
• Results plotted in conventional Hess screen
format allow the clinician to establish whether
a deviation is concomitant or incomitant and
which muscle is affected.
• A variety of analytical tools to help the
clinician form a diagnosis
• Built in database allows results to be archived
for future reference
• Results can be printed or pasted into referral
letters and reports
• Runs on a standard PC Fig. 6.24A Patient is seated in front of a PC Hess screen
• Voice instructions, possible. after wearing red and green goggles.

Operating methods
Digital Hess screen is a computer program
which is designed to run on any computer.
Steps of use are as below:
• Patient wears red and green goggles and is
positioned in front of the computer screen at
the appropriate distance (Fig. 6.24A).
• Room lights are extinguished and a red and a blue
circle are displayed on the screen (the right eye
sees the red circle and the left the blue).
Initially the red circle is placed in the top left
of the screen and the patient is instructed to
move the blue circle using the mouse until it
appears to be centred on the red circle.
• As the eyes are dissociated, any deviation in this Fig. 6.24B Misalignment of the circles seen due to any
direction of gaze will result in a misalignment deviation in the direction of gaze.
 Evaluation of a Case of Strabismus and Orthoptic Instruments 125

Fig. 6.25 Hess screen plots superimposed to assess longitudinal changes and the exact amplitude of any deviation.

• A number of analytical tools can then be applied

to the data to help the clinician establish a
diagnosis. For example, the program will
automatically calculate the relative areas of the
plots for the left and right eyes, helping the
clinician to determine which eye has a palsied
muscle and providing an index for monitoring
the progression of an incomitant deviation.

3. Lees screen test

Lees screen, also known as the Hess-Lees screen,
is another modification of the original Hess
screen. The Hess-Lees screen (Fig. 6.26) consists
of two tangent screens made of white
translucent material placed at a right angle with
a plane mirror bisecting this right angle and
dissociating the fields of the two eyes. The
tangent pattern similar to the original Hess
screen printed in black dots (fixation points) on
a white background is placed just behind both
Fig. 6.26 Lees screen.
the translucent screens, and is seen only when
the translucent screens are illuminated for
performing the test. with this horizontal line by an adjustable chin
rest. The patient is given a pointer with a ring at
Procedure its tip. The examiner has another pointer with a
To perform the test with right eye fixing, the small disc at its tip, half the diameter of the
patient sits facing the left tangent screen at patient's pointer. The right screen is illuminated
50 cm from it with his/her forehead leaning and left screen is kept non-illuminated. The
against the central vertical rim of the mirror. The patient's right eye vision is intercepted by the
line bisecting the mirror horizontally lies in the mirror, in which he/she sees the right screen
same plane as the centre of the horizontals of projected forward as a virtual image that
the tangent screens. Patient's pupils are levelled appears to be superimposed upon the left screen.
 126 Theory and Practice of Squint and Orthoptics

The patient's left eye sees the left screen directly

(he/she is facing that screen) but cannot see the
right screen, situated laterally. The examiner
now places his pointer on the zero (central) point
of the right screen. Its image that appears to be
superimposed on the left screen is seen by the
patient's right eye. Patient is asked to super-
impose his/her pointer on the examiner's
pointer with his/her left hand on the left screen.
By means of a foot pedal, the left screen is
illuminated for 1–2 seconds so that the examiner
can plot on the diagnostic Hess chart the precise
location of the patient's pointer on the left screen.
The 8 dots of the inner square are then plotted
in sequence and, wherever necessary, this is
followed by the plotting of the 16 dots of the
outer square.
To perform the test with left eye fixing, patient
sits facing the right screen which is kept
unilluminated while looking with his/her left
eye into the mirror, where he/she sees the
virtual image of the left (illuminated) screen. The
procedure described above is then repeated.

Diagnostic interpretation of the Hess chart

The diagnostic interpretation of the Hess chart
is done by comparing the two fields, i.e. one, of
the left eye plotted while the right eye fixing and
other, of the right eye plotted while the left eye
is fixing. The interpretation should be done as
follows:
1. Compression of the space between the two
plotted fixation points indicates underaction
of a muscle acting in that direction.
2. Expansion of the space between the two
plotted fixation points indicates overaction of
the muscle acting in that direction.
3. Smaller field belongs to the eye with the Fig. 6.27 Hess chart of a patient with right lateral rectus
palsy.
paretic muscle (Fig. 6.27A).
4. Non-affected eye shows the larger field (underaction). In many cases, displacement in
expressing the overaction of the contralateral the direction of the field of the antagonist (due
synergist (Fig. 6.27B). to contracture) may also be seen.
5. Fields of similar shape and size are suggestive 7. In greater field, the greatest displacement
of comitant deviation, while the fields of (expansion) away from the normal cardinal
dissimilar shape and size indicate incomitance. direction will indicate the overacting muscle
6. In the smaller field, the greatest displacement (contralateral synergistic or yoke muscle of the
(compression) away from the normal cardinal paretic muscle). In many cases (especially in those
direction will indicate the paretic muscle of long duration), there may also be displacement
 Evaluation of a Case of Strabismus and Orthoptic Instruments 127

of the field away from the direction of the Procedure

antagonist of this muscle (due to inhibitional The test is performed on the perimeter using a
palsy of the contralateral antagonist). central chin rest. The patient fixates a small
(3.5 mm) movable white target in the primary
IV. FIELD OF BINOCULAR FIXATION position, which is then moved along the arc until
It should be tested in patients with incomitant diplopia results or it goes out of the fixation
squint, where applicable, i.e. if patient has some limits. This point is recorded. The arc is then
field of binocular single vision. The area of moved on successively in 15° steps and the test
binocular single vision is opposite to the repeated for each position until the whole field
direction in which ocular motility is impaired. has been examined. Normal field of fixation is
In general, the field of binocular fixation shown in Fig. 6.28.
represents the extreme limits of conjugate If the patient has diplopia in central position
movement of the eyes in all directions in the fixation, the target is moved in the periphery till
absence of any movement of the head. the target becomes single or the target goes out

Fig. 6.28 Field of fixation: A, left eye; B, right eye and C, binocular.
 128 Theory and Practice of Squint and Orthoptics

of fixation point. The record of binocular field V. BIELSCHOWSKY'S PHENOMENON TEST

of fixation is completed as above. This test is performed for confirming the
The diplopia as well as binocular fixation is diagnosis of alternating sursumduction
better appreciated, when the test is performed suspected on alternating cover test. The eye
using red and green goggles in front of the eyes under cover deviates upwards and extorts. When
and a movable spot of white light as target. In the cover is removed, the eye slowly rotates
the area of binocular single fixation, the target downward to return to its previous position.
spotlight will appear as mixture of red and To perform the Bielschowsky phenomenon
green, and when binocular fixation is lost it will test, patient is asked to fixate a spotlight with one
appear red or green. eye and the other eye is covered by an occluder.
It is standard to shade the area of binocular It is observed that the eye under cover moves up
single vision after plotting on the chart. In the and extorts. Then, a filter is held before the
presence of suppression, the test can be fixating eye, keeping a watch on the eye under
performed with filters, but this may diminish cover, which moves downward and intorts. This
the fusional response. In general terms, the field influence of changing the light stimulus in the
of binocular fixation is more or less circular with fixating eye on the deviation of the covered eye
a radius of about 45–50° from the fixation point is known as the Bielschowsky phenomenon. Its
in the primary position, except below when it is presence confirms the diagnosis of alternating
restricted on either side by the nose (Fig. 6.33C). sursumduction made on alternate cover test.
In a patient with paresis of an extraocular
muscle, it may be helpful to record a patient's VI. BIELSCHOWSKY'S HEAD TILT TEST
successive fields of binocular fixation on the same This test was originally recommended by
chart, thus making it easy to observe the clinical Bielschowsky to differentiate between superior
cause of the condition. As an example, Fig. 6.29 oblique palsy in one eye and superior rectus palsy
shows field of binocular fixation before and after in the contralateral side. The modified three-step
operation (on the same chart) in a patient having technique of Bielschowsky's head tilt test is used
3rd nerve palsy of 2 years duration. for the diagnosis of paretic vertical recti and
oblique muscles. For details, see pages 293–297.

SENSORY EVALUATION: ASSESSMENT FOR

BINOCULAR CO-OPERATION AND
SENSORY ANOMALIES
Normal binocular single vision consists of three
grades: Simultaneous perception, fusion and
stereopsis. It is maintained with central fixation
and normal retinal correspondence.
There are variety of sensory adoptations that
occur in response to clinical situations that
disrupt binocular vision. The devlopement of a
specific type of sensory adaptation depends on
when (age of the patients) the sensory anomaly
occurred and the severity and type of binocular
disruption.
Visually mature patients may develop
following sensory adaptations:
• Diplopia,
Fig. 6.29 Field of binocular fixation before and after ex- • Confusion, or
traocular muscles surgery in a patient with third nerve palsy. • Rivalry
 Evaluation of a Case of Strabismus and Orthoptic Instruments 129

Visually immature patients may develop B. TESTS FOR THE STATE OF RETINAL CORRESPONDENCE
following sensory adaptations: Assessment for the state of retinal corres-
• Monofixation syndrome, pondence is neccessary only in the presence of
• Anomalous retinal correspondence (ARC), or a constant manifest deviation. It is absolutely
• Large regional suppression essential to know the state of monocular fixation,
Amblyopia, not actually a sensory adaptation whether it is eccentric or central, so that this can
may occur as a consequence of suppression. be taken into account, when evaluating the
Sensory adaptations and amblyopia are results of the various tests.
described in details on page 177–201. In the absence of normal retinal corres-
Tests for binocular co-operation and sensory pondence (NRC), a patient with strabismus may
anomalies are as given below. develop anomalous retinal correspondence
(ARC). ARC is an unstable secondary adaptation
A. TESTS FOR FIXATION BEHAVIOUR of sensory interaction between the two eyes that
Fixation behaviour should be tested in each has developed under conditions of everyday
patient with strabismus having vision less than stimulation and exists under these conditions.
6/6 Snellen's. It can be tested with the help of a The tests employed to evaluate state of retinal
visuscope (page 140) or fixation star of the correspondence are described here in decreasing
order of their similarity to normal circumstances.
ophthalmoscope. Patient is asked to cover one
eye and fix the star with the other eye. Fixation 1. Striated glass test (Bagolini test)
may be centric (normal on the fovea) or eccentric
This test, performed with the Bagolini striated
(which may be unsteady, parafoveal, para-
glasses, is closest to everyday visual conditions.
macular, centrocaecal, paracaecal or temporal;
The eyes are not dissociated during the test and
Fig. 6.30). The preliminary checking of fixation
can be observed by the examiner.
should be done without dilating the pupil, since
this would be an obstacle to the pursuit of the Bagolini's striated glasses (sometimes referred
rest of the diagnostic tests. However, in the end, to as lenses) are in fact glass plates without
pupils should be dilated and fixation test refractive power. The glass plates contain
repeated along with the detailed fundus extremely fine parallel striations on the surface.
examination. When looking through them, a spotlight appears
as a fine streak of light perpendicular to the
A steady central foveal fixation is a good
striations. The principle basically is the same as
prognostic sign. An unsteady but central foveal
for the Maddox rod except that the patient can
fixation indicates a possibility of good vision
actually see through Bagolini's glasses. The
with conventional occlusion while a steady
glasses are mounted so that they can be inserted
paramacular or peripheral eccentric fixation
into a trial frame. Marks on the glass indicate
indicates a poor prognosis. the direction of the streak seen by the patient.
Procedure to perform the test (Fig. 6.31).
Preferably the test should be performed in a
room with subdued light. The test is performed
for distance (6 metres) as well as near (33 cm).
Patient is instructed to fixate on a spotlight. The
striated glasses are placed in a trial frame with
their axis oriented respectively at 45° and 135°,
so that a normal subject would see two streaks
of light forming a × intersecting at the spotlight
(Fig. 6.31A). In a patient with strabismus, one
of the following observations may be made:
1. A patient with a constant tropia having
normal retinal correspondence (NRC) with no
Fig. 6.30 Types of fixation. demonstrable suppression will experience
130 Theory and Practice of Squint and Orthoptics

diplopia, i.e. will see two spotlights each one 2. In the presence of suppression of one eye, the
crossed by one streak of light (Fig. 6.31B). patient will see the spotlight crossed by the line
According to the deviation, they will be seen in front of the non-suppressing eye only
either in crossed or in uncrossed diplopia. (Fig. 6.31C).

Fig. 6.31 Bagolini's striated glass test (for explanation see text).
 Evaluation of a Case of Strabismus and Orthoptic Instruments 131

3. A patient with harmonious anomalous retinal • When the patient sees one red light and one
correspondence will see a perfect cross, as seen white light, it indicates either normal retinal
by a normal person (Fig. 6.31A), but the cover correspondence or unharmonious ARC (if the
test will show the presence of a tropia. separation of the images is not compatible
4. Two streaks, but only one crossing through with the angle of deviation).
the centre of the light (the other one being • When the patient sees only one red light, it
displaced away from the light with a portion of indicates suppression of the deviating eye.
it missing), indicate a suppression area with • When the patient sees a mixture of red and
either normal retinal correspondence or white or a light red light, it indicates
unharmonious ARC (Fig. 6.31D). probability of harmonious ARC.
5. In the presence of a small angle tropia, if the
patient sees a perfect cross as seen by a normal Advantages
person (Fig. 6.31A), and there is no movement The test is very simple and can be performed in
on cover test, NRC is indicated (although there children of average intelligence who are as
may be lack of bifoveal fixation). Parks records young as 4 years of age.
such cases as having 'unknown retinal Disadvantages
correspondence'.
It is difficult to differentiate between fusion of
Advantages of Bagolini's test the images and suppression of the deviating eye,
• This test is closest to the everyday visual since even in binocular vision, the fixating eye
conditions, i.e. there is minimal interference will be dominant and the image will tend to
with normal visual condition since the patient appear red.
can see with both eyes.
• It is a simple and easy test both for the patient 3. Prism bar and red filter test
and the examiner. Even a child can describe To perform this test, patient is asked to fixate a
exactly what he sees. spotlight at 6 metres distance and a prism bar
• The test can be performed for any fixation cover test is carried out with prism bar in front
distance. of the fixating eye, till the deviation is neutra-
• Since the eyes are not dissociated during the lized. The prism bar reading at this point equals
test, these can be observed by the examiner. the objective angle of squint. A red filter is then
Disadvantages placed in front of the deviating eye and the
• The test is only qualitative since the angle of patient is asked to describe what he/she sees.
anomaly cannot be measured. The various possibilities are as below:
• Small angles of anomaly may be over- 1. Patient may suppress one eye, i.e. he/she does
looked. not see red light or a mixture of red and white
light. This makes the test useless.
2. Diplopia test 2. In the presence of normal retinal corres-
To perform this test, patient's deviation is first pondence (i.e. when the foveae have a
determined objectively and the diplopia test is common visual direction), the patient may see
then performed under the same conditions (i.e. the light as a blend of red and white.
same fixation distance and refractive correction) 3. In the presence of ARC, i.e. when the foveae
to permit comparison. In the diplopia test, the have different visual directions, the patient
patient fixates a spotlight on the centre of a will see two lights, a white and a red one. In a
tangent scale through a red filter and the patient with esodeviation, the diplopia will be
deviating eye is uncovered. To begin with, each crossed and with exodeviation uncrossed
eye is covered alternately, so as to show him, that (paradoxical diplopia).
the fixation light and the tangent scale or screen 4. To measure the angle of anomaly, in the
is seen with one eye and red spot of light with presence of ARC, the prism bar is now moved
the other eye. When both eyes are uncovered, the slowly (decreasing the base-out strength for
patient may see one or two lights as follows: esodeviations or the base-in strength in
 132 Theory and Practice of Squint and Orthoptics

exodeviations) until the diplopia disappears

or until the type of diplopia is reversed. The
prism bar value at this point equals the
subjective angle of squint. The difference
between the objective and subjective angles
represents the angle of anomaly.

4. Synoptophore test
To detect ARC by synoptophore method,
objective and subjective angles of the squint are
measured using dissimilar slides (e.g. lion and
the cage) as discribed on page 112, respectively,
and the results are interpreted as below:
1. If the objective and subjective angles of the
squint coincide, normal retinal correspondence
(NRC) is present.
2. If the objective angle is greater than subjective
angle, the anomalous retinal correspondence
(ARC) is present; and the difference between
these angles is called the angle of anomaly, when
the angle of anomaly is equal to the objective
angle, i.e. when subjective angle is zero, the ARC
is harmonious. In unharmonious ARC, angle of
Fig. 6.32 Worth's four-dot test.
anomaly is smaller than the objective angle.

5. Worth's four-dot test 4. If the patient sees only three green lights, he/
she has right suppression (Fig. 6.32D).
For this test, patient wears red-green goggles
5. When the patient sees three green lights and
with red lens in front of the right eye and green
two red lights alternately, it indicates presence
lens in front of the left eye and views a box with
of alternating suppression.
four lights—one red, two green and one white
(Fig. 6.32A). Since the lights are of the colours 6. If the patient sees five lights (2 red and 3
complementary to those of the filters before the green), he has diplopia (Fig. 6.32E).
patient's eye, he/she can see the red light only
6. Bielschowsky’s after image test
through the red filter and the two green lights
only through the green filter. The white light can In this test, patient's right fovea is stimulated
be seen with both eyes. with a vertical bright light and left fovea with a
horizontal bright light (Fig. 6.33A) for 15 seconds
Depending upon the patient's observation, the
each and the patient is asked to draw the
results are interpreted as below:
position of after images. Perception of the after
1. If the patient sees all the four lights (one red, images is easiest, when the patient closes his/
two green and one white or red or green or her eyes or when he/she looks at a blank screen.
mixture of red and green) in the absence of The results are interpreted as below:
manifest squint, he/she has normal binocular 1. A patient with normal retinal corres-
single vision (Fig. 6.32A). pondence will draw a cross (Fig. 6.33B).
2. With abnormal retinal correspondence (ARC), 2. A right esotropic patient with ARC will draw
patient sees all the four lights as above even vertical image to the left of horizontal image
in the presence of a manifest squint (Fig. 6.33C).
(Fig. 6.32B). 3. A right exotropic patient with ARC will draw
3. If the patient sees only two red lights, he/she vertical image to the right of horizontal
has left suppression (Fig. 6.32C). (Fig. 6.33D).
 Evaluation of a Case of Strabismus and Orthoptic Instruments 133

Fig. 6.33 Bielschowsky's after image test (for explanation, see text).

4. A response showing the images in a crossed patient sees vertical and horizontal lines
position in exotropia (Fig. 6.33E) and in an alternately.
uncrossed position in esotropia (Fig. 6.33F)
indicates the presence of paradoxic diplopia Disadvantages
in the presence of ARC with eccentric fixation. 1. The after image test is the most unphysiologic
5. The patient may draw only vertical image (in of all the tests for ARC, since an after image
left suppression) or only horizontal image (in and a normal visual stimulation are so
right suppression). In alternate suppression, different that they cannot even be compared.
 134 Theory and Practice of Squint and Orthoptics

2. Small children do not understand what they fixate through a plane mirror or prism (which
should observe. changes the direction of fixation) (Fig. 6.34A).
The examiner projects the star of the visuscope
7. Cupper's binocular visuscope test on the patient's fovea and asks the patient to tell
In this test, the patient sits 5 metres away from its position on the Maddox scale in respect to
a Maddox scale and is asked to fixate the light the central fixation light. The results are
on the centre of scale with fixing eye and the interpreted as below:
examiner looks the images of the visuscope on 1. In the presence of normal retinal correspon-
the retina of patient's deviated eye. Since it may dence, the patient sees star superimposed on
be difficult for the examiner to look through the the fixation light (Fig. 6.34B).
visuscope without blocking the patient's view 2. In the presence of ARC, patient sees star to
of the fixation object, i.e. the patient is asked to the right or left of the fixation light depending

Fig. 6.34 Cupper's binocular visuscope test (for explanation, see text).
 Evaluation of a Case of Strabismus and Orthoptic Instruments 135

upon the deviation. The number on the A. Diagnostic uses of orthoptic instruments
Maddox scale coinciding with the star gives 1. Measurement of angle of deviation (subjective
the angle of anomaly (Fig. 6.34C). and objective).
After the presence of ARC is established, the 2. Measurement of range of fusion.
examiner moves the visuscope until the star and 3. Measurement of accommodative conver-
the fixation light coincide, and at this point, the gence/accommodation (AC/A) ratio.
examiner notes the position of the star on the 4. To know the sensory status of binocular vision
patient's retina. This peripheral point on the and to detect the sensory anomalies such as
retina of the patient's deviated eye has acquired suppression, amblyopia and ARC.
a common visual direction with the fovea of the 5. To evaluate for stereoacuity.
dominant eye. This point is not always the same 6. To evaluate the motor status of binocular
as the one used for eccentric fixation. In other vision.
words, the angle of anomaly is not always B. Therapeutic uses of orthoptic instruments
identical with the distance between the fovea 1. Exercises to improve the fusional range.
and the retinal point used for fixation. 2. Exercises to improve the relative convergence
Disadvantages or relative accommodation.
The binocular visuscope test is difficult to 3. Anti-suppression exercises.
perform with young children. 4. Amblyopia therapy.
Evaluation of tests for retinal correspondence Working principle of orthoptic instruments
A great disparity between the results of various Working of most orthoptic instruments is based
tests performed for evaluation of state of retinal on the fact that they either allow or detect the
correspondence is reported in the literature. In dissociation of fusion of binocular vision.
general, as stated earlier, the tests that interfere The common modes by which an orthoptic
least with the ordinary conditions of seeing (e.g. instrument can cause dissociation of two eyes
Bagolini's test) show more ARC response and the are as follows:
tests which cause most dissociating conditions
1. Use of septum so that each eye sees the
(e.g. after image test) show less ARC response.
different half of the field, as in Maddox wing,
C. ASSESSMENT FOR GRADES OF BINOCULAR diploscope, Remy separator, cheiroscope and
SINGLE VISION pigeon-cantonnet stereoscope.
Assessment for grades of binocular single vision 2. Use of two tubes, one in front of each eye as
(BSV) is essential, since its achievement is the in synoptophore.
ultimate goal in the management of a case with 3. Use of red and green complimentary glasses
strabismus. As stated earlier, the three grades of one in front of each eye.
BSV include simultaneous perception (first 4. Use of polaroide glasses.
grade), fusion (second grade) and stereopsis 5. Use of striations as in Bagolini's glasses.
(third grade). Various tests employed to assess 6. Use of cylinderical lenses as in Maddox rod.
the state of BSV have been described on page 58.
Types of orthoptic instruments
D. TESTS TO ASSESS SUPPRESSION AND AMBLYOPIA • Conventional, i.e. non-computerised orthoptic
See pages 171 and 186. equipment, and
• Computerised orthoptic programs see
page 149.
ORTHOPTIC INSTRUMENTS
GENERAL CONSIDERATIONS CONVENTIONAL (NON-COMPUTERISED)
Uses ORTHOPTIC INSTRUMENTS
The orthoptic instruments are required for Like any other branch of science, the science of
diagnostic, therapeutic or both purposes. orthoptic and strabismus is also advancing and
 136 Theory and Practice of Squint and Orthoptics

changing fast. With time, certain instruments 2. Teller acuity cards with screen
have become obsolete and some have become 3. Optokinetic nystagmus drum
less important. For example, even synoptophore 4. VER and electronystagmography
is no more considered an essential equipment 5. System perimeter
for orthoptic set-up. However, its persence do 6. Camera for documentation
adds grace to the orthoptic clinic. Description
Non-priority additional orthoptic instruments
of certain instruments which are used only for
1. Livingston binocular gauge
diagnostic purposes has been given along with
2. Remy separator
the diagnostic tests under the evaluation of a
case of strabismus. 3. Reading bars
A few other important orthoptic instruments 4. Cheiroscope
which have not been described elsewhere will 5. Neutral density filters and graded density
be described in this section. Orthoptic instru- bar
ments can be grouped as below: 6. Maddox wing (page 111)

I. Essential orthoptic instruments IV. Orthoptic instruments not used presently

The bare minimum equipment required for the 1. Bishop-Harman diaphragm
clinical work of a patient with strabismus are: 2. Stereoscope (Holmes, Keystone)
1. A refraction trial set with prism of 1–8 D 3. Projectoscope
2. Snellen's vision chart and single letter E-chart. 4. Visuscope
3. Prism bars, horizontal and vertical (page 116) 5. Euthyscope
4. Loose prism set 6. Co-ordinator
5. Fixation targets, for near and distance 7. CAM vision stimulator
6. Occluders
8. Pigeon-Cantonnet stereoscope
7. Bagolini's striated lenses (pages 129)
9. Tibbs binocular trainer
8. Red and green goggles
9. Maddox rods (page 109) 10. Diploscope
10. Direct ophthalmoscope
SYNOPTOPHORE
11. Transparent foot ruler
Synoptophore (major amblyoscope) is a
II. Desirable orthoptic instruments haploscopic device. Though not an essential
These instruments, when present, add grace and instrument but its presence is most desirable in
completeness to the orthoptic clinic. These an orthoptic clinic. It essentially consists of two
include: tubes, having a right-angled bend, mounted on
1. Synoptophore a base having a chin rest and a forehead rest
2. Random dot stereo test (page 85) (Fig. 6.35). Each tube contains a light source for
3. Hess screen (page 123)
4. RAF rule (page 118)
5. Worth four dot test (page 132)
6. Indirect ophthalmoscope
7. Spielman’s occluder

III. Additional orthoptic instruments

There is no limit to additional orthoptic
instruments. Additional orthoptic instruments
can be grouped as below.
Priority additional orthoptic instruments
1. Haidinger brushes and after images
attachment for synoptophore. Fig. 6.35 Synoptophore.
 Evaluation of a Case of Strabismus and Orthoptic Instruments 137

Synoptophore slides
The pair of slides used to perform various
diagnostic and therapeutic purposes include the
following.
1. Simultaneous perception slides. Two dissimilar
slides, such as one having picture of a bird and
the other of the cage, constitute a pair of
simultaneous perception slides (Fig. 6.37A).
Each slide is presented separately to each eye.
Ideally, the pictures should not have
overlapping contour since this will induce
suppression. These slides are graded by their
Fig. 6.36 Optical principle of synoptophore. size into three groups:
a. Simultaneous foveal perception (SFP) slides. This
illumination of slides and a slide carrier at the pair consists of small sized pictures, the
outer end, a reflecting mirror at the right-angled images of which do not exceed the size of
bend and an eyepiece of +6.5D at the inner end the fovea.
(Fig. 6.36). The two tubes can be converged, b. Simultaneous macular perception (SMP)
diverged and moved vertically separately or slides. The pictures in this pair of slides are
together by means of knobs. The tubes can also slightly larger than those on the SFP slides.
be adjusted to the patient's interpupillary c. Simultaneous paramacular perception (SPP) slides.
distance. Each slide carrier can be rotated to These slides have the largest pictures and form
adjust for any torsion. The horizontal, vertical images that extend into paramacular areas.
and torsional positions of each tube with regard
to normal zero position can be read on scales in (Note: As a routine, if possible, the smallest
either degrees or prism dioptres. slides should be used. However, the larger slides
The graduations from the zero mark inward may be required in the presence of suppression
represent base-out prisms or degrees of or amblyopia).
convergence (+), while those from the zero mark 2. Fusion slides. Fusion slides consist of two
outward represent base-in prisms or degrees of similar pictures, each of which is incomplete in
divergence (–). one small detail. For example, there are two
Light switches permit the simultaneous or rabbits each lacking either a tail or a bunch of
alternate illumination of the tubes, useful for flowers. If fusion is present, one complete rabbit
performing the cover tests. with tail and holding a bunch of flowers will be

Fig. 6.37 Synoptophore slides for simultaneous perception (A), fusion (B) and stereopsis (C).
 138 Theory and Practice of Squint and Orthoptics

seen (Fig. 6.37B). In the presence of suppression, 3. Eccentric fixation.

either tail or bunch of flowers will be missing in 4. Accommodative esotropia (dissociation
the respective eye. training).
Grading.The fusion slides are also graded 5. Heterophorias and intermittent heterotropias
according to the size in the same way as the (improvement of fusional amplitude).
simultaneous perception slides.
3. Stereoscopic slides. Stereoscopic slides consist Measurement of anlge kappa with synoptophore
of two pictures of the same object which have To measure the angle kappa with synoptophore,
been taken from slightly different angles, i.e. the a special slide is placed in front of the eye under
picture for one eye is in part dissimilar from that observation. This slide consists of a row of
for the other eye. These dissimilar parts are numbers and letters (4 3 2 1 0 A B C D) and
imaged on disparate retinal areas in the two eyes animal pictures (for small children and illiterate
and, when the entire picture is fused, the patients) placed at 1° intervals (Fig. 6.38). The
disparity gives rise to the perception of patient is asked to focus on the ‘0’ mark while
stereopsis of the dissimilar portions (Fig. 6.37C). the examiner looks for the corneal reflex. If the
corneal reflex is on the nasal side of centre of
Uses of synoptophore pupil, the angle is positive; if it is on the temporal
Diagnostic uses side, it is negative. The patient is then asked to
1. Measurement of the objective and subjective look in turn either one letter or one number until
angles of deviation (page 112). the reflex is centred. The degree of deviation
corresponding to the letter or number is then
2. Measurement of the primary and secondary
recorded. For example, if the left eye is being
deviations.
tested and the corneal reflex is centred, when
3. Measurement of deviation in cardinal
the patient looks at the number 3, the patient
directions of gaze.
has 3° negative angle kappa in the left eye.
4. Measurement of interpupillary distance (IPD)
5. To investigate the state of retinal corres- Measurement of interpupillary
pondence (page 129). distance with synoptophore
6. Estimation of grades of binocular vision To measure the IPD, arms of the synoptophore
(page 57). are placed at zero and the patient is instructed
7. To estimate presence and type of suppression to look at the centre of the picture in the right
(page 168). hand tube with his/her right eye. The examiner,
8. Measurement of range of fusion or vergence with his right eye closed, aligns the central white
(page 116). line which is on the mirror unit of the tube, with
9. Measurement of angle kappa.
the reflection of the light on the centre of the
Therapeutic uses patient’s pupil. The same procedure is repeated
It is used in the treatment of: with the patient fixing with the left eye and the
1. Suppression (page 168). examiner closing his right eye. The IPD is then
2. Abnormal retinal correspondence. read on the millimetre scale.

Fig. 6.38 Synoptophore slide for measurement of angle kappa.

 Evaluation of a Case of Strabismus and Orthoptic Instruments 139

Digital synoptophore Procedure

Digital synaptophore, introduced recently, is Before examination, the interpupillary distance
likely to soon replace the currently used electric (IPD) for the patient must be first measured in
synoptophore for sensory and motor assessment millimetres. The distance between the eyepieces
of strabismus patients as well as measurement is then set according to this measurement. The
of the angle of deviation. patient places his chin on the chin-rest and his
forehead against the headrest. The chin-rest is
Features of digital synaptophore adjusted in such a way that the eye level is at
Digital Synoptophore (Fig. 6.39) comprising of the centre of screen vertically.
a computer controlled display device consists
of both hardware and software components: Uses
Hardware component consists of various It is used to perform the following tests on
subunits as described below: patients with strabismus:
• Computer system with colour monitor. • Simultaneous perception (foveal, macular and
• Screen divider. An opaque vertical screen paramacular)
divider, to divide the display screen into 2 • Fusion
equal right and left halves. • Stereopsis
• Input device to move the generated screen • Assessing retinal correspondence
targets, such as mouse, trackball, etc. • After image testing
• Two tubes to carry the images from the display • Measure the angle of deviation
screen to each eye separately which contain a • Orthoptic exercises
lens system and eyepieces assembly with
adjustable interpupillary distance. LIVINGSTON BINOCULAR GAUGE
• Adjustable chin-rest and head-rest for stabilizing Livingston binocular gauge (Fig. 6.40) is an
the patients head. apparatus used for the measurement of
Software component consists of various computer convergence and accommodation. Basically, it
programs designed for various orthoptic test. consists of a 36 cm long wooden ruler marked

Fig 6.39 Hardware component of digital synoptophore

 140 Theory and Practice of Squint and Orthoptics

2. Measurement of subjective convergence. After

fitting the ruler on the patient's face, the central
convergence rod is removed and the box-like
attachment is kept farthest from the patient. The
patient is asked to look at the black vertical line
placed in the centre of vertical limb of cross-
opening while the box is slid towards the
patient's face. The point where the patient
Fig. 6.40 Livingston binocular gauge.
observes that the line has moved slightly to the
right or to the left or has become double is noted
in centimetres and half-centimetres. In the centre on the scale. This reading gives the measure-
of this ruler, from 6 cm mark to 21 cm mark, ment of subjective convergence. The normal
there is a slot into which is present a slidable value of subjective convergence is less than
convergence rod (white vertical rod, the centre 20 cm, but is almost invariably greater than that
third of which is painted black). One end of this of objective convergence.
ruler is so designed that when in position, it 3. Measurement of accommodation. The instru-
straddles the patient's nose and rests upon his/ ment is used in the same way as used for
her cheek bones. At this facial end of the ruler, a measuring subjective convergence; except that
detachable occluder is attached which can be now the patient is asked to look at the letters
used to occlude either eye while measuring placed corresponding to the horizontal limb of
uniocular accommodation. In this position, the the cross opening of the box-like attachment. The
markings on the ruler indicate distance from the point where the patient reports blurring of the
anterior surface of cornea. At the other end of letters gives the reading of his/her near point
the ruler, there is a box-like attachment which (punctum proximum).
can slide towards the eye. This box-shaped
attachment is 6 cm wide and has a cross-like VISUSCOPE
opening (cut in the surface facing the patient) It is an instrument similar to ophthalmoscope
through which the back surface of the box (Fig. 6.41) which is used to examine the fixation
consisting of a white rectangular card with a pattern of patients during monocular vision. It
central black vertical line (opposite the vertical was designed by Cupper. With the help of this
limb of the cross-opening) is seen. The black instrument, examiner projects a disc with a
vertical line is used for measuring the conver- green filter having a star in the centre and
gence. On either side of the black vertical line, surrounded by concentric rings on to the
there are three black horizontally placed patient's fundus. The distance between the
letters—ALT, opposite the horizontal limb of the concentric rings is ½o. The patient is asked to
cross-opening. These letters are used for look into the star after occluding the eye not
measuring accommodation.

Uses of livingston binocular gauge

1. Measurement of objective convergence. The
ruler is fitted on the patient's cheek bone. The
convergence rod is kept farthest from the patient,
who is asked to continuously look at the central
black section of the rod which is moved steadily
towards the patient's eyes. The examiner notes
the scale reading, where the patient's one or both
eyes diverge on the loss of binocular fixation.
Normally, the objective convergence varies
between 6 and 10 cm in young adults. Fig. 6.41 Examination of fixation pattern with visuscope.
 Evaluation of a Case of Strabismus and Orthoptic Instruments 141

being examined. Normally, the foveolar reflex with white background. On this card are printed
of the patient coincides with the star if the letters DOG, with a green sqaure placed
fixation is central. In the presence of eccentric centrally above the O and a red square centrally
fixation, the star, will not coincide with the below the O. About 6.5 cm in front and parallel
foveolar reflex and can be anywhere on either to the card holder is mounted a metal septum
the nasal or temporal retina or above or below which is perforated by four holes, each 8 mm in
the fovea (Fig. 6.20). The degree of eccentricity diameter. The two holes are situated hori-
may be known from the concentric ring with zontally 15 mm apart from each other and at an
which the star coincides. equal distance from the centre of the septum.
The other two holes situated vertically, one
EUTHYSCOPE below the horizontal left-hand hole and other
Euthyscope is a modified form of ophthal- above the horizontal right-hand hole.
moscope (Fig. 6.41) which is used in pleoptics When in use, the septum dissociates the two
for the re-education of the fovea that has lost its eyes in such a way that each eye can see only
principal visual direction in eccentric fixation. two of the three letters on the white card and
This instrument projects an approximately 30o only one of the two-coloured squares. The left
wide beam of light in the centre of which an eye sees the letters OG and the lower red square,
opaque 3° or 5° disc can be moved to cause a while the right eye sees the letters DO and upper
black dot. This serves to shield the fovea during green square (Fig. 6.43).
the exposure of the surrounding retina. Since the
light intensity used is moderate, the peripheral Uses
retina is not dazzled but only stimulated enough 1. Suppression and the presence or lack of
to produce an after image. A green filter disc binocular vision can be detected. With normal
enables the examiner to locate the fovea without retinal correspondence and bifoveal fixation,
dazzling the patient's retina. Since pleoptic when the two images of O are fused, the patient
treatment is now obsolete, so this instrument is will see three holes with the word DOG in them
also not used in the modern orthoptic clinics. (Fig. 6.43).
DIPLOSCOPE 2. The main use of the instrument is to exercise
for relative convergence, when binocular
The diploscope consists of a 25 cm long metal single vision is present. To perform exercise,
shaft supported by a handle having face-piece patient is asked to move his/her eyes in
at one end and a card holder at the other end. relation to septum and card at four different
Depending upon the model, the face-piece can positions (described below). As the patient
rest on the nose (Fig. 6.42), the cheek bones, or does so, he/she sees a change in the relative
the upper lid. The card holder contains a card position of the letters and colours as perceived
by each eye simul-taneously. This movement
of letters into a definite pattern is utilized in
training the patient to appreciate and control
the position to which his/her eyes are
directed. Thus, it teaches the patient to switch
easily from distant to near fixation and vice
versa improving the fusional amplitudes
which are essential for a comfortable binocular
single vision.

Procedure
The four positions of fixation and the various
kinds of physiological diplopia, when practising
Fig. 6.42 Diploscope. with the diploscope, are as follows (Fig. 6.43).
 142 Theory and Practice of Squint and Orthoptics

Left eye Right eye

Fig. 6.43 Principle of diploscope and observations made by the patient while in use at positions 1, 2, 3 and 4 (for explanation,
see text).

Position 1. The point of fixation is central letter Position 2. The second point of fixation is the
O on the card. In this position, letter D falls on a centre of metal septum midway between the two
point temporal to the fovea in the right eye and horizontally placed holes (Fig. 6.43). When the
is projected to the left of O, while G falls on point patient's eyes converge on this point, the images
of O no longer fall on both foveas, but on a retinal
temporal to the fovea in the left eye and is
element nasal to the fovea in each eye. Consequen-
projected to the right of O. Thus, the letters DO tly, the O will be seen in uncrossed (homonymous)
are seen with the right eye and the letters OG diplopia and the patient sees DO and OG. When
with the left eye and in the presence of binocular the patient will exert a greater amount of
single vision patient will perceive three holes convergence, he/she may see only DG, because
with the word DOG in them. the D and O and the G and O will overlap.
 Evaluation of a Case of Strabismus and Orthoptic Instruments 143

Position 3. The point of fixation is tip of a pencil and will be seen superimposed by the patient
or other object held midway between the septum (Fig. 6.45).
and his/her eyes. When the patient's eyes Uses. The instrument is designed in such a
converge on this point, the images of both D and way that when used properly, it teaches the
O in the right eye and G and O in the left eye fall patient to relax his/her convergence and
on a retinal element nasal to fovea in each eye strengthen the fusional divergence.
and thus will be projected temporally, and the
patient will see OGDO on the card. READING BARS
Position 4. The point of fixation is an object (such
Reading bars are simple devices used to train
as a picture on the wall) situated beyond the
the patient for subjectively controlling the
printed card. When the patient fixates at this
maintenance of binocular vision.
distant point, the images of D and O and G and
O fall on retinal element temporal to fovea in Principle. All reading bars are based on the
each eye and thus will be projected nasally, and principle of physiologic diplopia.
the patient will see D O O G. Common reading bars include thumb bar reader,
Note: The aim of exercise with diploscope is zig-zag bar reader, the Mayan bar reader, the
to teach the patient to obtain and maintain all Jaual grid, Tibb's physiologic diplopia reader.
four positions with ease so that effortless Method. By introducing a bar between the
convergence and divergence is fully established. patient's eyes and the reading material, the
It is advisable to practise for 2 to 3 minutes for 2 patient is made aware of physiologic diplopia
to 3 times a day. Position four is quite useful in (Fig. 6.46). As the patient reads the print
improving the fusional divergence (fusional binocularly, he/she perceives the bar in crossed
negative convergence). diplopia, each image of bar hiding on position of
the print from one eye, but not the other, so that
REMY SEPARATOR the print can be read normally. Maintaining the
Remy separator is a simple instrument which correct position of his/her eyes despite this
consists of a septum with a handle having a obstacle will strengthen the binocular vision of
transparent slide holder at one end and a nose- the patient. This is a very useful and simple
piece at other end (Fig. 6.44). The patient resting home exercise.
the septum on his/her nose is instructed to look
through the slides at an object beyond them. If
his/her eyes are properly focused for the distant
object, the picture of the slides (such as a star
and a circle) will be imaged one on each fovea

Fig. 6.44 Remy separator. Fig. 6.45 Optical principle of Remy separator.
 144 Theory and Practice of Squint and Orthoptics

Procedure. The picture to be copied is placed in

the picture carrier and a sheet of paper on the
base of the instrument. The patient should look
with his/her fixating eye into the mirror and
with the suppressing one on the paper. He/she
is instructed to trace the picture (which has black
outlines) on the paper using a pencil (a red-
coloured pencil is very helpful, especially in the
beginning). Steady fixation of the picture should
be stressed to prevent rapid alternation, which
can be suspected, if the patient's drawing is
Fig. 6.46 Zig-zag bar reader in use. either smaller or larger than the picutre or if
parts of the picture are missing.
CHEIROSCOPE TIBB'S BINOCULAR TRAINER
The cheiroscope (Fig. 6.47) is an instrument for The Tibb's binocular trainer (Fig. 6.48) is a
anti-suppression exercises. It consists of a haploscopic instrument designed for home use.
working base, a picture carrier to one side, a However, in offices where a major amblyoscope
headrest containing a pair of +7.0D spherical is not available, it can also be used as a
lenses, and an obliquely placed septum diagnostic instrument. It consists of three parts:
extending from the centre point between the A middle septum and two wingboards that fold
lenses to the base of the picture carrier. A plane together like a book. The middle septum has a
mirror is attached to the septum on the side mirror on both sides so that it can be used with
where the picture is located. The distance either eye fixating. Each of the wingboards has
between lenses and the working base is 14 cm, a vertical scale of 20Δ base-up and base-down
which is the focal length of each lens and the and a horizontal scale of 40Δ-base in and base-
eyes are consequently focused for distance. Most out. Four cards (target carriers) come with the
cheiroscope models may be turned around so instrument and consist of peripheral, macular,
that the mirror is in front of the right or left eye, and foveal superimposition and fusion pictures.
depending upon which eye is required to fixate. Uses. It can be used for both diagnosis and
Uses. The instrument can be used in cases of treatment of suppression and abnormal retinal
heterophorias, intermittent tropias, or small correspondence and for increasing fusional
tropias (in the latter, prisms to neutralize the amplitudes.
deviation have to be taped over the eyepiece). Procedure. One wingboard is placed so that it
rests along the table at a slight angle to it. The
vertical wingboard is to the right side, when the

Fig. 6.48 Tibb's binocular trainer. Direct cover test depicting

Fig. 6.47 Cheiroscope. left exotropia.
 Evaluation of a Case of Strabismus and Orthoptic Instruments 145

right eye is the fixating one; it is to the left side, • Computer orthoptics programe by HTS INC
when the left eye is fixating. The patient places solutions.
the bridge of his/her nose against the curved • Computer orthoptics.
part of the septum so that his/her visual axis is
perpendicular to the table. His/her head should I. COMPUTERIZED ORTHOPTIC DIAGNOSTIC
not be tilted. PROGRAMS AND INSTRUMENTS
One target carrier (such as the dog) is taped Several applications are available for smart-
on the vertical wingboard so the zero mark phones, tablets, laptop, and PCs that reproduce
shows in the window. This target is seen in the many eye tests. These can be used by
mirror. The patient places another target on the optometrists, ophthalmologists and some
horizontal wingboard (such as the cage for programs by the patients also. Computer
superimposition or another dog for fusion) and orthoptics includes complex monocular and
moves it until the two images are superimposed. binocular stimuli, which allow automatic testing
This target is not viewed in the mirror. and measurement of the following skills:
Oculomotor (pursuits and saccades); fusional
ranges; phorias; motor fields; fixation disparities,
COMPUTER-BASED ORTHOPTIC
suppressions; retinal correspondence; accommo-
PROGRAMS AND INSTRUMENTS
dative facility; stereopsis, visual memory and
Computer-based orthoptic programs have aniseikonia.
brought a revolutionary change in the diagnosis Some common such programs are described
and management of orthoptic disorders. briefly.
Computer-based orthoptic practice includes: Electronic vision testing programs. Electronic
• Computerised orthoptic-diagnostic programs charts are available for far as well as near vision
for sensory as well as motor evaluation, and testing. Few examples are:
• Computer-based orthoptic therapy programs • Chart Pro (www.eyechartprotoapp.com)
for vision therapy, and neurovision therapy. • Optos’ chart remote (blog.optos.com/index.php/
Recently many computer based systems have optos.chart.remote.ipad.app)
been developed for orthoptic practice which are • Vision Test (https://itunes.apple.com/ca/app/
either only diagnostic, or therapeutic, or combined vision-test/id380288414?mt=8 or https://
diagnostic and therapeutic programs. Some of play.google.com/store/apps/details?
these are listed below: id=com.threesidedcube.visiondroid)
I. Computer-based diagnostic programs • With sight Book (www.digisight.net/patients/
vision_testing)
• Electronic vision testing programs.
• AAPOS Vision Screening App.(www.aapos.org/
• Optodrum [Software for optokinetic drum ahp/aapos_vision_screening_app) has optotype
(OKN)]. for both adults and children, and can be used
• Digital Hess chart and diplopia chart by anyone including healthcare workers.
• BVA (Binocular Vision Assessment Program) Optodrum. The Optodrum (www.linsay.com/
• PTS (perceptual therapy system) Linsay_associates_Medical/Optodrum.html) is
• ReadAlyzer eye movement recording system a good alternative to the expensive and bulky
• TOVA (test of variable attention) optokinetic drum for adults and children. It even
has a version for the iPad that uses its camera to
• Visagraph
record a video of the patient’s eye movement
II. Computer-based therapeutic programs while looking at moving patterns.
• Vision therapy programs
BVA is a stand-alone binocular vision screening
• Neurovision therapy programs. program. It is capable of automatic testing of
III. Computer-based combined diagnostic and heterophorias, fusional range, saccades,
therapeutic systems pursuits, accommodation, suppressions, fixation
• TRYe vision therapy software disparities and an asthenopia survey.
146 Theory and Practice of Squint and Orthoptics

PTS test. Computerized perceptual therapy activities that preserve the patient’s present level
system allows automatic testing for speed of of function and/or prevent regression of that
information processing, visual sequential function. Maintenance begins when the thera-
processing, visual simultaneous processing. peutic goals of a treatment plan have been
ReadAlyzer eye movement recording system. achieved, or when no additional functional
This system allows fixations, regressions, fixa- progress is apparent or expected to occur.
tion duration, reading speed, cross correlation
between right and left eye, play-back of recorded Perceptual visual tracking program (PVT)
eye movements. This program is designed to improve specific
tracking deficits that are often found in persons.
TOVA (test of variables attention). It assesses
Perceptual visual tracking skills are basic to all
ADD, ADHD and impulsivity.
aspects of reading and other academic areas.
Visagraph an eye movement recording system Improvement in tracking is often accompanied
is capable of computerized recordings of reading by improvement in reading, spelling, attention,
eye movements, saccades and fixations. and speed of working. PVT contains a variety of
Digital Hess screen See page 123. visual tracking programs that have been clinically
proven. They are game-like in nature so that
Digital syneptophore Described on page 139.
improvement takes place almost effortlessly.
II. COMPUTER-BASED VISION THERAPY AND Individuals of all ages from five years up can
NEUROVISION THERAPY PROGRAMS benefit from PVT. It is a sophisticated yet user-
friendly computer application that will run on
VISION THERAPY PROGRAMS almost any personal computer.
Vision therapy is effective for:
• Eliminating amblyopia Vision builder: Computer vision training
• Breaking suppressions A home vision training program designed to
• Improving oculomotor skills treat both binocular vision disorders such as
• Improving visual memory convergence problems (i.e. eye-teaming problems),
• Improving accommodative facility accommodation problems (i.e. non-refractive
• Altering retinal correspondence; increasing focusing difficulties), suppression (i.e. “lazy
fusional ranges; and/or treating strabismus. eye”) and eye-tracking difficulties, as well as
The gamepad and mouse allow the patient’s visual perceptual or visual information
therapy responses to alter the target demands. processing difficulties that can impact on a
The Computer Orthoptics graphics are instantly student’s learning by limiting their ability to
moved, rotated or changed to create any base- understand and remember what they see.
in base-out disparity. A few computer-based
Eyeport @ Vision Training System
orthoptic programs are mentioned below.
Has revolutionized the way people look at their
Computer vergence system (CVS) vision. To read more comfortably, learn more
This program uses random dot stereograms to easily, work less painfully, and play sports more
form pictures that require bi-foveal fixation to effortlessly, people everywhere are doing daily
stimulate the vergence system. The program eye exercises with the EYEPORT.
gradually increases the amount of vergence
required to appreciate the stereogram picture Eyelights
and can monitor progression on line. This may Target the weaker functioning side of the brain
be used as part of the home therapy program via the non-dominant eye. Light stimulation
and the results of the computer program are directly to the non-dominant brain causes an
often followed by an eye care professional with excitatory barrage to travel to the mesen-
print outs that can be brought in to the office cephalon, the most metabolic area of the brain,
visit. A maintenance program consists of where an increase in cellular activity takes place.
 Evaluation of a Case of Strabismus and Orthoptic Instruments 147

The excitatory barrage travels also to the can be preformed monocularly or monocularly
parietal, temporal, and occipital lobes of the in a binocular field (Fig. 6.50A&B).
brain, while collateral fibers lead to the pineal • The AmbP iNet programme provides a
gland, pituitary gland, and hypothalamus. cumulative graph depicting each session’s
performance. The computer denotes date,
AmbP iNet Program time, duration and denotes when the patient
An amblyopia hand-eye coordination program has performed the assigned tasks as well as if
which uses principles of operant conditioning they were performed correctly.
and behavior modification to appropriately alter PTS II iNET. It is a home-based computerized
stimuli characteristics to improve visual acuity perceptual therapy program that has been
(Fig. 6.49A&B). designed to address a variety of visual
• Patients begin therapy with targets that are perceptual information processing domains,
easily seen and become progressively smaller including simultaneous processing, sequential
as therapy progresses. Correct responses are processing, speed-of-information processing,
reinforced with subsequent reduction in the visual temporal processing, and rapid auto-
size of the stimuli. Therapy is directed to matized naming,
improve resolving ability with concomitant • Dyslexia
use of hand-eye coordination tasks. Therapy • Ordinary reading disability

Fig. 6.49A and B AmbP iNet program.

Fig. 6.50A and B Protocol of therapy with AmbP iNet program.

148 Theory and Practice of Squint and Orthoptics

• Word decoding difficulty Brainware safari. Therapy for 14 cognitive skills

• Problems in reading comprehension in six areas.
• Spelling problems Play attention. Improves attention.
• Memory disorders Sanet vision untegrator. Procedures for saccadic
• Laterality-directionality deficits trainer, tactile feedback, hand speed function,
• Slow speed of information processing “visual search” saccadic, metronome, tachisto-
• Non-verbal learning disability scope functions.
• Mathematics difficulty
Sub iNet. Addresses subitizing deficits to
• Above average intelligence but not achieving improve math skills.
up to potential
Track and read. Twelve therapy procedures for
• Acquired brain injury with perceptual-
developing saccadic eye movements, span of
cognitive deficits
recognition, and visual sequential memory skills.
• Attention disorders
• Diagnosis of perceptual/visual information NEUROVISION THERAPY PROGRAMS
processing deficits (Fig. 6.51A&B).
1. Revital vision. Concept of neurovision
Therapy procedure. PTS II iNet presents the therapy is based on the visual plasticity, which
patient with a grid that shows the different is the ability of the visual system to change its
difficulty levels for each procedure. Each time responses in order to adapt to the changes in
the patient meets or exceeds a therapy goal they the visual input. Revital vision is a pereptual
receive a gold star. The program gives the Learning therapy program developed by
patient verbal reinforcement. It prompts them “Revital Vision Technology”. Perceptual
to “Get Ready” before a stimulus is presented. learning is an alternative treatment option which
PTS II iNet also verbally reinforces correct and modifies visual function in adult amblyopia. The
incorrect responses. For example, it will perceptual learning therapy program (Revital
encourage them by saying “Good Job” after they Vision) is a non-invasive software-based patient-
input a correct response. specific, interactive perceptual learning tool
Dynamic reader. The dynamic reader is a home based on visual stimulation. It facilitates neural
Vision Training program designed to improve connections at the cortical level through a
reading eye movements and thus reading computerized training regimen using Gabor
fluency and comprehension. patches to improve contrast sensitivity and
visual acuity. The term perceptual learning
ADR iNet. Moving text, standard, and whole describes a process whereby practicing certain
line dynamic reading visual tasks leads to an improvement in visual

Fig. 6.51A and B Diagnosis of perceptual/visual information processing deficit.

 Evaluation of a Case of Strabismus and Orthoptic Instruments 149

performance. As visual perception depends on recommended for fusional vergence testing

both the optical input received from the eye and and training.
the neural processing of that input in the visual • ‘Office vision therapy’ (TrYe-Doctor’s module)
cortex, Revital vision technology improves program for dysfunctions of binocular vision,
quality of vision (visual acuity and contrast accommodation and ocular motor system.
sensitivity) by enhancing neural processing in
• Home based vision therapy program (TrYe-
the primary visual cortex.
Patient’s module) for vision therapy at
The typical building blocks of the visual stimulus home.
in the field of visual neuroscience are:
• Gabor patch, 2. Computer orthoptics program by HTS INC
• Neuronal lateral interactions, solutionsTM. HTS iNet/Computer Vergence
• Brain (neural plasticity), and System (CVS) is a sophisticated yet easy-to-use
vergence exercise computer application that the
• Perceptual learning.
patients can run on Windows and Mac
Effects of revital vision–perceptual learning. computers. It encourages and motivates patients
Neurovision technology (Revital) is formed by continuously evaluating their progress and
around proprietary algorithms and has proved making the exercises more challenging
successful in the following ways: whenever the goals are achieved.
• Improvement of lateral interactions in To assure compliance, CVS uses random-dot
amblyopia stereograms. Each exercise requires bifovial
• Improvement of CSF in amblyopia fixation for correct responses. The responses
• Improvement of CSF in non-amblyopic required are a simple choice of four arrows keys;
groups up, down, left or right. The results of each
• Improvement of VA exercise session are documented as the program
• Transfer to improvement of binocular vision. notes the date, duration and vergence demand
of each exercise. All results are available for
2. Neurovision rehabilitator used for those with
your review via the Internet. The HTS iNet
brain injury is useful as ocular vestibular
program is fully automated and easy to use.
integrator, visual motor enhancer, visuomotor
Procedures include:
integrator, dynamic ocular motor processing,
fixation anomalies. • Pursuits
• Saccades
III. COMPUTER-BASED COMBINED DIAGNOSTIC • Base-in/base-out vergence
AND THERAPEUTIC PROGRAMS • Auto Slide Vergence
1. TrYe (Train your eyes) vision therapy software • Jump ductions
TrYe (Train your eyes) vision therapy software • Base-up/base-down vergence
has been designed and developed by ‘Digital • Accommodative rock
Works Technology Private Ltd’, using the
domain expertise of ‘Sankra Nethralya, Chennai, 3. Computer orthoptics. It includes both
India’. This program has the ability to run on orthoptic therapy and diagnostic procedure:
multiple platforms, such as PC, Laptop, Mobile • Therapy procedures include smooth vergence,
and Tablet. rotations, jump ductions, multiple choice
Uses of TrYe software include: vergence, accommodative rock, pursuits,
• Binocular vision assessment, and thus addresses saccades, visual memory, cheiroscope and
to the binocular vision dysfunctions. Binocular amblyopia therapy procedures.
vision assessment programmein TrYe is based • Diagnostic procedures for heterophobia,
on aperant conditioning using random dot fusional ranges, accommodative facility,
stereograms (RDS) as targets. RDS targets are pursuits, saccades, Worth 4 dot, motor field,
devoid of monocular cues and have been fixation disparity, visual memory, aniseikonia.
 150 Theory and Practice of Squint and Orthoptics

15. Cooper J, Feldman J, Eichler R. Relative Strength

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Review. Pediatrics 1998; 102(5):1217–1219A S, Horn D. Pupillary Dilation andFunduscopy
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 7
Anomalies of Convergence,
Divergence and Accommodation
CONVERGENCE DIVERGENCE
• Types of convergence • Fusional divergence
• Angle of convergence Anomalies of divergence
• Near point, far point, range and amplitude of • Divergence insufficiency
convergence
• Divergence paralysis
Anomalies of convergence • Acquired motor fusion deficiency
• Convergence insufficiency ACCOMMODATION
• Convergence insufficiency • Accommodation and related terms
associated with accommodative • Assessment of accommodation
insufficiency • Age-related changes in accommodation
• Convergence paralysis • Anomalies of accommodation
• Convergence spasm • Insufficiency of accommodation
• Paralysis of accommodation

CONVERGENCE convergence involved in normal visual

activities; and that not every one is capable of
Convergence is a disjugate movement in which doing or learning this.
both eyes rotate inward so that the lines of sight Comedians learn to invoke voluntary
intersect in front of the eyes. It allows bifoveal convergence to cross their eyes. Some patients
single vision to be maintained at any fixation learn to use voluntary convergence to produce
distance. Convergence remains more or less convergence nystagmus.
same throughout life. It does not deteriorate
with increasing age, unlike accommodation, but II. Reflex convergence
may deteriorate under certain abnormal It is the convergence of visual axes which is not
circumstances. The power of convergence can under complete voluntary control. Reflex
be increased by exercises. convergence has four components: Tonic,
fusional, accommodative and proximal conver-
TYPES OF CONVERGENCE gence.
Convergence is a very complex process. It may 1. Tonic convergence
be voluntary or reflex. It is that part of reflex convergence which results
from some inherent innervational tone of the
I. Voluntary convergence extraocular muscles, when the patient is awake.
It is that amount of convergence of visual axes It is the sum of excitatory and inhibitory
that can be produced at will. It is not a part of influences from different sources such as cortical
normal convergence movement as it occurs in centres, subcortical centres and vestibular
everyday vision. Voluntary convergence is thus organs. It is independent of fusion or object
a separate phenomenon from the reflex proximity. This is a form of proprioceptive eye
152
 Anomalies of Convergence, Divergence and Accommodation 153

position control that keeps the eye converged nerve impulse to accommodate is discharged to
even after one eye is occluded for a while. It is the eyes. Thus, the stimulus for accommodative
very important in determining the position of a convergence is blurred retinal images rather
person's eyes, i.e. under the influence of tonic than the retinal disparity that stimulates fusional
convergence, the eye position will be more convergence. In contradiction to fusional
convergent than before, but from an absolute convergence, accommodative convergence is
point of view, it will still be divergent. Tonic not dependent on binocular vision and occurs
convergence is most prominent in childhood even if one eye is occluded. Patients with one
and decreases with age. The emotional energy blind eye still show convergence of the eyes,
level of the individual may affect tonic when accommodating on near objects. In fact,
convergence. It disappears under deep general the accommodative convergence is a part of the
anaesthesia and after patching one eye for 30 to triad of synkinetic near reflex complex. Other two
60 minutes. components of this neurosynkinesis being
2. Fusional convergence accommodation and miosis. The quantitative
relationship between the accommodative
Fusional convergence, also called positive convergence and accommodation is expressed
fusional convergence, is the convergence that is as the AC/A ratio. This relationship is a linear
produced to ensure that similar retinal images one and is thought to be relatively stable
are projected onto corresponding retinal areas. throughout life. In it, the accommodative
It occurs without a change in refractive state of convergence is measured in prism dioptres and
the eye and is initiated by a bitemporal retinal the accommodation in lens dioptres. The AC/A
image disparity. In other words, fusional ratio, therefore, is expressed as so many prism
convergence implies a responsiveness to
dioptres per one dioptre of accommodation. The
disparate stimuli lying outside the Panum's
normal AC/A ratio is about 3 to 5 prism dioptres
fusional area. It is not a voluntary process, but
for one dioptre of accommodation.
one of the optomotor reflexes and thus forms a
kind of fusion reflex or motor fusion. Fusional The fact that AC/A ratio remains almost normal
vergence, in general, forms an important in presbyopic persons, indicates that it is the
mechanism for the achievement of bifoveal stimulus for the accommodation that evokes the
single vision. And that a fusional vergence may response of accommodative convergence rather
be a convergence, a divergence or a vertical than the amount of accommodation that actually
vergence movement. takes place. The majority of myopes have a high
AC/A ratio and hypermetropes have a low AC/
Fusional convergence is the most important
A ratio as compared with the emmetropes.
type of convergence in the study of motor
However, there is no correlation between the
anomalies. It has been found that the amplitude
degree of myopia, hypermetropia and the
of fusional convergence is greater, when attention
magnitude of AC/A ratio. The pupillary distance
is directed between the two disparate retinal
must also be considered in the determination of
images than when the attention is directed at only
the AC/A ratio, since the convergence require-
one of the two images. The normal fusional
ment for an individual with a wide interpupillary
convergence amplitude for distance is about 18D
distance is greater than for a patient with a
and for near it is 35 D. Fusional convergence helps
narrow interpupillary distance looking at the
to control exophoria (latent divergent squint). The
same fixation distance.
fusional convergence may be decreased by
fatigue or illness, converting a phoria into a Abnormalities of the AC/A ratio are very
tropia. The amplitude of fusional convergence important causes of strabismus. A high AC/A
can be improved by orthoptic exercises. ratio may cause excessive convergence and
produce a convergent squint (esotropia) during
3. Accommodative convergence accommodation on a near object. A low AC/A
It is that component of convergence which ratio may cause a divergent squint (exotropia),
occurs, when the eyes accommodate, or when a when the patient looks at a near object.
 154 Theory and Practice of Squint and Orthoptics

4. Proximal convergence The effect of IPD on the angle of convergence

Proximal convergence, also known as is usually negligible and so practically not taken
instrument convergence, is that component of into consideration while measuring the
reflex convergence which is induced by the convergence angle. Convergence angle can be
proximity of the object of regard or the measured in metre angles or in prism dioptres.
awareness of the proximity of a near object. It Metre angle
appears to be initiated by psychological factors, One metre angle convergence is exerted by each
since it occurs also when a subject just believes eye, when the eyes are directed to an object at a
that he/she is looking at a near object although distance of one metre of the meridian line
he/she actually is not. For example, while using between the two eyes (Fig. 7.2). The convergence
the haploscope optically set at infinity, proximal exerted in metre angle (ma) by each eye is inversely
convergence is often induced. proportional to the distance in metres the object is
There exists a linear relationship between infront of the eyes, i.e. it would be 0.5 ma at
proximal convergence and the changes in 2 metres and 2 ma at half metre (Fig. 7.2).
fixation distance, similar to accommodative In an emmetropic eye, the number of dioptres
convergence. Here the change in fixation of accommodation required to see an object
distance is expressed as changes in the vergence clearly is equal to the number of metre angles
of light, i.e. in dioptres. Thus a change in fixation through which each eye must converge to see
from infinity to 1 metre is a change of 1D; as is a the object singly. Thus, one dioptre of accommo-
change from 1 metre to 0.5 metre. It has been dation is associated with one metre angle of
found that for about each dioptre of change of convergence of each eye.
fixation distance, an approximate change of 1.5
Convergence in prism dioptres
occurs in proximal convergence.
When a converging prism (base out) is placed
in front of the eye, it will deviate the rays of light
ANGLE OF CONVERGENCE entering the eyeball outwards and will tend to
It refers to the angle that is formed between the produce diplopia. To maintain a binocular single
primary lines of sight during convergence vision, the eye will turn inwards (converged)
(Fig. 7.1A). Its size depends on the fixation through a corresponding degree (Fig. 7.3). The
distance, becoming smaller with increasing convergence required to see singly an object
distance of fixation object (Fig. 7.1B) and on the placed at one metre distance from the eyes with
interpupillary distance (IPD), becoming larger a prism of one dioptre placed in front of one eye
with increasing IPD (Fig. 7.1C). is called one prism dioptre convergence. It has

A B C
Fig. 7.1 Angle of convergence (A) which becomes smaller with increasing fixation distance (B), and becomes larger with
increasing interpupillary distance (C).
 Anomalies of Convergence, Divergence and Accommodation 155

Far point of convergence refers to relative

position of the eyes when they are completely
at rest. It is usually infinity. At rest, eyes may be
in slight divergence and so the far point of
convergence may be in negative (behind the
eyes).
Range of convergence is the distance between
far point of convergence and near point of
convergence. The part of the range of conver-
gence between the eye and infinity is called
positive convergence, and the part beyond infinity,
i.e. behind the eye (when eyes are in slight
divergence) is called negative convergence or
divergence.
Amplitude of convergence refers to difference in
the convergence power exerted to maintain the
eye in a position of rest and in a position of
maximum convergence.
Fig. 7.2 Convergence in metre angles. Measurement of Amplitude of Convergence
and Near Point of Convergence
See pages 117 and 118 respectively.

ACCOMMODATIVE CONVERGENCE/
ACCOMMODATION (AC/A) RATIO
See page 119.

ANOMALIES OF CONVERGENCE
CONVERGENCE INSUFFICIENCY
Convergence insufficiency is the inability to
obtain and/or maintain adequate binocular
convergence for any length of time without
undue effort. It is the most common cause of
ocular asthenopic symptoms.

Fig. 7.3 Convergence in prism dioptres. Etiology

1. Primary or idiopathic. In many cases, exact
been estimated that roughly 1 metre angle etiology of the convergence insufficiency is not
convergence is equal to 3 convergence. known. It may be associated with a wide inter-
pupillary distance and delayed or inadequate
NEAR POINT, FAR POINT, RANGE AND functional development. General debility,
AMPLITUDE OF CONVERGENCE psychological instability, over work and worry
Near point of convergence (NPC) is the closest may be the precipitating factors.
point at which an object can be seen singly during 2. Refractive errors. Convergence insufficiency
bifoveal vision. In other words, it is the point at may be associated with uncorrected high
which the two foveal lines of sight intersect, hypermetropia and myopia. Diseases of
when maximum convergence is exerted. It is accommodative convergence mechanism result
always closer than the near point of in convergence insufficiency in such patients as
accommodation and is usually less than 8 cm. follows:
 156 Theory and Practice of Squint and Orthoptics

• High hypermetropes (more than 5D) usually • Eye strain and a sensation of tension in and
make no effort to accommodate and thus there around the globes is a common complaint of
is deficient accommodative convergence as such patients.
well. • Headache and eye ache after prolonged use of
• Myopes may not need accommodation and eyes especially for near work, which are
thus lack accommodative convergence. relieved, when the eyes are closed for a while.
• Patients who have worn too full a plus Some patients may show even migrainous
spherical correction may also exert less tendencies.
accommodation and thus less accommodative • Difficulty in changing the focus from distant to
convergence. near objects.
3. Presbyopia. With the advent of presbyopia, • Itching, burning and soreness of eyes and even
near point of eye recedes and so there is less use hyperaemia of the nasal half of the conjunctiva
of convergence. Neglect of presbyopia may lead may occur after prolonged close work.
to fixation of this anomaly. 2. Symptoms due to failure to maintain binocular
On the other hand, patients may also develop vision
convergence insufficiency with the first time use
• Blurred near vision and crowding of words
of presbyopic correction. This has been
while reading.
explained by the fact that the relief of sustained
• Intermittent crossed diplopia for near vision
accommodative effort afforded by the use of
under conditions of fatigue is not uncommon.
presbyopic correction causes a decrease of
accommodative convergence. • Characteristically, one eye will be closed or
4. Muscular imbalances. Extraocular muscular covered while reading to obtain relief from
imbalances in the form of exophoria, visual fatigue.
intermittent exotropia and vertical muscle Diagnosis
imbalances, if neglected for a long time may be
associated with convergence insufficiency. Diagnosis of convergence insufficiency is
5. Consecutive convergence insufficiency may confirmed by:
occur following either recession of medial recti 1. Remote near point of convergence (NPC).
or resection of lateral recti muscles. Convergence insufficiency is said to exist, if NPC
is more than 10 cm from the baseline.
Clinical features 2. Decreased fusional convergence for near.
Convergence insufficiency becomes a clinical When measured on synoptophore, the conver-
problem in children with increased school work, gence insufficiency is said to exist, if there is
prolonged periods of reading, desk workers and difficulty in attaining 30° of convergence.
percision workers. It is usually not a problem in 3. Prism convergence is low but prism diver-
farm and manual labour workers. gence is normal.
Symptoms of convergence insufficiency are 4. Exophoria at near with orthophoria at
similar to that of heterophoria and in general distance may occur. However, convergence
the term asthenopia is used to denote the insufficiency may be associated with ortho-
symptom complex. Unsuitability of the glasses phoria and even exophoria.
is the most frequent complaint of patients using 5. Near point of accommodation (NPA) is
glasses and having asthenopic symptoms. Such normal and corresponds to the age of the patient.
patients change their refractionist and glasses However, measurement of NPA is essential in
frequently without any satisfaction. Asthenopic each case to diagnose and manage patients
symptoms may be grouped as given below. suffering from a combined insufficiency of
convergence and accommodation. Further,
1. Symptoms of muscular fatigue rarely accommodative spasm may occur, if
These result due to continuous use of the voluntary accommodation and convergence are
neuromuscular power and are usually marked stimulated in an effort to overcome the
with near work. These include: convergence insufficiency.
 Anomalies of Convergence, Divergence and Accommodation 157

Differential diagnosis 1. Optical treatment

Convergence insufficiency needs to be differen- Proper refraction should be carried out and the
tiated from the following conditions presenting correct glasses should be prescribed for any
with almost similar symptoms. associated refractive error. Myopes are given full
correction and hypermetropes undercorrection
1. Convergence insufficiency versus convergence to stimulate their accommodation which will
paralysis simultaneously stimulate convergence. In adults
• In convergence paralysis, there is total lack of above the age of 40 years, proper presbyopic
ability to overcome any amount of base-out correction should also be done.
prism; while in convergence insufficiency 2. Orthoptic treatment
several dioptres of convergence amplitude can
Aim of orthoptic exercises is to improve the
be demonstrated.
binocular convergence and to increase the
• On receiving a convergence impulse, a patient amplitude of fusional convergence. ‘Vision
with convergence paralysis will show therapy’. The term used for orthoptic treatment
pupillary constriction but inability to includes:
converge; in a patient with convergence A. Conventional or non-computerised excercises for:
insufficiency, pupillary constriction will occur
• In-office vision therapy, and
while converging on an approaching target,
• Home vision therapy
followed by dilation of the pupil, when
B. Computerised orthoptic excercises for:
convergence can no longer be maintained.
• In-office vision therapy, and
2. Convergence insufficiency versus accommoda- • Home vision therapy
tive effort syndrome Note. Conventional, i.e. non-computerised
• Usually an exophoria at near is associated with excercises are described here. For computerised
convergence insufficiency, while patients with exercises see page 146.
accommodative effort syndrome have
esophoria. Conventional or non-computerised exercises
• Convergence insufficiency is helped by the Orthoptic treatment for convergence insuffi-
–3D test, which compensates for the lack of ciency is by and large same as for exophoria and
good fusional convergence; while a patient includes the following exercises:
with accommodative effort syndrome breaks (a) Exercises to improve near point of
into a tropia during this test. convergence
• Plus lenses will worsen the convergence i. Advancement exercises (see page 215).
insufficiency due to relaxation of accommo- ii. Jump convergence exercises (see page 215).
dative convergence, while they will improve
(b) Exercises to increase amplitude of fusional
the symptoms in the accommodative effort
convergence
syndrome for the same reason.
i. Convergence exercises with prisms (see page
214).
Treatment ii. Convergence exercisess using synoptophore (see
Convergence insufficiency has an excellent page 214).
prognosis in the majority of cases. Children are iii. Exercises using convergence card. Convergence
treated, when fusional vergences are poor and card consists of dots on either side; so also
the patient is showing signs of becoming known as physiologic dot card. On one side
exotropic. Adults with this condition receive of the card, dots are coloured red and on the
treatment only in the presence of symptoms. other side blue. Dots identical in size are in
Treatment of convergence insufficiency includes: the same place on each side of the card and
optical treatment, orthoptic treatment, prismo- these are of three sizes—large, medium and
therapy and surgery. small.
 158 Theory and Practice of Squint and Orthoptics

To perform the exercise, the card is put in front to and fro, the distance between the lights
of the patient's eyes with one end of the card increases or decreases. Patient is asked to
resting on his/her nose, with the large dots maintain the two lights apart as long as possible.
farthest away, so that he/she will see the red dots The finger may again be brought in, if the two
with one eye and the blue with the other eye. lights become single as soon as the finger is
The patient is instructed to look at the large dots removed. This exercise is completed, when the
and to see them fused or blended together, then patient is able to double the lights without the
the middle dots and finally the smallest ones. aid of the finger. Development of voluntary
The patient must be aware of heteronymous convergence goes a long way in relieving
physiologic diplopia on those dots between his/ symptoms.
her eyes and the fused ones; and homonymous
(d) Relaxation exercises
physiologic diplopia on those beyond the fused
Relaxation using relative negative convergence
one.
may be carried out after the treatment by any of
With the use of convergence card, there is a
the following methods:
great deal of retinal rivalry. If the patient is
unable to do the exercises as instructed above, i. Stereogram in crossed position (see page 214).
it may be easier for him/her, if the three dots are ii. Divergence with prisms (see page 213).
connected with a black line on both sides of the iii. Synoptophore exercises (see page 214).
card (Fig. 7.4). Now, when fixating on the large Criteria for good orthoptic management
size dots, he/she should see the lines as . When
• Patient should be symptom free.
he/she is able to fuse the large dots easily, he/
she may then fixate on the middle size dots and • There should be good binocular convergence.
see the black lines as X. Lastly, he/she should • Voluntary convergence should be possible
fixate the small size dots and see the black lines easily.
as . • Patient should have good fusional reserves.
iv. Physiologic diplopia exercise using stereogram 3. Prismotherapy
in the uncrossed position (see page 214).
When all the exhaustive orthoptic exercises fail,
v. Convergence exercise using diploscope (see then prismotherapy may be tried to relieve
page 141). symptoms.
(c) Training of voluntary convergence • Base in prism reading glasses or bifocals with
It is very helpful, if the patient is intelligent and prism in the lower segment are useful as
co-operative. It aims at developing the control relieving prisms.
of the position of eyes. Patient is made to • Relieving prisms and bifocals in young age
understand physiological diplopia which he/she should be avoided.
practices. If a finger is brought in the field of
vision while the patient is fixing a distant light, 4. Surgical Treatment
there will appear two fingers. Now, if the patient As a last resort, when all other measures fail,
fixes at the finger, then there will appear two especially when convergence insufficiency is
lights at the distance. While the finger is moved associated with a large exophoria at near vision,
medial rectus muscle resection can be performed
in one or both eyes. In some cases, exophoria at
near fixation tends to recur.

Convergence insufficiency treatment trial

Convergence insufficiency treatment trial (CITT)
was conducted to compare three forms of vision
therapy with office-based placebo therapy:
• Office-based vision therapy with a trained therapist
Fig. 7.4 Convergence card. along with home reinforcement. Children in this
 Anomalies of Convergence, Divergence and Accommodation 159

group came to the office once per week for a Clinical features
60-minute therapy session with a trained 1. Symptoms of the patients are similar to those
therapist. During these sessions, the children of functional convergence insufficiency.
worked on 4–6 procedures designed to 2. Near point of convergence (NPC) is reduced.
improve the ability to converge the eyes. The 3. Near point of accommodation (NPA) is
children in this group also did home therapy reduced drastically.
for 15 minutes, 5 days per week to practice
4. Accommodative convergence/accommo-
the procedures learned during the office visits.
dation (AC/A) ratio may be low or even
• Home-based pencil push-ups therapy. In this
absent.
group, the child had to follow a small letter
on a pencil as the pencil was moved toward
Treatment
the bridge of his nose. His goal was to keep
the letter clear and single, but to stop if the 1. Orthoptic exercises alone are usually not
letter became double. The child was told to much effective. Exercises need to be combined
try and get the pencil closer and closer to the with reading glasses.
bridge of his nose each day. This was practiced 2. Plus lenses for reading and base-in prism is
for 15 minutes, 5 days per week. the treatment of choice. The reading spectacle
• Home-based computer vision therapy and pencil prescription should be titrated according to
push-ups. In this group, the child was given patients need. The minimal power necessary to
complex exercises using a computer program achieve comfortable vision should be pres-
plus pencil push-ups. cribed. Fresnel membrane prisms glued on glass
• Office-based placebo therapy. This group was lenses in the lower segment of bifocals may be
given placebo vision activities designed to useful, since a frequent change may be necessary
simulate office-based therapy. before the final adjustment is made. Alter-
CITT concluded that office-based vision therapy natively, two piece executive bifocals with
with a trained therapist plus at-home reinforce- decentered plus lenses for prism power in the
ment was most effective in treating CI in lower half may be prescribed.
children 9 to 17 years old. 3. Surgery is usually not indicated for this
condition. However, it has been reported that
CONVERGENCE INSUFFICIENCY resection of both medial rectus muscles followed
ASSOCIATED WITH ACCOMMODATIVE by prescription of bifocal adds may be helpful
INSUFFICIENCY in untreatable cases.
Convergence insufficiency in some patients may
be secondary to accommodation insufficiency. CONVERGENCE PARALYSIS
Therefore, before treating the patient for a Convergence paralysis refers to a total lack of
functional convergence insufficiency, it is ability to overcome any amount of base-out
important to rule out associated accommodation prisms. It is an uncommon entity and should
insufficiency. not be confused with functional convergence
Etiology insufficiency which is very common.
Secondary convergence insufficiency associated
Etiology
with primary accommodation insufficiency has
been reported to occur in following conditions: Convergence paralysis occurs secondary to
1. Early Adie's syndrome some organic diseases of the brain in the region
2. Sequelae to head trauma, particularly of corpora quadrigemina or the nucleus of third
posterior occipital or whiplash injury. cranial nerve. The organic brain lesions reported
3. Subclinical viral encephalopathies. to be associated with convergence paralysis are
4. Infectious mononucleosis as follows:
5. Diphtheria • Head injury
6. As a conversion reaction. • Encephalitis
 160 Theory and Practice of Squint and Orthoptics

• Disseminated sclerosis Treatment

• Tabes dorsalis 1. Base-in prisms are prescribed at near to
• Narcolepsy alleviate the diplopia at near.
• Tumours 2. Plus lenses with base-in prisms may be required
in patients having weakness of accommo-
Clinical features dation.
3. Occlusion of one eye at near may be indicated
Convergence paralysis is characterized by
in patients where it is not possible to restore
following features:
comfortable single binocular vision.
1. Convergence is completely absent. There is
4. Eye muscle surgery is contraindicated in this
acute onset of convergence failure.
condition.
2. Exotropia and crossed diplopia occur on
attempted near fixation only. CONVERGENCE SPASM
3. Adduction is normal. Convergence spasm refers to a condition
4. Accommodation is usually normal. But in some characterized by intermittent episodes of maxi-
cases it may be reduced or even absent. mal convergence usually associated with spasm
of accommodation.
Parinaud's syndrome refers to convergence
paralysis associated with vertical gaze paralysis. Etiology
Pretectum-posterior commissure syndrome 1. Functional causes. It has been reported that
(dorsal midbrain syndrome) which is commonly in most of the cases, convergence spasm is
caused by tumour in the pineal region includes: functional in origin. It occurs in patients with
• Convergence paralysis hysteria or neurosis.
2. Organic causes. Rarely convergence spasm
• Vertical gaze paralysis
may be secondary to some underlying organic
• Pupillary areflexia to light with light near lesion. It has been reported to occur after head
dissociation. trauma, encephalitis, tabes, pituitary adenomas,
• Bilateral fourth nerve paralysis may be present posterior fossa neurofibroma and Arnold-Chiari
sometimes. malformation.
• Lid retraction may also occur in some patients.
Clinical features
Diagnosis In most of the cases, the condition is episodic.
Bielschowsky's criteria for diagnosis of the In between the attacks, patients are normal.
convergence paralysis include: (1) evidence of During the episode of convergence spasm, a
intracranial disease, (2) history of sudden patient may exhibit following clinical features:
onset of crossed horizontal diplopia at near 1. Extreme convergence. Eyes may be fixed in a
fixation, (3) reproducible findings on sub- position of extreme convergence resembling
sequent exami-nations and (4) preservation of bilateral abducens palsy.
accommodation and pupillary reaction on 2. Homonymous diplopia may be experienced.
attempts to converge. Patient may give history of intermittent diplopia.
3. Blurring of vision. During the attack, patient
Differential diagnosis may have blurred vision for near due to
Convergence paralysis can be differentiated associated spasm of accommodation. Patient
from functional convergence insufficiency may come with a complaint of difficulty in
by use of base-out prisms. A patient with reading.
convergence paralysis will immediately have 4. Miosis. Pupils usually become miotic, as a part
diplopia, while in convergence insufficiency of near reflex.
several dioptres of convergence amplitude can 5. Induced myopia to the tune of 6D (confirmed
be demonstrated. by retinoscopy) has been reported due to
 Anomalies of Convergence, Divergence and Accommodation 161

associated spasm of accommodation. Thus, When fusional divergence amplitudes are

during the attack, patient may also have a measured with a prism bar, the normal range is
reduced distance visual acuity. 4D to 6D at distance and 8D to 12D at near. As
discussed earlier, in the case of fusional
Psychiatric examination may reveal the
convergence, the end point of the amplitude
underlying hysteria and neurosis in many cases.
(i.e. the point of maximal convergence) is
marked by an identical phenomenon, that is,
Management
blurring of vision both for distance and near.
Neurological evaluation. Organic lesions are a However, in fusional divergence, the end point
rare cause of spasm of convergence; however, of amplitude differs in character for near
each patient should undergo detailed neuro- (blurring of vision) and distance (double vision)
logical evaluation to rule out associated as follows:
condition, if any. • End point of amplitude of fusional divergence for
Treatment of functional spasm of convergence distance: Beyond the maximal limit of fusional
includes: divergence, there occurs diplopia (end point).
1. Prolonged atropinization with plus lenses in lower • End point of amplitude of fusional divergence for
segment of bifocals for near work may be near: In near vision, as we know, the eyes are
required to break the cycle. accommodated and also converged. Under
2. Alternate monocular occlusion may be these circumstances, when measuring the
considered as an alternative to atropinization. amplitude of fusional divergence beyond the
3. Psychiatric work-up and therapy is useful as a limits of maximal fusional divergence the
long-term measure. accommodative convergence is relaxed to
prevent retinal disparity. But along with the
relaxation of accommodative convergence, the
DIVERGENCE accommodation is also relaxed resulting in
As mentioned earlier, divergence refers to blurring of vision (end point).
disjugate but symmetrical and simultaneous Measurement of amplitude of divergence
outward rotation of the two eyes from a given
See page 116.
position. It is unknown whether voluntary
divergence exists. Certainly, it is possible to relax
one's convergence voluntarily and to produce ANOMALIES OF DIVERGENCE
in this way a more divergent position of the eyes. DIVERGENCE INSUFFICIENCY
Thus, from clinical point of view, the only
significant form of divergence is fusional Divergence insufficiency is not synonymous
divergence. with divergence paralysis, but is a separate
clinical entity.
Fusional divergence Etiology
Fusional divergence, also called negative Exact etiology is not known. It has been assumed
fusional convergence, is the divergence that is that perhaps in divergence insufficiency, a pre-
produced (like the positive fusional convergence) existing esophoria at distance fixation caused by
to ensure that similar retinal images are an anomalous position of rest becomes manifest.
projected onto corresponding retinal points of
two eyes. It occurs without a change in refractive Clinical features
state of eye and is initiated by a binasal retinal Characteristic features are as follows:
image disparity. In other words, fusional 1. Esodeviation. Intermittent or constant eso-
divergence implies a responsiveness to deviation is much larger at distance than
disparate stimuli lying outside of Panum's near. Usually, patients maintain the fusion
fusional area. Similar to fusional convergence, at near. Esodeviation is comitant in all fields
it is a kind of optomotor reflex. of gaze.
 162 Theory and Practice of Squint and Orthoptics

2. Ductions and versions are normal in all the centre has been proved to be divergence centre
directions. that abduction is completely normal bilaterally.
3. Fusional divergence is markedly reduced both 2. Convergence spasm may also be confused with
at distance and near fixation. divergence paralysis because in both the
4. Absence of any neurologic disease (e.g. conditions patients have uncrossed diplopia at
divergence paralysis). distance fixation. However, in convergence spasm
visual acuity is reduced due to induced myopia
Treatment and the divergence fusional range is normal.
1. Prismotherapy. Divergence insufficiency is a
self-limiting condition. However, to alleviate the Management
patient of symptoms, prism base-out is prescribed A thorough neurologic work-up is required for
to provide comfortable single vision at distance. any associated disease. However, divergence
Prism power is decreased stepwise in months paralysis has no localizing significance for the
and ultimately discarded. neurologist. Further, despite the association of
2. Surgical treatment. In the form of resection of neurologic diseases, mostly divergence paralysis
both lateral rectus muscles is indicated, rarely is self-limiting and disappears within 5 to 6
when the prismotherapy fails. months. During this period, prismotherapy as
described for divergence insufficiency is
DIVERGENCE PARALYSIS required. Bilateral lateral rectus resection may
Divergence paralysis is a rare entity charac- be indicated in some cases where divergence
terized by a sudden onset esotropia at distance paralysis persists beyond 6 months.
fixation and a homonymous diplopia. ACQUIRED MOTOR FUSION DEFICIENCY
Etiology Acquired motor fusion deficiency refers to a
combined deficiency of the fusional convergence
Divergence paralysis may be associated with a
and fusional divergence.
head trauma or other neurologic diseases such
as encephalitis, tabes, disseminated sclerosis, Etiology
vascular disorder, neoplasm and raised It has been assumed that, perhaps, a midbrain
intracranial pressure. lesion accounts for the acquired motor fusion
deficiency. The lesions reported to be associated
Clinical features
with this rare entity are as follows:
Clinical features of divergence paralysis are • Closed head trauma
similar to divergence insufficiency except that • Cerebrovascular accidents
it is of sudden onset and usually associated with • Intracranial tumours
some organic neurologic diseases. • Brain surgery
Differential diagnosis Clinical features
1. Unilateral or bilateral sixth nerve palsy. 1. Asthenopia. Most patients complain of severe
Clinically, a paralysis of fusional divergence is asthenopic symptoms.
differentiated from the bilateral sixth cranial 2. Diplopia is usually intractable and variable, i.e.
nerve paralysis by the fact that in the former, at may be crossed at one moment and uncrossed
a given distance, the deviation is comitant and at another.
that the distance between the double images 3. Fusional amplitudes (convergence as well as
remains the same irrespective of the position of divergence) are either markedly decreased or
the object of regard, as it is moved laterally in completely absent.
the radius of a circle with the patient at the 4. Sensory fusion and stereopsis are intact during
centre. This observation indicates that perhaps the brief moments and that such patients may
a separate divergent centre exists. However, still be able to superimpose the double images.
no specific midbrain or central nervous system 5. Accommodation range may be decreased.
 Anomalies of Convergence, Divergence and Accommodation 163

Differential diagnosis Far point, near point, range and amplitude of

Acquired motor fusion deficiency needs to be accommodation
differentiated from following conditions: The nearest point at which small objects can be
1. Combined convergence and accommodation seen clearly is called near point or punctum
deficiency (see page 159). proximum and the distant (farthest) point is
called far point or punctum remotum. The
2. Motor fusion deficiency in adults due to
distance between the near point and the far point
prolonged unilateral use of the eye as seen is called range of accommodation. The difference
with unilateral cataract or untreated unilateral between the dioptric power needed to focus at
aphakia. near point (P) and to focus at far point (R) is
3. Malingering: Some patients may malinger for called amplitude of accommodation (A). Thus, A =
diplopia-like acquired motor fusion deficiency P – R.
following work-related trauma to get compen- Far point and near point of the eye vary with
sation. Measurement of fusional amplitudes the static refraction of the eye. In hypermetropic
with a rotatory prism helps in differentiating eye, far point is virtual and lies behind the eye,
the two conditions. while in myopic eye, it is real and lies in front of
the eye (Fig. 7.6). In an emmetropic eye, far point
Treatment is at infinity and near point varies with age;
Treatment of this condition is usually frustrating. being about 7 cm at age of 10 years, 25 cm at the
Prisms cannot be employed because of frequent age of 40 years and 33 cm at the age of 45 years.
Thus the amount that the eye can alter its
adjustments for near and distance. The only
refraction is greatest in childhood and slowly
way to get relief from symptoms is unilateral
decreases until it is lost in middle age.
occlusion. Infrequently, a spontaneous recovery
Amplitude of accommodation in dioptres as
may occur.
function of the age, as studied by Duane is
depicted in Fig. 7.7.
ACCOMMODATION
ACCOMMODATION AND RELATED TERMS
Accommodation
As we know that in an emmetropic eye, parallel
rays of light coming from infinity are brought
to focus on the retina, with accommodation at
rest. Our eyes have been provided with a unique
mechanism by which we can even focus the
diverging rays coming from a near object on the
retina in a bid to see clearly (Fig. 7.5). This
mechanism is called accommodation. In it, there
occurs increase in the power of the crystalline
lens.

Fig. 7.5 Effect of accommodation on divergent rays entering Fig. 7.6 Far point in emmetropic eye (A), hypermetropic
the eye. eye (B), and myopic eye (C),
 164 Theory and Practice of Squint and Orthoptics

ANOMALIES OF ACCOMMODATION
Anomalies of accommodation are not uncommon.
These include:
I. Deficient or decreased accommodation
1. Presbyopia (physiological deficiency of
accommodation).
2. Insufficiency of accommodation (patho-
logical deficiency of accommodation).
3. Paralysis of accommodation
II. Excessive accommodation or spasm of accommo-
dation

INSUFFICIENCY OF ACCOMMODATION
The term insufficiency of accommodation is
used, when the accommodative power is
significantly less than the normal physiological
limits for the patient's age. Therefore, it should
Fig. 7.7 Decrease in the amplitude of accommodation not be confused with presbyopia in which the
with age in human (from Duane A : Arch Ophthalmol 54: physiological insufficieny of accommodation is
566–587, 1925). normal for the patient's age.

Causes
ASSESSMENT OF ACCOMMODATION 1. Premature sclerosis of lens.
See page 118. 2. Weakness of ciliary muscle due to systemic
causes of muscle fatigue such as debilitating
illness, anaemia, toxaemia, malnutrition,
AGE-RELATED CHANGES IN ACCOMMODATION diabetes mellitus, pregnancy stress and so on.
As discussed earlier, in an emmetropic eye, far 3. Weakness of ciliary muscle associated with
point is infinity and near point changes with age, primary open angle glaucoma.
being about 7 cm at the age of 10 years, 25 cm at
Clinical features
the age of 40 years, 33 cm at the age of 45 years
All the symptoms of presbyopia are present, but
and about 50 cm at the age of 50 years. Therefore,
those of asthenopia are more prominent than
at the age of 10 years, amplitude of accommo- those of blurring of vision.
dation (A) = 100/7 (dioptric power needed to
see clearly at near point) – I/a (dioptric power Treatment
needed to see clearly at far point); i.e. A (at age 1. The treatment is essentially that of the
10) = 14 dioptres. Similarly, A at age 40 years = systemic causes.
(100/25 – I/a) = 4 dioptres; at age 45 years, A = 2. Near vision spectacles in the form of weakest
3 dioptres and at 50 years = 2 dioptres. Since, convex lens which allows adequate vision
we usually keep the book at about 25 cm, so we should be given till the power of accommo-
dation improves.
can read comfortably up to the age of 40 years
3. Accommodation exercises help in recovery, if the
and after that the near point recedes beyond the
underlying debility has passed.
normal reading or working range. This
condition of failing near vision due to related PARALYSIS OF ACCOMMODATION
decrease in the amplitude of accommodation or Paralysis of accommodation, also known as
increase in the near point (punctum proximum) cycloplegia, refers to complete absence of
is called presbyopia. accommodation.
 Anomalies of Convergence, Divergence and Accommodation 165

Causes illumination, bad reading position, lowered

1. Drug-induced cycloplegia results due to the vitality, state of neurosis, mental stress or
effect of atropine, homatropine or other anxiety.
parasympatholytic drugs.
Clinical features
2. Internal ophthalmoplegia (paralysis of ciliary
muscle and sphincter pupillae) may result 1. Defective vision due to induced myopia.
from neuritis associated with diphtheria, 2. Asthenopic symptoms are more marked than
syphilis, diabetes, alcoholism, cerebral or the visual symptoms.
meningeal diseases.
3. Paralysis of accommodation as a component of Diagnosis
complete third nerve paralysis may occur due to Diagnosis is made with refraction under atropine.
intracranial or orbital causes. The lesions may
be traumatic, inflammatory or neoplastic in Treatment
nature. 1. Relaxation of ciliary muscle by atropine for a
few weeks and prohibition of near work allow
Clinical features prompt recovery from spasm of accommo-
1. Blurring of near vision. It is the main complaint dation.
in previously emmetropic or hypermetropic 2. Correction of associated causative factors,
patients. Blurring of near vision may not be prevent recurrence.
marked in myopic patients.
2. Photophobia (glare) due to accompanying BIBLIOGRAPHY
dilatation of pupil (mydriasis) is usually
1. Convergence Insufficiency Treatment Trial
associated with blurring of near vision.
(CITT) Study, Group. “The convergence
3. Examination reveals, abnormal receding of insufficiency treatment trial: design, methods,
near point and markedly decreased range of and baseline data.”. Ophthalmic epidemiology.
accommodation. 2008;15 (1): 24–36.
2. Cooper J, Duckman R. Convergence insufficiency:
Treatment
incidence, diagnosis, and treatment. J Am Optom
1. Self-recovery occurs in drug-induced Assoc 1978; 49:673-80.
paralysis and in diphtheric cases (once the 3. Cooper J. Accommodative dysfunction. In: Amos
systemic disease is treated). JF, ed. Diagnosis and management in vision
2. Dark glasses are effective in reducing the glare. care. Boston: Butterworths, 1987:431–59.
3. Convex lenses for near vision may be 4. Daum KM. Divergence excess: characteristics and
results of treatment with orthoptics. Ophthalmic
prescribed, if the paralysis is permanent.
Physiol Opt 1984; 4:15–24.
5. Duane A: Studies in monocular and binocolar
SPASM OF ACCOMMODATION accommodation with their clinical applications.
Spasm of accommodation refers to exertion of Am J Ophthalmol 1922;5:865.
abnormally excessive accommodation. 6. GK. Van Norden, “Anomalies of convergence
and divergence,” in Binocular Vision and Ocular
Causes Motility, G. K. Von Norden, Ed., Mosby, 4th
1. Drug-induced spasm of accommodation is edition, 1990.
known to occur after the use of strong miotics 7. Goss, DA. Ocular accommodation, convergence,
and fixation disparity: a manual of clinical
such as echothiophate and DEP.
analysis, 2nd ed. Newton, MA: Butterworth-
2. Spontaneous spasm of accommodation is Heinemann, 1995:14.
occasionally found in children who attempt 8. MattiWestman; M. Johanna Liinamaa. “Relief of
to compensate for a refractive anomaly that asthenopic symptoms with orthoptic exercises
impairs their vision. It usually occurs, when in convergence insufficiency is achieved in both
the eyes are used for excessive near work in adults and children”. Journal of Optometry. 2012;5
unfavourable circumstances such as bad (2). pp. 62–67.
166 Theory and Practice of Squint and Orthoptics

9. Morgan MW Jr. Relationship between accommo- 12. Scheiman M, Mitchell GL, Cotter S, Kulp MT,
dation and convergence. AMA Arch Ophthalmol Cooper J, Rouse M, Borsting E, London R,
1952;47:745–759. Wensveen J. “A randomized clinical trial of
10. Rutstein RP, Daum KM, Amos JF. vision therapy/orthoptics versus pencil pushups
Accommodative spasm: a review of 17 cases. J for the treatment of convergence insufficiency
Am OptomAssoc 1988; 59:527-38. in young adults”. Optom Vis Sci. 2005; 82 (7):
11. Scheiman M, Mitchell GL, Cotter S, Cooper J, 583–95.
Kulp M, Rouse M, Borsting E, London R, 13. Wilson ME, Saunders RA, Berland JE. Dissociated
Wensveen J, Convergence Insufficiency horizontal deviation and accommodative
Treatment Trial Study Group (Jan 2005). “A esotropia: treatment options when an eso- and
randomized clinical trial of treatments for an exodeviation co-exist. Journal of Pediatric
convergence insufficiency in children”. Arch Ophthalmology and Strabismus. 1995;32(4):228–
Ophthalmol. 123 (1): 14–24. 230.
 8
Adaptations to
Strabismus and Amblyopia
INTRODUCTION • Prevention fo amblyopia
SENSORY ADAPTATIONS • Treatment of amblyopia

Suppression Abnormal retinal correspondence

• Physiological suppression • General considerations
• Pathological suppression • Harmonious versus unharmonious ARC
• Mechanism and seat of suppression • Development of ARC
• ARC and suppression
• Retinal areas of suppression
• Quality of binocular vision of ARC
• Tests for detection of suppression
• Clinical phenomena associated with ARC
• Measurement of depth • Tests for ARC
• Treatment • Management of ARC
Monofixation syndrome
MOTOR ADAPTATIONS
• Definition and causes
• Characteristic features • Control of ocular deviation by an alteration of
the tone of extraocular muscles
Amblyopia • Compensatory head posture
• Classification and terminology • Blind spot syndrome
• Pathogenesis and pathophysiology • Blind spot mechanism
• Clinical characteristics and
laboratory findings PSYCHOLOGIC ADAPTATION
• Clinical evaluation and diagnosis • Ignoring

INTRODUCTION • Confusion occurs due to formation of image of

two different objects on the corresponding
When the visual functions are normally deve- points of the two retinae (Fig. 8.2).
loped, the individual possesses a binocular • To escape the disabling and troublesome situation
single vision, which implies a point-to-point created by diplopia and confusion, the
normal correspondence of two retinae with strabismic patients (below the age of 7–9 years;
foveas being the principal corresponding point. with immature visual system) develop certain
With the occurrence of strabismus, the adaptations which allow them to have a
alignment of the corresponding retinal points is comfortable single vision. Older patients who
disturbed. Consequently, the fixation point is develop strabismus for the first time suffer
imaged in the centre of the fovea of the non- from diplopia and confusion for a long time
deviating eye and some extrafoveal (peripheral till they learn to ignore (psychological
retinal) point in the deviated eye. This results in adaptation).
diplopia and confusion.
• Diplopia occurs due to formation of image on Adaptations to strabismus. To avoid repetition,
dissimilar points of the two retinae (Fig. 8.1) and adaptations to strabismus are being discussed
167
 168 Theory and Practice of Squint and Orthoptics

• Suppression
• Anomalous retinal correspondence (ARC)
Note. Amblyopia, not actually a sensory
adaptation, may occur as a consequence of
suppression, and so is described in this chapter.
The term strabismic amblyopia is used for the
amblyopia seen in patients with unilateral
constant squint.
Motor adaptations
• Compensatory head posture
• Blind spot syndrome and mechanism
Psychological adaptations
• Ignoring

SENSORY ADAPTATIONS
A sensory adaptation may be defined as the
Fig. 8.1 Diplopia due to formation of image on dissimilar
points of the two retinae. manner in which a patient makes sensory
adjustments to an interruption in the normal
binocular single vision caused by the occurrence
of squint. In other words, sensory adaptations
are nature's way out of trouble but at the cost of
binocular single vision. Sensory adaptations are
more frequent in patients with strabismus of
childhood onset as compared to the patients
with strabismus of adult onset.
Sensory adaptations include the following:
• Suppression
• Monofixation syndrome
• Amblyopia (not actually an adaptation but a
consequence of suppression)
• Anomalous retinal correspondence (ARC)

SUPPRESSION
Suppression may be defined as a temporary
active cortical inhibition of the image of an object
formed on the retina of the squinting eye. This
phenomenon occurs (to avoid diplopia and/or
confusion) only during binocular vision (i.e.
Fig. 8.2 Confusion due to formation of image of two different with both eyes open). However, when the
objects on the corresponding points of two retinae. fixating eye is covered, the squinting eye fixates,
i.e. suppression disappears (Fig. 8.3).
before the clinical description of different
TYPES OF SUPPRESSION
varieties of strabismus. Adaptations to
strabismus include the following: Suppression has been variously classified:
Sensory adaptations I. Depending upon the etiopathogenesis
• Monofixation syndrome 1. Physiological suppression
 Adaptations to Strabismus and Amblyopia 169

Fig. 8.3 Suppression in esotropia: A, Central suppression in left esotropic eye obviates confusion; B, Nasal retinal
suppression scotoma obviates diplopia. Suppression in exotropia: C, suppression scotoma in left fovea obviates confusion;
and D, Temporal retinal scotoma obviates diplope; Suppression in left esotropic eye during binocular vision.

2. Pathological (abnormal) suppression. It is 1. Foveal suppression

further of two types: 2. Macular suppression
a. Facultative suppression 3. Peripheral suppression
4. Large regional suppression
b. Obligatory suppression
III. Depending upon the constancy
II. Depending upon the retinal area where image is 1. Intermittent suppression
suppressed 2. Constant suppression
 170 Theory and Practice of Squint and Orthoptics

IV. Depending upon the eye involved Obligatory suppression. It refers to constant
1. Monocular suppression suppression of the image from one eye which
2. Alternating suppression occurs under all conditions and remains even
when the fixating or dominant eye is covered.
Physiological suppression Obligatory suppression in the long run leads to
Physiological suppression refers to the supp- amblyopia and decreased visual acuity in the
ression present in everyday life of every affected eye.
individual having normal binocular single
Central versus peripheral suppression
vision. It occurs due to retinal rivalry and to
avoid physiological diplopia. As a matter of fact, Central suppression occurs to suppress the
the physiological suppression is very helpful in image formed at the fovea of the deviating eye
everyday life, since it allows greater concen- to avoid confusion (Fig. 8.3A and C).
tration on the object of interest. The specific Peripheral suppression, i.e. suppression of the
examples of physiological suppression in image formed at some peripheral area of the
everyday life while using one eye with both eyes retina of the deviating eye that corresponds to
open are as follows: the image falling on the fovea of the fixating
• While using monocular microscope, the image occurs to prevent diplopia (Fig. 8.3B and D).
of the other is suppressed.
Size and shape of suppression scotoma
• A watchmaker keeps both eyes open but
normally suppresses one eye while viewing Size and shape of suppression scotoma are
the delicate parts of a watch through a different in esotropia as compared with an
monocular magnifying instrument with the exotropia.
other eye. • In esotropia, a small round scotoma involving
nasal retinal area corresponding to the fovea
• While looking down the barrel of a rifle with
of fixating eye is produced (Fig. 8.3B). Rarely
the dominant eye, the person may suppress
this scotoma can be bit larger.
his nondominant eye.
• In exotropia, a comparatively much larger
Pathological suppression scotoma occurs on temporal retina of the
deviated eye (Fig. 8.3D). This is explained by
It refers to the suppression of the image of one
the fact that intermittent exotropia, slowly
eye that occurs to avoid diplopia and/or
increases in size and so is the area of
confusion. It occurs in patients with strabismus
suppression scotoma.
and anisometropia. It can be described as:
• Facultative versus obligatory suppression, PATHOGENESIS
and Mechanism of suppression
• Central versus peripheral suppression Retinal rivalry, i.e. struggle for dominance of each
Facultative versus obligatory suppression eye is considered a prerequisite to suppression.
Retinal rivalry is a situation that occurs when
Facultative suppression. It occurs to avoid dissimilar objects are presented to the two eyes.
diplopia and/or confusion under conditions of That is, two complete images of the dissimilar
binocular vision but ceases, when the fixating objects are not seen. In an area of the field, part
or the dominant eye is covered. It may occur of one image predominates while the corres-
under following situations: ponding part of the second image is suppressed
• Latent squint as shown in Fig. 8.4. This condition is constantly
• Intermittent squint changing and the different parts of the image
• Alternating squint (there is alternate sup- are suppressed in turn.
pression) Burian popularized the concept that sup-
• Manifest deviations of recent onset with pression is merely an exaggeration of the same
normal retinal correspondence. process which is involved in blocking out certain
 Adaptations to Strabismus and Amblyopia 171

A B C D
Fig. 8.4 Retinal rivalry occurring due to dissimilar objects being present to the left (A) and the right (B) eyes. Patient will
not see the picture as shown in (C) but will see as shown in (D) due to suppression.

parts of the image seen by each eye in binocular (Fig. 6.32). In the presence of alternate suppre-
rivalry. However, Smith and coworkers conclu- ssion, patients will see alternately two red lights
ded that though the retinal rivalry may be an and three green lights.
important phase in the development of the Disadvantages. Worth's four-dot test is not a
strabismic suppression, but the suppression and very useful test for suppression because of the
the retinal rivalry are mediated by different following reasons:
mechanisms. • It does not detect foveal suppression.
Present knowledge is far from complete to • Since the eyes are easily dissociated with red-
authentically designate the primary seat of the green glasses, a patient with unstable but
suppressive mechanism. However, most studies functionally useful binocular vision may
implicate the cortex as being the probable seat exhibit a suppression response with this test.
of suppression, since normal ERG with • In a patient having ARC, a normal fusion
reduction in the amplitude of the VER has been response (the patient sees all four dots in a
reported in patients with suppression. Results rectangular arrangement) occurs even in the
of some of the electrophysiological and presence of suppression.
psychophysiological studies are mentioned in 2. The 4D base-out prism test (Fig. 8.5). This
the discussion of amblyopia. test popularized by Jampolsky is performed for
Selective suppression. Burian believes that detection of small angle heterotropias and the
suppression may be selective even with regard presence of central suppression scotoma.
to a specific retinal function; that is, the ability Technique. To perform this test, patient fixates
to resolve only contours may be defective a penlight (Fig. 8.5A). Then a 4D prism is placed
momentarily. with base-out in front of the right eye and the
examiner observes the presence of a biphasic
Suppression pattern usually corresponds with
corrective movement of the left eye (Fig. 8.5B
the deviation pattern, i.e. suppression is
and C). This is absent in the presence of a central
intermittent in patients with intermittent squint,
suppression scotoma (Fig. 8.5D).
monocular and constant in patients with
uniocular constant deviation and it is alternating Mechanism of biphasic corrective movements
in patients with alternating squint. can be explained as below:
• The prism displaces the image towards its
TESTS FOR SUPPRESSION base, in other words, from the fovea of the
right eye towards a point on the temporal half
Tests for detection of suppression of the retina (4D or 2° away from the fovea).
1. Worth's four-dot test. This test can be The relaxation movement of the right eye will
employed to diagnose the suppression involving elicit conjugate movements of both eyes to the
the peripheral retina. As described in detail on left (levoversion), if the right eye has no foveal
page 131, the patients having left suppression suppression (Fig. 8.5B).
will see only two red lights and that having right • This displaces the image in the left eye from
suppression will see only three green lights the fovea to the temporal retina and thus the
 172 Theory and Practice of Squint and Orthoptics

Fig. 8.5 The 4D base-out prism test with its optical principle (for explanation, see text).

left eye now makes a fusional movement in 3. Diplopia test or red glass test. See page 131.
the opposite direction, if no foveal suppression 4. Bagolini’s striated glass test. A foveal
is present (Fig. 8.5C). In the presence of central (central) suppression scotoma in orthotropic
suppression scotomal this will be absent patient or a fixation point scotoma in the
(Fig. 8.5D) presence of microtropia can be detected with
• The test should be repeated with prism over Bagolini’s striated glass test. As described on
the left eye (Fig. 8.5E) and observation for a page 129 and shown in Fig. 6.31, a patient with
biphasic corrective movement in the right eye fixation point suppression will see a central
should be made. interruption of the light streak.
 Adaptations to Strabismus and Amblyopia 173

5. Visual acuity test with Project-O-Chart slide suppression can produce obligatory suppression
of American optical. It is a very effective test to probably depends upon the age of the child,
detect foveal suppression in patients with when facultative suppression begins. The
microtropia or in patients with subnormal deeper the suppression, more difficult it is to
binocular vision after surgical correction of overcome.
essential infantile esotropia. In this test, visual Depth of suppression can be measured with
acuity of each eye is measured under binocular the help of red filter ladder (Fig. 8.6) which
conditions with the Project-O-Chart slide of contains a series of red filters of increasing
American optical. Presence of decreased visual density. The red filter ladder usually consists of
acuity in one eye that is not present, when the gelatine fibres, beginning with one layer and
eye is tested under monocular conditions increasing to six or eight layers. The more the
indicates the foveal suppression. layers, the darker the filter.
6. Synoptophore test. Suppression can be To measure the depth of suppression, the
diagnosed with the use of simultaneous patient is asked to fixate a small light, and the
perception slides (e.g. a cage and a lion) as well filters in increasing density are placed in front
as fusion slides (e.g. identical pictures of a rabbit, of the fixating eye till the patient sees double
one having a candle and the other having lights. Some patients see double with a filter
flower). When simultaneous perception slides made of single layer; while the others require
are used, normally patient should see the lion filters of three or more layers depending upon
in the cage. In the presence of suppression, the depth of suppression. The greater the
patient sees either lion or cage. number of layers needed, the deeper is the
To begin with, simultaneous foveal perception suppression.
(SFP) slides are used. When foveal suppression
is present, the simultaneous macular perception TREATMENT OF SUPPRESSION
(SMP) slides are used. In the presence of macular Indications
suppression, simultaneous paramacular per- The role of antisuppression orthoptic therapy is
ception (SPP) slides are used and observations controversial, since it is not clear whether
are made. While using fusion slides, if the patients treated by antisuppression orthoptic
patient sees a rabbit with both the candle and therapy gain better functional results than others
flower, it indicates normal binocular single who receive passive treatment such as alter-
vision. In the presence of suppression, either the nating occlusion or no treatment at all. Further,
candle or the flower is absent. The fusion in some cases, antisuppression therapy may
pictures are graded according to the size in the cause either intractable diplopia or confusion as
same way as the simultaneous perception suppression disappears.
pictures.
In general, suppression should be treated only
Suppression scotoma can be mapped out, at
when one expects to achieve bifoveal single
least in the horizontal meridian with synopto-
vision. The suitable cases for suppression
phore. One arm of the instrument is rotated, and
therapy are:
the points are noted at which the target carried
by the moving arm disappears and reappears. 1. Patients with intermittent tropias in whom
fusion is present, when the deviation is
Test for measurement of controlled, are the most suitable cases. In
depth of suppression intermittent tropias, the suppression is more
Depth of suppression is not equal in all the superficial than in constant deviation and is,
patients. The degree to which facultative therefore, less difficult to disrupt.

Fig. 8.6 Red filter ladder used for measuring depth of suppression.
 174 Theory and Practice of Squint and Orthoptics

2. Patients with convergence insufficiency and deviation is sufficiently large, surgical

suppression are also suitable candidates for alignment should be considered first.
therapy. They should perform antisuppression • Third step is to make the patient learn fusion
exercises in conjunction with convergence on both an instrument and in free space.
exercises. • Fourth step is to improve the fusional amplitude.
3. Patients with constant tropias in whom
fusion ability can be demonstrated during (A) Diplopia exercises
examination, usually are those in whom the The aim is to make the patient aware of
deviation occurred after about 2½ years of age. physiological diplopia in heterophoria and inter-
4. Patients with fully corrected accommo- mittent tropia and of diplopia in heterotropia.
dative esotropia who continue to have an 1. Awareness of physiological diplopia.
intermittent deviation with suppression, should Awareness of physiological diplopia is an
receive suppression therapy. In such cases, anti- effective means of treating suppression in
suppression exercises should be combined with patients with phorias, intermittent tropias and
divergence exercises to increase fusional phorias recently converted from tropias by
divergence amplitude. surgery and/or orthoptics. Patients should be
5. Postsurgical patients with slight under taught to experience both homonymous and
correction who are suppressing and thereby heteronymous physiological diplopia (as
inhibiting the potential for fusion also need described below). This will stimulate both the
antisuppression exercises. nasal and temporal elements of the retina and
thus will help the patient to overcome
Methods of treatment
suppression over the entire retina.
In fact, therapy for suppression involves the
I. Awareness of homonymous (uncrossed)
treatment of the strabismus as a whole which
diplopia. Patient is asked to make note of light
includes:
situated about 6 metres from him/her while
• Proper refractive correction wherever
fixating a light held 33 cm directly in front of
indicated to render both eyes emmetropic.
his/her nose. While doing so, by practice, patient
• Occlusion therapy to permit equal and
will learn to see one light at near (33 cm) and
alternate use of each eye and to overcome any
two lights at distance (6 metres) simultaneously.
amblyopia which may be present.
Now, if the orthoptist slowly covers and
• Alignment of the visual axes to permit simul-
uncovers his/her right eye several times with an
taneous stimulation of corresponding retinal
occluder, the patient should recognize that the
elements by the same object.
right light at distance intermittently disappears.
• Antisuppression orthoptic exercises may be
Similarly, if the left eye is covered, the left light
needed before and/or after the surgical
at distance disappears. Thus, patient experiences
therapy of strabismus.
homonymous (uncrossed) diplopia, due to
Antisuppression orthoptic exercises bilateral nasal retinal stimulation by the distant
light while patient is fixating on near light
Antisuppression orthoptic exercises are under-
(Fig. 8.7).
taken stepwise to overcome the tendency of the
image from one eye to suppress the images from II. Awareness of heteronymous (crossed)
other eye, when both eyes are open. Ideally, the diplopia. Patient is asked to make a note of light
steps involved in antisuppression orthoptic held 33 cm in front of his/her nose while fixating
exercises are as follows: a light situated 6 metres away. While doing so,
• First step is to make the patient aware of diplopia. by practice, patient will learn to see one light at
• Second step is to make the patient learn to hold distance and two lights at near simultaneously.
fixation with either eye, always seeing the Now, if the orthoptist slowly covers and
blurred image from the deviated eye off to the uncovers patient's right eye several times with
side (simultaneous perception). However, if the an occluder, the patient should recognize that
 Adaptations to Strabismus and Amblyopia 175

the room (Fig. 8.9A). Patient should see two

pencils and one distant object (awareness of
heteronymous diplopia) (Fig. 8.9B). Now the
patient is asked to move the pencil away from
his/her nose. During this procedure, patient will
note that two pencils are moving closer to each
other. Similarly, when the pencil is moved
towards the nose, its two images will be seen
moving farther apart from each other. Once the
patient learns this, he/she is asked to fix at
different distant objects in the room and move
the pencil backward or forward so that the
distant object is framed between the two pencils,
i.e. the object is centred with pencils touching
its each side (Fig. 8.9C).
ii. Bar reading. Bar reading exercise is also based
on the principle of awareness of crossed
Fig. 8.7 Homonymous (uncrossed) diplopia.
physiological diplopia. Patient is asked to hold
the left light situated at near intermittently bar (thumb, finger, pencil or any other bar) about
disappears. Similarly, if the left eye is covered, 5–6 cm from his/her nose and asked to read a
the right light at near disappears. Thus the print kept at about 33 cm from his/her eyes
patient experiences heteronymous (crossed) (Fig. 6.43). As the patient reads the print
diplopia due to bilateral temporal retinal binocularly, he/she perceives the bar in crossed
stimulation by the near light while patient is diplopia, each image of the bar hiding a portion
fixating at distant light (Fig. 8.8). of the print from one eye, but not the other, so
that patient can read the print normally. Such a
III. Exercises utilizing physiological diplopia practice of maintaining the correct position of
i. Framing. Framing is a good home exercise his/her eyes despite the obstacle will strengthen
based on physiological diplopia. Patient is asked the binocular vision of the patient. However, till
to make note of a pencil held at 15 cm in front of suppression is present, he/she will see only one
his/her nose while fixating at a distant object in bar, which will hide some of the print and thus
continuous reading will be impossible. So, the
patient will either shift his/her head or move the
bar to see the print.
The better way to eradicate suppression with
this exercise is to start the patient with a few large
letters on a card. Slowly the amount of letters on
a line may be increased and the size of the letters
decreased as the suppression disappears until he/
she can easily read a page of small print.
2. Diplopia exercises with coloured filters. These
exercises are based on the fact that a patient with
strabismus suppresses similar images in contrast
to normal person who tends to suppress
dissimilar images. In this exercise, patient is
asked to fixate a white light and a red filter is
placed in front of one eye and a green filter in
front of the other eye. The dissimilar images
Fig. 8.8 Heteronymous (crossed) diplopia. produced by the filters are sufficient to
 176 Theory and Practice of Squint and Orthoptics

Fig. 8.9 Framing — a home exercise for suppression (for explanation, see text).

overcome suppression in most instances and (depending upon his/her age and interest) is
produce diplopia. After the diplopia is evoked, asked to do any of the following exercises:
patient is asked to look alternately from one • Colour, trace or draw with a red pencil
image to the other. During this process, first one matching the red filter.
filter and then the other is removed in an attempt • Little girls may follow red design drawn on a
to have the patient notice diplopia without the white cloth with a needle and a light red or
help of filters. When patient is able to do so, the pink thread.
fixation light is exchanged for another fixation • Children can draw lines with a red pencil
object which is a less intense stimulus. In this using the number-to-number games in their
way, patient should be taught diplopia during colouring books.
both near and distance fixations. • Children can also select red beads for a
3. Diplopia exercise with prisms. In this necklace
orthoptic exercise, a vertical prism (base up or • The boys can play ping-pong with a red ball.
down) sufficient enough to displace the image 2. To treat suppression at distance, patient may
outside the suppression scotoma is placed in be asked to watch colour television with the red
front of one eye. Consequently, the patient will filter over the dominant eye.
have diplopia. Gradually, the prism power is
reduced until finally the patient has diplopia (C) Exercises with major amblyoscope
without any vertical displacement of one image. 1. Macular massage. This exercise stimulates
Once the patient learns to perceive diplopia the retina of deviated eye. It is accomplished by
without prism, the fixation light is then replaced moving the visual target on the major amblyo-
by a less intense stimulus. In this way, patient scope back and forth across the suppression
should be taught diplopia during both near and scotoma (macular massage) as below:
distance fixations. The major amblyoscope tubes having slides
of paramacular simultaneous perception are
(B) Exercises with use of red filter locked at the patient's objective angle. While he/
1. To treat suppression at near, a red filter is she looks constantly straight ahead, the tubes
placed over the dominant eye and patient are moved rapidly from side to side over a large
 Adaptations to Strabismus and Amblyopia 177

arc. Since they are at the objective angle, the (E) Exercises with Tibb's binocular trainer
images moving over the retina will always Tibb's binocular trainer is an excellent instru-
stimulate normally corresponding areas. In the ment for antisuppression exercise, especially for
periphery, they will be superimposed. When home. For details see page 144.
approaching the suppression area, however, one
picture will disappear or the two will separate. MONOFIXATION SYNDROME
The excursion of the tubes is gradually reduced
Definition and causes
until eventually the patient can maintain
superim-position while looking at the pictures, Monofixation syndrome, also known as Park’s
when tubes are held still. syndrome, is the term coined by Marshal Park
for the sensory adaptation occurring in patients
2. Crossing technique. A pair of paramacular
with microtropia (angle of strabismus <10 PD).
simultaneous perception slides is put in the
Other clinical situations associated with
tubes of major amblyoscope. Patient is asked to
monofixation syndrome are:
fixate the target viewed by the dominant eye,
• Anisometropic amblyopia, unilateral astig-
while the target in front of the suppressed eye
matism and
is moved in from the periphery of the field
towards the suppression scotoma. It will • Unilateral partial cataract.
disappear, when it reaches the suppressed area Characteristic features
and reappear on the other side after the entire
It is characterized by absence of bifoveal fusion
scotoma has been crossed. This back and forth
(unilateral central suppression) with the presence
movement of the target across the suppression
of peripheral fusion.
area is continued until this area has decreased
• Central suppression in the microtropic eye
to such an extent that the patient can
occurs because the central retina has small
simultaneously perceive both targets and can
receptive field and high spatial resolution
superimpose the two images. In normal
potential, so relatively small difference in
correspondence, peripheral size targets may be image clarity or retinal image position are
used first followed by macular and foveal size recognized. Patients with monofixation
targets. syndrome usually have stereo acuity in the
range of 30 to 70 seconds of arc and the central
3. Chasing technique. It is a technique of
scotoma measures between 2° and 5°.
subjective exercise using the smallest
simultaneous perception slides that the patient • Peripheral fusion is maintained because in the
peripheral field slight interocular image
can superimpose. The two arms of the major
differences are not detected since the
amblyoscope are loosened and the patient is
peripheral retina has large receptive field and
asked to hold the handle of the tube in front of
relatively low spatial resolution. Small retinal
the suppressed eye. The examiner moves the
image discrepancies between fellow eyes are,
picture tube that is in front of the fixating eye in
therefore, not disruptive in the peripheral
a random position. The patient is asked to chase
fields and so peripheral fusion is maintained.
it and superimpose the two pictures by moving
the other tube. This chasing technique
is exercised repeatedly. As the patient's AMBLYOPIA
performance improves, increasingly smaller Amblyopia, by definition refers to, a partial loss
pictures are used until he/she can superimpose of sight in one or both eyes, caused by abnormal
the foveal slides. visual development secondary to abnormal
visual stimulation in the absence of ophthal-
(D) Exercises with cheiroscope moscopic or other marked objective signs.
The cheiroscope is a very good instrument for Literally speaking, amblyopia is a spectrum of
antisuppression training. For details see page visual loss, ranging from missing a few letters
144. on the 20/20 lines to hand motion vision.
 178 Theory and Practice of Squint and Orthoptics

However, for practical purposes, amblyopia is manage to maintain fusion in some positions
labelled, when there is at least two Snellen lines of gaze with an anomalous head posture.
difference in the visual acuity between the eyes. • Patients with alterante strabismus do not have
Amblyopia occurring in a patient with amblyopia but they do have abnormal
strabismus is not a sensory adaptation per se, binocular function.
but the consequence of suppression which is a 2. Stimulus (visual) deprivation amblyopia. It
sensory adaptation. is caused by those conditions wherein one eye
is prevented from seeing early in life. Such
CLASSIFICATION AND TERMINOLOGY conditions would include:
The self-explanatory terms used for amblyopia • Monocular congenital or traumatic cataract
in present day orthoptic practice are as follows: • Complete ptosis
1. Strabismic amblyopia. The term strabismic • Corneal opacity and
amblyopia is used for the amblyopia seen in • Prolonged patching of the normal eye for
those patients with unilateral constant squint treatment of amblyopia (occlusion amblyopia).
who strongly favour one eye for fixation from Constant monocular occlusion of the visual
birth to 6 years of age. Peak age for development axis for more than a weak per year of life places
of fixation preference in strabismic children is a child at significant risk for the development
about 1 year (range 9 months to 24 years). of stimulus deprivation amblyopia until about
Strabismic amblyopia is a common form of 5–6 years of age.
amblyopia and typically shows following features
Deprivation amblyopia is characterized by
that are uncommon in other forms of amblyopia:
following features:
• Grating acuity is often considerably less
• It is the least common but most damaging and
reduced than the Snellen's acuity. This is
difficult to treat form of amblyopia.
because forms seen by the affected eye are in
• Amblyopic visual loss resulting from
a twisted or distorted manner that interfere
unilateral deprivation is worse than that
more with letter recognition than with the
produced by bilateral deprivation of similar
simpler task of determining whether a grating
degree. This is because of the fact that in the
is present or not.
unilateral deprivation, interocular effects add
• Neutral density filter effect, i.e. when illumi- to the direct developmental impact of severe
nation is decreased, the acuity of an eye with image degradation.
strabismic amblyopia does not decline further
while it does so in organic amblyopia. Bilateral deprivational amblyopia may develop
in small children with bilateral media opacities,
• Laterality, strabismic amblyopia is always
e.g.:
unilateral and is caused by an active inhibition
within the retinocortical pathways of visual • Bilateral congenital cataract
input originating in the fovea of the deviating • Bilateral corneal opacities (Peter's anomaly)
eye. Strabismic amblyopia follows through a • Bilateral vitreous haemorrhage
stage of suppression, giving rise to the term 3. Refractive amblyopia occurs due to consistent
suppression amblyopia. defocus of the retinal image in one or both eyes
• Type of strabismus. Strabismic amblyopia is due to presence of refractive error. It can be of
seen far more often in esotropes than the following subtypes:
exotropes. This might be related to the fact that • Anisometropic amblyopia. The term anisome-
in esotropia, the fovea of the deviating eye has tropic amblyopia refers to the amblyopia
to compete with the strong temporal hemifield occurring in an eye having higher degree of
of the fellow eye; while in exotropia, the fovea refractive error than the fellow eye.
of the deviating eye has to compete with the • Anisohypermetropic versus anisomyopic
weaker contralateral nasal hemifield. amblyopia. It has been reported that amblyopia
• Strabismic amblyopia occurs very rarely in is more common and is of a higher degree in
patients with hypertropia, as they usually patients with anisohypermetropia than in those
 Adaptations to Strabismus and Amblyopia 179

with anisomyopia. Even 1.5 to 2 dioptre are also differences in the severity and
hyperopic anisometropia may cause amblyopia reversibility of the various types of amblyopia.
while up to 3 dioptre myopic anisometropia However, the most pertinent factual knowledge
usually does not cause amblyopia. However, about the changes occurring in amblyopia which
unilateral high myopia (–6D or more) often has been obtained through experimental studies
results in severe amblyopia. It has been presumed (electrophysiologic and histopathologic) in
that both the forms, vision deprivation as well kittens suggests that the structural and functional
as the abnormal binocular interaction that is involvement of the afferent visual pathway in
caused by unequal foveal images in the two different forms of amblyopia is the same as is the
eyes, might be playing role in the development sensitive period during which amblyopia occurs,
of anisometropic amblyopia. regardless of the etiology. Therefore, on the basis
Strabismus is frequently associated with of this recent neurophysiologic reasearch, largely
anisometropia. In such cases whether amblyopia done in experimental animals, it has been
occurs due to anisometropia or strabismus or postulated that the basic amblyogenic
both is very difficult to determine. mechanisms are—light deprivation, foveal form
• Meridional amblyopia. This refers to vision deprivation, abnormal binocular
amblyopia occurring in patients with interaction and active cortical inhibition.
uncorrected astigmatic refractive error due to The initial development of amblyopia from
selective visual deprivation for visual stimuli of any cause rarely occurs in children older than
a certain spatial orientation. Thus, meridional about 5–6 years. However, once amblyopia has
amblyopia is a selective amblyopia for a specific developed and has been treated with therapy,
visual meridian. 1.25D of arriso astigmatism it may recur until about 9–11 years of age.
may cause amblyopia. Pathophysiology of amblyopia thus can be
• Isoametropic amblyopia. Isoametropic discussed under following headings:
amblyopia is bilateral amblyopia occurring in • Amblyogenic factors,
children with bilateral uncorrected high • Role of retina in the development of amblyopia,
refractive error. Hyperopia of more than +5.0D and
and myopia in excess of –10.0D have a risk of • Active cortical inhibition
inducing bilateral amblyopia. Such amblyopia
is usually of milder form. It is supposed to result [A] AMBLYOGENIC FACTORS
from the effect of blurred retinal images alone The basic mechanisms responsible for amblyopia,
(pattern vision deprivation). Bilateral meridional which have been recognized on the basis of
amblyopia is caused by bilateral astigmatism. neurophysiologic research, largely done in
Significant meridional amblyopia occurs with experimental animals, are as follows:
astigmatism greater than –2.5D.
• Deprivation of form vision
• Light deprivation
PATHOGENESIS AND PATHOPHYSIOLOGY OF
• Abnormal binocular interaction
AMBLYOPIA
Despite an enormous research in this field, 1. Deprivation of form vision
pathogenesis and pathophysiology of amblyopia • Monocular deprivation of form vision during
is still not elucidated fully. However, the clinical the critical period of visual development results
features and laboratory findings in eyes with in amblyopia of the deprived eye. Since the
amblyopia permit certain conclusions for deprived eye becomes dominated by the normal
understanding the nature of the processes eye so a competitive amblyopia of profound
underlying amblyopia and its treatment. intensity develops. Monocular visual deprivation
Psychophysical studies in human strabismic, works as an amblyogenic factor in strabismic,
anisometropic and visual deprivation amblyopia anisometropic, stimulus deprivation ambloypia.
show differences between the function of the • Binocular deprivation of form vision during
fovea versus the retinal periphery. Further, there the critical period of visual development results
 180 Theory and Practice of Squint and Orthoptics

in bilateral deprivational amblyopia. Since in Neurophysiologic basis of

binocular deprivation, the binocular interaction normal binocular vision
is not disrupted so a competitive amblyopia is Hubel and Wiesel are pioneers in the neuro-
not superimposed; so the resultant amblyopia not physiologic studies of binocular vision. They
so severe as in unilateral deprivation. Binocular have concluded that in normal cat or kitten:
deprivation plays the role of amblyogenic factor • Approximately 80% neurons of striate cortex
in children with bilateral cataract, ametropia and are derived from each eye, 10% from the
bilateral high refractive errors. contralateral eye and 10% from the ipsilateral
eye only. The two receptive fields of
2. Light deprivation
binocularly driven cortical cells are found to
Light deprivation works as an amblyogenic have corresponding location in the two
factor in children with unilateral as well as retinae.
bilateral complete cataracts.
• Of the binocularly driven cortical neurons,
3. Abnormal binocular interaction only 25% are stimulated equally well from
each eye, while the remaining 75% show
Abnormal binocular interaction is highly
amblyogenic. It produces a profound amblyopia graded degree of influence from the right or
due to a competition amblyopia. Abnormal left eye (disparity sensitive binocular cells).
binocular interaction plays the role of amblyo- Stereopsis has been linked with horizontal
genic factor in children with strabismic, disparity sensitive binocularly driven cortical
anisometropic and unilateral stimulus depri- neurons.
vation amblyopia. • It has been reported that at birth (i.e. without
Thus, the amblyogenic factors for different previous visual experience or training) the
types of amblyopia are same. However, their visual system of kittens gives responses which
contribution to each may vary and so is the are in no way different from those obtained
severity of amblyopia (Table 8.1). in adult cats. From this, the conclusion was
drawn that the basis for visual recognition and
Neurophysiological basis of
binocular interaction is already fully
role of amblyogenic factors
developed at birth. The immaturity of visually
The above conclusions about the role of various dependent behaviour in young animals, such
amblyogenic mechanisms have been drawn as the lack of pursuit movements, therefore,
from the various experimental studies done in cannot be explained by incomplete develop-
cat, kitten and monkey. A few key studies to ment but must be due to other factors,
understand the neurophysiologic basis of possibly lack of interpretation/visuomotor
normal binocular vision and amblyopia are cooperation.
described briefly here.

Table 8.1 Role of amblyogenic factors in different types of amblyopia

Amblyogenic factors
Types
of Light Deprivation of Abnormal Severity of
amblyopia deprivation form vision binocular amblyopia
interaction
• Stimulus deprivation amblyopia
– Unilateral (e.g. cataract) + + + +++
– Bilateral (e.g. cataract) + + – ++
• Strabismic amblyopia – + + ++
• Anisometropic amblyopia – + + ++
• Ametropic amblyopia – + – +
 Adaptations to Strabismus and Amblyopia 181

• To understand the pathophysiologic mecha- with the peripheral retina and is concerned with
nisms concerned with binocular vision and the location of objects in space, which enables
development of amblyopia, the visual fixation movements to be made. This system
pathway carrying visual sensations from the projects to both the lateral geniculate nuclei and
retina to visual cortex have been classified in superior colliculus.
X, Y and W-system (Fig. 8.10) as follows: W-cell system (slow system). It is believed to
X-system. The fibres from the retinal ganglion be the pursuit system. It projects only to superior
cells carrying the sustained response to visual colliculus.
stimuli are found in the X-cell system—the
medium velocity system. The X-system is Neurophysiologic studies in
experimental and clinical amblyopia
associated with central form vision and,
therefore, central visual acuity. Cells of this Neurophysiology of amblyopia is a complex
system project only to the lateral geniculate mechanism and its understanding is far from
nucleus. complete. Some of the observations made from
Y-system. The fibres from the retinal ganglion the study of experimental modification of visual
cells carrying a transient response are in the experience in animals and laboratory testing of
Y-cell, or fast system. The Y-system is associated amblyopic human beings are given below.

Monocular deprivation studies

Methods. Monocular deprivation was produced
in experimental animals (kitten and monkey) by
suturing the eyelids of one eye during the critical
period of development.
Observations are as below:
• Deprived eye developed amblyopia, probably
because this eye was dominated by the normal
eye due to the interruption in binocular co-
operation.
• Changes observed in visual system neuron
functions were as follows:
– In the lateral geniculate body, cells in those
layers receiving input from the deprived eye
showed a profound shrinkage. Larger cells
shrank more than the smaller ones.
– Cells of primary visual cortex either lost
their innate ability to respond to stimulation
or showed significant functional deficiency.
It has been reported that there occurs a
selective reduction in the number of cortical cells
driven by Y-input from retina, suggesting that
perhaps visual deprivation selectively involves
the Y-system.
Note. Similar experiments in adult animals did
not reveal such changes.
Conclusions. These studies indicate that uniocular
visual deprivation produces amblyopia by
changes in the visual system neurons. It has also
Fig. 8.10 X, Y and W-system of cells in the visual cortex. been concluded that deprivation during the
 182 Theory and Practice of Squint and Orthoptics

early part of critical period of development is deprived monkeys, both the monocular and
more deleterious than at a later stage. This binocular portions of the lateral geniculate
experimental work of uniocular deprivation nucleus showed shrinkage. Thus, two different
closely resembles the clinical situation of neural mechanisms appear to underlie the
unilateral cataract, severe congenital ptosis or deprivational and strabismic amblyopia.
corneal opacity producing profound amblyopia. However, it has been postulated that visual
deprivation (since central fixation is not used in
Binocular deprivation studies strabismus), abnormal binocular interaction and
Technique. Binocular visual deprivation was active cortical inhibition similar to that occurs,
made by bilateral tarsorrhaphy in kittens and in suppression, all play a role in the amblyopia
monkeys during sensitive period of development associated with strabismus. While this seems
and also in visually mature animals and reasonable intuitively and laboratory findings
following observations made were as below: suggest this, but definitive proof is still lacking.
• A mild bilateral amblyopia occurred in infant
animals due to visual deprivation. Perhaps Exprerimental anisometropic amblyopia
amblyopia was mild due to the fact that the Experimental anisometropic amblyopia was
competition amblyopia was not superimposed produced in primates by putting high plus
since the binocular interaction was not convex lens in one eye and/or atropine.
abnormal in binocular equal deprivation. Observations made were as below:
• Changes observed in visual system neuron • Visual cortex cells of striate cortex layer IV and
functions were as follows: outside layer IV (driven by the involved eye
– Cell shrinkage produced in lateral geni- and a fraction of binocular driven cells) had
culate body was less than that produced in reduced contrast sensitivity.
uniocular deprivation. • Lateral geniculate body showed anatomical
– There occurred a decrease in the specific changes in the form of diminution of cell size
responsiveness of cells and an increase in limited only to the parvicellular layers that
number of cells that respond sluggishly or subserve high spatial frequency.
abnormally. In addition, there were many
cells that did not respond at all. [B] ROLE OF RETINA IN
DEVELOPMENT OF AMBLYOPIA
– The number of Y-cells was reduced by 307%.
– It was postulated that in binocular There is some evidence that the retina itself is
abnormal in amblyopia. Whether retinal
deprivation, there may be a competition for
abnormality is the effect or cause of amblyopia is
synaptic space in the cortex between the
debatable. The views have differed, if retinal
X-cells and the more disadvantaged Y-cells.
threshold and sensitivity have been affected in
Conclusions. The amblyopia produced by amblyopia. It is, however, widely believed and
binocular deprivation was less severe than that proved experimentally that there is a decreased
produced by uniocular deprivation, owing to sensitivity of foveal cones in amblyopia. Even
the fact that binocular interaction was not flicker fusion threshold and differential
abnormal. This work closely resembles the thresholds are considerably affected. However,
clinical situations of bilateral amblyopia seen in reduction of foveal cone and retinal sensitivity is
children with bilateral cataract and also to some much less than the reduction in visual acuity.
extent the ametropic amblyopia. Also electroretinography is found to be normal
in amblyopic eyes. It, therefore, is improbable that
Experimental strabismus amblyopic a functional defect of foveal cones would be
In monkeys made artificially strabismic, by responsible for reduced visual acuity. However,
disinserting lateral or medial rectus, only the amblyopic eye is not at its best under photopic
binocular portion of the lateral geniculate body condition than under mesopic conditions. In fact
showed cell shrinkage, while in visually it has been observed that there is a quicker dark
 Adaptations to Strabismus and Amblyopia 183

adaptation in such eyes suggesting takeover of in amblyopia, active cortical inhibition might be
rod functions from decreased retinal foveal cones. mediated by GABA led the researchers to
It is, therefore, logical to believe that reduced perform certain experiments.
inputs from the rods and cones in the affected Duffy et al. produced deprivation amblyopia
eye cause certain neurophysiological changes, in kittens and studied the effects of some anti-
transmitted aberrantly to the CNS which triggers GABA agents. Following observations were
the onset of amblyopia. The vicious circle made by them:
continues, till the process is reversed. It may – Intravenous injection of biculline (an anti-GABA
become irreversible in later stages. agent) led to stimulation of 60% visual cortex
That retinal sensitivity is related to onset of cells which were otherwise unresponsive due
amblyopia is further proved by the fact that eyes to deprivation amblyopia. However, convul-
which had relatively poor sensitivity, responded sions were noted as a complication of this
to amblyopia treatment better than those which drug.
had better retinal thresholds. Perhaps a different – Intravenous injection of naloxone, another anti-
mechanism is also responsible, i.e. a central GABA agent, also restored binocular inputs
suppression exists in amblyopia. in the visual cortex cells which were otherwise
unresponsive due to deprivation amblyopia.
[C] ACTIVE CORTICAL INHIBITION Kasamatsu and Pettigrew produced depletion
The neurophysiologic research points out that of brain catecholamines by using 6-hydroxy-
visual deprivation and active cortical inhibition dopamine and norepinephrine and observed a
are the two fundamental mechanisms for the failure of ocular dominance shift after
development of amblyopia. The role of active monocular occlusion in kittens.
cortical inhibition is evidenced by following The above studies provide a pharmacologic
studies. evidence of the role of active cortical inhibition
1. Physiologic evidence. In one study, in experi- in the amblyopia. However, further studies will
mental animals, deprivation amblyopia was reveal as to where we stand in understanding
produced in one eye. After 5 months of the pathological basis of onset of amblyopia.
deprivation, these animals, were divided into two
groups. In group-I animals the normal eye was CLINICAL CHARACTERISTICS AND
enucleated, while in group-II, the normal was LABORATORY FINDINGS IN AMBLYOPIA
retained and following observations were made: 1. Visual acuity. Amblyopia, by definition,
In group-I animals (with normal eye enuclea- refers to a partial loss of sight in one or both
ted), the amblyopic eye was found to drive 31% eyes in the absence of ophthalmoscopic and
of the visual cortex cells. While in group-II other marked objective signs. It has been
animals, in which normal eye was retained, only recommended that a difference of two lines on
6% of the visual cortex cells were driven by the a visual acuity chart should be there to diagnose
amblyopic eye. In other words, after removal of amblyopia. However, strictly speaking, any
the normal eye, the deprived eye showed difference between the two eyes especially in
marked capacity for recovery indicating thereby strabismic amblyopia should be considered
that perhaps the normal eye may be responsible significant.
for an active cortical inhibition in unilateral Certain clinical characteristics associated with
amblyopia. visual acuity in patients with amblyopia are as
2. Pharmacologic evidence. It has been ascert- follows:
ained that under normal circumstances, most of i. Recognition acuity (Snellen's or similar
the excitatory synapses in the visual cortex are charts) is more affected than the resolution acuity
cholinergic. Further, it has also been reported (Teller's chart or VER) and the detection acuity
that the visual cortex is inhibited by the gamma (Catford drum test or Bailey-Hall cereal test).
aminobutyric acid (GABA)—an inhibitory ii. Snellen's acuity and grating acuity are
neurotransmitter. The assumption that perhaps affected equally in anisometropic amblyopia
 184 Theory and Practice of Squint and Orthoptics

whereas in strabismic amblyopia, the grating Amblyopia with central fixation. In amblyopia
acuity is affected to half the extent of Snellen's with foveolar fixation, the foveola has preserved
acuity. Thus, strabismic amblyopia is under- the principal visual direction and its zero
estimated on grating test. retinomotor value. Amblyopia is secondary to
iii. Effect of neutral density filter. It has been a central suppression scotoma.
reported that when visual acuity is tested with
Amblyopia with eccentric viewing. In
a neutral density filter placed in front of the
amblyopia with eccentric viewing, patients
affected eye, the visual acuity improves by one
prefer to view with an extrafoveal point bacause
or two lines in patients with developmental
of the deep suppression scotoma, but the fovea
amblyopia; while in patients with organic
has still not lost its principal visual direction. In
amblyopia, the visual acuity decreases by two
eccentric viewing, patients look past the object
to three lines. Therefore, the neutral density filter
they have been asked to fix. This can be
test has been recommended to differentiate
demonstrated during visuscopic examination of
between developmental amblyopia and organic
a co-operative patient, who will tell the examiner
amblyopia.
that he/she is aware of the fact that he/she has
The neutral density filter test is based on the to look over to one side to see the star clearly
fact that under photopic conditions, visual and when he/she looks straight ahead the
acuity of amblyopic eye is less than that under fixation target appears blurred. The examiner
scotopic conditions. Since the neutral density can also observe that in eccentric viewing,
filter, when placed in front of an eye, produces patient will place the image of fixation target
a state of scotopic conditions, the vision of first on the fovea and then immediately from
amblyopic eye improves.
the fovea on to the paramacular retinal elements.
iv. Crowding phenomenon. Crowding pheno-
menon, also known as separation difficulty, refers Amblyopia with eccentric fixation. In amblyopia
to the inability of an amblyopic eye to with eccentric fixation, the fovea has lost its
distinguish letters (or other symbols) crowded principal visual direction, its retinal motor value
together. Therefore, the vision in an amblyopic is no longer zero and an extrafoveal point is now
eye is better, when tested with isolated the bearer of these properties. Patients report
optotypes than when tested with line or Snellen's that they are looking straight at an object
acuity charts having rows of letters. In other stimulating non-foveolar retinal area. If the
words, single optotype visual acuity is better image of an object is placed on the patient's fovea
than linear visual acuity. The larger the (by means of an instrument), this object is sensed
discrepancy between the linear and single letter as being in some other direction than straight
acuity, the poorer the prognosis. ahead.
Crowding phenomenon is the result of
Types of eccentric fixation. Depending upon the
contour-interaction between the neighbouring
retinal area with which the eyes appear to fixate,
test targets because of decreased lateral
the eccentric fixation may be of following types
inhibition in amblyopia.
(Fig. 8.11):
2. Fixation pattern. Amblyopia may be associated • Parafoveolar—just outside the foveal reflex.
with central fixation, eccentric viewing or
eccentric fixation. In a normal eye, three • Parafoveal—outside but close to foveal wall.
characteristics of foveolar area, which appear to • Paramacular—on or just outside the rim of the
be responsible for maintaining the fixation reflex macula. Many workers have now abandoned
central, are: the use of this term because of vague ophthal-
a. Peak visual acuity in the foveolar region, moscopic definition of the macula.
b. A principal occulocentric direction of straight • Peripheral—outside the macula, anywhere
ahead, and between the macula and extreme retinal
c. A retinomotor value of zero. periphery.
 Adaptations to Strabismus and Amblyopia 185

not be possible in amblyopic, patients with

unsteady fixation.
4. Localization of an object of regard. Locali-
zation of an object of regard is normal in patients
having amblyopia with central as well as
eccentric fixation. However, in patients having
amblyopia with eccentric viewing, localization
of an object of regard is faulty.
5. Colour vision. Colour vision anomalies may
occur in patients with amblyopia only if visual
acuity is markedly reduced below 6/36. Ano-
Fig. 8.11 Types of fixation pattern. malous colour vision in such cases has been
related to peripheral eccentric fixation, i.e. a
Steady versus wandering fixation. Central as peripheral retinal area is being used for fixation
well as eccentric fixation may be steady or rather than foveola.
wandering. Wandering fixation, which occurs
only upon covering the sound eye, must be 6. Light perception. There occurs a dissociation
distinguished from the monocular, sponta- of the form vision and light perception in
neous, pendular and vertical oscillations that are amblyopia, since form vision is abnormal
occasionally found in deeply amblyopic eyes. (especially under photopic condition) while
This condition has been designated as the absolute light threshold is found to be normal.
Heimann-Bielschowsky phenomenon. It is clinically However, differential threshold (i.e. how much
similar to other forms of monocular nystagmus brighter the test field must be than its
that may occur in connection with posterior surrounding so that a difference is perceived) is
fossa or brainstem disorders. elevated in amblyopia.
Paradoxical eccentric fixations. Ordinarily, 7. Pupillary light reflexes. Generally speaking,
there develop nasal eccentricity in esotropic and pupillary light reflex is normal in amblyopes.
temporal eccentricity in exotropic patients. However, rarely in patients with a deep
However, sometimes the eccentric fixation may amblyopia, an afferent pupillary defect may
be paradoxical, i.e. reverse of the expected occur. It has been suggested that perhaps the
situation. In other words, there may be nasal afferent pupillary defect may result from
eccentricity in exotropic and temporal eccen- synaptic inhibition in the retina, since this
tricity in esotropic patients. Such a situation can pathway does not reach the geniculate body.
occur under following circumstances: 8. Light and dark adaptation. Usually, dark
• Following surgical overcorrection of the adaptation is not abnormal in amblyopes,
deviation. though a significant difference in adaptation
• In patients with spontaneous reversal of the between amblyopic and normal eyes has been
deviation. found in the region of Kohlrausch's bend (the
• Following prolonged occlusion of the sound kink or bend in the adaptation curve normally
eye in amblyopia. produced by increased sensitivity of the rods).
• With no obvious cause (rarely). 9. Critical flicker frequency (CFF). It has been
3. Absolute central scotoma. Monocular scoto- reported that in amblyopia, central CFF tends
metry on visual field charting may plot an to approach the CFF of peripheral retina or of
absolute central scotoma. Visual field charting rod mechanism. It has also been reported that
for this purpose should never be done CFF is significantly faster in amblyopic eyes that
binocularly, otherwise, a facultative binocular fixated eccentrically than in those with foveal
suppression scotoma (present only with fixation. Some workers have reported that
binocular viewing) may be mistaken as the examination of CFF with a simple apparatus is
absolute central scotoma. The scotometry may a useful tool to distinguish reduced visual acuity
 186 Theory and Practice of Squint and Orthoptics

in maculopathies from amblyopia, since in the Methods for evaluating visual potential in
former the thresholds are below normal. infants and very young children (up to 2½ years
10. Electroretinography (ERG) and electro- of age). Infancy and early childhood is probably
oculography (EOG). An enormous data is the most important age to be bothered, since it
available on ERG studies in amblyopia, how- is the most sensitive period to develop
ever, till date it has not been definitely answered amblyopia. At the same time, testing of vision
whether ERG is normal or abnormal in amblyo- during this period is also not so easy. However,
pia. Many studies report that ERG is essentially untiring efforts should be made to detect
normal and EOG shows unsteadiness of fixation unequality of vision in two eyes. Certain useful
in amblyopia. methods are as follows:
Fixation behaviour test. Fixation behaviour test
EVALUATION AND DIAGNOSIS is a reliable and useful test in infancy to obtain
Diagnosis of amblyopia is made by a reduced a rough estimate of visual acuity. Each eye is
best corrected visual acuity that cannot be covered alternately and behaviour of the infant
entirely explained on the basis of physical ocular is noticed. If vision is equal or nearly equal in
abnormalities. Clinical evaluation of a suspected both eyes, an infant or very young child will not
case of amblyopia should include the following: object to having either eye covered. However,
1. Evaluation of visual acuity. if the visual acuity is reduced in one eye, the
2. Neutral density filter test. child will show objection (in the form of a cry or
3. Test for crowding phenomenon. pushing the occluder away) when the normal
4. Thorough ocular examination including eye is covered. In such cases, one should suspect
fundus examination. any ocular disease, high refractive error or
5. Refraction. amblyopia.
6. Evaluation for central versus eccentric A rough estimate of visual acuity can be made
fixation. by testing with brightly coloured toys of varying
size while occluding one eye. It is noticed
7. Tests for other sensory anomalies.
whether the child can fix and follow the toy.
1. Evaluation for visual acuity. As mentioned
above, clinical evaluation of visual acuity is most Binocular fixation pattern (BFP). The binocular
important for the diagnosis of amblyopia. fixation pattern, indicating strength of
Generally speaking, a difference of two lines preference for one eye or the other under
between the best corrected visual acuity of the binocular viewing conditions, is generally relied
two eyes (e.g. OD 6/6, OS 6/12 or OD 6/5, OS upon for estimating the relative level of vision
6/9) is considered diagnostic for amblyopia. For in two eyes for very young children with
practical purposes, particularly after amblyopia strabismus. It is important to note that, when
treatment has started, any acuity difference is the infant's binocular fixation pattern is tested,
considered amblyopia. an accommodative target such as small toy
should be used. A child with extremely unequal
Severity of amblyopia. In the "Amblyopia
vision will show great preference for the good
treatment study (ATS) group trials, amblyopia
eye. A child with nearly equal vision will have
has been graded as below:
only mild preference for one eye. Five grades of
• Mild to moderate amblyopia is defined as visual binocular fixation pattern described while
acuity in the amblyopic eye of 20/80 or betta.
making the patient fix with the deviated eye
• Severe amblyopia is defined as visual acuity in (Table 8.2).
the amblyopic eye of 20/100 to 20/400. Binocular fixation pattern test is quite
Methods employed to evaluate visual acuity sensitive for detecting amblyopia but is
depend upon the age of the patient and have sometimes false positive (showing a strong
been described in detail on page 48. However, preference, when vision is equal or nearly equal
for a ready reference, important points for in the two eyes), particularly with small-angle
different age groups are mentioned as follows. strabismic deviations.
 Adaptations to Strabismus and Amblyopia 187

Table 8.2 Grading of binocular fixation pattern in strabismic scotoma complex, thus allowing the patient to
patients fixate with either eye.
Grade Description of response CSM method of rating monocular fixation. CSM
Grade 0 : Spontaneous alternation (no pre- method has been used to describe the fixation
ference for one eye). pattern of a too young patient for visual acuity
Grade 1 : Holds fixation through blink (simply measurement by some workers after examination
prefers one eye but can use the other with a handlight as follows:
eye with nearly equal frequency). • C: Stands for 'central' which refers to the fact
Grade 2 : Holds fixation until blink, i.e. that angle kappa appeared equal in direction
habitually fixing eye resumes fixation and magnitude.
with the next blink (moderate fixation • S: Stands for 'steady' which means that fixation
preference).
is not aimless or wandering as in amblyopia
Grade 3 : Holds fixation for 1–2 seconds but
and also that nystagmus is absent.
switches before blinks (strong
fixation preference but the other • M: Stands for 'maintained', meaning thereby
is used briefly for fixation.) that there is no shift on the cover test, i.e. a
Grade 4 : Immediately switches fixation on manifest squint is not present.
removal of cover from non- It has been reported that rating of monocular
deviating eye (strong fixation
fixation pattern as central, steady and maintained
pattern, and patient uses only one
provides limited information. An eye with
eye for fixation.)
extremely poor visual acuity may also have
central, steady and maintained fixation.
Prism-induced tropia test. Prisms can be used in a
Therefore, use of CSM should be avoided,
variety of ways to induce a tropia, thus allowing
particularly, if it replaces a visual acuity
the binocular fixation pattern to be assessed in
notation. Similarly the 'maintained' is no
children with small angle strabismus:
alternative to cover and cover-uncover test to
• 25 dioptre base-in prism test (Cassin, 1982). A detect manifest deviation.
25D base-in prism is introduced over one eye
Preferential looking test, optokinetic nystagmus
and the child's eye preference is noticed. The
and visually evoked potential. These tests are
prism is then placed over the other eye and
also used to measure visual acuity in infants and
preference is noted. This prism induces a large
very young children (for details see page 40).
esotropia that cannot be overcome by most
children and results in diplopia. Therefore, a Methods of estimating visual acuity in
child with equal vision will ordinarily use the preschool children (2½ to 4 years). Commonly
eye without the prism to fixate regardless of employed tests are listed below (for details see
which eye is viewing through the prism. If a page 45):
child shows preference for one eye to fixate • Marble game test
through the prism, the nonpreferred eye is • Hand chart test
considered amblyopic. • Illiterate E-game test
• Vertical prism test (induced tropia test). Ten to • Allen’s preschool vision test
fifteen dioptre vertical prism test has also been
• Sheridan Gardiner test
recommended to assess eye fixation preference
• Stycar matching test
by producing tropia with diplopia, similar to
25D base-inprism test. Methods of estimating visual acuity in school
Note that the vertical prism test rectifies the children and adults (age 5 and older). Most
high rate of misdiagnosis of amblyopia by commonly used tests are as follows (for details
standard fixation preference testing in patients see page 48):
with small-angle strabismus and monofixation • Snellen's test types
syndrome. This is because the vertical prism • E-chart for illiterate
breaks up the peripheral fusion and central • Landolt's broken-C chart
 188 Theory and Practice of Squint and Orthoptics

2. Neutral density filter test. Whenever possible,

it is imperative to illucidate this important
characteristic of amblyopic eye—that the
amblyopic eye sees better under mesopic
conditions (between scotopic and photopic
condition). This can be tested with neutral
density filter test. For details see page 184.
3. Test for crowding phenomenon should be
performed to establish the separation
difficulties—another important feature—
exhibited by amblyopic eyes. For the detail see
page 184.
4. Thorough ocular examination including
fundus examination. A thorough ocular
examination including a detailed fundus
examination is very important to rule out any
cause, other than amblyopia, of reduced visual
acuity.
5. Refraction. The importance of a meticulous
refraction cannot be overemphasized in the
clinical evaluation of squint and amblyopia (see
page 102).
6. Evaluation for central versus eccentric
fixation. About one-half of all amblyopic eyes
are associated with eccentric fixation. The
fixation pattern can be evaluated by following
methods: Fig. 8.12 (A) Angle kappa (OPA) is formed between the
visual axis (OF) and central pupillary line (AP). However,
i. Angle kappa method. An idea about eccentric
clinically angle kappa (OXA) is measured at a point on the
fixation can be made by comparing the angle cornea (X) that lies in the central pupillary line. (B) Angle
kappa in each eye. Though it is commonly used kappa is labelled positive when the corneal light is displaced
but comparatively less accurate method of nasally and negative when it is displaced temporally.
detecting eccentric fixation. Angle kappa can be
estimated by following methods: It is not an accurate method. Small degree of
eccentric fixation is often missed. However, it is
• Hand light method. After occluding the non-
the only available method of testing eccentricity
fixing eye, patient is made to fix a hand light
in infants.
held directly below the examiner's eye to avoid • Arc perimeter method. In this technique, after
an inaccuracy due to parallax. The location of occluding one eye, patient is asked to fix a
corneal reflex is noted. The same procedure is centre mark on the perimeter, and a very fine
repeated on the other eye. The angle is positive, light is moved along the perimeter arc until
when the corneal reflex is displaced nasally and the light reflex is centred on the cornea.
negative, when it is displaced temporally Location of light on the perimeter arc tells the
(Fig. 8.12). A positive angle kappa of up to 5° is angle kappa in degrees.
physiologic in emmetropic eyes. • Major amblyoscope method. Angle kappa is
– In central fixation, the corneal reflex is located measured using special slides with synopto-
in a similar position in each eye. phore (see page 138).
– In eccentric fixation, a significant difference in ii. Visuscope method. In most clinical practice,
the location of corneal reflex in fixing and visuscope or its ophthalmoscopic alternative is
nonfixing eye will be noted. the most commonly employed method for
 Adaptations to Strabismus and Amblyopia 189

testing eccentric fixation (see page 140). How- Vision screening examinations should start at
ever, this technique requires patient's birth and continue as part of routine check ups
cooperation and thus can be used in patients by primary care physicians.
above 4–5 years of age. • Acronym I-ARM (Inspection—Acuity, Red
iii. Haidinger's brushes method. Patient is made reflex, and Motility) can be a helpful reminder
to perceive the entoptic pattern of the Haidinger of the essential parts of a paediatric screening
brushes and then asked to touch its centre with examination. Table 8.3 summarizes the I-ARM
a pointer. In the presence of central fixation, screening eye examination for neonates,
patient will easily do so. However, if fixation is babies, and children.
eccentric, a gross error will be made and patient • Most important test for the newborn is the red
will be unable, despite repeated attempts to reflex test. If an abnormal red reflex is present,
correct the error. Being cumbersome, this then an immediate referral to an ophthalmo-
method is not used routinely. logist is required.
iv. Maxwell's spot method. Maxwell spot is a • Infant screening examination takes less than a
round, dark, purplish spot of about 3 arc degrees minute, but this brief examination is quite
in diameter. It is perceived entoptically, when powerful. If performed properly, it can detect
the eye is exposed to a homogenous blue or the vast majority of eye pathologies.
purple field. In central fixation, this spot is
centred over the fixation target. In eccentric Children with risk factor for amblyopia should
fixation, the Maxwell spot is displaced to the side have a comprehensive ophthalmic examination.
of fixation target by an angular amount Some risk factors include:
equivalent to the degree of eccentricity. Like • Family history of amblyopia or strabismus
Haidinger's brushes method, this is also • Childhood cataract or glaucoma
sparingly used in common clinical practice. • Premature birth of lens than 30 weeks
7. Tests for other sensory anomalies. Amblyopia gestation and/or less than 1500 gm weight
may be associated with ARC. Therefore, the tests
employed for suppression (see page 171) and Brückner reflex test
ARC (see page 129) may also be required for a The red reflex test is the single best vision
thorough clinical evaluation of amblyopia. screening exam for infants and young children.
It is best performed using the Brückner
PREVENTION AND EARLY DETECTION OF modification, which is simply a simultaneous
AMBLYOPIA bilateral red reflex. Use the direct ophthal-
Early detection as well as early intervention is moscope and view the patient’s eyes at a distance
most essential for the effective treatment of of approximately 2 feet from the patient. Use a
amblyopia. The best way for prevention and broad beam so that both eyes are illuminated at
early diagnosis of amblyopia is adoption of some the same time. Dim the room lights and have the
screening programme. child look directly into the ophthalmoscope light.

Table 8.3 Screening eye examination: I-ARM

Step Neonate Babies Children
(Birth–2 months) (3 months–2 years) (3 years and older)
Inspection Symmetry face and eyes Face turn or head tilt Face turn or head tilt.
Acuity Poor fixation, pupillary Good fixation and Visual acuity: Allen cards,
response smooth pursuit E-game,
Red reflex Red reflex test Binocular red reflex Bilateral red reflex test
(Brückner) (Brückner)
Motility Gross alignment (70% Good alignment, light Good alignment, light reflex and
small exotropia but reflex and Brückner Brückner (any misalignment
esotropia probably (esotropia is abnormal misalignment is abnormal)
abnormal) after 2 months of age).
 190 Theory and Practice of Squint and Orthoptics

Start with the ophthalmoscope on low • Significant congenital cataract should be

illumination then slowly increase the illumination removed during the first 2–3 months of life.
until a red reflex is seen. The examiner will • In symmetric bilateral cases, the interval
observe a red reflex that fills the pupil and a small between operations on the first and second
(approximately 1 mm) white light reflex that eyes should not be more than 1–2 weeks.
appears to reflect off the cornea . The white light • Acutely developing severe traumatic cataracts
reflex is actually a reflex coming from just behind in children under 8–10 years of age should be
the pupil and is called the “corneal light reflex” removed within a few weeks of injury, if possible.
or the “Hirschberg reflex.” Thus, the Brückner • Refractive correction for aphakia following
test gives both a red reflex and the corneal light cataract surgery in childhood must be
reflex simultaneously. Blockage of the retinal provided promptly with no further delay.
image or large retinal pathology will result in an
Severe congenital ptosis should be corrected at
abnormal red reflex.
the earliest.
• Cataract can either block the red reflex or
Corneal opacity should be treated by penetrating
reflect light to give a white reflex.
keratoplasty.
• Retinoblastoma has a yellowish-white colour
and will produce a yellow reflex. 2. Correction of refractive error
• Anisometropia (difference in refractive error) and spectacle adaptation
will result in an unequal red reflex. Refractive error, if any, should be fully corrected
• Strabismus will cause a brighter red reflex in as determined with cycloplegic refraction before
the deviated eye, and the corneal light reflex starting the amblyopia therapy.
will be decentered.
Spectacle adoption for 3 to 4 weeks should be
Note. The key sign of a normal exam is symmetry. tried in anisometropic amblyopia before starting
occlusion therapy. Refractive correction alone
TREATMENT OF AMBLYOPIA
may improve vision in many cases.
Goal of amblyopia treatment is to maximise and
potentially normalise visual acuity. II. CORRECTION OF OCULAR DOMINANCE
Strategies to treat amblyopia include: Correction of ocular dominance is done by
• Elimination of the cause of visual deprivation stimulating the amblyopic eye with the use of
and provision of clear retinal image in the following modalities alone or in combinations:
amblyopic eye, • Occlusion therapy
• Correction of ocular dominance • Penalization
• Near visual activity, and • Active stimulation
• Perceptual training. • Pleoptics
• Pharmacological manipulation
I. ELIMINATION OF THE CAUSE OF VISUAL
DEPRIVATION AND PROVISION OF CLEAR 1. Occlusion therapy
RETINAL IMAGE Occlusion of sound eye is the most powerful
means of treating amblyopia by forcing the
1. Media clearance
patient to use amblyopic eye. Occlusion therapy
Media clearance, whenever required, is the first has been the mainstay of treatment since 18th
step of amblyopia management. century.
Childhood cataract, when present should be
operated as early as possible with appropriate Methods of occlusion
aphakic correction depending upon the circum- Occlusion can be accomplished by an adhesive
stances. Correction of amblyopia in congenital patch on skin, gauze pad and tape, use of
cataract is a challenge full of frustration. Doynes rubber occluder which can be stuck to
Important guidelines for treatment of childhood spectacle lens, opaque contact lense, adhesive
cataract are as follows: tape on glasses or any method that excludes the
 Adaptations to Strabismus and Amblyopia 191

use of occluded eye (Fig. 8.13). Adhesive skin patch Previously, many workers recommended that
is the best method. However, problem may arise in the presence of eccentric fixation, first one
in children with sensitive skin. If application of should occlude the amblyopic eye for some time,
tincture of benzoin before the patch is applied so that the eccentric fixation becomes less fixed.
on the skin also does not help, then other However, after long observations, now only
methods may be tried as a substitute for a patch. direct occlusion is recommended even in the
presence of eccentric fixation as discussed above.
Programmable electronic glasses
The lenses are liquid crystal display (LCD), they Full-time versus intermittent (part-time) occlusion
can also be programmed to turn opaque, Full-time occlusion involves placing the occluder
occluding vision in the left or right eye for over the eye as soon as the child gets up in the
different time intervals, acting like a digital patch morning and removing only after the child goes
that flickers on and off. Amblyz™ occlusion to bed at night. Earlier, constant and total
glasses were used for 4 hours daily in a study, occulsion was considered the choice for initial
where the lens over the eye with better vision treatment of amblyopia. Standard teaching has
switched from clear to opaque every 30 seconds been that children need to be observed at
(presented at AAO but not published). intervals of 1 week per year of age, if undergoing
full-time occlusion to avoid occlusion amblyopia
Direct versus inverse occlusion in the sound eye. A simplified schedule for initial
Direct occlusion refers to occlusion of the sound treatment of amblyopia previously recom-
eye and inverse or indirect occlusion refers to mended with a combination of full-time direct
occlusion of the amblyopic eye. and inverse occlusion is shown in Table 8.4.

A B C

D E

Fig. 8.13 Methods of occlusion: A, Elastoplast occluder; B, Eye pad and sticking tape method; C, Spectacle occlusion
method; D, Doynes rubber occluder method; and E, Ground glass as occluder.
 192 Theory and Practice of Squint and Orthoptics

Table 8.4 A simplified schedule for initial occlusion therapy for amblyopia
Age of the patient (in yrs.) Period of occlusion (days) Follow-up after every
Direct : Inverse
Up to 2 2 : 1 15 days
3 3 : 1 15 days
4 4 : 1 1 month
5 5 : 1 1 month
6 and older 6 : 1 1 month

Part-time (intermittent) occlusion involves should be continued till the amblyopic eye has
use of the occluder for a short time each day. not only developed equal vision but also equal
Amblyopia treatment studies (ATS) have preference of fixation compared to the normal
demonstrated: eye. On an average, it may take 3–6 months,
• In children aged 3–7 years with severe amblyopia depending upon the age of the patient and
(visual acuity between 20/100 and 20/400), initial level of vision. The younger the patient
full-time patching produced a similar effect the better is improvement in the visual acuity,
to that of 6 hours of patching per day. when the occlusion is started, and the shorter
• In children aged 3–7 years with moderate is the duration of occlusion required.
amblyopia (visual acuity better than 20/100), • In patients with no improvement with occlusion
2 hours of daily patching produced an on three consecutive monthly follow-up visits,
improvement in visual acuity similar to that further occlusion is unlikely to be fruitful.
of 6 hours. In this study, patching was However, it is essential to once again rule out
prescribed in combination with 1 hour of near any organic disease and carefully recheck the
visual activities. refraction. Incomplete response to occlusion
• In children aged from 7 years to younger than tends to be associated with anisohyper-
13 years, prescribing 2–6 hours a day of metropia and anisoastigmatism.
patching can improve visual acuity, even if the
Maintenance occulsion treatment is done as
amblyopia has been previously treated.
below:
• In patients aged from 13 years to younger than • Once the vision has been equalized, the
18 years, prescribing 2–6 hours a day of maintenance occlusion should be continued
patching might improve visual acuity, when till the amblyogenic age, i.e. up to at least
amblyopia has not been previously treated; 9 years of age and sometimes even till the child
however, this is likely to be of little benefit, if has reached early teens.
amblyopia was previously treated with
• Maintenance occlusion is accomplished by a
patching. Long-term results from these studies
part-time occlusion for 2–3 hours in a day with
are still pending.
active vision exercises at home.
How to go about occlusion
2. Penalization
Compliance is the keyword of success in occlusion
therapy and should be ensured by motivating the Principle. The word penalization literally means
child and parents. The initial phase is the uphill to punish or to inhibit. The principle is to force
route; once the near vision and then the distance the amblyopic eye to a greater use for distance,
vision start improving, the task is easier. near or both by penalizing the sound eye for
distance, near or both with the help of glasses
How long to continue occlusion is decided as and a cycloplegic drug.
below:
Prerequisite to penalization is that the eyes
• In patients with improvement in vision, assessed
should be straight and hence is best used in
at monthly follow-up visits, the occlusion
anisometropic amblyopia without deviation or
 Adaptations to Strabismus and Amblyopia 193

with deviation after it has been corrected or ii. Optical penalization

surgically with prisms. Optical penalization is based on over-plussing
(prescribing more plus sphere than needed) the
Indications. In the past, penalization therapy sound eye to force fixation to the amblyopic eye
was reserved for children who would not for distance targets; the patient will usually use
wear a patch or in whom compliance was an the sound eye for near targets. Optical
issue. The amblyopia treatment studies, penalization works well for mild amblyopia;
however, have demonstrated that atropine however, some children will look over the tops
penalization in patients with moderate of their glasses to use their sound eye.
amblyopia (defined by the study as visual
acuity better than 20/100) is as effective as 3. Active stimulation therapy
patching. The amblyopia treatment studies Active stimulation therapy using CAM vision
were performed in children aged 3–7 years. stimulator has been used in the past.
Further, penalization may also be used for The word CAM stands for Cambridge
maintaining vision obtained through (England), where this vision stimulator device
previous occlusion therapy. was first used in 1978.
Methods. Penalization can be done by two Method. In this technique, after occluding
methods are given as follows. the sound eye, amblyopic eye is stimulated for
7 minutes by slowly rotating high contrast
i. Atropine penalization, and
square wave grating of different spatial
ii. Optical penalization. frequencies. The treatment is carried out once
Note. Atropine penalization is a stronger in a week for 3 to 4 weeks. The sound eye
method. remains open between the weekly treatment
sessions—an advantage over the conventional
i. Atropine penalization of non-amblyopic eye occlusion therapy.
a. Near penalization. It is most commonly used. Principle on which this treatment is based is the
For near penalization, the fixing eye is assumption that rotating grating provides
atropinized and fully corrected for distance specific stimulation for cortical neurons.
vision, while amblyopic eye is overcorrected However, equally good results are reported
with +2.0 to +3.0D. This forces the amblyopic using grey discs instead of grating. Therefore, it
eye to be used in near vision and stimulates may well be assumed that the combination of
alteration of two eyes for near and distance occlusion and near work requiring visual
fixation. concentration is responsible for the improvement.
Alternately, sound eye is atropinized and 1% Present status of CAM vision stimulator.
pilocarpine is used in amblyopic eye. Pilocarpine Though initial workers reported good results
gives pinhole effect due to constriction of pupil but subsequent workers reported that this
and improves near fixation due to facilitation of technique is not as effective as conventional
accommodation. occlusion therapy. So it has failed to replace the
b. Distance penalization. Fixing eye is atropi- time tested conventional occlusion therapy for
nized and overcorrected by +3.0D lens while the the treatment of amblyopia. However, some
amblyopic eye is fully corrected. In this way, workers believe that this method can be used as
sound eye is penalized for distance and is used supplementary to occlusion therapy in co-
for near only, while amblyopic eye is used for operative patients with supportive parents who
distance. can carry out the treatment at home.
c. Total penalization. Fixing eye is atropinized
and undercorrected by 4.0 to 5.0 D, while 4. Pleoptics
amblyopic eye is fully corrected. This prevents The word pleoptics is derived from a Greek
the fixing eye being used for near as well as word meaning full vision. This technique
distance. generated a lot of excitement during 1950s and
 194 Theory and Practice of Squint and Orthoptics

1960s. But nowadays it is only of historical there are questions regarding long-term stability
interest. of vision.
• Pleoptics was used for active stimulation of
Role of Citicholine. See page 405
the fovea to overcome eccentric fixation and
improves the visual acuity. Role of omega fatty acid is also reported in
• In this technique, the peripheral retina development of vision and cognitive development.
including the eccentrically fixing area around
III. ACTIVE THERAPY (NEAR VISUAL ACTIVITIES)
the fovea is dazzled with an intense light while
protecting foveal area. Active thrapy, in the form of near visual
activities, using amblyopic eye has been
• Supposedly, after the light source is turned
suggested as an important supplement to
off, the fovea functions better because the
occlusion therapy based on the assumption that
surrounding retinal area is in a state of hypo-
these activities stimulate the visual system and
function.
thus causes easy recovery.
• This can be followed by direct stimulation of
fovea as by pleoptophore (Bangerter's Active vision exercises by the amblyopic eye
method) or indirectly by producing after- during occlusion therapy, which may enhance
image (Cupper's method). the success of occlusion include:
• The excitement over this technique abated, • Simple tasks like dotting the o's and encircling
when it became obvious that the technique is the e's in the newsprint, joining dots to make
complex and requires an absolute co- drawing, tracing, colouring threading beads,
operation of the patient, his/her intelligence watching television, reading comics and story
to appreciate after-images (which is possible books may be quite useful and enchance the
in older children) and daily sittings for a recovery.
longer period of time (with its own socio- • Computer games, especially designed for active
economic problems) are required. vision exercises, are beging considered a very
• Since occlusion of the dominant eye is a very useful adjunct with occlusion therapy. The
successful simple and inexpensive method of television, video games and mobile games for
treating eccentric fixation, so most practi- near vision activity are easily available and
tioners have abundoned use of pleoptic have become very popular with the children.
methods.
IV. ROLE OF PERCEPTUAL LEARNING IN
5. Pharmacological manipulation AMBLYOPIA TREATMENT

Role of levodopa. Studies indicate that plasticity Gibson (1963) defined perceptual learning as
of visual system during the sensitive period is “Any relatively permanent and consistent
dependent on input from non-adrenergic change in the perception of stimulus array
neurons and thus can be subjected to following practice or experience with this
pharmacological manipulation. This aspect has array….”
been tried with the use of levodopa as a Over the last 15 years, number of studies suggest
pharmacologic manipulator. Levodopa/ that ‘perceptual learning (PL) may provide an
carbidopa has been traditionally used to treat important new method for treating amblyopia.
Parkinson’s disease. Levodopa is a precursor for Perceptual learning is reported to operate via a
the catecholamine dopamine, a neurotrans- reduction of internal neural noise and/or
mitter/neuromodulator known to influence through more efficient use of stimulus informa-
receptive fields. Levodopa/carbidopa has been tion by returning the weighting of the information.
studied as an adjunct to patching for the Perceptual learning employes repeatedly
treatment of amblyopia. However, the role of practicing a visual discrimination task, e.g:
levodopa remains controversial, as the visual • Positional acuity,
acuity improvement has been relatively small, • Contrast sensitivity,
not clearly better than with patching alone and • Stereoacuity, etc.
 Adaptations to Strabismus and Amblyopia 195

Recommended period for preceptual learning is of acupuncture appears promising, the

2 hrs/day, 5 days/week, for a period of 9 months. mechanism underlying its success as a treatment
Role of preceptual learning is still controversial, for amblyopia remains unclear. Acupuncture at
but utility is reported in adult amblyops. vision-related acupoints may modulate the
activity of the visual cortex. Moreover,
V. BINOCULAR APPROACH FOR acupuncture has been shown to be effective in
AMBLYOPIA TREATMENT increasing blood flow to the cerebral and ocular
Recently, amblyopia is being considered a vasculatures (including the choroid), stimulating
binocular problem caused by active suppression the expression of retinal nerve growth factors
that’s converts structurally intact binocular and leading to metabolic changes in the central
system into a functionally monocular system. So, nervous system.
treating unilateral amblyopia with a binocular
approach using dichoptic stimuli is under active Transcranial magnetic brain stimulation in
consideration.The dichoptic treatment presents amblyopia
a stimulus to each eye separately and the brain Transcranial magnetic stimulation (TMS) is a
is forced to integrate the images into a single non-invasive method for stimulating parts of the
perception. In these treatment modalities; under brain by use of weak electric current that are
binocular conditions, the signal strength coming induced into the tissue by use of rapidly
into the patient’s good eye is reduced enough changing magnetic currents. TMS produces
so that it cannot suppress the amblyopic eye. effects that last slightly longer than the actual
The result is binocular perception in a patient time of stimulation.
with otherwise deep suppression of the
amblyopic eye. Over time, the viewing conditions AMBLYOPIA TREATMENT STUDY REPORTS
are changed and the image seen by the good eye Reports of various ‘amblyopia treatment studies
is suppressed less until both eyes see (ATS)’ carried out under the 'Paediatric Eye
approximately the same image. There are many Disease Investigation Group (PEDIG)' are
popular softwares that are developing now-a- summarised in Table 8.5.
days promoting binocular vision therapy with
3D gaming and at the same time stimulating PROBLEMS IN AMBLYOPIA TREATMENT
perceptual learning. These are believed to treat 1. Problems in applying the occluder
amblyopia not only in children but also in
adults. Though occluding the sound eye may appear
to be a simple procedure, and in many cases it
Examples of dichoptic stimuli are: is nevertheless, in the practical application of this
• Hess et al have developed antisupprression simple treatment, several problems may arise.
therapy as dichoptic stimuli using a ‘Faling Some of the important difficulties encountered
Blocks game’ on an iPad. during this treatment are discussed as follows.
• Li et al treated amblyopia with dichoptic Occluder attached to spectacle lens is easy to
stimuli using red-green glasses with reduced wear but usually ineffective, since children are
contrast for the non-amblyopic eye. able to look over, around and under the patch.
Further, most children will simply take their
VI. ROLE OF OTHER MODALITIES IN glasses off, when unobserved, and for this
AMBLYOPIA TREATMENT reason, most workers rarely use this method of
Acupuncture in amblyopia occlusion.
Acupuncture is a potentially useful comple- Adhesive patch applied directly over the
mentary treatment modality that may provide skin (Fig. 8.13) is the best method for a total and
sustainable adjunctive effect to refractive full-time occlusion. However, problems of skin
correction for anisometropic amblyopia in reactions may arise in some children. Following
young children. Although the treatment effect measures have been recommended:
 Table 8.5 Summary of amblyopia treatment study reports 196

Study Aim Age No. of Type of study Follow up Results

patients
ATS 1 Occlusion versus 3–<7 yr 419 Randomised 2 year Improvement was initially faster in the patching group,
pharmacologic therapy comparative but after 6 months the difference in visual acuity between
for moderate amblyopia trial treatment groups was insignificant; 3.16 lines (patching
to compare atropine and group) vs 2.84 lines (atropine group). Visual acuity
patching for moderate >20/30 and/or improved by 3 lines in 79% of
amblyopia occlusion group and 74% of atropine group
ATS1-Ext Atropine vs patching for 3–<7yr 188 Randomised 15 years At 15 years of age, most children treated for moderate
treatment of moderate comparative of age amblyopia when younger than 7 years have good
amblyopia: follow-up at trial visual acuity, although mild residual amblyopia is
15 years of age of a common. The outcome is similar regardless of initial
randomized clinical trial treatment with atropine or patching.
ATS 2A A randomized trial 3–7yr 175 Randomised 4 months 6 hrs of patching equally effective as full-time patching.
comparing part-time comparative 86% patients in the 6-hour group and 82% patients in the
versus minimal-time trial full-time group had improved by 3 lines from baseline.
patching for moderate
Theory and Practice of Squint and Orthoptics

amblyopia
ATS 2B A randomized trial 3–7yr 189 Randomised 4 months 6 hrs of patching as effective as 2 hrs of patching. At
comparing part-time comparative 4 months, no difference in amblyopic eye acuity between
versus full-time patching trial groups
for severe amblyopia
ATS 2C An observational study 3–7 yr 156 Observational 52 weeks Recurrence occurred in 35 (24%) of 145 cases and was
on recurrence of amblyopia similar in patients who stopped patching (25%) and
after disconti nuation of in patients who stopped atropine (21%). In patients
treatment treated with moderately intense patching (6 to 8 hours
per day), recurrence was more common (11 of 26, 42%)
when treatment was not reduced prior to cessation than
when treatment was reduced to 2 hours per day prior to
cessation. Approximately one-fourth of successfully-
treated amblyopic children experience a recurrence
within the first year off treatment.

(Contd...)
 Table 8.5 Summary of amblyopia treatment study reports (Contd...)
Study Aim Age No. of Type of Follow up Results
patients study
ATS 3 To evaluate the 7–13 yr 507 Observational 6 months In the 7 to <13-year-old, 53% of the treatment group
effectiveness of phase and were responders compared with 25% of the optical
optical correction alone randomised correction group (P<0.001). In the 13 to <18-year-old,
vs 2–6 hr/day of trial phase the responder rates were 25% and 23% respectively
patching combined overall (adjusted P = 0.22), but 47% and 20% respectively
with near visual activities among patients not previously treated with patching
plus atropine and/or atropine for amblyopia. For patients 7 to <13 years
old, prescribing 2 to 6 hours per day of patching with
near activities and atropine can improve visual acuity
even if the amblyopia has been previously treated. For
patients 13 to <18-year-old, prescribing patching 2 to
6 hours per day with near activities may improve visual
acuity when amblyopia has not been previously treated
but appears to be of little benefit if amblyopia was
previously treated sheet with patching.
ATS 4 A randomized trial 3–7 yr 168 Randomised 4 months Weekend atropine provides an improvement similar
comparing daily atropine comparative to that provided by daily atropine in moderate amblyopia.
versus weekend atropine trial
for moderate amblyopia
ATS 5A Prospective non- 3–7 yr 84 Prospective Up to 30 Amblyopia improved in 77% by optical correction and
comparative trial to non-comparative weeks resolved in 27%.
evaluate 2 hours of daily trial
patching for amblyopia
(eye glass only phase
study)
ATS 5B Randomized trial to 3–7 yr 180 Randomised 5 weeks Refractive correction alone improves visual acuity in
evaluate 2 hours daily trial many cases and results in resolution of amblyopia in at
patching for amblyopia least one-third of 3 to <7-year-old children with
(randomization phase) untreated anisometropic amblyopia. Following a period
of treatment with spectacles, two hours of daily patching
Adaptations to Strabismus and Amblyopia

combined with one hour of near visual activities modestly

improves moderate to severe amblyopia in children 3 to
7-year-old.
197

(Contd...)
 Table 8.5 Summary of amblyopia treatment study reports (Contd...)
198

Study Aim Age No. of Type of Follow up Results

patients study
ATS 6 A randomized trial of 3–7 yr 425 Randomised 17 weeks No difference in visual acuity improvement between
near versus distance comparative children performing near activities and distance
activities while patching trial activities during patching.
for amblyopia in
children 3 to < 7-
year-old
ATS 7 Bilateral refractive 3–11 yr 113 Randomised 1 year Bilateral refractive amblyopia improves with spectacle
amblyopia comparative correction. Binocular visual acuity of 20/25 or better was
treatment study: Response trial achieved by 73%
to treatment of previously
untreated presumed
bilateral refractive
amblyopia
ATS 8 To compare weekend 3–7 yr 180 Randomised 18 weeks Augmentation of weekend atropine with a plano lens
atropine augmented by comparative does not substantially improve amblyopic eye
Theory and Practice of Squint and Orthoptics

a plano lens with weekend trial acuity

atropine alone for moderate
amblyopia.
ATS 9 To compare patching with 7–12 yr 193 Randomised 17 weeks Atropine and patching achieve similar results among
atropine eyedrops in the comparative older children with unilateral amblyopia.
treatment of moderate trial
amblyopia
ATS 10 Randomized trial 3–10 yr 186 Randomised 24 weeks Bangerter filters and patching was less than half a line,
comparing bangerter filters comparative and there was lower burden of treatment on the child
vs part time occlusion trial and family, Bangerter filter treatment is a reasonable
for the treatment of option to consider for initial treatment of moderate
moderate amblyopia amblyopia
ATS 11 Randomized trial to 3–10 yr 55 Randomised 10 weeks Amblyopic eye VA improved similarly in both groups
evaluate combined trial suggestive of no additional benefit of combined
patching and atropine treatment in residual amblyopia
for residual amblyopia

(Contd...)
 Table 8.5 Summary of amblyopia treatment study reports (Contd...)
Study Aim Age No. of Type of study Follow up Results
patients
ATS 12 A randomized trial 7–<13 yr 19 Randomised 17 weeks 16-week treatment trial of vision therapy was feasible
comparing patching trial with respect to maintaining protocol adherence;
with active vision therapy however, recruitment under the proposed eligibility
to patching with control criteria, necessitated by the standardized
vision therapy as approach to vision therapy, was not successfull
treatment for amblyopia
in children 7 to <13 year
old; to determine the
feasibility of conducting
a full-scale randomized
clinical trial
ATS 13 Nonrandomized prospec- 3–7 yr 146 Non 28 weeks Treatment effect was greater for strabismic amblyopia
tive trial of glasses alone for randomised than for combined mechanism amblyopia
strabismic and strabismic- prospective
anisometropic amblyopia trial
ATS 14 A Pilot Study of Levodopa 8–18 yr 33 Randomised 10 weeks The results suggested that levodopa/carbidopa
dosage as treatment for trial therapy for residual amblyopia in older children
residual amblyopia and teenagers is well tolerated and may improve visual
acuity. There was a suggestion of partial regression of
the improvement in visual acuity after treatment was
discontinued.
ATS 15 Randomized trial of 3–8 yr 169 Randomised 12 weeks More improvement in VA after 10 weeks compared with
increasing patching trial continuing 2 hours daily.
for amblyopia
ATS 16 Augmenting atropine 3–8 yr - Not published
treatment for amblyopia,
the effectiveness of adding
a plano lens (a lens without
any prescription) to
weekend atropine treatment
Adaptations to Strabismus and Amblyopia

after a patient’s visual

acuity has stabilized but
199

amblyopia is still present.

(Contd...)
 Table 8.5 Summary of amblyopia treatment study reports (Contd...)
200

Study Aim Age No. of Type of Follow up Results

patients study
ATS 17 Compare the efficacy and 7–13 yr 139 Randomised 18 weeks For children 7 to 12 years of age with residual amblyopia
safety of oral levodopa trial after patching therapy, oral levodopa while continuing
and patching versus oral to patch 2 hours daily does not produce a clinically or
placebo and patching statistically meaningful improvement in VA compared
at 18 weeks, after 16 weeks with placebo and patching.
of treatment followed by
a two-week taper of oral
medication.
ATS 18 Compare the effectiveness 5–<12 385 Non-inferiority 16 weeks In children aged 5 to younger than 13 years, amblyopic-
of 1 hour/day of binocular years randomised trial eye VA improved with binocular game play and with
game play 7 days per week patching, particularly in younger children (age 5 to
with 2 hours/day patching <7 years) without prior amblyopia treatment.
7 days per week Although the primary noninferiority analysis was
indeterminate, a post hoc analysis suggested that VA
improvement with this particular binocular iPad
Theory and Practice of Squint and Orthoptics

treatment was not as good as with 2 hours of prescribed

daily patching.
ATS 20 To compare the efficacy of 4–13
1 hour/day of binocular years Not published
game play 5 days per week
plus spectacle correction
only, for treatment of
amblyopia
 Adaptations to Strabismus and Amblyopia 201

• Tincture benzoins may be applied to the skin be eliminated in about the same time it took to
before applying the patch. This forms develop by changing the occlusion to the other
a protective layer over the skin and also eye.
increases adhesiveness of the patch so Now, it has been established that chances of
that child is less likely to remove it. developing amblyopia in sound eye are more
• Opticlude patch is claimed to be hypo- with the penalization technique than with total
allergenic and can be replaced for ordinary occlusion. It is because the blurred diffuse
sticking patch. stimulus (a white noise type of stimulus)
• If a skin problem does develop, the patch produced by penalization is more amblyogenic
should be left on the eye until it falls off than the total occlusion.
rather than removing it every night, since ii. Occlusion esotropia. Sometimes, in anisome-
repeated removal of the patch aggravates tropic amblyopia, when no deviation exists and
the skin irritation. bifoveal single vision is present, constant
Occluding soft contact lenses have been occlusion may so disrupt binocular vision that
recommended where the above two methods an esotropia results. The parents should be
prove to be frustrating because of one or the warned of this possibility, but it should be
other reason. However, soft contact lenses have explained that the risk is worthwhile in the
their inherent problem of difficulty in interest of good visual acuity. The use of
application and complications associated with intermittent occlusion or partially transparent
soft contact lens use. occulsion (which allows binocular fixation) is
preferable to total occlusion as a precaution
2. Problem of parental co-operation against the development of this complication. If
A full co-operation of the parents is essential in a deviation does develop, it may not spon-
keeping the sound eye of the child occluded. taneously disappear once occlusion is
Many parents fail to co-operate for one or the discontinued.
other reason—may be their inability to devote
time for their child or may be a lack of under- 4. Recurrence of amblyopia
standing. Therefore, it is the duty of treating Once amblyopia has been corrected, chances of
person to hammer on the parents' mind the recurrence are always there until child is visually
importance of patching for the sake of their mature (10 years of age). Therefore, a careful
child, so much so, that even the parents should monitoring every month up to the age of 1 year,
be warned that they will be held responsible for every 2 months up to the age of 2 years and then
a permanent loss of vision of their child. every 4–6 months up to the age of visual
Instilling a feeling of guilt in the parents is not maturity is required. Not only this, a mainte-
wrong keeping in view the results of rightly and nance occlusion therapy (see page 192) should
timely performed occlusion therapy. also be carried out.

3. Complications of occlusion PROGNOSIS

Two complications may accompany occlusion— Prognosis of amblyopia therapy depends upon
occlusion amblyopia and occlusion esotropia. the age of patient, type of amblyopia and type
i. Occlusion amblyopia. In very young child- of treatment as below:
ren, occlusion amblyopia in the sound eye may • Younger the child better the prognosis.
occur in as short a time as 2 to 3 weeks. Not only • Amblyopia due to deprivation (e.g. due to
amblyopia but also eccentric fixation may congenital cataract) carries the poorest
develop in the previously normal eye when it is prognosis.
constantly occluded. It is, therefore, essential • Strabismic amblyopia has the best prognosis.
that visual acuity of the sound eye should be • Anisometropic amblyopia has a prognosis
carefully monitored during occlusion therapy. intermediate betwen strabismic amblyopia
If occlusion amblyopia does occur, it usually can and deprivation amblyopia.
 202 Theory and Practice of Squint and Orthoptics

• Presence of eccentric fixation worsens the From the above description, an impression is
prognosis. created as if ARC is a new point-to-point
• Amblyopia with unilateral high hyper- correspondence between the two eyes leading to
metropia has a poorer prognosis than the something like normal fusion, stereopsis, etc.
amblyopia with unilateral high myopia. However, in fact, this is not the case, rather the
• Prognosis is better, when treated with total ARC is a fragile, variable form of binocular co-
and full time occlusion than when treated with operation depending very much on the
penalization or other methods. momentary conditions of binocular vision.
Further, ARC does not replace NRC. In fact NRC
appears to be suspended since on some tests it can
ABNORMAL RETINAL CORRESPONDENCE be demonstrated that NRC co-exists with ARC.
GENERAL CONSIDERATIONS
Harmonious versus unharmonious ARC
As we know, in a state of normal single binocular
Before exactly defining harmonious and
vision, there exists a precise physiological
unharmonious ARC, we should revise the
relationship between the corresponding points
definitions of objective and subjective angles of
of the two retinae. And that the foveae of two
deviation and that of angle of anomaly.
eyes act as principal corresponding points and
have the same visual direction. This adjustment • Objective angle of deviation is the amount of
is called normal retinal correspondence (NRC). deviation measured, when no shift of the eyes
When squint develops, patient may experience is observed by the examiner on the prism and
either diplopia or confusion. To avoid these, alternate cover test. When measured on synopto-
sometimes (especially in children with small phore, it is the position of the instrument's arm
degree of esotropia), there occurs an active when no shift occurs as the lights in the tubes
cortical adjustment in the directional values of are alternately turned off and on.
the two retinae. In this state, fovea of the normal • Subjective angle of deviation when measured
eye and an extrafoveal point on the retina of the with the synoptophore, is denoted by the
squinting eye acquire a common visual direction position of the instrument's arms at which the
(i.e. become corresponding points). This patient can superimpose the images of dissimilar
condition is called abnormal retinal corres- test objects.
pondence and the child gets a crude type of • Angle of anomaly refers to the difference
binocular vision. Thus, abnormal retinal between the objective angle of deviation and
correspondence (ARC) is a binocular sensory subjective angle of deviation. In NRC, the
defence mechanism against peripheral diplopia objective and the subjective angles of deviation
and peripheral confusion. It is important to note are equal and so the angle of anomaly is zero. In
that ARC is entirely a binocular phenomenon, ARC, the subjective angle is always less than the
i.e. when the eyes are used monocularly, there objective angle and so the angle of anomaly is
is no change in visual direction of any retinal more than zero. Depending upon the value of
element. While eccentric fixation is a monocular angle of anomaly, the ARC is of two types as
phenomenon in which patient takes fixation follows:
with an extrafoveal point. If the fixating eye of a
patient with ARC is covered, he/she will turn 1. Harmonious ARC is present, when the angle
the deviating eye to fixate either with his/her of anomaly equals the objective angle of squint.
fovea (if he/she has central fixation) or with a In other words, in harmonious ARC, subjective
peripheral area (if he has eccentric fixation). angle of deviation is zero indicating total
Only in a minority of patients are ARC and compensation for the deviation.
eccentric fixation related in such a manner that 2. Unharmonious ARC is present, when the
the point of anomaly during binocular single angle of anomaly is less than the objective angle
vision is also the point of eccentric fixation of deviation. In other words, in unharmonious
during monocular vision. ARC, the subjective angle of deviation is
 Adaptations to Strabismus and Amblyopia 203

between zero and the objective angle, • Patients with constant angle of squint are more
indicating thereby that the ocular deviation has likely to develop ARC as compared to those
not been fully compensated. In fact, with variable angle of squint.
unharmonious ARC is presently considered an • ARC seems to develop more frequently, when
artifact of the more dissociating testing the angle of esotropia is between 10D and 20D.
conditions; since a more physiological test (e.g. This is because of the fact that the retinal area
Bagolini's test) reveals harmonious ARC and a of the squinting eye, which receives the same
more dissociating test (e.g. synoptophore or stimulation as the fovea of the fixing eye, is
red-green glasses) may reveal unharmonious close to the fovea and possesses good visual
ARC in the same patient. acuity, thus the false image is not only close to
the true one but is also relatively clear and the
DEVELOPMENT OF ARC resultant diplopia is troublesome, and in order
Factors affecting development of ARC to overcome this, ARC develops more rapidly.
ARC is a sensory adaptation that is brought
Natural course of development of ARC
about by an inherent desire for some form of
binocular vision and to avoid diplopia and • During natural course of development of
confusion that would otherwise take place. ARC, the angle of anomaly gradually
However, ARC does not develop in each and increases until it equals the amount of
every case with strabismus. The factors that have objective deviation and the ARC becomes
been reported to favour and unfavour the harmonious.
development of ARC are as follows: • During development of ARC, the NRC is not
immediately and rarely totally suppressed.
1. Age of onset of squint. ARC develops only in
NRC and ARC may both coexist in some
visually immature children who have acquired
patients, especially in those with intermittent
binocular single vision. Therefore, chances of
exotropia. Such patients may show NRC while
ARC development are more, if the squint occurs
fusing and ARC while tropic.
between 1 and 6 years of age. Therefore, patients
• The actual development of ARC appears to
in whom binocular single vision is not developed
occur slowly. But once established the shift
due to presence of early infantile esotropia do not
from ARC to NRC and back again can occur
develop ARC. On the other hand, visually mature
very rapidly.
patients (beyond 6–7 years of age) who acquire
strabismus are also incapable of developing ARC. ARC AND SUPPRESSION
In other words, in young children (below
6–7 years), with binocular single vision, the Both ARC and suppression are the sensory
instability of the binocular reflexes leads to adaptations to prevent diplopia and confusion.
replacement of normal reflex development by They may occur alone or may coexist in the same
abnormal binocular reflexes. patient. Following observations have been made
about occurrence of ARC and suppression:
2. Patient profile. ARC develops more 1. In patients with large deviations suppression
frequently in patients where binocular single is the rule without associated ARC.
vision has previously existed and the patient has
2. In patients with low degrees of strabismus
a high degree of general adaptability and
(30D or less), ARC and/or suppression may
intelligence.
develop.
3. Type and amount of strabismus 3. The presence of suppression does not prevent
• ARC develops more commonly in esotropes the development of ARC. Rather some studies
than exotropes and is less common with quote that suppression is a prerequisite for
vertical deviations. establishment of ARC.
• ARC develops more commonly in patients 4. Co-existence of ARC and suppression can be
with uniocular squint than in patients with demonstrated, when Bagolini's striated
alternating squint. glasses are used to test an esotropic patient.
 204 Theory and Practice of Squint and Orthoptics

As shown in Fig. 8.14, when the foveola of

the non-deviating eye is aligned with an
extrafoveal point in the deviating eye
(Fig. 8.14B), the streak seen by the deviating
eye has a gap in it (Fig. 8.14C). This gap corres-
ponds to a suppression scotoma in the devia-
ting eye. Since the patient sees the diagonal
streaks as a cross and the streak seen by the
deviating eye is in line with the fixation light,
this indicates that in the periphery, ARC
coexists with the central scotoma (Fig. 8.14).
5. Suppression and ARC when co-exist, are
complementary mechanisms, since the extra-
foveolar suppression in the deviating eye
protects against central diplopia, while ARC
eliminates peripheral diplopia and peripheral
visual confusion.

QUALITY OF BINOCULAR VISION IN ARC

Simultaneous perception of images in the two
eyes occurs in ARC due to correspondence of
fovea of one eye with some extrafoveal area of
the other eye.
Fusion in ARC. Possibly, restoration of some form
of motor fusion occurs with the development of
ARC. Since ARC provides a low-quality type of
peripheral fusion, the occurrence of fusional
movements might be expected in ARC. However,
when a change in the strabismic angle occurs,
patients with ARC should be expected to change
the angle of anomaly to avoid peripheral diplopia.
Peripheral fusion is thereby achieved without a
fusional movement. Thus, the fusional movements, Fig. 8.14 Bagolini's striated glass test depicting co-existence
if they do occur in ARC, are probably limited. of suppression and ARC (for explanation, see text).
Further, some workers believe that the fusional
movements noted in ARC may not be true It is important to remember that:
fusional movements and instead might be the • Normal stereopsis (40 seconds or better),
manifestations of covariations of the angle of indicates bifixation, i.e. perfect central and
anomaly. The covariation of angle of anomaly peripheral fusion.
with the angle of deviation is believed to be • Gross stereopsis (67–3000 seconds of arc),
possible because of a point-to-area relationship indicates monofixation, i.e. peripheral fusion
in ARC. Point-to-area relationship means that without central fusion.
numerous retinal elements in the deviating eye • Stereopsis is absent in peripheral fusion with
can apparently be coupled with a single retinal arc. However, it has been reported that a gross
element in the non-deviating eye. stereopsis (usually less than 120 minutes of
Stereopsis and ARC. It has been concluded that ARC), is a common finding in patients with
ARC and stereopsis do not coexist. The absence ARC and small angle esotropia or microtropia
of stereopsis is indicative of the low quality of which may occasionally even be demonstrable
ARC peripheral fusion. with random dot stereograms.
 Adaptations to Strabismus and Amblyopia 205

CLINICAL PHENOMENA the same basis. Following three stages have been
ASSOCIATED WITH ARC postulated for the occurrence of changes in ARC
after surgical correction of strabismus:
Paradoxical diplopia
1. Stage of ARC (continues for some time).
Paradoxical diplopia is the one which is not
expected in a particular type of deviation. 2. Stage of rivalry between normal and anomalous
Obviously, it occurs because of abnormal pro- correspondence (sometimes patient may
jection. In ARC, paradoxical diplopia occurs complain of monocular diplopia during this
under following circumstances: stage).
1. It can be elicited in patients with ARC, when 3. Stage of normal correspondence (develops in
both foveas are simultaneously stimulated with favourable cases). It has also been reported
major amblyoscope. It can also be elicited with that all patients may not develop NRC after
after-image test (see page 132 and Fig 6.33). surgical correction of strabismus.
2. Paradoxical diplopia can also be perceived by Factors which influence the postoperative changes
a patient in which ARC continues after surgical in correspondence are as follows:
correction of deviation. Though, in clinical • Age of the patient at the time of operation.
practice, paradoxical diplopia is a fleeting • Depth of ARC.
phenomenon limited to the immediate • Use of the eyes made by the patient.
postoperative period, which usually disappears
• Individual adaptability.
after a few days or weeks of surgery. However,
rarely it can persist long and may make the
TESTS FOR ARC
patient's life miserable.
See page 129.
Monocular diplopia
MANAGEMENT OF ARC
Monocular diplopia is a condition which
sometimes occurs in patients with ARC, when The important points to be noted are:
an object stimulating a retinal area is projected • Great emphasis used to be laid on the treatment of
in two different visual directions, i.e. the normal ARC in the past before the surgical treatment of
one and the abnormal one. Under binocular strabismus. However, according to the current
viewing, the patient may have binocular views, orthoptic treatment for ARC in patients
triplopia. In rare cases, binocular triplopia with strabismus is not required.
occurs spontaneously, but just like paradoxical • Treatment modalities used for ARC in the past
diplopia, binocular triplopia can frequently be (now of historical interest only) included:
provoked instrumentally, e.g. by appropriate Occlusion therapy, prismatic overcorrection
stimulation with synoptophore. of the ocular deviation and use of major
amblyoscope for retinal stimulation (retinal
Postoperative changes in correspondence massage).
Now most of the workers believe that in ARC • Presently, it is believed that the most effective
there occurs point-to-area correspondence, i.e. treatment for terminating ARC, is surgical
numerous retinal elements in the deviating eye realignment of the eyes.
can apparently be coupled with a single retinal • A small-angle, cosmetically inconspicuous
element in the deviating eye. Thus, with the residual strabismus with ARC is now
change in the angle of deviating eye, the retinal considered an acceptable or even desirable end
point of the non-deviating eye starts corres- stage of therapy in infantile esotropia.
ponding with some other retinal point of the
deviating eye. Thus, the angle of anomaly may
MOTOR ADAPTATIONS
adapt to considerable variations in the angle of
strabismus. Disappearance of ARC after surgical As discussed above, suppression and ARC are
correction of strabismus has been explained on the sensory adaptations which help the
 206 Theory and Practice of Squint and Orthoptics

strabismus patients to deal with annoying • Chin is elevated in A-pattern esotropia and V-
diplopia and/or confusion. These mechanisms pattern exotropia.
are available only to the visually immature 3. Nystagmus patients may place their head in
children who acquire strabismus after deve- such a way that the eyes are positioned in the
loping singular binocular vision. The older, null zone, thereby improving binocular visual
visually mature patients, have no capacity to acuity.
develop sensory adaptations. However, these
patients may deal with the confusion and/or BLIND SPOT SYNDROME
diplopia by developing motor adaptations,
The blind spot syndrome is a motor adaptation
which include:
to cope up with the diplopia and/or confusion
• Control of ocular deviation by an alteration
in patients with esotropia. It occurs, when the
in tone of extraocular muscle,
esotropia is of small to moderate degree with
• Compensatory head posture, annoying diplopia and without sufficient
• Blind spot syndrome, and divergence to regain foveal fixation. In this
• Blind spot mechanism. condition, a motor response tends to move the
eye further to the esotropic side, projecting the
CONTROL OF OCULAR DEVIATION BY AN image seen by the deviating eye on to the blind
ALTERATION OF TONE OF THE EXTRAOCULAR spot (optic disc) (Fig. 8.15).
MUSCLES
Occasionally, a small degree of ocular deviation Characteristics of blind spot syndrome
may be controlled by an alteration of tone of • Esotropia of 12° to 18° (25 to 35).
extraocular muscles, which may be of the nature • Good visual acuity in each eye.
of an active contraction or an active relaxation. • Normal retinal correspondence (NRC).
The alterations in the extraocular muscles are • No suppression other than the fovea of the
induced by the compelling influence of the fusion deviated eye.
reflexes so that there is a disappearance of the • Normal fusional vergences.
squint and a consequent absence of any diplopia • Diplopia is elicited, when the deviation is
or confusion. In this way, a manifest deviation is prismatically reduced.
converted into a latent one. The success of such • Occasional diplopia and/or confusion in
an adaptation, however, may be frustrated to casual seeing. Transitory diplopia is most
some extent by an inadequacy of the fusional
reserves, so that the deviation is controlled only
at the expense of significant discomfort.
However, sometimes this adaptation permits a
satisfactory degree of functional control.

COMPENSATORY HEAD POSTURE

Compensatory head posture is a common motor
adaptation used by visually mature patients to
deal with the diplopia and/or confusion. It
occurs under following circumstances:
1. Paralytic strabismus is a particularly
common situation in which compensatory head
posture is used to achieve fusion of the images
seen by the two eyes. For details see page 289.
2. A- and V-pattern strabismus may also be
associated with an abnormal head posture.
• Chin is depressed in A-pattern exotropia and Fig. 8.15 Blind spot syndrome or mechanism. Note, image
V-pattern esotropia. in the right esotropic eye is formed on the optic disc.
 Adaptations to Strabismus and Amblyopia 207

commonly noted, when approaching car lights 4. Bagolini, B: Anomalous correspondence: defi-
are seen at night. nition and diagnostic methods. Doc Ophthalmol
• Good fusion potentialities demonstrated on 23:346, 1967.
haploscopic devices. 5. Bagolini, B: I Sensorial anomalies in strabismus
(suppression, anomalous correspondence
amblyopia). Doc Ophthalmol 41:1, 1976.
BLIND SPOT MECHANISM
6. Bagolini, B: 11. Sensorio-motorial anomalies in
The blind spot mechanism is a coincidental type strabismus (anomalous movements). Doc
of esotropia in which the image of the fixated Ophthalmol 41:23, 1976.
object falls on the blind spot (optic disc) of the 7. Baker, FH, Grigg, P and Noorden, GK von:
deviated eye (Fig. 8.15). Effects of visual deprivation and strabismus on
the response of neurons in the visual cortex of
Characteristics of blind spot mechanism the monkey, including studies on the striate
• Amblyopia of the non-dominant eye prestriate cortex in the normal animal. Brian
• Abnormal retinal correspondence Res. 66: 185, 1974.
• Deep suppression 8. Bielschowsky A: Lectures on motor anomalies,
V Development and causes of strabismus. Am
• ARC and suppression may coexist
J Ophthalmol. 22:38, 1939.
Presence of the above abnormal sensory 9. Boeder, P: Anomalous retinal correspondence
findings makes the differential diagnosis refuted. Am J Ophthalmol 58:366, 1964.
between the blind spot syndrome and blind spot 10. Bradley, A, and Freeman, RD: Is reduced vernier
mechanism. acuity in amblyopia due to position, contrast,
or fixation deficits. Vision Res. 25:55, 1985.
11. Burian, HM: Sensorial retinal relationship in
PSYCHOLOGICAL ADAPTATION concomitant strabismus. Trans Am Ophthalmol
IGNORING Soc. 81: 373, 1945.
12. Burian, HM: Normal and anomalous correspon-
Ignoring is a psychological adaptation which is dence. In Allen, JH, editor: Strabismus
learnt by some visually mature patients to deal ophthalmic symposium I, St. Louis, 1950,
with the annoying diplopia. It probably falls Mosby-Year Book, Inc., p130.
somewhere between conscious and unconscious 13. Burian, HM: Anomalous retinal correspondence:
thought. All patients cannot learn to ignore Its essence and its significance in diagnosis and
diplopia. Further, it has also been reported that treatment. Am J Ophthalmol 34:237, 1951.
the patients with excellent acuity in each eye 14. Burian, HM: Adaptive mechanisms. Trans Am
may be able to ignore the weaker image while Acad Ophthalmol Otolaryngol 57:131, 1953.
the patients with poor vision cannot. How this 15. Burian, HM: Thoughts on the nature of
occurs is not known exactly. Perhaps the amblyopia exanopsia. Am Orthopt J 6:5, 1956.
individual's psychologic factors play an 16. Burian, HM: The behavior of the amblyopic eye
under reduced illumination and the theory of
important role in the development of rare
functional amblyopia. Doc Ophthalmol 23: 189,
phenomenon of ignoring. 1967.
17. Burian, HM, and Cortimiglia, RA: Visual acuity
BIBLIOGRAPHY and fixation pattern in patients with strabismic
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amblyopia Invest Ophthalmol Vis. Sci. 26:88, in microtropia. Arch Ophthalmol 81:753, 1969.
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2. Assaf AA. The sensitive period: transfer of of the near point of convergence and its signi-
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Br J Ophthalmol 66:64, 1982. ciency. Am Orthopt J 2:40, 1952.
3. Bagolini B. Presentazione di una sbarra di filtri 20. Chavasse, BF: Worth's squint or the binocular
a densita scalare assorbenti i raggi luminosi reflexes and the treatment of strabismus,
Boll Ocul 36:638, 1957. Philadelphia, 1939 P Blakiston's Son & Co. Inc.
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21. Crawford MLJ, visual deprivation syndrome. 31. Noorden, GK von: Pathogenesis of eccentric
Ophthalmology (Rochestter 85: 465, 1978. fixation. Doc Ophthalmol. 23:263, 1967.
22. Firth, AY: Pupillary responses in amblyopia. Br 32. Noorden, GK von: The etiology and patho-
J Ophthalmol 74:676, 1990. genesis of fixation anomalies in strabismus.
23. Garey L, Blakemore C: Monocular deprivation: Trans. Am. Ophthalmol. Soc. 67:698, 1969.
Morphological effects on different classes of 33. Noorden, GK von: Histological studies of the
neurons in the lateral geniculate nucleus. Science visual system in monkeys with experimental
195:414, 1977. amblyopia Invest Ophthalmol 12:727, 1973.
34. Noorden, GK von: Mechanisms of amblyopia.
24. Grunwald MJ, Parks MM: Amblyopia. In
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Tasman W, Jaeger EA (eds): Duane's clinical
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Ophthalmology, Philadephia, JB Lippincott
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25. Hielschowsky, A: Application of the afterimage
36. Noorden, GK von: Idiopathic amblyopia. Am J
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26. Hubel, DN, and Wiesel, TN: Receptive fields, disciplinary approach (Proctor Lecture). Invest.
binocular interaction and functional architecture Ophthalmol. Vis, Sci. 26:1704, 1985.
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38. Noorden, GK von, and Crawford, MLI:
160:106, 1962
Morphological and Physiological changes in
27. Hubel, DH, and Wiesel,TN: Receptive fields of the monkey visual system after short-term lid
single neurons in the cat's striate cortex. J Physiol suture. Invest Ophthalmol Vis. Sci. 17:762, 1978.
148:574, 1959 39. Noorden, GK von, and Maumenee, AE: Clinical
28. Hubel, DH, and Wiesel, TN: Stereoscopic vision observations on stimulus deprivation amblyopia
in macaque monkey, cells sensitive to binocular (amblyopia exanopsia). Am J Ophthalmol 65:
depth in area 18 of the macaque monkey cortex. 220, 1968.
Nature 225:41, 1970 40. Noorden, GK von, and Middleditch PR:
29. Jampolsky, A: Characteristics of suppression in Histology of the monkey lateral geniculate
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 Heterophoria 209

9
Heterophoria

HETEROPHORIA Symptoms
Definition • Compensated heterophoria
Etiology
• Decompensated heterophoria
Evaluation
Types
Treatment
• Esophoria
• Exophoria • Indications of treatment
• Hyperphoria • Choice of treatment
• Cyclophoria • Treatment modalities

HETEROPHORIA TYPES OF HETEROPHORIA

Depending upon the direction of deviation,
DEFINITION heterophoria may be:
Heterophoria, also known as 'latent strabismus', • Esophoria
is a condition wherein the tendency of the eyes
to deviate is kept latent by the fusion reflex. • Exophoria
However, when the fusion is interrupted, for • Hyperphoria, and
example, by occluding one eye, the visual axis • Cyclophoria.
of the occluded eye deviates from its position
and if the occluder is removed, the fusion reflex
will turn the eye back into the orthoposition. ESOPHORIA
While, orthophoria is a condition of perfect In this condition, when fusion is interrupted, the
alignment of the two eyes which is maintained non-fixating eye becomes convergent, i.e.
even after the removal of influence of fusion. deviates nasally. Esophoria is less frequent than
However, orthophoria is just a theoretical ideal. exophoria. Only about 33% of all patients with
Practically, a small amount of heterophoria is a distance phoria are esophoric. Near esophorias
of universal occurrence and is known as occur even less frequently.
'physiological heterophoria'. The incidence of
distance heterophoria in primary position has
been reported to be as high as 80% and that for Clinical types
near phorias close to 100%. When tested in Esophoria may be:
different directions of gaze, almost everyone is 1. Convergence excess type. Esophoria is greater
found to have small degree of heterophoria. on near fixation than on distant fixation.
ETIOLOGY 2. Divergence weakness type. Esophoria is greater
In general, the factors which play role in the on distant fixation than on near fixation.
etiology of heterophoria are the same as for 3. Non-specific type. Esophoria which does not
heterotropia. For details see page 97. vary significantly in degree for any distance.
209
 210 Theory and Practice of Squint and Orthoptics

Etiological types complain that during reading letters run

The etiological factors have been discussed in together. The blurring is due to accommodation
general etiology of heterophoria. Common and occurs when fusional convergence becomes
etiological types of esophoria are as follows: insufficient to cope up with the deviation, e.g.
during prolonged close work.
1. Accommodative esophoria. It may be
2. Divergence excess type. Exophoria is greater
associated with:
on distant fixation than on near fixation. In such
a. Uncorrected high hypermetropia. In this condi- patients, asthenopic symptoms occur during
tion, excessive use of accommodation in a bid to activities connected with distant vision.
clear the blurred retinal image is associated with
3. Non-specific type. This type of exophoria does
increased accommodative convergence. It may
not vary significantly in degree for any distance.
result in esophoria for distance and near in
proportion to the accommodation used and the
Relation of near exophoria to
patient's fusional divergence.
distance exophoria
b. High AC/A ratio may be associated with
The basic deviation in exophoria is measured
esophoria. Effect of AC/A ratio on basic
while the patient fixates a distant target using
esophoria is depicted in Table 9.1.
his/her full optical correction and near
Table 9.1 Effect of AC/A ratio on basic esophoria exophoria is measured, when patient fixates a
AC/A ratio Esophoria target at 33 cm. The patient's AC/A ratio
influences the magnitude of near phoria (X') in
Normal E1 slightly smaller than E
High E1 almost equal or greater
relation to distance phoria (X) as depicted in
than E Table 9.2.
Low E1 smaller than E Table 9.2 Effect of AC/A ratio on near vs distance
exophoria
2. Tonic esophoria. Most of the non-accomm- AC/A ratio Exophoria
odative esophorias are due to increased tonic
Normal X1 slightly greater than X
convergence which may be due to either over- High X1 almost same or smaller
activity or insufficient inhibition of the involved than X
centres of the central nervous system. Low X1 greater than X
3. Esophoria due to other causes includes those
due to anatomical and innervational factors. HYPERPHORIA
It is a vertical deviation occurring on disso-
EXOPHORIA
ciation in which one eye rotates upwards or the
In this condition, the non-fixating eye becomes other downwards depending upon the fixation.
divergent on dissociation, i.e. when fusion is In orthoptic practice, the term hypophoria is
interrupted. However, under condition of avoided and it has become customary to use the
bifoveal single vision, the deviation is corrected term right or left hyperphoria depending upon
by the fusional convergence. Exophoria is the the eye which remains up as compared to the
commonest variety of heterophoria. It consti- other. For example, in a right hyperphoria, the
tutes about 60% of all cases of heterophoria for right eye rotates upwards, when it is not fixating
distance and about 80% of all cases of and when the right eye is fixating, the left eye
heterophoria for near. rotates downwards, and reverse to it occurs in
left hyperphoria.
Types
The incidence of hyperphoria has been
Exophoria may be of following types: reported to be about 30%. Frequently, hyper-
1. Convergence weakness type. Exophoria is phoria is combined with horizontal phorias.
greater on near fixation than on distant fixation. Since vertical fusional vergences are of small
In this condition, symptoms primarily occur amplitude, asthenopic symptoms are more
during close work. Such patients frequently marked in hyperphorias than the horizontal
 Heterophoria 211

phorias. For the same reason, even the minor Decompensated heterophoria. It is associated
prismatic action of glasses (due to vertical with multiple symptoms which may be grouped
decentring) may be difficult to compensate. as under:
A hyperphoria may be comitant or incomitant. 1. Symptoms of muscular fatigue. These result
Paresis of an extraocular muscle and anatomic due to continuous use of the reserve neuro-
anomalies of muscles and tendons and/or their muscular power and are usually more marked
insertions constitute the major causes of after the day's work. These include:
hyperphoria. Since, more frequently, hyperphoria
• Headache and eye ache after prolonged use of
is incomitant, measurements should be taken:
eyes, which is relieved when the eyes are
(a) with either eye being the fixating eye, (b) in
closed for a while.
the cardinal positions, and (c) in the reading
position. The latter is obviously more important • Difficulty in changing the focus from near to
since a special therapeutic solution has to be distant objects of fixation or vice versa.
found, if the patient's deviation in the reading • Photophobia, itching and burning may also be
position differs from that in the primary experienced by some patients. Photophobia
position. due to muscular fatigue is not relieved by
using dark goggles, but is relieved by closing
CYCLOPHORIA one eye.
It is the tendency of the either eye to wheel 2. Symptoms due to failure to maintain
rotate around the anteroposterior axis on binocular vision. These include:
dissociation. When the 12 O'clock meridian of • Blurring or crowding of words while reading.
cornea rotates nasally, it is called incyclophoria • Intermittent diplopia due to temporary manifest
and when it rotates temporally, it is called deviation under conditions of fatigue.
excyclophoria. Excyclophoria is of more frequent • Intermittent squint (without diplopia) may
occurrence than the incyclophoria. In general, occur in patients who involuntarily learn to
cyclophorias of clinical significance are of rare suppress. It is usually noticed by the patient's
occurrence and are frequently associated with close relations or friends.
vertical deviation. Asthenopic symptoms are
most marked with cyclophorias. 3. Symptoms of defective postural sensations
cause problems in judging distances and posi-
SYMPTOMS OF HETEROPHORIA tions, especially of the moving objects. This
difficulty may be experienced particularly by
Symptoms that may be caused by phorias are cricketers, tennis players and pilots during
of a very non-specific nature and they may also landing.
occur for other reasons. In general, the term
asthenopia is used to denote the symptom
EXAMINATION (EVALUATION) OF
complex produced by heterophoria. As already
A CASE OF HETEROPHORIA
mentioned, asthenopia is most marked with
cyclophoria followed by hyperphoria. Small It should include the following tests:
degrees of esophoria and exophoria produce 1. Testing of visual acuity and refraction. It is
little or no symptoms. of prime importance in management of
Depending upon the symptoms, heterophoria heterophoria.
can be divided into compensated and decomp- 2. Testing for ocular movements. Uniocular as
ensated. well as binocular movements should be tested
Compensated heterophoria. It is associated with meticulously (see page 113).
no subjective symptoms. Compensation of the 3. Cover-uncover test. It is quite useful in
phoria depends upon the reserve neuro- detecting the presence of a heterophoria. When
muscular power to overcome the muscular using an occluder to dissociate the eyes, the
imbalance and individual's desire for maintenance fusion reflex innervation does not immediately
of binocular vision. close completely covering one eye, although all
 212 Theory and Practice of Squint and Orthoptics

fusional stimuli are excluded by the cover. The power of the fusional vergence that
Therefore, the cover should be kept in front of opposes and overcomes the phoria during
the eye for a few moments before proceeding bifoveal vision is very important in planning the
with the test. treatment of phoria. The fusional vergence
4. Measurement of heterophoria. A hetero- available in excess of the amount necessary to
phoria should be measured for both distance overcome the phoria and to bring the eyes into
and near. To obtain the basic measurement of a orthoposition is called the fusional reserve or the
heterophoria, the patient should fixate a distant relative vergence. Though there does not exist
target and should not accommodate. It is any relationship between the amount of fusional
necessary, therefore, that he/she wears full reserve and degree of phoria producing
optical distance correction. It should also be kept symptoms, but, in general, bifoveal vision is
in mind that lenses that are decentred before the comfortable, when the fusional reserve is twice
eye have a prismatic effect and that this may as large as the phoria.
obscure the true situation because the patient 7. Assessment of state of binocular vision. It is
has to overcome such an effect during binocular also helpful in planning the management of a
vision through fusional vergence. Thus a phoria heterophoria. For details of the various tests, see
may be simulated or depending on the direction page 83.
of prismatic displacement, an existing phoria TREATMENT OF HETEROPHORIA
may be exaggerated or minimized, if lenses of
sufficient power are decentred to a significant INDICATIONS OF TREATMENT
degree during the phoria test. • In adults, treatment is indicated only if the
The near phoria should be measured at a patient is suffering from symptoms. Some-
fixation distance of about 33 cm using a target times it is difficult to decide whether or not
which requires accommodation rather than a the asthenopic symptoms are due to the
flashlight. In presbyopes, the near phoria should phoria. In such cases, occlusion may be used
be determined while they fixate through their to diagnose. If this relieves the symptoms, it
reading glasses. is very likely that the phoria is the cause.
Commonly employed tests to measure the heterophoria • In children, however, treatment is indicated, if
are as follows: the phoria is showing a tendency to decom-
Objective tests: Prism and cover test (page 105) pensate into a manifest deviation, whether
symptoms are present or not.
Subjective tests include:
• Maddox rod test (page 109) CHOICE OF TREATMENT
• Maddox wing test (page 111) The treatment modalities employed in hetero-
• Double prism test (page 113) phoria includes, glasses, miotics, orthoptic
5. Measurement of convergence, accommodation exercises, prismotherapy and surgery. The
and AC/A ratio. A knowledge about these para- choice of a particular modality in a given patient
meters is quite useful in the management of depends upon different factors, some of which
heterophoria. are as follows:
1. Age and cooperation of the patient, e.g. in a
6. Measurement of fusional reserve. It is
small child, miotics may be used to facilitate
essential to examine the patient's fusional
accommodation.
vergence amplitude to form a meaningful
opinion of the significance of his/her phoria. 2. Etiology of heterophoria
During this examination, an excessive demand • If a phoria is entirely or partly caused by a
is placed on the vergence mechanism. This may refractive error, glasses will eliminate or
induce a transient phoria in some patients. improve the condition.
Therefore, in clinical practice, the phoria should be • When a phoria is due to abnormal AC/A ratio,
measured first and the vergence amplitude orthoptic exercises to increase the fusional
determined afterward. amplitudes are the method of choice.
 Heterophoria 213

• Surgery is indicated in phorias of anatomic or to achieve a prismatic effect thus relieving the
paretic origin for which other treatment stress on patient's vertical vergence control.
methods cannot be successful. • In cyclophoria, the best efforts should be made
3. Size and type of heterophoria to correct the astigmatic refractive error, when
associated.
• When the heterophoria is too large to be
controlled easily with other methods, surgery 2. Orthoptic treatment
will be necessary. Aim of orthoptic treatment of heterophoria is to
• When vertical phorias are associated with improve fusional reserves so that the bifoveal
horizontal phorias, an attempt should be made single vision can be maintained without efforts.
to eliminate the vertical deviation first, since Orthoptic treatment also known as ‘vision
in a number of instances, this will enable the therapy’ includes:
patient to control the horizontal phorias with
A. Conventional or non-computerised excercise for:
much less effort.
• In-office vision therapy, and
4. The speed of recovery to binocular single vision • Home vision therapy
on cover test. Symptomatic phorias with very
B. Computerised orthoptic excercises for:
slow recovery require surgical treatment more
often than those with a quick recovery. • In-office vision therapy, and
• Home vision therapy
TREATMENT MODALITIES Note: Conventional, i.e. non-computerised
excercises are described here. For computerised
1. Optical correction
exercises see page 146.
In all cases, a meticulous refraction should be
performed and correct glasses prescribed. Orthoptic exercises for esophoria
Optical correction may eliminate the underlying In general, the orthoptic treatment of esophoria
cause of the phoria in some patients. General is aimed at improving the amplitude of fusional
guidelines for prescribing glasses are as follows: divergence (relative negative convergence). This
• Astigmatic errors and spherical differences can be accomplished by the following exercises:
between the two eyes should be eliminated a. Divergence exercises with prisms. Prisms of
completely. increasing strength are placed base-in before one
• In exophoria, both eyes may be undercorrected eye while he/she is fixating an object at any
by an equal amount of spherical plus power. distance (preferably at a distance where the
This forces the patient to accommodate esophoria is maximum). Patient is trained to
constantly and accordingly induces accommo- maintain a single vision by relaxing the conver-
dative convergence. However, it should be gence.
kept in mind that constant accommodation Loose prisms, a prism bar or rotatory (Risley)
itself may lead to eye strain. prisms may be used for this purpose. A prism
• In esophoria, the patient should receive as much bar should be preferred. Prism exercises are
spherical plus correction as is compatible with performed for a few minutes at each weekly
his/her best visual acuity. Bifocal glasses visit.
decrease or eliminate the need for accom- b. Divergence exercises on synoptophore. These
modation during near vision and thus may be exercises are performed on synoptophore using
useful in patients having esophoria of stereopsis slides, because they provide the
convergence excess type. Bifocals should be strongest stimulus to fusion. After fusing the two
used as a temporary aid to orthoptic treatment pictures, patient is trained to maintain a single
aiming to reduce the bifocal segment as soon vision (by relaxing convergence) while the
as possible. instrument tubes are diverged. These exercises
• In hyperphoria, if feasible, the lenses of the should be performed for about 5 minutes at each
patient's optical correction may be decentred weekly visit.
 214 Theory and Practice of Squint and Orthoptics

Fig. 9.1 Stereogram card.

c. Physiologic diplopia exercises using

stereograms in the crossed position. To perform
this exercise, patient is first trained to appreciate
crossed physiological diplopia with a flash light
or pencil. Once the patient is trained, the exercise
is performed as below:
While the patient is fixating a distant object, the
stereogram card (Fig. 9.1) is held about 25 cm in
front of his/her eyes. Patient will perceive four
pictures (because of crossed physiological
diplopia). He/she is trained to adjust its position
until the two central pictures are fused and patient
perceives three pictures (Fig. 9.2). Patient is trained
to maintain the joined pictures and to see it clearly.
While doing so, patient is converging for the
distant target but accommodating for near
(distance of the card) and thus relatively relaxing Fig. 9.2 Physiologic diplopia excercises using stereogram
his/her convergence. in the crossed position.
Once the patient is trained to perform this
Orthoptic exercises for exophoria
exercise, he/she can practice at home for a few
minutes several times a day. The aim is to improve the fusional convergence
(relative positive convergence). This can be
d. Exercises using diploscope. For details see
accomplished by the following:
page 141.
a. Convergence exercise with prisms. It is similar
e. Exercises using Remy separator. For details see to that described for esophoria, except that in it,
page 143. prisms are placed base-out in front of the eyes.
f. Reading bar exercises. For details see page 143. While the patient is performing this exercise, the
Orthoptic exercises for esophoria should be orthoptist should watch the patient's eyes to make
carried out weekly in the clinic and should be certain that he/she is converging and has not
supported by home exercises as discussed above. diverged and suppressed.
It is important to ensure that constant efforts to b. Convergence exercise using synoptophore. It is
relax convergence have not resulted in an inability performed as described for esophoria, except that
to obtain a normal near point of convergence. This in it, the instrument arms are slowly converged,
should be checked at regular intervals. beginning at an angle at which patient can fuse
It is more difficult to improve relative negative the picture.
convergence than relative positive convergence. c. Physiologic diplopia exercise using stereogram
Therefore, esophorias respond less well to in the uncrossed position. To perform this exercise,
orthoptic treatment alone. the stereogram card is held at arm's length in front
 Heterophoria 215

of the patient and a pencil (fixation point) is placed i. Advancement exercise. It is a good convergence
midway between his/her eyes and the card. When exercise which can be done at near. In it, patient
he/she looks at the pencil, he/she will notice is asked to hold a target (preferably a small
diplopia (uncrossed) of the card and will see four detailed picture or fine print) away from his/her
instead of two pictures. Patient is trained to adjust nose where fusion is possible. He/She is asked
the position of the pencil in such a way that the to slowly advance the target towards his/her
two central pictures are joined into one so that nose until diplopia is appreciated. At this point,
now he/she sees three pictures (Fig. 9.3). The he/she is asked to stop and try to converge more
patient is trained to see the fused central picture and, thus to unite the two images again. If he/
clearly. In doing so, he/she is converging for the she cannot do this, he/she should move the target
fixation object and accommodating for the distance back to a small distance to get single vision and
of the card, i.e. he/she is converging relatively then try to bring it closer again. This should be
more than he/she is accommodating. Patient can repeated until the patient can converge to his/
practise this exercise at home for a few minutes her nose or at least reasonably close to it.
several times a day. ii. Jump convergence exercise. Jump convergence
d. Convergence exercise using diploscope. See is more elaborate and more effective form of the
page 141. 'picture-to-nose' convergence exercise. It trains
e. Exercises for improving near point of the patient to achieve bifoveal single vision
convergence. These are as follows: following a sudden change in the convergence
requirement. This is usually possible only after
convergence has been improved to some extent
by other exercises and therefore, is not used
before the fourth week of convergence training,
depending upon the progress. This exercise may
be carried out by any of the following methods:
• The simplest way of doing this exercise is to
have two fixation targets, one fixed target at a
distance of about 6 metres and other movable
target held about 33 cm in front of the eyes.
The patient is asked to change his/her fixation
repeatedly between the distance and near
targets. Subsequently, the near target is brought
closer to the eyes until he/she can eventually
change from distant fixation to fixation at 5 cm
while keeping one image of the near object,
even though it may be very blurred.
• Another way of performing jump convergence
exercise is the use of prism. Patient is asked
to fixate an object and a 10D prism is placed
base-out in front of one eye. Patient is
instructed to try to maintain single vision. In
doing so, he/she has to converge his/her eyes.
The prism is then removed and convergence
is relaxed for a few seconds. This is repeated
several times with prisms of increasing
strength until eventually the patient can
converge to overcome a 40D prism.
Fig. 9.3 Physiologic diplopia excercises using stereogram • Jump convergence exercise can also be
in the uncrossed position. performed with synoptophore. Patient is asked
 216 Theory and Practice of Squint and Orthoptics

to join the two pictures from stereopsis slides. can be tolerated. There is no fixed rule as to
He/she is then asked to look up on a distant the amount of prism correction to be given in
target, while the orthoptist moves the tubes a particular patient. However, in practice,
of instrument into slightly more convergent prism is prescribed with apex towards the
position. Patient is then asked to look back at phoria to correct only half or at the most two-
the pictures. Patient will have to converge to thirds of total heterophorias.
obtain single vision. This is repeated several
times, converging the instrument tubes more 5. Surgical treatment of heterophoria
each time, until the patient can converge to Indications
overcome an angle of 60D. • Horizontal phorias. Surgery becomes necessary
3. Role of miotic drugs in only when symptoms cannot be relieved by
the treatment of heterophoria other methods. This is most frequently true,
when the basic deviation is too large to be
Miotic drugs are the treatment of choice in near controlled despite good fusional vergence. An
esophoria due to a high AC/A ratio. Miotics operation is also indicated in phorias of
facilitate accommodation, so that less than anatomic or paretic origin for which other
normal innervation is necessary to obtain a given treatment methods cannot be successful.
accommodative response and consequently • Vertical phorias. In many hyperphorias,
there occurs less accommodative convergence. especially those of paretic origin and those too
Therefore, miotics are useful aid to orthoptic large to be corrected by prisms, surgery is the
treatment in convergence excess type of best treatment.
decompensating esophorias, if orthoptic
• Cyclophorias. Surgery is the only treatment of
treatment alone is insufficient to relieve the
cyclophorias.
symptoms.
Miotics do not affect the distance deviation, Amount of surgery
for distance vision, no accommodation is It is an erroneous attitude to think that lesser
necessary, if full correction is provided by amount of surgery is required in heterophorias in
glasses. However, if for some reasons, full comparison to heterotropias. In fact, the amount
optical correction has not been provided, the of surgery must be aimed at the basic deviation
distance esophoria will also show a decrease. and the goal to align the eyes, regardless of
Usually, phospholine iodide 0.06%, or 0.125% whether it is a latent, intermittent or manifest
is used once daily. deviation. However, a conservative approach has
4. Role of prisms been recommended when considering surgery in
patients beyond 50 years of age.
• Prisms may be used as a training device for
orthoptic exercises as discussed above. They
may also be incorporated as a permanent
BIBLIOGRAPHY
correction in the patient's glasses, or they may 1. Crone RA: A new theory about heterophoria.
be clipped on by the patient for specific Ophthalmologica 1971;162:199.
purposes only. 2. Flynn JT, Grundmann S, Mashikian M:
• Role of prisms as a permanent correction in Binocular suppression of scotoma: its role in
horizontal phorias is debatable. They should phorias and intermittent tropias. Am Orthopt J
be considered only after other measures have 1970;20:54.
3. Jampolsky A, Flom B, Fried A: Fixation disparity
failed to relieve the symptoms. For exophoria,
in relation to heterophoria. Am J Ophthalmol
base-in prisms and for esophoria base-out 1957;43:97.
prisms are incorporated into the glasses. 4. Palmer EA, Noorden GK von: The relationship
• Primarily, prisms are prescribed as a between fixation disparity and heterophoria. Am
permanent correction in the treatment of J Ophthalmol 1978;86:172.
comitant vertical phorias. A vertical prismatic 5. Scobee RG: The oculorotary muscles, 2nd ed.,
correction of 10D is the maximum amount that St. Louis. 1952, Mosby-Year Book, Inc.
 10
Concomitant
Esotropias and Exotropias
CONCOMITANT ESOTROPIAS CONCOMITANT EXOTROPIAS
• Definition • Congenital (infantile) exotropia
• Classification • Primary exotropia
• Infantile esotropia – Etiology
• Accommodative esotropias – Classification
– Refractive – Intermittent exotropia
– Nonrefractive – Constant exotropia
– Hypoaccommodative • Sensory exotropia
– Partial accommodative • Consecutive exotropia
• Acquired non-accommodative esotropias
• Sensory esotropia
• Consecutive esotropia

CONCOMITANT ESOTROPIAS 3. Stage of constant esodeviation. When the fusional

divergence amplitude becomes inadequate to
DEFINITION maintain the latency of deviation, a permanent
Concomitant esotropia is the term used to manifest esodeviation, i.e. constant esotropia occurs.
describe any manifest convergent deviation of Constant esotropia may be unilateral esotropia or
the visual axes in which the amount of devia- alternating esotropia.
tion in the squinting eye remains constant Note. Since the fusional divergence mechanism
(unaltered) in all the directions of gaze and is much weaker (amplitude 4 to 6 PD) than
there is no associated limitation of ocular fusional convergence (amplitude >30 PD), the
movements. esodeviations usually become constant with
In general, development of an esodeviation poor stereoacuity. While in contrast, exode-
has got three stages: viations tend to remain intermittent with good
stereoacuity for a longer period.
1. Stage of latent esodeviation. In this stage, the
esodeviation is kept latent by the control of
CLASSIFICATION
fusional divergence reserve. It is also called the
stage of esophoria. It has been described in detail Concomitant esodeviations can be classified into
on page 209. following clinico-etiological groups:
2. Stage of intermittent esodeviation. In this 1. Infantile (congenital) estropia. It is a large
stage, the fusional divergence reserves which angel estropia that is constantly mainfest and
usually keep the deviation latent, become occurs during first few months of life.
inadequate intermittently resulting in inter- 2. Accommodative esotropia. The deviation
mittent manifest esodeviation (intermittent increases, when accommodation is exerted. It
esotropia). may be:
217
 218 Theory and Practice of Squint and Orthoptics

i. Refractive accommodative esotropia profound amblyopia and normal retinal

ii. Non-refractive accommodative esotropia correspondence prevail.
– Hyperaccommodative (high AC/A ratio)
Alternating esotropia
iii. Hypoaccommodative (weak accommodation)
iv. Mixed or partially accommodative esotropia. • In alternating esotropia, either eye is used
alternately for fixation while the other one
3. Acquired non-accommodative esotropia. The
assumes the deviated position.
acquired esotropia, in which deviations not
• The patients frequently use crossfixation,
affected by the state of accommodation,
especially when the angle of deviation is large.
includes:
• Visual acuity is almost equal in both eyes.
i. Essential acquired or late onset non-accommo-
dative esotropia, which can be: • Sensory adaptations in alternating esotropia
• Basic esotropia are such that while one eye fixates, the
• Convergence excess esotropia deviating eye is either suppressed or
participates through anomalous retinal
• Divergence insufficiency esotropia
correspondence (ARC) in binocular vision.
ii. Acute concomitant esotropia
Incidence of ARC is high and is found with
iii. Microtropia greater frequency in patients with small angle
iv. Nystagmus blockage syndrome deviations than in those with large angles in
v. Cyclic esotropia whom NRC prevails.
vi. Divergence pareses • Since either eye is used for fixation part of the
vii. Stress-induced esotropia time, there is no amblyopia.
viii.Esotropia in myopia
ix. Esotropia due to spasm of near reflex. INFANTILE ESOTROPIA
4. Sensory esotropia. It results from a poor Infantile esotropia (old name—congenital
vision in one eye. esotropia) is a distinct clinical form of esotropia
5. Consecutive esotropia. It results following which usually presents at 1–2 months of age.
surgical over correction of exotropia. However, it may be detected shortly after birth
or any time within the first 6 months of life.
Unilateral versus alternating esotropia Previously, it was known as 'congenital
Primary constant esotropia of any type may be esotropia'.
either unilateral or alternating depending upon
ETIOLOGY
the type of fixation. Some of the important points
worth mentioning about each type are given The exact etiology of infantile esotropia is
below. obscure. Following factors have been implicated:
1. Innervational disturbance in the form of an
Unilateral esotropia imbalance between tonic convergence and
• It occurs more frequently than alternating divergence is being considered the probable
esotropia. cause rather than the anatomic cause. This
• In it, one eye is habitually preferred for view has been derived from the observation
fixation and the other eye is always deviated. that many a time, the eyes of such a patient
• If an anisometropia exists, almost invariably become straight or even divergent under
the eye with the lesser spherical or astigmatic anaesthesia.
error will be fixating eye. 2. Role of accommodation. It has also been
• In patients with small angle esotropia (up to reported that an accommodative element may
about 15 ), harmonious anomalous retinal be responsible partially or wholly in some cases
correspondence and moderate degree of of infantile esotropia. Further, in some cases, an
amblyopia are found more frequently than in accommodative element may be superadded
those with larger deviations, in whom over the basic deviation at the age of 2–3 years.
 Concomitant Esotropias and Exotropias 219

3. General and environmental factors include given. However, following examination techniques
low birth weight, prematurity, perinatal may help in demonstrating the presence of
hypoxia, maternal-smoking, drugs and alcohol abduction:
abuse. a. Doll's head phenomenon test. This is simple,
easy and best method of demonstrating presence
CLINICAL FEATURES
of abduction in an infant. In this technique, the
1. Time of onset. In most of the cases, squint examiner grasps the top of the infant's head and
manifests within first 6 months of birth in an quickly turns it in horizontal direction. During
otherwise normal infant. this manoeuvre, an observation for the
2. Angle of deviation. Most patients with occurrence of abduction is made in the eye
infantile esotropia (Fig. 10.1) have a large angle opposite to head turn, i.e. when head is
suddenly turned towards the right, the
observation should be made in left eye and vice
versa.
b. Rotating the child for demonstrating abduction.
In this technique, the examiner holds the infant
in his hands and rotates him completely first in
one direction and then the other. While rotating
towards his right the examiner should look for
Fig. 10.1 A child with infantile esotropia. a quick abductive movement in the infant's right
of deviation for both distance and near fixation. eye and vice versa (Fig. 10.3).
In half of the patients, the deviation measures c. Alternate patching. If the above described two
30° or more. The angle of deviation is usually techniques fail to demonstrate abduction, then
stable except in a few cases having an an alternate patching of the either eye should
accommodative element in the etiology. be done for several hours. If there is no lateral
3. Fixation pattern. Usually an alternate fixation rectus palsy in the unpatched eye, the ability to
occurs in primary gaze and crossed fixation in abduct will become apparent (Fig. 10.4).
lateral gaze, i.e. the infant fixates the objects in the 5. Visual acuity is normal and equal in both
left field with right eye and the objects in the right eyes of patients who freely alternate fixation. If,
field with the left eye (Fig. 10.2). however, one eye is preferred, amblyopia will
4. Apparant limitation of abduction. The cross- develop in the other eye.
fixation makes the abduction of either eye 6. Refractive errors. The refractive errors are not
unnecessary and thus on initial examination, the prominent, but are consistent with the patient's
impression of bilateral sixth nerve palsy may be age group.

Fig. 10.2 Diagrammatic depiction of crossfixation in a patient with infantile esotropia.

 220 Theory and Practice of Squint and Orthoptics

muscles may be seen in about 50–60% of patients

above one year of age. Before this age, it is rarely
seen suggesting that probably it represents an
evolutionary stage in the development of
infantile esotropia syndrome.
2. Dissociated vertical deviation (DVD). It is an
important component of the syndrome, seen in
about 70 to 90% of cases. For details of DVD,
see page 264.
3. Latent nystagmus. A jerk type of latent
nystagmus having a decreasing exponential
slow phase is another component of the
syndrome seen in about 33% cases. The
nystagmus occurs in both eyes, on occluding one
eye, with the fast component directed towards
the fixating (uncovered) eye. In patients having
alternate squint, nystagmus may occur while
both eyes are open, with a fast component
Fig. 10.3 Rotation of an infant to determine lateral rectus directed towards the fixating eye. Under these
palsy (for explanation see text). circumstances, it might be confused with the
true congenital nystagmus and thus electro-
Associations oculography may be indicated for differentiation.
Infantile esotropia is frequently associated with 4. Lack of bifixation. Inherent lack of capacity
other ocular abnormalities. Under such for bifixation is another component of the
circumstances, the condition is labelled as syndrome. Therefore, many patients with
infantile esotropia syndrome. Components infantile esotropia syndrome, even after full
other than esotropia of this syndrome are as surgical correction performed in time, do not
follows: achieve normal stereopsis. This is one of the
1. Inferior oblique overaction. A marked degree causes of monofixation syndrome which has been
of overaction of one or both inferior oblique described on page 235.

Fig. 10.4 Patch test to detect ability of abduction in an infant: A, momentary occlusion of the fixing eye may not suffice to
force the fellow eye to take up fixation; B, patching for several hours may be required to detect abduction ability in the
originally non-fixing eyes.
 Concomitant Esotropias and Exotropias 221

MANAGEMENT • Tight medial rectus muscle revealed on forced

Management of a case of infantile esotropia can duction test.
be considered under following heads: • Vision is most comfortable with the eye in
• Clinical evaluation and differential diagnosis adduction leading to face turn towards the
• Non-surgical treatment fixing eye.
• Surgical treatment 5. Congenital fibrosis syndrome, also known as
• Surgical results and post-surgical treatment strabismus fixus (see page 350) may present as
large angle congenital esotropia with severe
A. Clinical evaluation and differential diagnosis limitation of abduction of one or both eyes. This
is a congenital restrictive strabismus, often
The essential infantile esotropia usually mani-
inherited as an autosomal dominant trait.
fests between birth and 6 months of age and thus
needs to be differentiated from following 6. Congenital sixth nerve palsy unilateral or
conditions: bilateral also needs to be differentiated from
infantile esotropia.
1. Pseudoesotropia or aparant esotropia may
occur in infants due to following associations: Clinical work-up which may help in arising at
the diagnosis should include:
• Prominent epicanthal folds (Fig. 5.3)
1. Examination of anterior segment to rule out
• Small interpupillary distance
corneal pathology and congenital/develop-
• Negative angle kappa, and mental cataract.
• Excessively broad nasal bridge 2. Estimation of visual acuity in an infant can
2. Infantile accommodative esotropia. Though be made by OKN, PVT or VEP as decribed on
accommodative esotropia usually occurs page 40.
between 2 and 3 years of age but can occur in 3. Examination of fundus and media is quite
infants as young on 2 months of age and is often useful in diagnosing retinoblastoma and other
diagnosed as infantile esotropia. The key abnormalities responsible for sensory squint.
features which help to differentiate infantile 4. Refraction under atropine cycloplegia helps
accommodative esotropia from infantile in discovering refractive accommodative
(congenital) esotropia are: esotropia due to high hypermetropia. In
essential infantile esotropia, cycloplegic
• Presence of straight eyes for the first 2–3
refraction characteristically reveals only 1–2 D
months of life,
of hyperopia. Repeat refractions are important
• Variable angle of strabismus, and
because an accommodative component is
• Hypermetropia >2D. sometimes discovered on follow-up in patients
3. Sensory esotropia may occur rarely in infants with infantile esotropia.
with early onset retinoblastoma and other 5. Measurement of AC/A ratio. The lens gradient
disorders of optic nerve and retina. method (see page 119) can be used to know the
approximate AC/A ratio.
4. Ciancia syndrome, though a variant of
6. Measurement of deviation. Usually, an appro-
infantile esotropia, is a distinct clinical entity
ximate idea is made by observation for distance
characterized by following features:
deviation, since it is not possible to get coopera-
• Large angle esotropia (>60 PD) with cross-
tion from the infant for a distance fixation.
fixation and both eyes appear to be stuck in
However, attempts may be made to measure the
towards the nose.
near deviation with the help of Krimsky test
• Face turn towards fixing eye. (described on page 107).
• Limited abduction bilaterally with good 7. Differentiation from simulated bilateral sixth
abduction saccades. nerve paralysis can be made by Doll's head
• Jerk-end-point nystagmus on attempted phenomenon test, i.e. rotating the child to
abduction with minimal or no nystagmus on demonstrate abduction or by alternate patching
adduction. test as described earlier (Fig. 10.2).
 222 Theory and Practice of Squint and Orthoptics

Note. Most of the times, the above examinations • In general, it has been reported that the sooner
and the typical clinical features are helpful in the child can be operated after fulfilling the
establishing the diagnosis of essential infantile above criteria, the better it is.
esotropia. Once diagnosed, treatment of choice for • Peripheral fusion (monofixation syndrome) is
infantile esotropia is surgical alignment of the eyes. achieved in most cases (80%), if the surgery is
However, some non-surgical treatments may be done by the age of 2 years and in some patients
needed before the surgery is performed. if it is done by the age of 4 years (20%).
• After the age of 4 years, a functional cure in the
B. Non-surgical treatment
form of peripheral fusion is unlikely.
Non-surgical measures needed before the
surgery is undertaken include the following: Choice of surgery
1. Correction of refractive error. Usually correc- • Bimedial recessions are preferred over the
tion of physiologic hypermetropia of 2D to 3D unilateral recess-resect procedure in the
has little effect on the deviation. However, it has absence of amblyopia. However, by and large,
been advocated that when hypermetropia is it is surgeon's perference.
more than +1.5D, glasses should be prescribed • It has recently been recognized that a maxi-
and child should be reassessed after 6 weeks. mum of 8 mm of medial rectus recession may
This time it may be possible to determine the be performed without crippling its function
AC/A ratio accurately, because accommodation as against the traditionally suggested upper
will now be controlled by the patient's glasses. limit of 5.5 mm.
Miotics may be tried as an alternative to • Unilateral recess-resect operation on the non-
spectacles in unco-operative infants. dominant eye may be performed in patients
2. Treatment of amblyopia. Amblyopia, when who have failed to respond to amblyopia
present, should always be treated rigorously treatment.
before rather than after the surgery. For
• Amount of muscle surgery to be performed for
treatment of amblyopia, see page 190. Failure to
horizontal deviation depends upon the
treat amblyopia will compromise a stable
angle of squint, age of the patient, duration of
surgical alignment of the eyes.
squint, visual status and the surgeon's pre-
C. Surgical treatment vious experience. However, the figures given
in Table 10.1 can serve as a rough guideline for
Time of surgery
the beginners. From the Table 10.1, it is clear
It has been and still continues to be a controversial that now the surgeon has an alternative to three
and debatable question. However, experienced and four muscles surgery for a large angle
strabismologists have recommended that before esotropia.
surgery is performed for infantile esotropia,
following prerequists should be ascertained:
• Deviation should be constant and stable. Table 10.1 Rough guidelines for amount of surgery in
• Fixation should be alternating or only a mild infantile esotropia
fixation preference should be present. Deviation Monocular surgery Binocular surgery
in prism in mm in mm
• Accommodative element should be absent. dioptres Recession Resection Bilateral MR
• Sensory esotropia should have been ruled out. of MR of LR recession
• Amblyopia should have been treated. 15 3.0 4.0 3.0
• Associated vertical deviation or A/V patterns 20 3.5 5.0 3.5
25 4.0 5.0 4.0
should be revealed.
30 4.5 6.0 4.5
The above information can be obtained 35 5.0 7.0 5.0
between the age of 6 months and 2 years 40 5.5 7.0 5.5
depending upon the cooperation of the child as 50 6.0 8.0 6.0
well as patience and understanding of the 60 6.5 9.0 6.5
70 7.0 10.0 7.0
examiner.
 Concomitant Esotropias and Exotropias 223

• Recession of both inferior oblique muscles, if • Small-angle esotropia or exotropia (less than
indicated, should be performed along with the 15) which is cosmetically acceptable. There
horizontal muscle surgery. is less stability of angle.
• Surgical treatment of DVD, if associated • About 80% patients have anomalous retinal
with infantile esotropia, may be postponed for correspondence.
a later date. • Stereopsis is of low grade or absent.
Treatment. No further treatment is required
D. Surgical results and post-surgical treatment except amblyopia prevention.
Post-surgical treatment will depend upon the
4. Large-angle residual esotropia (undercorrection) is
outcome of surgery. Von Noorden has graded
the surgical results of infantile esotropia as an unacceptable treatment result. Its features are:
follows: • Large-angle esotropia (more than 20 D) is
1. Subnormal binocular vision. It is the best
usually cosmetically unacceptable and needs
possible therapeutic result that can be expected repeat surgery.
following surgery for infantile esotropia. It is • Suppression amblyopia is of frequent
usually achieved, if surgery is performed before occurrence.
2 years of age. It is characterized by: • Stereopsis is absent.
• Orthophoria or asymptomatic heterophoria; Post-surgical treatment: Resurgery may be
alignment is stable. required for large residual esotropia and should
• Normal visual acuity in both eyes. be done within 3 months of origin surgery. Prism
• Peripheral fusion develops, which allows a adaptation test may be preformed before surgery
low grade of stereopsis from 67 to 3000 to identify patients with binocular potential and
seconds of arc as well as normal fusional to uncover the maximum underlying deviation
vergence amplitudes. prior to any further surgery.
• Normal retinal correspondence.
Choice of surgery
• Foveal suppression in one eye in binocular
vision. • Bilateral LR resection may be performed, if the
primary surgery was bilateral MR recession.
Post-surgical treatment. Such patients do not
• When the primary surgery was LR resection
require further treatment, regular follow-up is
and MR recession, then the similar surgery
required for:
should be performed in the other eye.
• Correction of refractive error,
• Maintenance amblyopia therapy, and 5. Large-angle consecutive exotropia (over-
• Late development of under or overcorrection. correction) should be managed as below:
2. Microtropia. Postoperative microtropia i. Children below 2 years of age having fusion
(deviation less than 10) is the desirable treatment potential with consecutive exotropia of more than
result. It is characterized by: 15 PD, surgery should be taken up early to give
the child a chance of developing peripheral fusion.
• Inconspicuous shift or no shift on cover test.
Principle: Copper's dictum, i.e. a decision about
Alignment is usually stable and thus no
type of surgery should be based on thorough
further treatment except amblyopia prevention
reassessment as a new case of exotropia.
is required.
• Mild amblyopia is common. Surgical options include
• Peripheral fusion usually develops, which • LR recession with advancement of the previously
may allow a low grade stereopsis and normal recessed MR is an effective treatment.
fusional vergence amplitudes. • Bilateral LR recession is another equally
3. Small-angle residual esotropia or small-angle effective option.
exotropia due to overcorrection is considered an • LR recession + MR resection of the other eye may
acceptable treatment result. It is characterized by: also be considered.
 224 Theory and Practice of Squint and Orthoptics

Note REFRACTIVE ACCOMMODATIVE ESOTROPIA

• The diagnostic value of preoperative prismatic Definition
correction of the deviation is very limited. Refractive accommodative esotropia refers to
• Exotropia drift is common after consecutive that type of esotropia which:
exotropia surgery. • Varies in degree depending on the amount of
• Therefore, a suitable ocular alignment accommodation exerted,
immediately after surgery for consecutive • Is caused by hypermetropia, and
exotropia is a small-angle esotropia of 5– • Wearing of spectacles (to correct the
10 PD. hypermetropia) eliminates the esotropia in all
ii. In patients above 8 years of age, i.e. visually fixation distances and in all gaze positions.
mature group with bifoveal fusion, wait for 6 to
8 weeks and perform surgery, if deviation is Etiopathogenesis
more than 20 PD. If adduction is near normal, All children with uncorrected hypermetropia do
the decision of whether or not to perform more not develop accommodative esotropia. This
surgery should be based purely on the desire implies that in addition to hypermetropia, there
for improved cosmesis. are some other factors also which play an
important role in the occurrence of accommo-
ACCOMMODATIVE ESOTROPIAS dative esotropia.
Accommodative esotropia refers to esotropia Factors implicated are:
which is caused by over convergence in response • Uncorrected hypermetropia,
to accommodation. Therefore, the convergent • Fusional divergence amplitude,
deviation of the eyes varies in degree according • AC/A ratio, and
to the amount of accommodation exerted.
• Child's personality
Characteristics. All accommodative esodevia-
Course of events, depending upon the interplay
tions are acquired and have the following
of above factors as described by von Noorden
characteristics in general:
and Helveston (Fig. 10.5), is as follows:
• Onset age is usually between 2 and 3 years.
Most cases occur between 6 months and 1. It is unequivocal that the uncorrected
7 years of age. However, rarely it may occur hypermetropia causes a retinal image blur. Thus
as early as 3–4 months of age (infantile the patients with hypermetropia must
accommodative esotropia). accommodate to clear the retinal image at
distance. Whether the child will accommodate
• Esodeviations is usually intermittent at onset
to clear the image or not depends less on the
becoming constant with passage of time.
degree of hypermetropia than on the child's
• Often hereditary. personality. It has been reported that the more
• Precipitated sometimes by trauma or illness. relaxed and easy going children may not
• Amblyopia is frequently associated. accommodate and thus remain orthotropic
(because they prefer blurred vision over the
Types constant effort to accommodate excessively).
However, these patients may develop bilateral
Depending upon the mechanism of occurrence,
ametropic amblyopia with or without accommo-
accommodative esotropia is of following types:
dative deficiency (Fig. 10.5A).
1. Refractive accommodative esotropia (high
2. Many patients with uncorrected hyper-
hyperopia with normal AC/A ratio)
metropia (especially fastidious and exacting
2. Non-refractive accommodative esotropia children) will accommodate to clear the retinal
(high AC/A ratio) image blur. Because of the near synkinesis, the
3. Hypoaccommodative esotropia accommodation in turn will cause excessive
4. Mixed or partially accommodative esotropia accommodative convergence. The final outcome of
 Concomitant Esotropias and Exotropias 225

Fig. 10.5 Course of events in patients with uncorrected high hypermetropia.

this excessive use of accommodative conver- convergence induced by excessive accommo-

gence, depending upon the fusional divergence dation is normal or even subnormal (Fig. 10.5D).
amplitude of the patient, may be any of the three:
i. Refractive accommodative esotropia will develop Clinical characteristics
in children with insufficient fusional divergence 1. Time of onset. Most of the times, the accommo-
to handle the excessive accommodative conver- dative esotropia manifests between the age of
gence and a normal AC/A ratio (Fig. 10.5B). 2 and 3 years. However, in rare circum-stances,
ii. Intermittent esotropia and esophoria. Esophoria it may develop anytime between birth to 7 years
will develop in children with sufficient fusional of age.
divergence amplitude to handle the excessive 2. Hypermetropia. Children developing refractive
accommodative convergence and thus have accommodative esotropia usually have hyper-
straight eyes under binocular vision (Fig. 10.5C). metropia ranging between 2 and 6 dioptres with
However, at times patient's fusional reserve may a mean of 4.75 dioptres.
be depleted resulting in intermittent esotropia. 3. Ocular deviation. Development of accom-
Factors affecting fusional reserve are: modative esotropia usually passes through
• Any severe illness stages of esophoria and intermittent esotropia.
• Emotional stress However, in a child features of these stages may
• Fatigue, etc. not be noticed every time and constant esotropia
may be the first complaint by the parents.
iii. Orthotropia will be there in children with low
or flat AC/A ratio who may not become esotropic Features during stage of intermittent esotropia. Parents
inspite of uncorrected hypermetropia, since the often give a history that the eyes are straight,
 226 Theory and Practice of Squint and Orthoptics

however, sometimes when the child is tired or the child's behaviour becomes less fretful and
focusing at near object the eyes cross. The main he/she no more closes one eye.
feature of intermittent esotropia is occurrence of • Abnormal retinal correspondence (ARC) may
transient diplopia. Children usually react to it by develop, if the squint is not treated for a long
fretfulness and irritability and by closing of one eye. time. This protects the patient from any further
Features of constant esotropia (Fig. 10.6A). sensory symptoms and allows a crude form
Esotropia, which is initially intermittent, quickly of binocular single vision. In a patient with
increases to become a constant deviation. Ocular intermittent squint, when the eyes are straight
deviation is usually variable and may be slightly normal retinal correspondence (NRC) will be
larger at near than at distance fixation. Distance present and when squinting, ARC will be
versus near deviation is usually within 10 prism present.
dioptres. Constant esotropia may remain • Amblyopia ensues, if unilateral constant
unilateral or may become alternate. Degree of esotropia develops with moderately strong
crossing is usually moderate in magnitude, fixation preference.
20–40 prism dioptres, but may be more or less. • Alternate fixation with no binocular single
Typically, it is smaller than infantile esotropia. vision occurs in some patients developing
Refractive accommodative esotropia is usually alternate convergent squint. These patients
fully corrected by optical correction (Fig. 10.6B). usually have good and equal vision in both
eyes, with no amblyopia.
4. AC/A ratio is usually normal.
6. Associations. Patients with refractive accom-
5. Development of sensory adaptations. modative esotropia may have following
• Suppression develops to take care of the associated strabismic abnormalities:
problems associated with diplopia during • Vertical deviations
stage of intermittent squint. It usually develops
• A–V pattern
within a week of the onset of squint and then
Clinical evaluation and diagnosis
Accommodative esotropia usually develops at
the age of 2–3 years and needs to be differen-
tiated from sensory esotropia. Rarely, it may
develop early and must be differentiated from
essential infantile esotropia. Each case needs a
detailed work-up as described on page 100, but
the key features which need a special mention
are as follows:
1. Measurement of deviation for far and near
and in all cardinal positions of gaze is important.
2. Cycloplegic refraction is the most important
investigation in the evaluation of a case of squint
with a special reference to refractive accommo-
dative esotropia. It is preferred to use 1% atropine
eye ointment, three times a day for three
consecutive days to achieve cycloplegia. Use of
atropine not only allows a complete cycloplegia,
but also provides a brief period of relaxed accom-
modation during which child starts tolerating the
Fig. 10.6 A patient with accommodative esotropia (A), glasses. Further, incidence of systemic compli-
corrected with glasses (B). cations is much less with the use of atropine eye
 Concomitant Esotropias and Exotropias 227

ointment than with the eyedrops. However, These infants should be seen every 2–3 weeks
recently some clinicians contest that 1% until it is certain that the glasses have controlled
cyclopentolate is good enough for the cycloplegia. the deviation. If the esotropia persists,
3. Measurement of fusional divergence amplitude retinoscopy should be repeated and if additional
is also an important parameter in such cases, hypermetropia is discovered, the glasses should
since it plays an important role in the be changed. However, if no change in
development of refractive accommodative retinoscopy is observed, the diagnosis should
esotropia (see etiology). Normal fusional be changed to infantile type.
divergence amplitude at distance is 4–6 and at ii. From 6 months to 6 years. Esotropic children
near it is 8–12. of this age group having hypermetropia of more
4. Examination of fundus and ocular media is than +1.5 D should be prescribed full
quite useful in diagnosing retinoblastomas and retinoscopic finding without any additional plus
other abnormalities responsible for sensory lens and should be followed every month till
squint. Fundus examination should be performed their deviation is stabilized. Once a patient is
along with the cycloplegic refraction. stabilized under 1 year of age, retinoscopy
should be repeated at least every 3 months. For
Treatment the children between 1 and 5 years of age,
1. Optical correction retinoscopy should be repeated every 6 months.
• A full optical correction usually eliminates the iii. Above 6 years of age. In children above 6
refractive accommodative esotropia (Fig. 10.6) years of age, the optical prescription should
and converts it into a phoria. However, it may include the minimum power lens that should
take several weeks before the glasses become provide both binocular single vision with
fully effective. esophoria and maximum visual acuity. At this
• Role of atropinization. A child who has never stage, to obtain esophoria rather than orthophoria
worn glasses and has been accommodating for is useful since with the former fusional
years may not accept glasses immediately. divergence, amplitude continues to be exercised.
Such children require atropinization for a These patients should be followed every
period of a few weeks to relax their accommoda- 6 months for 2 years and then every year.
tion before the glasses are tolerated. Note. When, after full hypermetropic correction
• Importance of full time wear of spectacle correction for 4 to 8 weeks, the residual esotropia is greater
must be emphasized to the parents. A common than 15 PD, the diganosis should be revised as
cause of treatment failures or partial responses below:
to anti-accommodative management is • Partial accommodative esotropia is labelled,
inconsistent spectacle wear. when residual esotropia is for both distance
Method of prescribing optical correction and and near. In such cases, surgery is indicated
follow-up (see page 232).
The follow-up regime varies slightly from age • High AC/A ratio should be considered, when
to age, as follows: eyes are aligned for distance and residual
esotropia is present for near. Such patients
i. From birth to 6 months. An infant below
need bifocals (see page 230).
6 months (with a definite intermittent or
constant esotropia) usually has non-accommo-
dative infantile esotropia. However, all such 2. Role of miotics
infants having hypermetropia of +2.0 dioptres Though some people have recommended use of
or more should be given glasses. The prescription topical miotics, but it is best to avoid their use in
should include full retinoscopic findings plus refractive accommodative esotropia. However, in
an additional +1.5D. The additional plus will extremely uncooperative and hyperexcited
provide clear vision up to 66 cm which is the children, miotics may be prescribed as a short-
usual limit of the young infant's world. term alternative to spectacles.
 228 Theory and Practice of Squint and Orthoptics

3. Amblyopia therapy Course of untreated accommodative esotropia

It is indicated, when a child is brought late with If anti-accommodative therapy is delayed for
a constant unilateral accommodative esotropia several weeks to months:
with amblyopia. For details of amblyopia, see • Some or all of the esodeviations will not
page 190. However, in general, full hyper- respond fully to appropriate anti-accommo-
metropic correction and occlusion therapy may dative therapy.
improve the vision. • When hypermetropia is greater than 5.0D, there
is a major risk of bilateral ametropic amblyopia.
4. Orthoptic treatment
Once the occlusion therapy improves visual NON-REFRACTIVE ACCOMMODATIVE ESOTROPIA
acuity, the orthoptic treatment aims at over- (ESOTROPIA WITH HIGH AC/A RATIO)
coming the suppression and improving the Definition
negative fusional convergence. Before starting
It refers to that type of accommodative esotropia
orthoptic treatment, it should be ensured that:
which is caused by a high AC/A ratio and thus
a. Child has worn glasses for several weeks; the esotropia is significantly greater at near than
b. Amblyopia, if any, has been treated; and distance fixation. It is unrelated to refractive
c. Child is cooperative enough for exercises. error and the near point of accommodation is
• Antisuppression exercises. For details, see page normal for the age of the patient.
174.
Etiopathogenesis
• Exercises to improve negative fusional convergence
(fusional divergence) include divergence High AC/A ratio. The principal factor in the
exercises with prisms, exercises with synopto- etiopathogenesis of non-refractive accommodative
phore, physiological diplopia exercises using esotropia is occurrence of an excessive amount of
stereogram in the crossed position, exercises accommodative convergence associated with a
using diploscope, exercises using Remy normal amount of accommodation (i.e. high AC/
separator and bar reading exercises. For A ratio).
details, see page 143. Amplitude of fusional divergence modifies the
Antisuppression and fusional divergence deviation as follows:
exercises combined with the full hypermetropic • Esophoria for near will occur, when the fusional
correction may be successful in maintaining divergence is sufficient to cope up with the
single binocular vision. increased convergence at near fixation.
• Esophoria for distance and esotropia for near will
5. Role of surgery
occur, when the fusional divergence is able to
• As a general principle, surgery for refractive cope up with the convergence exerted during
accommodative esotropia should not be distance fixation but is insufficient to cope up
recommended, since it causes more harm than with the excessive convergence at near
any benefit. Therefore, it would be an error of fixation.
commission to surgically correct accommo- • Esotropia greater at near than at distance fixation
dative esotropia. occurs, when the fusional divergence is
• Patients, who have an associated vertical insufficient to counter the convergence exerted
deviation of A–V-pattern, will require surgery even for distance fixation.
for these strabismic abnormalities in addition
to the proper refractive correction for their Clinical characteristics
accommodative esotropia. 1. Time of onset. Like the refractive accommo-
• In the rare event of deteriorations despite a dative esotropia (RAE), the peak age of onset of
satisfactory initial response to optical correction non-refractive accommodative esotropia (NRAE)
for the hypermetropia, a recession of both medial is also 2–3 years, but may occur anytime
rectus muscles will restore fusion. between 6 months to 7 years of age.
 Concomitant Esotropias and Exotropias 229

2. Ocular deviation. Like the RAE, to begin that child is fully accommodating. This can be
with, deviation in NRAE is also small and achieved with the use of a fixation target that
intermittent. However, unlike RAE (where requires full accommodation to identify small
distance and near esotropias are approximately details.
equal), in NRAE, the near esotropia is typically 2. Cycloplegic refraction. Cycloplegic refraction
much greater than the distance esodeviation. In should be carried out in each case. Non-refractive
fact, there is little or no deviation for distance in accommodative esotropia may occur in
NRAE. emmetropia, hypermetropia or even myopes.
Near-distance disparity, depending on the However, moderate degree of hypermetropia is
degree, can be classified as below: a more frequent association.
• Grade I: 10–19 PD more deviation for near, 3. Measurement of AC/A ratio. It can be
performed with lens gradient method or
• Grade II: 20–29 PD more deviation for near,
heterophoria method, former being more reliable.
and
Normal AC/A ratio is 3 to 4 : 1 (For details, see
• Grade III: 30 PD more deviation for near.
page 119).
3. AC/A ratio. It is characteristically high. 4. Measurement of fusional divergence ampli-
4. Development of sensory adaptations tude. It is also an important factor. Its role has
been discussed in etiopathogenesis. For details,
• Suppression. During periods of manifest
see page 116.
deviation (near fixation), the patient soon learns
5. Examination of fundus and ocular media is
to suppress to avoid problem of diplopia.
quite useful in differentiating from sensory
Before the development of suppression, child
deviation. Fundus examination should be
may be irritable, fretful and may close one eye.
performed along with cycloplegic refraction.
• Abnormal retinal correspondence. Development
Diagnosis of NRAE is usually based on following
of suppression is soon followed by develop-
ment of abnormal retinal correspondence observations:
(ARC). Thus, in such patients, retinal corres- • Near esotropia is typically much greater than the
pondence is normal (NRC) for distance distance esotropia with the refractive error
fixation and abnormal (ARC) for near fixation. fully corrected.
• Amblyopia. Like RAE, development of amblyo- • Establishment of high AC/A ratio by lens
pia is a particular danger in patients with high gradient method in the presence of a normal
AC/A ratio esotropia. A proper optical near point of accommodation.
correction with regular follow-up is must to • Special care and caution is needed in not to
avoid the amblyopia. confuse a V-pattern esotropia with NRAE due
to high AC/A ratio. In V esotropia, the
5. Associations. Like RAE, patients with NRAE
deviation increases characteristically in down
may also have following strabismic abnormalities:
gaze at either distance or near fixation. While
• Vertical deviations in esotropia due to high AC/A ratio, deviation
• A–V-pattern increases at near fixation irrespective of the
position of the eyes.
Clinical evaluation and diagnosis
Each case of strabismus needs a complete work Treatment
up (see page 100). The most important aspects 1. Amblyopia therapy. Before treatment is
of strabismic work for non-refractive accommo- begun, any amblyopia should be eliminated or
dative esotropia which need special mention are improved.
as below: 2. Bifocal glasses. Since near deviation is the
1. Measurement of deviation. It should be per- primary obstacle to normal binocular vision, a
formed for far and near by prism and alternate bifocal add of about +3.0 D over the full
cover test. While measuring deviation for near cycloplegic refraction with simultaneous
fixation, special care should be taken to ensure orthoptic exercises is extremley useful in the
 230 Theory and Practice of Squint and Orthoptics

treatment of non-refractive accommodative

esotropia.
Prerequisites for use of bifocals
• Non-refractive accommodative esotropia
usually occurs at 1½ to 2½ years of age.
However, sometimes it may occur even in an
infant. Bifocals need not be prescirbed in cases
below 6 months of age. As a general principle,
all infants below 6 months with definite
Fig. 10.7 Use of executive type of bifocal glasses in a
esotropia (intermittent or constant) should be patient with high AC/A ratio.
prescribed the full retinoscopic finding plus
an addition of +1.5D as a single vision glasses. At each follow-up visit, retinoscopy should be
Bifocals are not required below 6 months performed and new glasses should be
because visual acuity of such infants is usually prescribed, if there is difference of 1D or more.
limited to 66 cm. These infants should be Wearing of bifocals
followed up as described for refractive If, with the use of bifocals, bifoveal single vision
accommodative esotropia (see page 227). can be maintained at near, the fusion reflex may
• Above 6 months of age, a focal add of +3.0D gain in stability. Under these circumstances, the
should be given. bifocals should be continued up to 5 years of age,
• Bifocals should be prescribed only in patients after which the bifocals may be reduced step by
with marked esotropia for near and ortho- step in decrements of +0.75 to +1D. The aim is to
tropia or minimal esotropia for distance with maintain the patient's fusion while leaving him/
full cycloplegic refraction worn for at least 4– her esophoric at near so that patient excercises
8 weeks. divergence amplitude continuously. Preferably,
• Bifocals should be continued in patients the patient should be completely weaned from
whose near esotropia is fully corrected with the bifocal segment by 10 years of age.
their use. During the weaning period, the antisupp-
• Bifocals should not be prescribed in the ression exercises and exercises to increase
presence of amblyopia. Rather amblyopia fusional divergence amplitudes may be helpful
should be treated first. for maintaining fusion.
Prescription of bifocals Some patients may become dependent on
Usually about +3.0D add is required in bifocal bifocals and weaning may not be possible even
glasses to correct the non-refractive accommo- in early teens. Under these circumstances, a
dative esotropia. However, the exact power of Faden or bilateral MR recession may be
bifocal add is determined by hit and trial method. preferred to remove the near add. However,
To begin with, +1 D is added to the distance spectacles without near add still may be
correction in the trial frame and then the power required.
is increased in steps of +0.5D up to a maximum
3. Miotics
of +3.0D till all the near esotropia is corrected.
Mechanism of action. Miotics facilitate accommo-
The success of bifocal therapy depends largely
dation and thus reduce the accommodative
on the proper bifocal segment. Executive type of
convergence. Because of this action, miotics are
bifocals in which the separation line between the
quite useful in patients with non-refractive
distance and near segments is properly located
accommodative esotropia due to high AC/A
so that it bisects the pupil or touches its lower
ratio.
border, when the child looks straight ahead
(Fig. 10.7) should be preferred. Suitable cases. Presently bifocals are preferred
Follow-up examination should be done every over miotics in general, but some physicians do
3 months up to one year of age, every 6 months prefer use of miotics. Perhaps following are the
up to 2 years of age and thereafter every year. guidelines for suitable cases for use of miotics:
 Concomitant Esotropias and Exotropias 231

• Patients of younger age group than bifocals. but immediate cessation of miotic treatment will
• When the child is unlikely to wear glasses for lead to the spontaneous disappearance of the cysts.
the entire day. 4. Orthoptic exercises
• Probably, the best and most appropriate use Orthoptic exercises rarely achieve results alone.
of miotics is to assist in withdrawing bifocals. These should be best combined with bifocal
• May be used for residual deviations post- glasses and/or miotics. Orthoptic exercises
operatively. require good co-operation from the patient.
• Miotics should not be used unless some Some exercises are only suitable for older
degree of binocularity can be achieved. A children. So, in practice, it is often impossible to
slight reduction in the angle of esotropia is of carry out orthoptic treatment satisfactorily.
no benefit to the patient with respect to The aim of orthoptic treatment is to overcome
restoring normal binocular function. suppression and to improve negative fusional
Preparation and dosage schedule for miotics is convergence, i.e. fusional divergence (see pages
as below: 174 and 213).
• Two most commonly used miotics are These orthoptic exercises have been described
phospholine iodide (0.06%, 0.125%) solution in the section of refractive accommodative
and disopropyl fluorophosphate (DFP 0.025% esotropia (see page 228).
ointment). 5. Surgery
• To begin with, higher strength of phospholine Surgery is indicated in patients with large angle
iodide may be used. Where, with the help of of squint in which deviation cannot be corrected
miotics, bifoveal single vision can be achieved by above described measures.
for near, continue the drug for several weeks. Generally, these patients respond well to
Gradually discontinue, reducing strength of bilateral medial rectus recessions. Occasionally,
the phospholine iodide. recession may have to be combined with
• It is unequivocal to say that a hypermetropic bilateral Faden operation on medial recti. The
patient must wear the full distance correction amount of surgery should always be based on
during treatment with miotics so that the need the near deviation.
for accommodation during near vision is
minimum. Any significant astigmatism HYPOACCOMMODATIVE ESOTROPIA
should also be corrected since clear images Hypoaccommodative esotropia is the term
promote better vision. coined by Costenbader for the accommodative
• While using miotics, patient should be esotropia which is associated with weakness of
reviewed at fortnightly intervals and checked accommodation. Costenbader hypothesized that
for the side effects. to overcome accommodation, there is an
Complications of miotics include increased accommodative effort which in turn
i. Anaesthetic risk. Inadvertent use of depolarizing results in increased convergence resulting in
muscle relaxant such as succinylcholine, during near esotropia.
general anaesthesia, may result in life-threatening
Clinical characteristics
apnoea. Therefore, anaesthetist must be informed
about the use of these medicines. Hypoaccommodative esotropia is characterized
ii. Systemic complications such as headache, by following features:
stomach cramps, hallucinations, nausea, • Esotropia is large for near fixation and small
vomiting and diarrhoea have been reported with for distance fixation.
the use of topical anticholinestrase agents, but • Esotropia is not related to uncorrected
are rare. hypermetropia.
iii. Ocular side effects include pain on instillation, • AC/A ratio is not high.
iris cyst formation, spasm of accommodation, • Near point of accommodation (NPA) is definitely
anterior subcapsular cataract and rarely retinal remote, i.e. there is weakness of accom-
detachment. Iris cyst formation is not uncommon, modation.
 232 Theory and Practice of Squint and Orthoptics

Treatment 3. Surgery. It is important to note that only non-

1. Bifocal glasses. A near add of plus lenses to accommodative part of the squint needs to be
compensate for the weak accommodation may corrected surgically.
be useful. • Measurement should, therefore, be taken with
2. Orthoptic exercises to improve accommo- full optical correction for near and distance;
dation. and the amount of surgery should be based
on the amount of residual esotropia measured
PARTIALLY ACCOMMODATIVE ESOTROPIA at distance with full correction.
Definition • Parents should also be explained that even
Partially accommodative or mixed esotropia is after the surgery, the child will require glasses
the term used to describe an esotropia which is to correct the esotropia.
partly due to some accommodative factors • Bilateral medial rectus recession should be
(hypermetropia, or high AC/A ratio) and partly preferred. However, in the presence of
due to some non-accommodative factors. uncorrected amblyopia, parents may insist for
surgery on the affected eye only.
Clinical types
• In the presence of high AC/A ratio, it has been
In clinical practice, two types of partially observed that a slightly larger recession is
accommodative esotropia are described: required. For example, if ordinarily bilateral
1. Infantile esotropia with superadded accommo- medial rectus recession of 3 mm is required
dative esotropia. In this clinical situation, child to correct an esotropia of 18D at distance, this
first develops typical infantile esotropia before should be increased to bilateral 3.5 mm
6 months of age which is non-accommodative recession. Bilateral Faden operation on the
(for details see page 218). This is superadded by medial recti may also be required.
accommodative esotropia, usually, by the age
• To prevent undercorrection in patients with
of 2–3 years, often accompanied by a larger
the so-called decompensated, deteriorated,
hypermetropia than was first measured.
residual or non-accommodative portion of the
2. Decompensated accommodative esotropia mixed mechanism squint, following measures
with superadded non-accommodative esotro- have been recommended:
pia. In this clinical situation, the accommodative
– Surgery after prism adaptation. Patient is given
esotropia is well corrected with glasses or bifocal
full hyperopic correction as usual with
lenses. But, after some period of initial alignment
added press-on prism to neutralize any
of the eyes, the esotropia again develops due to
residual esodeviation. The patient wears the
some superadded non-accommodative factors.
glasses with press-on prism for 1 or 2 weeks
It has been postulated that in decompensated
and is then re-examined. If the esodeviation
accommodative esotropia, either increased
increases with the prism, new prisms are
convergence tone or mechanical factors such as
prescribed to neutralize the deviation. In
hypertrophy or contracture of the medial rectus
some patients, the esodeviation will increase
muscles, conjunctiva or Tenon's capsule may
as the patient 'eats-up' the prism. Surgery is
play a role.
planned for the full prism-adapted deviation.
Note: Partially accommodative esotropia is usually
– Enhanced or augmented surgery has been
constant and, therefore, typically associated with
advocated by some workers as an alternative
suppression, ARC and amblyopia.
to surgery with prism adaptation.
Treatment Note. Various formulas are used to augment
1. Correction of accommodative part of esotropia standard surgery. A simple and effective
should be carried out first. method to determine the target angle is by
2. Amblyopia is usually associated and should averaging the near deviation without correction
be treated (as described on page 190) before the (largest deviation) and the distance deviation
surgery is undertaken. with correction (smallest deviation).
 Concomitant Esotropias and Exotropias 233

INFANTILE ACCOMMODATIVE ESOTROPIA • Basic esotropia

Infantile accommodative esotropia is the term • Non-accommodative convergence excess
used for rare cases of accommodative esotropia esotropia
occurring at 3 to 4 months of age. • Divergence insufficiency esotropia
Differential diagnosis from essential infantile Basic esotropia
esotropia can be made by:
Clinical features
• Presence of straight eyes for the first 2 to 3
months, 1. Onset is in childhood (early a few years of
life) but decidedly after 6 months of age.
• Variable angle of deviation at the onset, and
2. Deviation is usually equal at distance and
• Presence of hypermetropia >2D.
near. At the onset, the angle of deviation is
Treatment consists of: smaller than essential infantile esotropia but the
• Full hypermetropic correction should be done angle may increase to 30 to 70.
immediately after cycloplegic refraction. 3. Accommodative factor is characteristically
• Amblyopia should be treated, when present. absent.
• Surgery is indicated when spectacles correction 4. Refractive error is insignificant.
cannot allign the eyes to within 10 PD (see 5. AC/A ratio is normal.
partially accommodative esotropia). Etiopathogenesis
ACQUIRED NON-ACCOMMODATIVE In view of the above clinical features,
ESOTROPIAS Costenbader postulated that this deviation is
due to excessive convergence tonus and so
This group includes all the acquired esode-
suggested the term 'acquired tonic esotropia' for
viations in which the amount of deviation is not
basic esotropia.
affected by the state of accommodation. These
include the following clinical entities: Clinical work-up
• Essential acquired or late onset esotropia • It should be on the general lines for work up
• Acute concomitant esotropia of any squint patient.
• Microtropia • A special word of caution has been conveyed
• Nystagmus blockage syndrome by many reports in the literature to rule out
• Cyclic esotropia any possibility of an underlying lesion of
• Divergence paresis malformation in the central nervous system
• Stress-induced esotropia in all patients with 'acquired non-accommo-
• Esotropia in myopia dative esotropia'.
• Esotropia due to spasm of near reflex
Treatment
ESSENTIAL ACQUIRED ESOTROPIA 1. Amblyopia, when present, should be treated
OR LATE ONSET ESOTROPIA first of all (see page 150).
In general, all forms of non-accommodative 2. Surgery is the ultimate treatment for basic
esodeviations other than essential infantile esotropia. It should be performed as early as
esotropia may be labelled as 'acquired non- possible after the amblyopia therapy. Results,
accommodative esotropias'. However, in in terms of binocular visual potential, are better
strabismic practice, the term acquired non- than the infantile esotropia.
accommodative esotropia or the late onset non-
Non-accommodative convergence
accommodative esotropia or essential acquired
excess esotropia
esotropia by common use has come to mean a
non-accommodative esotropia that occurs Clinical features
during the first few years of life and that cannot It is characterized by following features:
be grouped with other forms of esotropia. It 1. Onset of deviation is usually between 2 and
includes following clinical types: 5 years of age.
 234 Theory and Practice of Squint and Orthoptics

2. Deviation is characteristically large-angle element. It is typically associated with diplopia.

esotropia for near with a small angle esotropia/ Voluntary closure of one eye may often be the
esophoria or orthophoria at distance (similar to only sign in preverbal infants. Following two
non-refractive accommodative esotropia). Near forms of acute concomitant esotropia have been
distance disparity is 15 PD. reported.
3. AC/A ratio determined by lens gradient
method is normal or even low and the use of 1. Acute strabismus after artificial
bifocals has no effect on near deviation (unlike interruption of fusion
non-refractive accommodative esotropia due to • It has been reported to occur in patients with
high AC/A ratio). In view of the above, no previous history of squint, after interruption
determining the AC/A ratio by heterophoria of fusion under following conditions:
method (by comparing the near and distance – Prolonged bandaging of one eye for any
deviation) is likely to miss such a condition and surgery or perforating injuries.
patient may be wrongly prescribed bifocals to – Occlusion of one eye for treatment of
which they will not respond. ambylopia without squint (e.g. aniso-
4. Near point of accommodation is within metropic amblyopia).
normal limits. This observation differentiates the – Swelling of the lids following blunt trauma.
condition from the hypoaccommodative type of • It has been postulated that perhaps these
non-refractive accommodative esotropia. patients have latent esodeviation and/or
5. Refractive error. Patients are usually hyper- uncorrected hypermetropia which was fully
metropic or emmetropic. controlled by the well-functioning fusion
Treatment
mechanism without any symptoms. However,
the deviation becomes manifest after the
Surgical correction by bilateral medial rectus fusion is disrupted and the compensatory
recession with Faden operation (suturing the mechanism is thus suspended following
muscle to the sclera 12 mm behind the muscle occlusion of one eye.
insertion) is the treatment of choice. von
Noorden has reported that conventional Treatment
recession procedure of bilateral medial rectus • Spontaneous improvement occurs in some
(4 to 5 mm) with or without Faden operation is patients after the occlusion is removed.
usually ineffective. Therefore, a large recession
• Correction of underlying hypermetropia may
between 5 and 8 mm should be planned.
straighten the eyes in other patients.
Divergence insufficiency esotropia • Surgery may be required to correct esotropia
Clinical features in a few patients.
• It is characterized by a greater deviation for
2. Acute concomitant esotropia without
distance than near. Near-distance disparity
preceding disruption of fusion
should be 15 PD.
Etiopathogenesis
• It is usually comitant; but the possibility of an
underlying neurological lesion causing mild It has been postulated that perhaps such patients
sixth nerve palsy should always be ruled out. have an asymptomatic esophoria with only a
slim reserve of fusional amplitude that
Treatment maintains alignment of the eye over the years
Base-out prism may be tried to help the patient. but that may become lost under the influence of
However, often surgical treatment with bilateral physical or emotional strain.
rectus resection is required.
Clinical features
ACUTE CONCOMITANT ESOTROPIA • Onset is acute with diplopia.
It refers to sudden onset of large-angle • Deviation is relatively large.
concomitant esotropia without any paralytic • Refractive error is insignificant.
 Concomitant Esotropias and Exotropias 235

• Disruption of fusion is not the associated factor. Consistent findings

• No signs of paralysis of lateral rectus muscle. 1. Amblyopia. A microtropia should always be
• Good potential for binocular co-operation is suspected in unilateral decrease of visual acuity
there. (usually not worse than 6/18) for which no
organic cause can be found in patients without
Treatment apparent strabismus or a history of such and
Excellent functional results are obtained in such without significant refractive error or
patients with surgical treatment. anisometropia. Amblyopia is not the cause but
a result of microtropia.
MICROTROPIA 2. Abnormal retinal correspondence (ARC) as deter-
Depending upon the degree of deviation, Lange mined with: (i) Bagolini's striated glasses (page 129)
classified heterotropia as follows: and (ii) Foveo-foveal test of Cupper (page 134).
• Microtropia: 1°–5° 3. Relative scotoma on the fovea or in the case of
parafoveal fixation, the fixation point of the
• Small angle esotropia: 5°–12°
deviated eye. Tests for identifying a monocular
• Large angle esotropia: >12° scotoma in the binocular visual field include:
• Worth four-dot test (page 132)
Monofixation syndrome versus microtropia
• Binocular scotometry (page 185)
The sine quo non of the monofixation syndrome
• 4 base-out prism test (page 171)
is the absence of bifoveolar fusion with the
presence of peripheral fusion. There are various • Bagolini striated glasses (page 129)
causes of monofixation syndrome. Microtropia • A–O Vectographic Project-O-Chart slide
is the most common cause of monofixation (page 173).
syndrome (Park's syndrome). Thus, microtropia 4. Normal or near normal peripheral fusion with
is a cause and not synonymous with mono- amplitudes is present in patients with microtropia
fixation syndrome as used previously in the despite the fact that such patients have 3°–5°
strabismic literature. central scotoma in one eye during binocular
An array of other terms have also been used viewing and retinal image disparity of up to 10.
to describe the microtropia. Some of these are: This is possible because the Panum's fusional
Fixation disparity, fusion disparity, mono- space is wide in the peripheral retina (up to 5°)
fixational esophoria, retinal slip, retinal flicker as compared to central retina (only 20 minutes
and minisquint. of arc). It has been reported that peripheral
fusion begins at some point in the retina that is
Diagnostic features anywhere from 9° to 12° from the foveola.
Based on the fixation pattern, Lang has 5. Defective stereoacuity. Stereoacuity for near
described three types of microtropia: should at least be 3000 seconds of arc. A minimal
requirement for gross stereopsis of distance is
• Type-I—central fixation, not necessary for diagnosis. However, about
• Type-II—eccentric fixation without identity, one-third patients with monofixation syndrome
and have a distance stereoacuity between 240 and
• Type-III—eccenteric fixation, with identity, i.e. 120 seconds of arc.
angle of anomaly is same as the eccentricity
of fixation. Variable findings

Features of microtropia as described by Lange 1. Size of deviation can measure up to 10 

and also adopted by von Noorden et al can be horizontally and up to 3 vertically.
grouped as: 2. Fixation pattern is non-foveal but may be
foveal. Diagnosis of microtropia is difficult in
• Consistent findings, and patients with minute degrees of fixation
• Variable findings. anomalies.
 236 Theory and Practice of Squint and Orthoptics

3. Anisometropia may or may not be present. 2. Older children or adults with microtropia
Identification of microtropia is more difficult in need not be treated, since they have comfortable
isometropic patients. and nearly normal binocular vision with good
4. Cover test may be positive or negative. In peripheral fusional amplitude.
patients with positive cover test, diagnosis is
NYSTAGMUS BLOCKAGE SYNDROME
clearly established by a very small fixation
movement (flick) of the deviated eye upon Definition and etiology
covering the fixating eye. When the cover test is The term 'nystagmus blockage syndrome' has
negative (Fig. 10.8), special diagnostic procedures been suggested for the occurrence of esotropia
are required to differentiate a microtropia with in a child with congenital nystagmus. It has been
identity from non-strabismic causes of reported that in a bid to dampen the nystagmus,
decreased vision in one eye. there occurs adduction or excessive convergence
which results in esotropia. Others believe that
Treatment nystagmus is not the sole cause of esotropia.
1. In young patients who are visually immature Such patients usually have a static angle infantile
(age 6 or under), full-time occlusion therapy esotropia unrelated to nystagmus on which is
should be done to treat amblyopia after full superadded a dynamic angle due to conver-
refractive correction. Even microtropia is reported gence for dampening nystagmus.
to disappear following energetic occlusion
therapy. In patients showing recurrence, part- Clinical features
time occlusion should be continued for a long 1. Esotropia. Nystagmus blockage syndrome is
time. characterized in its acute form by an esotropia

Fig. 10.8 Microtropia with identity: A, Eyes appear straight and both eyes take up fixation; the right esotropic eye has
parafoveal fixation due to central scotoma; B, on cover test, right eye continues to fixate with the same parafoveal point and
thus there occurs no fixation movement.
 Concomitant Esotropias and Exotropias 237

of early onset with a variable angle, changing Onset. Cyclic esotropia can be acquired at
from orthotropia with manifest nystagmus during virtually any age but most frequently occurs
periods of visual inattention to esotropia without between 2 and 6 years of age.
nystagmus during visual attention. Eventually Cyclic nature of strabismus may last from
esotropia may become constant. 4 months to several years, after which the cycle
2. Nystagmus intensity is inversely proportional breaks and esotropia becomes constant.
to the angle of deviation. Nystagmus appears
as the fixing eye moves from adduction to Clinical features
abduction. This is an important distinguishing 1. During strabismic phase (24 hours)
feature from infantile esotropia with associated • Deviation is usually large, 40 to 70, and is
latent nystagmus which lacks inverse relation- consistent on subsequent examinations.
ship. • Suppression occurs in deviated eye, so usually
3. Pseudoparalysis of both lateral recti results due there is no history of amblyopia.
to maintaining the eyes in a position of • Fusional amplitudes are defective or absent.
convergence. It can be differentiated from a true 2. During non-strabismic phase (next 24 hours)
paresis of lateral recti by means of the Doll's • Deviation. There is no manifest deviation,
head manoeuvre (see page 219). however, esophoria may be present.
4. Fixation occurs with the adducting eye. • Fusion and stereopsis are both normal.

5. Head turn may exist towards the side of Treatment

fixating eye. When one eye is covered, a face turn • Surgery in the form of either bilateral medial
in the direction of the uncovered eye may be rectus recessions or recession of the medial
demonstrated. A periodic shifting of the head rectus and resection of the lateral rectus
from side to side may be observed in the constitutes the treatment.
presence of alternate fixation. • Amount of muscle surgery is based on the
6. Visual acuity is increased with adduction due degree of deviation during strabismic phase.
to dampening of nystagmus. Therefore, best • No overcorrection is seen on alternate days,
visual acuity is at near fixation. as is expected.

DIVERGENCE PARESIS
Treatment
It is a poorly understood condition characterized
Treatment is surgical but unsatisfactory. by comitant esodeviation present at distance
1. In the presence of a face turn, a recession of the fixation in patients having normal ductions and
medial rectus combined with Faden operation versions.
and a resection of the lateral rectus of the
adducting eye should be performed. This Etiology
procedure will relieve the face turn and also 1. Idiopathic. Etiology is not known in many
correct esotropia. cases. These cases are usually self-limiting.
2. In the absence of face turn, a bilateral medial 2. Neurological disorders may be associated in
rectus recession with Faden operation should some cases, so neurologic consultation is
be preferred. indicated for all such cases. A few reported
causes include tabes, encephalitis, disseminated
CYCLIC ESOTROPIA sclerosis, poliomyelitis, influenza, pontine
It is rare but fascinating form of esotropia tumour, increased intracranial pressure, trauma,
characterized by a strabismic and a non- and Arnold-Chiari syndrome.
strabismic phase of 24 hours each. This 48 hour
cycle is encountered most commonly, but Clinical features
72 hours and 96 hours cycles have also been 1. Diplopia. There is history of sudden onset of
reported in the literature. uncrossed diplopia at distance fixation. When
 238 Theory and Practice of Squint and Orthoptics

an object is brought near to the patient, the ETIOPATHOGENESIS

images become closer, and patient fuses them Sensory esotropia results from monocular lesions
at 25–50 cm. (from infancy to childhood) which either prevent
2. Deviation. Esotropia is usually more for the development of normal binocular vision or
distance with a near-distance disparity of 15 interfere with its maintenance. Examples of such
PD. The angle of esotropia remains unchanged lesions are: Congenital or acquired cataracts,
or may decrease on lateroversion. paediatric aphakia, corneal opacities, anisome-
3. Abduction and versions are completely tropia, optic atrophy, retinoblastoma, macular
normal bilaterally. lesions, severe congenital ptosis and so on.
4. Field of fixation is unrestricted. It has been reported that if the loss of vision
in one eye occurs in the first few months of life,
Differential diagnosis there may occur sensory esotropia or exotropia;
1. Sixth nerve palsy. The condition is differen- and after that till childhood, usually there occurs
tiated from either unilateral or bilateral sixth sensory esotropia. Occurrence of poor vision in
nerve palsy by the fact that in diversence paresis, adulthood due to any reason tends to cause a
the deviation is equal in both right and left sensory exotropia. However, Sidikaro and von
lateral gaze and that abduction is completely Noorden have reported that up to 5 years of age,
normal bilaterally. there are almost equal chances of getting sensory
2. Convergence spasm. Occasionally, divergence esotropia and sensory exotropia; and after
paralysis may be confused with convergence 5 years definitely there are more chances of
spasm, since in both the conditions patient developing sensory exotropia.
presents with uncrossed diplopia at distance Mechanism of sensory heterotropia. The exact
fixation. However, presence of unimpaired mechanism of development of sensory hetero-
fusional divergence and decreased visual acuity tropia is not known; however, following views
for distance are diagnostic features of have been put forward:
convergence spasm. Further, the esotropia will 1. Chavasse's theory. He reported that possibly
be more for near (15 PD) than distance. strong tonic convergence during childhood and
perhaps the diminishing tonic convergence with
Treatment
age contribute to the direction of sensory
Note. Neuroimaging studies, as well as heterotropia.
neurologic consultation are indicated to rule out 2. Bielschowsky's theory. He explained the
possible neurologic disease. increased incidence of sensory exotropia with
1. Prismotherapy. Since in most of the cases, advancing age as a gradual change of topo-
divergence paralysis is self-limiting and graphic anatomic orbital factors in adolescence,
disappears within 5–6 months, so base-out prisms favouring divergence rather than convergence.
may provide adequate relief from diplopia. The This explanation is difficult to reconcile with the
minimum power that gives the patient observation by Mann that the orbital axes
comfortable single vision at distance fixation actually converge rather than diverge.
should be used and should be decreased 3. Worth's theory. Worth speculated that the
periodically as the deviation improves. direction of a sensory heterotropia is determined
2. Surgery. If the condition does not disappear by the refractive error of the sound eye; that is,
within 6 months, resection of both lateral rectus the blind eye will diverge, if the sound eye is
muscles should be considered. myopic, and will converge, if the sound eye is
hypermetropic. However, this view has not been
SENSORY ESOTROPIA supported by other workers. Sidikaro and von
Noorden have reported an equal distribution of
DEFINITION refractive errors in patients with sensory squint.
It refers to the esotropia which develops due to 4. Spielmann's theory. He reported that there is
poor visual function in one eye in the childhood. a frequent association between monocular vision
 Concomitant Esotropias and Exotropias 239

loss, esotropia and manifest latent nystagmus. He 1. Surgical overcorrection of exotropia, is the
suggested that these signs together with cause of consecutive esotropia in almost all the
optometeric asymmetry are manifestations of cases. Its management has been discussed in
optokinetic immaturity occurring from lack of detail on page 248.
normal binocular inputs during early infancy. 2. Spontaneous consecutive esotropia, i.e.
From the above discussions, it is quite clear that change of exotropia into esotropia without any
still the mechanism of sensory esotropia is illusive. exogenous mechanical factor or an acquired
paralysis of lateral rectus muscle is an extremely
CLINICAL FEATURES rare condition. Hardly any such case has been
1. Monocular visual loss due to any cause is reported in the literature.
always associated.
2. Deviation. Sensory esotropia is always CONCOMITANT EXOTROPIAS
comitant. However, limitation of abduction due
to contracture of medial rectus or conjunctiva Concomitant exotropia is the term used to
or both may occur in long-standing cases. describe any manifest divergent deviation of the
Vertical deviation due to overaction of inferior visual axes in which the amount of deviation in
oblique muscle is a frequent association. the squinting eye remains constant (unaltered)
3. Amblyopia may be superimposed over the in all the positions of gaze and there is no
originally caused organic visual loss. associated limitation of ocular movements.
Concomitant exotropia may be divided into
TREATMENT four types:
• Surgical treatment is usually required to improve • Congenital (infantile) exotropia
cosmetic appearance, since visual loss is due • Primary exotropia
to some intractable organic lesion. However, • Sensory exotropia
in all such cases, the refractive error and • Consecutive exotropia
accommodational status of the straight eye
needs to be evaluated before contemplating CONGENITAL (INFANTILE) EXOTROPIA
cosmetic surgery. Congenital (infantile) exotropia is an extremely
• In children with sensory esotropia due to un- rare condition.
corrected aphakia or traumatic cataract, Systemic associations. It is reported to occur in
functional results may be obtained sometimes. patient with:
In these cases, cataract surgery, treatment of • Craniofacial anomalies,
aphakia and occlusion therapy for amblyopia • Ocular albinism, and
should be tried first, followed eventually by
• Cerebral palsy
squint surgery.
• Prematurity
• Medial rectus recession with or without lateral
rectus resection depending upon the size of Characteristic features of congenital exotropia
deviation. Should always be performed on the include:
eye with poor vision. Inferior oblique weaken- • Onset, usually before 6 months of age
ing should be performed for associated • Large angle constant exodeviation mostly more
overaction of this muscle. than 35 PD. Equal at distance and near
• Patients should always be informed that an • Fusion, prospectives are poor
esotropia may recur or a consecutive exotropia • Amblyopia, incidence is much higher than
may develop years after. intermittent exotropia
• Patterns. V-pattern more common than A-
CONSECUTIVE ESOTROPIA patten
Consecutive esotropia refers to occurrence of • Associations include dissociated vertical
esotropia in an eye which was previously deviation (DVD), primary inferior oblique
exotropic. It has been reported to occur under overaction (IOOA). Rarely superior oblique
following two clinical situations: overaction (SOOA) is also reported.
 240 Theory and Practice of Squint and Orthoptics

Differential diagnosis. Congenital exotropia needs include bright light, fatigue, ill health and day
to be differentiated from variable small-angle dreaming.
exodeviation seen in 70% of normal newborn
infants, which is a transient exodeviation and Stages of development of exodeviation
resolves by 2 to 4 months of age. In general, development of an exodeviation has
Treatment consists of: got three stages:
• Amblyopia therapy to be started at the earliest. 1. Stage of latent exodeviation. In this stage,
• Surgical treatment in the form of bilateral lateral exodeviation is kept latent by the control of
rectus recession should be performed after fusional convergence reserve. It is also called as
6 months of age, usually before the age of stage of exophoria. It has been described in detail
24 months. on page 209.
2. Stage of intermittent exotropia. In this stage,
PRIMARY EXOTROPIA the fusional convergence reserves which usually
Primary exodeviation is an idiopathic condition keep the deviation latent, become inadequate
in which the deviation is the essential feature, intermittently resulting in intermittent manifest
in contrast to other types in which divergence exodeviation (intermittent exotropia).
occurs as a result of certain obstacles in the 3. Stage of constant exodeviation. When the
development or maintenance of binocular single fusional convergence amplitude becomes
vision or due to defective action of the extra- inadequate to maintain the latency of deviation,
ocular muscles. a permanent manifest exodeviation, i.e. constant
exotropia occurs. Constant exotropia may be
ETIOLOGY unilateral or alternating.
Etiology of primary exotropia is speculative.
CLASSIFICATION
Following factors have been implicated:
I. In terms of the state of fusion, the exodevi-
Predisposing factors ations can be classified into:
1. Mechanical factors, which have been • Exophoria (see page 209),
implicated to predispose a person for develop- • Intermittent exotropia, and
ment of exotropia include: • Constant exotropia (unilateral or alternating)
• Shape and axes of the orbit, II. Duane's classification. It is based on the
• Interpupillary distance, assumption that divergence is an active process
• Size of the eyeball, rather than relaxation of the convergence with
• Mechanical properties of the conjunctiva or a return of the eyes to parallelism or a divergence
Tenon's capsule and position by mechanical or elastic forces. In
• Extraocular muscle characteristics. Duane's classification, the primary comitant
exodeviations (which include exophoria,
2. Innervational factors perhaps have been
intermittent exotropia and constant exotropia)
thought to play more important role than the
are further subdivided as follows:
mechanical factors in development of primary
exotropia. Duane hypothesized that primary 1. Basic exodeviation. Exodeviation is equal at
exotropias are caused by an innervational distance and at near, i.e. within 10PO of each
imbalance that upsets the reciprocal relationship other. It is thought to be associated with both
between active convergence and divergence divergence excess and convergence insufficiency.
mechanism. He suggested that exodeviation is Therefore, it is also called mixed type exodevia-
caused either by hypertonicity of divergence or tion.
convergence insufficiency or both. 2. Divergence excess type. The exodeviation is
at least 10 greater at distance than at near even
Precipitating factors after performing the patch test.
Precipitating factors which cause decompen- Note: Most of the patients with true divergence
sation of exophoria to intermittent exotropia excess type exotropia have high AC/A ratio, and
 Concomitant Esotropias and Exotropias 241

such patients are prone to postoperative Table 10.2 Phases of exodeviations and clinical
overcorrection, if the distance measurement is presentations
used as the target angle. Clinical presentation

3. Pseudodivergence excess type. Apparently, Phase Deviation Deviation Other features

at distance at near
the exodeviation appears to be greater at
distance than at near (e.g. XT 25D at distance I Exophoria Orthophoria
Asymptomatic
goes undetected
and 10D at near). However, with special tests II Intermittent Orthophoria Symptomatic
such as the patch for 30 minutes, the pattern is exotropia or exophoria for distance (no
shown to be basic type. (e.g. 25D at distance, suppression
25D at near). scotoma)
III Exotropia Exophoria or Binocular vision
This occurs because patients with pseudo- intermittent for near, suppress-
divergence excess have increased tonic fusional exotropia ion, scotoma for
convergence, which acts more at near. The distance
prolonged monocular patching dissipates tonic IV Exotropia Exotropia No binocular
single vision
fusional convergence, thereby disclosing the full
latent deviation. Short period of monocular
INTERMITTENT EXOTROPIA
occlusion that occur with alternate cover test is not
enough to break the tonic fusional convergence. Intermittent exotropia occurs much more
frequently than constant exotropia. It comprises
4. Convergence insufficiency type. The exo- between 70% and 90% of all exotropias.
deviation is at least 15 greater at near than at As discussed earlier, intermittent exotropia is
distance. usually preceded by a stage of exophoria. In
These four varieties of primary exodeviation intermittent exotropia, the deviation becomes
must not be considered as being in 'water-tight' manifest intermittentely when the fusional
compartments. convergence fails to control the deviation.
III. Phases of divergence excess type of
exodeviation: Calhounz et al. have described Clinical features
four phases of divergence excess type of 1. Time of onset. The onset of intermittent
exodeviation (Table 10.2). exotropia is usually in early childhood. About
IV. Kushner’s classification of intermittent 50% of the children develop intermittent
exotropia is depicited in Table 10.3. exodeviation before 6 months of age and nearly

Table 10.3 Kushner's classification of intermittent exotropia

Type Definition
Basic Distance and near measurements are equal
Tenacious proximal fusion Distance measurement initially exceeds near, but the near
measurement increases after 30–60 minutes of monocular occlusion
High AC/A ratio Distance measurement exceeds near measurement, even after 60
minutes of monocular occlusion and a high AC/A ratio is present
Proximal convergence Distance measurement exceeds near measurement, even after 60
minutes of monocular occlusion. AC/A ratio is normal
Low AC/A ration Near measurement exceeds distance measurement. A low AC/A
ratio is demonstrated
Fusional convergence insufficency Near measurement exceeds distance measurement. Patient has
poor fusional convergence amplitudes
Pseudoconvergence insufficiency Near measurement exceeds distance measurement, but distance
measurement increases with 60 minutes of monocular occlusion.
AC/A = Accommodative convergence/accommodation
 242 Theory and Practice of Squint and Orthoptics

70% develop within first two years of life. Some explained to occur owing to use of accommo-
begin at birth or shortly thereafter. Only a few dative convergence to control the exodeviation.
develop the exotropia after 5 years of age. 6. Deviation. At first, the deviation is typically
2. Sex distribution. Exodeviations are more manifest only at distance. With increasing age,
common in females (70.0%) than males (30.0%). the deviation progresses and there occurs an
The exact reason for female preponderance is increase in the duration and frequency of the
not known, possibly there might be some genetic tropia phase. Ultimately, a manifest deviation
factor. appears at near also (Tables 10.2 and 10.3).
Factors that may influence progression are:
3. Refractive errors. Earlier reports suggested
that myopia was more common in exotropia. • Decline in tonic convergence with increasing
However, recent view is that distribution of age,
refractive errors in exotropes resembles that in • Gradual lessening of accommodative power,
the orthotropes and that there is no role of • Development of suppression, and
underlying refractive errors in the etiology of • An increase in the divergence of the orbits
primary exotropia. with advancing age.
4. Precipitating factors. The heterotropic phase Associations
of intermittent exotropia most commonly occurs Intermittent exotropia may be associated with:
under conditions of fatigue, ill-health, bright light, • A–V-pattern (common)
day dreaming, drowsiness or visual inattention. • Comitant verstical deviation
5. Symptoms are as follows: • Dissociated vertical deviation
i. Transient diplopia may be experienced in the • Incomitant vertical deviation
beginning. However, suppression and later
Sensory adaptations
anomalous retinal correspondence (ARC) develop
quickly to protect the patient from diplopia. • Suppression and anomalous retinal corres-
pondence develop quickly to protect the
ii. Closing of one eye in bright light, conventionaly
patient from diplopia during exotropic phase.
referred to as photophobia is a conspicuous
• During non-strabismic phase, normal retinal
symptom of intermittent exotropia. The usual
correspondence is present.
history is that child closes one eye in bright light.
No convincing explanation is available in the • Deep amblyopia with eccentric fixation is a
literature for this phenomenon. Recent view is rare finding in exotropia.
that, perhaps, the bright light adversely affects Clinical evaluation
the amplitude of fusional convergence in
Detailed clinical evaluation should be carried
patients who maintain a delicate balance
out on the general lines (see page 100). However,
between exophoria and intermittent exotropia,
the points which need special attention are
causing them to close one eye.
mentioned here.
iii. Asthenopic symptoms may occur in the initial
phases, when fusion begins to succumb and the 1. History should provide information about:
eyes deviate momentarily from the ortho- Age of onset, change since onset, frequency of
position. Patient may experience eye strain, manifest phase and general health.
blurring, headache, difficulty with prolonged 2. Visual acuity is usually good. If unequal,
periods of reading and other asthenopic suspect: Anisometropia, microtropia or fundus
symptoms. However, soon the children become pathology.
asymptomatic due to development of sensory 3. Cycloplegic refraction and fundus exami-
adaptation. While adult patients with inter- nation should be carried out in each case.
mittent exotropia commonly have symptoms of 4. Cover test should be performed to assess at
decompensated exophoria. 1/3 m, 6 m and far distance.
iv. Micropsia is a comparatively less known 5. Measurement of deviation with prism bar
symptom of intermittent exodeviation. It is cover test (PBCT) should be performed in all the
 Concomitant Esotropias and Exotropias 243

cardinal positions of gaze at near and distance distance only or with at least 15  greater
fixation to discover any associated A- or V- exotropia at distance than near the occlusion test
pattern and presence of lateral gaze incomitance must be performed. Preferably one eye should
(LGI, i.e. 20% reduction in the angle of squint in be patched for 24 hours. However, recently it
lateral gaze). Detection of LGI is important to has been reported that only brief period of
prevent surgical overcorrection. occlusion (30 minutes to one hour) is sufficient.
Measurement of squint should also be made The detailed procedure of the occlusion test is
at a far distance beyond 6 metres since many a as follows:
time a larger angle of deviation may be detected. • First of all perform alternate cover test at
6. Measurement of stereopsis should be made distance and near to measure the angle of
during the phoric phase. Both near and distance exotropia (Fig. 10.9A to C). Let us presume
stereoacuity should be tested. A progressive this step has revealed that exotropia is
decline in stereopsis is a clear indication to significantly greater (15 or more) at distance
correct exotropia. Distance stereopsis than at near.
deteriorates earlier than the near stereopsis. • Patch is placed over one eye for one hour to
7. Occlusion test. It is very important for the dissociate the eyes thoroughly (Fig. 10.9D).
differentiation between true and simulated • After one hour, the fellow eye is covered with
divergence excess type of exotropia. Thus in an occluder and the patch is removed
every child with intermittent exotropia at (Fig. 10.9E and F). It is very important to

Fig. 10.9 Patch test for simulated divergence excess type exotropia (for explanation, see text).
 244 Theory and Practice of Squint and Orthoptics

prevent the patient from using both eyes • Fair control: Patient blinks or refixates to
simultan-eously even momentarily, since only control the deviation after disruption with
a brief binocular exposure may be sufficient cover testing.
to decrease the near deviation by fusional • Poor control: Patient who breaks spontaneously
convergence. without any form of fusion disruption.
• Again alternate cover test is performed at near Home control: At home, parents are told to keep
and deviation measured (Fig. 10.9 G and H). a chart noting the control of deviation in terms
In patients with simulated divergence excess of the percentage of waking hours the manifest
type exotropia, the angle of deviation for near deviation is noticed at home.
will increase markedly and will become equal
to the angle for distance (basic type); whereas II. Objective methods
with true divergence excess, the near deviation Distance stereoacuity testing is useful in noting
will remain unchanged, i.e. deviation for the deterioration of fusion, that occurs early in
distance will be 15 PD more than the near. this disorder. Normal distance stereoacuity
8. +3.0 D spherical lens test. In this test, indicates good control with little or no
measurement for near is performed with and suppression. The Mentor BVat II BVS assesses
without +3.0 DS lens in front of the exotropic distance stereoacuity using both contour circles
eye using an accommodative target which must and the ‘Random dot E test’ from 240 to
be seen clearly. This test is not an alternative to 15 seconds of arc disparity.
occlusion test in planning the surgical therapy Near stereoacuity: It does not correlate well with
(since, the occlusion test removes binocular the degree of control in intermittent exotropia
fusional stimuli, whereas +3.0 DS lenses provide and that performance in this test is only
only an indication of AC/A ratio). The +3.0 DS minimally affected by surgery.
lens test is useful in predicting how a patient
may respond to plus lenses, if surgical over- III. Newcastle scoring system
correction results. Since the clinic control does not take into account
9. Measurement of fusional amplitudes the duration of tropic phase and the fact that
• Convergence amplitudes are usually normal there is no standardization as to when to
at near and poor to good at distance. intervene, a novel method was put forth by H.
• Divergence amplitudes may be excellent to Haggerty and Richardson, the Newcastle
poor. Control Score. The revised New Castle Score
system seems to be useful in grading the
Assessment of control of intermittent exotropia severity of intermittent exotropia and as a
Assessment of control of intermittent exotropia criteria for surgical intervention.
before any intervention is useful in predicting The Newcastle Control Score (Table 10.4) takes
surgical outcome. Following methods of into consideration the subjective and objective
assessment are in vague: criteria to grade severity and quantify progress.
• Subjective methods The score is the sum total of scores obtained in
home control and in the clinic for near and far.
• Objective methods
Total score can vary from 0–7 and patients with
• Newcastle control score for intermittent a score of 3 or more are considered to need
exotropia surgical intervention. It is a consistent method
• Mayo scale for control in intermittent exotropia of rating severity and enables one to easily
monitor progress.
I. Subjective methods
Office control is graded as below: IV. Mayo scale for scoring of control in
• Good control: Patient “breaks” only after cover intermittent exotropia
testing and resumes fusion rapidly without Mayo scoring system (Table 10.5) is based solely
need for a blink or refixation. on times observations. An average of three score
 Concomitant Esotropias and Exotropias 245

Table 10.4 The Newcastle Control Score is taken to be more reliable. Score (0 to 5) is
measured both at distance and near fixation. So
Newcastle control score
a total score of 0–10 can be obtained.
Score Component
Home control Treatment
0 Squint/monocular eye closure never
1. Optical treatment
noticed
1 Squint/monocular closure seen occasionally • Myopia, when present, should be fully
(<50% of time) for distance corrected.
2 Squint/monocular eye closure seen • Hypermetropia up to +2.0 DS need not be
frequently (>50% of time) for distance corrected.
3 Squint/monocular closure seen for distance • Over minus lenses of 2 to 3D prescribed in
and near fixation emmetropes or over prescribed in myopes, is
Clinic control near reported to correct x(T) in some children.
0 Manifest only after CT and resumes fusion
without need for blink or refixation 2. Prismotherapy
1 Blink or refixate to control after CT Some strabismologists recommend use of base-
2 Manifest spontaneously or with any form in prisms to enforce bifoveolar stimulation. They
of fusion disruption without recovery correct one-half to one-third of deviation by
Clinic control distance prisms in order to stimulate fusional covergence.
0 Manifest only after CT and resumes fusion While other strabismologists like von Noorden
without need for blink or refixation do not prefer to use prisms. However, prismo-
1 Blink or refixate to control after CT therapy may be useful in children where surgery
2 Manifest spontaneously or with any form is to be postponed for some period.
of fusion disruption without recovery
The score varies from 0–7: score of 3 or more 3. Orthoptic treatment
significant Most of the strabismologists agree that, there is
not much role of preoperative orthoptics
treatment in patients with exotropia. However,
Table 10.5 Mayo score for scoring control of
following measures may sometimes be useful.
intermittent exotropia Aim is to make the patient aware of manifest
deviation and to improve the patient's control
Observation Score
over it.
Near Distance
i. Antisuppression exercises. An attempt
• No exotropia, unless 0 0 should be made to eliminate suppression so that
dissociated and recovers in the patient experiences diplopia whenever the
<1 second (exophoria) deviation becomes manifest. Suppression
• No exotropia, unless 1 1 scotoma usually and initially is amenable to
dissociated and recovers in therapy by flashes and then the BSV should be
1–5 seconds (exophoria) maintained by antisuppression exercises such
• No exotropia, unless 2 2 as bar reading, cheiroscope or on synoptophore.
dissociated and recovers in The methods of antisuppression treatment are
>5 seconds (exophoria) described in Chapter 8 page 174.
• Exotropia <50% of the 3 3
ii. Exercises to improve the patient's control of
examination before
the deviation by strengthening the fusional
dissociation
vergences. Improvement of fusional positive
• Exotropia > 50% of 4 4
relative convergence is of particular value in
the examination before
patients with intermittent exotropia at near only.
dissociation
The methods described in the treatment of
• Constant exotropia 5 5
exophoria (page 214) may be used with the aim
 246 Theory and Practice of Squint and Orthoptics

of ultimately obtaining a normal near point of von Noorden reports that alternate occlusion
convergence. Occasionally, a patient is unable for 3 months may be employed in lieu of surgery
to control the near exotropia with orthoptic with useful results in patients with small-angle
treatment, in which case, surgery should be intermittent exotropia.
indicated. In such cases, postoperative treatment
to improve the fusional vergences should be 4. Surgical treatment
given. Indications for surgery
Some people have questioned the effectivity i. Surgery is advisable, when the exotropia
of convergence exercises in controlling exode- occurs during more than 50% of waking hours,
viations. They cannot and do not affect the basic or causes asthenopic symptoms or when the
deviation but by improving the fusion control deviation exceeds 20.
decrease the manifestation of an exodeviation, ii. When the patient is exophoric most of the
a tropia being converted into a phoria. The lack time and becomes exotropic only two or three
of effect is only due to poor case selection (cases times a day, surgery should be preceded by
having suppression may require anti-supp- several months of observation, since the disease
ression exercises first) or improper method of does not progress in all patients. However,
exercising. Appreciation of physiological surgery should be undertaken, if during
diplopia should be taught as the first step. observation patient shows one or several of the
Secondly, training should be done to increase following signs and symptoms of progression:
both the phasic and tonic control, to improve • Gradual loss of fusional control as evidenced
the convergence sustenance. Just like any other by increasing frequency of the manifest phase
physical exercises, the results last till the of squint.
exercises are continued. Synoptophore exer-
• An increase in the size of basic deviation (more
cises may be desirable to start with but are
than 20).
insufficient, if not supplemented by proper
home exercises. • Development of secondary convergence
insufficiency with asthenopic symptoms.
For home exercises, special cards or a line on
• Development of suppression as indicated by
a plain paper may be made use of or a properly
absence of diplopia during manifest phase.
done "pencil-pushups" may be done.
• Gradual deterioration of stereopsis (it is a
It should be noted that no convergence frequently used argument for early surgical
exercises should be done by patients with therapy).
intermittent exotropia at distance only, in whom
surgery is planned, for this may lead to Age for surgery
postoperative overconvergence. There are two schools of thought:
iii. Occlusion therapy. It has been reported that i. Early surgery. Knap and many other workers
occlusion of preferred eye for 3 to 5 hours a day advocate early surgery. These workers state that,
for a long period is useful in decreasing the angle "surgery is dictated by the amount and fre-
of deviation. In one study, it has been reported quency of the exodeviation, not by the patient's
that about 40% patients with intermittent age. As long as the patient is 6 months or older,
exotropia become exophoric. The recommended surgery can be performed." However, they do
schedule of occlusion is as follows: caution that in visually immature children a
Initially, the results are evaluated after slight undercorrection should be attempted to
4 months of occlusion. If the angle of deviation prevent occurrence of mono-fixation syndrome
is decreased, the occlusion should be continued from consecutive esotropia.
and assessment made every 4 months until no ii. Delayed surgery. Jampolsky and a few other
further change occurs. In case, there is no workers recommend that surgery should be
improvement after initial occlusion for 4 months, delayed in visually immature infants to avoid
it should be discontinued. consecutive esotropia and occurrence of
 Concomitant Esotropias and Exotropias 247

monofixation syndrome. It is advisable that to Table 10.6 Rough estimate of amount of surgery to be
get good results, surgery may be delayed up to performed in patients with intermittent divergent squint
4 years of age. Till then child should be kept of true divergence excess type
under observation to watch: Visual acuity, Deviation in prism Bilateral lateral
convergences, and parent's observation of dioptres rectus recession
frequency of squint. Further, in the interim, 15 4.0
binocular vision should be reinforced with prisms 20 5.0
base-in or minus lenses. However, in case there 25 5.5
is very rapid functional deterioration of fusional 30 6.0
control in spite of prismotherapy and orthoptics, 35 6.5
an early surgical therapy may be considered. 40 7.0
50 8.0
60 9.5
General guidelines
70 8.0 + 8 mm MR resection
General guidelines adopted from the observation in one eye
of various workers are as follows: 80 8.0 + 8 mm MR resection
• In true divergence excess type of exotropia, a in both eyes
bilateral recession of lateral recti should be
Table 10.7 Rough estimate of amount of surgery to be
preferred. performed in patients with intermittent exotropia of
• In basic exotropia and simulated divergence excess basic and simulated divergence excess type
type, a unilateral lateral rectus recession and Deviation in Binocular surgery Uniocular surgery
medial rectus resection should be preferred. prism in mm in mm
However, Parks has shown that bilateral dioptres Bilateral LR LR + MR
rectus recession works for these patients also. recession recession resection
15 4.0 4.0 3.0
• In convergence insufficiency type of exotropia, a 20 5.0 5.0 4.0
bilateral medial rectus muscle resection may 25 5.5 6.0 4.5
be preferred. 30 6.0 7.0 5.0
35 6.5 7.5 5.0
• In the presence of lateral gaze inhibition (i.e. 20% 40 7.0 8.0 6.0
reduction in the amount of squint in right and 50 8.0 9.0 7.0
left lateral gaze), there is danger of over- 60 9.5 10.0 8.0
correction with the usual surgery especially 70 8.0 + 8.0 MR resection in one eye
so in visually immature patient. Therefore, in 80 8.0 + 8.0 MR resection in both eyes
the presence of LGI, a bilateral lateral rectus
Table 10.8 Rough estimate of amount of surgery to be
recession should be avoided and also, the
performed in patients with intermittent exotropia of
recession and resection done on the convergence insufficiency type
nonpreferred eye should each be 1 mm less. Deviation in prism Bilateral medial
• In small children (visually immature patients), a dioptres rectus resection
slight undercorrection should be attempted to 15 3.0
avoid hazards of consecutive esotropia. 20 3.5
25 4.5
• In visually mature patients, many strabismo- 30 5.5
logists feel that the surgeon should aim at an 35 6.0
overcorrection of 10D to 20D to ultimately 40 6.5
50 7.5
produce more stable results. However, it has 60 8.0
been observed that it is not possible to 70 8.0 LR recession of one eye + 8.0
accomplish this goal other than by pure 80 8.0 LR recession of both eyes + 8.0
chance.
• Single muscle surgery is almost useless in Amount of surgery
exotropias, it has been reported in the The actual amount of surgery performed will
literature. vary according to an individual surgeon's
 248 Theory and Practice of Squint and Orthoptics

technique. However, guidelines given in – Rerefraction should be done and if hyperme-

Tables 10.6, 10.7 and 10.8 may be useful as rough tropia is detected, it should be fully
estimate of amount of surgery to be performed. corrected.
– Bifocals or miotic therapy may be prescribed,
Management of associations if near esotropia is more than the distance.
1. Pattern deviations – Prismotherapy in the form of Fresnel
i. A-pattern exotropia (see page 285) membrane prism that fully correct the
ii. V-pattern exotropia (see page 285) deviation should be prescribed. The prism
power should be adjusted monthly
2. Comitant vertical deviation (see page 255) depending upon the latest examination
3. Dissociated vertical deviation (DVD). Though results.
very rare, but may be associated with • Re-surgery is indicated in patients where
exotropia (see page 264). overcorrection of more than 20 continues
even after the above described non-surgical
Surgical results and postoperative management
therapy for a period of 6 months.
After squint surgery, the eyes may be in It has been recommended that in planning
orthoposition, still exotropic (undercorrection) second surgery, Cooper's dictum (i.e. the
or consecutive esotropic (overcorrection). decision about choice and amount of surgery
Depending upon the position of the eyes, the should be made as if the present patient is a
postoperative management will be as follows: fresh case of squint) should be followed.
1. Orthoposition
B. In small children (i.e. visually immature
patients), as stated earlier, the consecutive
Undoubtedly, it is ideal to get a permanent esotropia is associated with a greater danger of
orthoposition, but there is always a tendency of developing monofixation syndrome and
the eyes to diverge postoperatively. Therefore, suppression amblyopia. So these patients need
it is extremely important to strengthen the positive a special care. Following measures should be
fusional convergence with orthoptic exercises in taken within 2 weeks of the surgery.
order to improve control of the newly acquired
• Re-refraction should be done and any
bifoveal single vision.
hypermetropic error should be fully corrected.
Orthoptic exercises required to strengthen the • Bifocals or miotics should be prescribed, if the
positive fusional convergence are the same as deviation is greater at near.
described for exophoria (page 214). • Occlusion therapy. Initially, when there is no
2. Consecutive esotropia (overcorrection) fixation preference, alternate occlusion should
A very large overcorrection with gross limitation be done for a few weeks. If the child develops
of ocular motility noted on the very next day is moderate fixation preference, conventional
possibly due to lost or slipped lateral rectus occlusion of the preferred eye should be used
muscle. Under such circumstances, patient till either there occurs alternate fixation or only
should undergo surgery again within 24 hours. a mild fixation preference.
• Prismotherapy in the form of 'Press on' base-
Small to moderate (10 to 50 ) overcorrection out Fresnel membrane prism should be started
needs to be managed depending upon the age at this stage to maintain bifoveal fixation. The
of the patient: prism power should fully correct the deviation
A. In adults (visually mature patients), as and be adjusted monthly depending upon the
described earlier, an overcorrection of 10 to 20 latest examination results.
is desirable to ultimately achieve stable results. • Re-surgery is indicated, if the child remains
• If overcorrection is more than 20 after at least overcorrected by 15 or more in spite of the
six weeks of surgery, then following non- above described non-surgical therapy.
surgical measures should be tried during the For a re-operation, the Cooper's dictum (as
wait and watch period of 6 months. stated above) should be followed.
 Concomitant Esotropias and Exotropias 249

3. Residual exotropia (undercorrection) Table 10.9 Plan of secondary surgical procedures in

i. Small residual exotropia (15 to 18) should patients with large residual exotropia
be managed by following non-surgical measures: Type of residual Primary Secondary
exotropia procedure procedure
• Optical correction. Refraction should be done
performed required
and if the patient is myopic, a full optical
Basic exotropia Recess-resect Recess-resect
correction should be ordered. (equal for near operation operation on the
• Cycloplegics. In hypermetropic or emmetropic and distance) other eye.
patient, 1% cyclopentolate eyedrops may be Bilateral Ipsilateral medial
instilled twice a day to stimulate accommo- lateral rectus rectus resection and
dative convergence. Once the alignment of the recession lateral rectus marginal
Divergence Bilateral myotomy
eyes and fusion are achieved, the frequency excess type lateral rectus Further recession
of instillation should be reduced progressively, (greater at recession or myotomies of
i.e. once a day, then on alternate day to every distance lateral recti.
third day. The regime may be continued for than near)
several months till full alignment is achieved. Convergence Recess-resect Recession of the
insufficiency procedure virgin lateral rectus
• Orthoptic exercises in the form of antisup-
type (greater and further recession
pression exercises (page 174) and fusional at near than or marginal myotomy
convergence exercises (page 214) should be distance) of the already recessed
continued till the goal is achieved. lateral rectus muscle
• Prismotherapy in the form of base-in prisms
that equals the undercorrection may be useful Failure
in visually mature patients. • Tropia at any distance or intermittent at two
of the four testing distances.
ii. Large residual exotropia (more than 15D to
18D) needs resurgery which can be performed • Absolute convergence less than 10 p.d. for
within 6–8 weeks of primary procedure. For a repeat distance.
surgery, the planning should be done as on a • Relative convergence less than 5 p.d. for
new case (Cooper's dictum). Depending upon distance and near.
the type of residual exotropia and primary • Near point of convergence less than 8 cm.
procedure done, the secondary procedure can • Peripheral and foveal suppression.
be planned as shown in Table 10.9. • No awareness of diplopia.
• Asthenopic symptoms.
Criteria for success of management
The criteria for classification of excellent and PRIMARY CONSTANT EXOTROPIA
poor success in the management of exotropia are Primary constant exotropia (Fig. 10.10) does not
as described below. occur as frequently as intermittent exotropia,
Excellent success
and both groups combinedly occur less
frequently than esotropias (constant exotropia,
• Phoria for distance and near in the primary aetiologically, may be primary, secondary,
position and reading position. sensory and consecutive). The number of
• Absolute convergence not less than 20 p.d. for primary constant exotropias is small compared
distance and near. to the number of secondary forms.
• Relative convergence not less than 15 p.d. for
distance and near. Clinical features
• Unlimited near point of convergence (5 cm or
1. Time of onset
closer).
• No suppression. Two types of primary constant exotropias have
• Excellent awareness of diplopia during testing been described depending upon the time of onset:
situation. 1. Primary constant, infantile exotropia. It is an
• Comfortable without asthenopic symptoms. extremely rare condition with onset shortly after
 250 Theory and Practice of Squint and Orthoptics

birth. It has been reported that this form of

exotropia is more common in the African than in
the white race. It has been described on page 239.
2. Primary constant exotropia due to decom-
pensated intermittent exotropia. The primary
constant exotropia almost invariably results from
decompensated intermittent exotropia, and thus
such patients usually do not possess a significant
amount of amblyopia. It has been reported that
in many patients intermittent exotropia may not
decompensate for a long period. Therefore, it has
been recommended that patients with inter-
mittent exotropia need to be evaluated over a
period of time to ascertain whether progression
is taking place and surgery is warranted,
particularly those in whom a constant deviation Fig. 10.10 A patient with primary alternate divergent squint
is present less than 50% of the time. (ADS): A, Right exotropia while fixing with left eye; and
B, left exotropia while fixing with right eye.
2. Fixation pattern
The patients with constant exotropia may fix suppressed. Thus the left eye would perceive
alternately, fix with one eye only or show a mild, the left half of the field but suppress the right
moderate or strong fixation preference. According half, while the right eye would see the right
to the fixation behaviour, constant exotropia may half and suppress the left. The fixation point
be classified as unilateral or alternating: and its vicinity, in this case, is seen by the
fixating eye and is included in the suppression
(a) Alternating exotropia area of the deviating eye.
i. Fixation. In constant alternating exotropia, the
(b) Unilateral exotropia
patient uses each eye alternately for fixation
(Fig. 10.10). i. Fixation. In constant unilateral exotropia, the
ii. Visual acuity. Usually, there is little or no same eye is used for fixation while the other is
difference between the visual acuity of the two deviated. Fixation preference may be mild,
eyes. moderate or strong:
iii. Deviation. The angle of deviation is usually • In mild fixation preference, one eye is preferred,
large and tends to be equal for distance and near but when it is covered, the other eye easily
fixation. The near deviation is determined by the takes up fixation and maintains it even when
effect of proximal and accommodative conver- the cover is removed from the first eye. Such
gence. Very frequently there is a secondary patients usually do not have a significant
vertical deviation, the deviating abducted eye amount of amblyopia.
being elevated. • In moderate fixation preference, one eye is
iv. Sensory adaptations. In cases with acquired preferred and when it is covered, the other eye
alternating exotropia, the patients had normal will take up fixation; but after the cover is
retinal correspondence and bifoveal fusion removed from the first eye, the other eye will
before the deviation became constant. If normal hold fixation only until the patient blinks or
correspondence persists, there occurs complete changes fixation.
suppression of the deviating eye. However, if • In strong fixation preference, one eye is preferred
ARC develops, both eyes co-operate in and when it is covered, the other eye will take
binocular vision. Sometimes a form of binocular up fixation, but only as long as the cover is
vision is achieved whereby each half of the left over the first eye.
visual field is perceived by the homolateral eye • In unilateral fixation, only one eye is preferred
while the information from the other half is and when it is covered, the other eye does not
 Concomitant Esotropias and Exotropias 251

take up fixation but makes irregular jerky Treatment

movements, suggesting loss of central fixation The treatment of constant exotropia is almost
and a deep amblyopia. always surgical. Orthoptic treatment is given
ii. Deviation is usually large. In patients with both preoperatively and postoperatively, if
long-standing neglected deviations, anatomic fusion ability can be demonstrated. Any patient
over 6 months of age can be taken for surgery.
changes may occur in the contracted muscles.
The type and amount of surgery to be performed
iii. Sensory adaptations. There is marked is similar to that for intermittent exotropia.
suppression in the deviating eye, but amblyopia
is less severe than in esotropia. ARC may be SENSORY EXOTROPIA
present but is of little importance since
Definition
suppression prevails. Since the majority of
unilateral exotropias are acquired, most of the It refers to unilateral exotropia that develops as
patients have had normal retinal correspon- a result of poor visual function in one eye.
dence previously.
Etiopathogenesis

Associations Sensory exotropia results secondary to some

sensory deficit such as anisometropia, unilateral
Primary constant exotropia may be associated cataract, unilateral aphakia, corneal opacity,
with: optic atrophy, macular lesion and any other
• A- or V-pattern (Fig. 10.11) with or without organic cause of unilateral loss of vision.
oblique muscle by function It has been reported that both infants and
• Comitant vertical deviation muscle dept young adults with poor vision in one eye develop
function sensory exotropia and the older children tend to
• Dissociated vertical deviation (DVD) develop a sensory esotropia. The mechanisms
and theories about development of sensory
Clinical evaluation esotropia and exotropia have been described on
The aim of clinical evaluation is to diagnose type page 238.
of deviation and to assess potential for binocular
single vision. The complete clinical evaluation Clinical features
should be carried out in each case of strabismus. 1. Monocular visual loss due to any cause is
Points pertinent to the evaluation of primary always associated.
constant exotropia are similar to those described 2. Deviation is unilateral and constant and
for the intermittent exotropia. involves the eye with poor vision (Fig. 10.12).

Fig. 10.11 Nine gaze photographs of a patient with alternate divergent squint with V-pattern with inferior oblique over action
(IOOA).
252 Theory and Practice of Squint and Orthoptics

Fig. 10.12 Nine gaze photographs of a patient with left sensory exotropia.

Treatment
• Cosmetic surgery is the treatment for most
sensory exotropias. However, some scope of
functional recovery may be there in children
with sensory exotropia due to anisometropia
including unilateral aphakia.
• Choice of surgery for sensory exotropia is
usually recess-resect operation on the same eye.
In large exodeviation, one can recess lateral
rectus up to 10 mm and can resect medial rectus
also up to 10 mm. Though, it may result in
limitation of horizontal movements in the
operated eye.
• Adjustable suture surgery may be preferred
in patients above 10–11 years of age with sensory
exotropia.

CONSECUTIVE EXOTROPIA
Consecutive exotropia refers to occurrence of
exotropia in an eye which was previously
esotropic. Fig. 10.13 A patient with esotropia (A), who developed
consecutive exotropia after surgery (B), which was surgically
Clinical types corrected to orthotropia (C).
It has been reported to occur under following
two clinical situations: medial rectus muscle. Spontaneous consecutive
1. Surgical overcorrection of esotropia may exotropia is known to occur more commonly
result in consecutive exotropia (Fig. 10.13). under following circumstances:
2. Spontaneous consecutive exotropia is change • Esotropia with poor vision in the deviating eye.
of esotropia into exotropia without exogenous • Infantile esotropia associated with a high
mechanical factors or an acquired paralysis of hypermetropia.
 Concomitant Esotropias and Exotropias 253

Treatment 12. Havener WH: Ocular Pharmacology, St. Louis,

1. Refractive error, if any, kind should be Mosby, 1978.
corrected. 13. Henson DB, Williams DE: Depth perception in
strabismus. Br J Ophthalmol 64:349, 1980.
2. Assess state of binocular sensory cooperation:
i. If normal retinal correspondence and bifoveal 14. Hiles DA: Surgery for congenital esotropia. Int
Ophthalmol Clin 16 (3) : 75, 1976.
fusion can be demonstrated (especially in
15. Hoyt WF, Daroff RB: Supranuclear disorders of
patients with overcorrected esotropia):
ocular control systems in man: Clinical,
• Overcome suppression
anatomical and physiological correlations. In
• Improve fusional convergence Bach-y-rita P, Collins CC, Hyde JE(eds): The
• Perform surgery Control of Eye Movements New York. Academic
ii. If bifoveal fusion cannot be demonstrated, assess Press, 1971, p 175.
cosmetic appearance: 16. Ing MR: Early surgical alignment for congenital
• If good, leave alone. esotropia. J Pediatr Ophthalmol Strabismus
• If poor, perform cosmetic surgery. 20:11-18, 1983.
17. Jotterand VH, Isenberg SJ: Enhancing surgery
BIBLIOGRAPHY for accommodative esotropia, Ophthalmic Surg
19:263-266, 1988.
1. Burian HM: Pathophysiology of exodeviations. 18. Krzystkowa, K and Paja Kowa J, The sensorial
In Manlev DR (ed): Symposium on Horizontal state in divergent strabismus. In orthoptics,
Ocular Deviations. St. Louis. Mosby, 1971. p 119 Proceeding of the second international
2. Burian HM, Noorden GK von: Binocular Vision orthoptics congress, Amsterdam, 1972, Excerpta
and Ocular Motility. St. Louis, Mosby, 1974. media foundation, p 72.
3. Burian HM: Exodeviations : Their classification, 19. Kushner BJ: Exotropic deviations: A functional
diagnosis and treatment. Am J Ophthalmol classification and approach to treatment. Am
62:1161, 1966. Orthoptic J 38:81-93, 1988.
4. Cooper El: Purposeful overcorrection in 20. Manle DR: Classification of the exodeviations, in
exotropia. In Arruga A(ed): International Manley DR (ed): Symposium on Horizontal
Strabismus Symposium (University of Geissen, Ocular Deviations. St. Louis. Mosby, 1971.p 128.
1966). Basel/New York, S Karger, 1968, p 311.
21. Nelson LB Bacal Da Burke MJ An alternative
5. Costenbader FD: Roundtable discussion. In
approach to the surgical management of
Allen JH (ed): Strabismic Ophthalmic
exotropia : the unilateral lateral rectus recession.
Symposium II. St. Louis, Mosby, 1958, p 484.
J.Ped. Ophthalmol strabismus 1992. 29 (6) 357–
6. Costenbader FD: Infantile esotropia. Trans Am
60.
Ophthalmol Soc. 59:397, 1961
22. Noorden GK von: Divergence excess and
7. Calhou JC, Nelson LB, Harley RD Atlas of Ped.
simulated divergence excess, diagnosis and
Ophthal Surgery Philadelphia Saunders. 1987
surgical management, Ophthalmologica 26: 719,
pp 8-10.
1969.
8. Crone RA. Everhard-Halm Y: Cyclofusion. In
Moore 5, Mein J (eds): Orthoptics: Past, Present, 23. Noorden GK von: Some aspects of exotropia,
and Future. New York, Stratton Intercontinental, presented before meeting of the Wilmer Resident
1976, p 409. Association, John Hopkins Hospital, April 26,
9. Dell' Osso LF, Ellenberger CJr, Abel LA et al: 1966.
The nystagmus blockage syndrome: Congenital 24. Noorden GK von: The nystagmus compensation
nystagmus, manifest latent nystagmus or both? (blockage) syndrome. Am J Ophthalmol 82:283,
Invest Ophthalmol Vis Sci. 24: 1580-87, 1983. 1976.
10. Duane A: A new classification of the motor 25. Noorden GK von: Indications of the posterior
anomalies of the eye based upon physiological fixation operation in strabismus. Ophthalmology
principles, together with their symptoms, (Rochester) 85:512, 1979.
diagnosis and treatment. Ann Ophthalmol 26. Parks MM: The monofixation syndrome. Trans
Otolaryngol 5:969, 1896; 6:84, 247, 1897. Am Ophthalmol Soc 67:609. 1969.
11. Helveston EM: Cyclic strabismus. Am Orthopt 27. Prism Adaptation Study Group: Efficacy of
J 23: 48-51, 1973. prism adaptation in the surgica management of
254 Theory and Practice of Squint and Orthoptics

acquired esotropia. Arch Ophthalmol 108:1228- exodeviations the immediate result and several
1256, 1993. years later Am Orthopt J. 1970: 20: 104-17.
28. Reinecke RD: Accommodative esotropia. J 31. Tychsen L, Lisberger SG: Maldevelopment of
Continuing Educ Ophthalmol 40:11,1978. visual motion processing in humans who had
29. Rogers GL, Chazen S, Fellows R, et al: strabismus with onset in infancy. J Neurosci
Strabismus surgery and its effect upon infant 6:2495-2508, 1986.
development in congenital esotropia. 32. Wright KW, Bruce-Lyle L: Augmented surgery
Ophthalmology 89:479-483, 1982. for esotropia associated with high hypermetropia.
30. Sanfilippo S. clahane AC The effectiveness of J Pediatr Ophthalmol Strabismus 30:167-170,
orthoptics alone in selected cases of 1993.
 11
Vertical Strabismus and
Cyclodeviations

VERTICAL STRABISMUS • Etiology

• Classification • Clinical characteristics
• Comitant vertical deviations
• Incomitant vertical deviations • Diagnostic tests
– Classification – Subjective tests
– Apparent oblique muscle dysfunction – Objective tests
• Dissociated vertical deviations • Treatment
• Dissociated horizontal deviation – Cyclovertical deviation
CYCLODEVIATIONS – Cyclodeviation without vertical deviation
• Classification

VERTICAL STRABISMUS III. Dissociated vertical deviation (DVD)

• Monocular DVD
CLASSIFICATION • Bionocular or alternating DVD
A. Depending upon constancy of deviation
1. Hyperphoria (H) COMITANT VERTICAL DEVIATIONS
2. Intermittent hypertropia (H [T]) The prevalence of comitant vertical deviations
3. Hypertropia (HT) in general is not low. Vertical deviations may
B. Depending upon the direction of deviation in occur as isolated anomalies or in association
the non-fixing eye with horizontal deviations.
1. Hypertropia Prevalence. Following observations have been
2. Hypotropia made regarding prevalence of vertical deviations:
C. Depending upon comitance of deviation • Approximately, half of the patients with
motility disorders have isolated vertical
I. Comitant vertical deviation
anomalies.
1. Induced (refractive) • Approximately, one-third of all patients with
2. End result of long-standing paralytic motility disorders have a combined horizontal
deviation and vertical deviation.
II. Incomitant vertical deviations • An associated vertical deviation has been
Depending upon the innervational etiology, reported in 43% of all exotropias.
the incomitant vertical deviations can be Types. Comitant vertical deviations include:
further subdivided into following types: 1. Hypertropia. In this condition, non-fixating eye
1. Apparent oblique muscle dysfunction is higher than the fixating eye.
2. Paretic vertical deviations 2. Hypotropia. In this condition, non-fixating eye
3. Restrictive vertical deviations is lower than the fixing eye.
255
 256 Theory and Practice of Squint and Orthoptics

Etiology vision, orthoptic treatment to eliminate

Exact etiology is not known. Following factors suppression may be indicated prior to surgery.
have been blamed: It is almost impossible to improve vertical
fusional vergence through orthoptic training.
• Correction of unequal refractive error may induce
comitant hyperdeviations. 2. Prismotherapy. Comitant vertical deviations
• Anomalous position of rest caused by orbital or smaller than 10D can be corrected with prism.
other anatomical anomalies or mechanical The prism power should be distributed equally
factors or abnormal innervation may be before the two eyes with a base-down prism in
causative mechanism. front of the hypertropic eye and a base-up prism
infront of hypotropic eye. The minimal prismatic
• Convertion of incomitant paralytic hyperdeviation
power that provides comfortable single
to comitant with the passage of time (as
binocular vision should be prescribed.
secondary changes occur) is also common to
find. 3. Surgery. Following procedures are indicated:
• A comitant vertical deviation up to 11D to 14D
Clinical features associated with horizontal deviation can be
• Symptoms can be marked in hyperdeviations, eliminated by simply lowering the horizontal
even when the magnitude is low. Patients muscle insertion (by 5 to 8 mm) of the
often present with frontal headache, diplopia, hypertropic eye or by raising the insertions of
ocular discomfort or pain, due to overuse of hypotropic eye; while performing the
fusional vergence. horizontal squint surgery.
• Hypertropias are more frequently intermittent • For large vertical deviations (between 15D and
than constant deviations. 25D), a 3 to 4 mm recession of the appropriate
• Suppression, amblyopia, or vertical anomalous vertical rectus muscle is recommended. For
retinal correspondence may occur. example, in a patient with right hypertropia
of 25 D, the right superior rectus and left
• In deviation of lesser magnitude, the patient
inferior rectus should each be recessed by
may obtain bifoveal fusion by tilting the head.
4–5 mm.
• Typically, patients with small comitant
vertical deviations have a moderate to large
horizontal strabismus (exotropia or esotropia) INCOMITANT VERTICAL DEVIATIONS
as well.
CLASSIFICATION
• Repeated measurements in the diagnostic
positions of gaze in majority of patients may Incomitant vertical deviations can be classified
reveal a paretic component or a primary as below:
overaction of one or several cyclovertical 1. Apparent oblique muscle dysfunction
muscles. i. Inferior oblique overaction (strabismus surso-
adductorious) is now termed as over-elevation
Note. Large primary comitant vertical deviations
in adduction (OEA). It can be:
are rare. The primary vertical deviation must be
differentiated from skew deviation, which is • Primary OEA, or
rather abrupt in onset, variable, and associated • Secondarry OEA.
with symptoms caused by intracranial or ii. Inferior oblique underaction, is now termed as
labyrinthin disease. under-elevation in adduction (UEA). It can be:
• Primary UEA, or
Treatment • Seconday UEA
1. Orthoptics. Amblyopia, when present, iii. Superior oblique overaction (strabismus
should be treated. If a bifoveal fusion potential deorsadductorious) is now termed as over-
and normal correspondence can be demon- depression in adduction (ODA). It can be:
strated in patients in whom the deviation was • Primary ODA, or
acquired after a period of normal binocular • Seconday ODA.
 Vertical Strabismus and Cyclodeviations 257

iv. Superior oblique underaction is now termed as • Due to fat adherence as seen after retinal
under-depresson in adduction (UDA). It can be: detachment surgery.
• Primary UDA, or • Post-radiation orbital scarring.
• Secondary UDA. • Due to conjunctival and Tenon’s capsule
scarring:
2. Paretic vertical deviations
• Congenital unilateral superior oblique paresis. – Postoperative
– Post-traumatic
• Non-congenital superior oblique paresis.
– Post-chemical burns
• Bilateral superior oblique paresis.
iii. Restrictive vertical deviation due to orbital mass
• Monocular elevation deficiency (MED), (old lesions, e.g. as in:
name: Double elevator palsy).
• Orbital tumours
• Monocular depression deficiency (MDD); (old
• Glaucoma explant with large bleb causing
name: Double depressor palsy).
mass effect.
• Superior rectus paresis (isolated).
Note. Incomitant vertical deviations caused by
• Inferior rectus paresis (isolated).
overaction of superior and inferior oblique
• Skew deviation. muscles are discussed here.
• Inferior oblique paresis Incomitant vertical deviations caused by
3. Restrictive vertical deviations weakness, paralysis or restriction of the
cyclovertical muscles are discussed in Chapter
A. Restrictive vertical deviations due to misdirected
12.
muscle force, as seen in:
i. Congenital cranial dysinnervation disorders APPARANT OBLIQUE MUSCLE DYSFUNCTION
(CCDDs) primarily affecting vertical ocular
INFERIOR OBLIQUE OVERACTION
motility.
• Congenital fibrosis of extraocular muscles Inferior oblique muscle overaction, also referred
(CFEOMs) to as strabismus sursoadductorious, is now termed
as over-elevation in adduction (OEA). It is
ii. Iatrogenic displacement of inferior oblique characterized by an upshoot of the eye in
muscle after its anteriorization. adduction.
B. Restrictive vertical deviation due to mechanical
restrictions as seen in: Etiology
i. Tight extraocular muscles, e.g. 1. Primary overaction of the inferior oblique
• Congenital Brown’s syndrome (new name muscle is etiologically not well understood.
restrictive hypotropia in adduction, i.e. Perhaps it may be due to mechanical or
RHA). innervational causes or a combination of the
• Hypotropia due to incarceration of two. It may occur as an isolated phenomenon
inferior rectus in blow-out fracture of or in association with esotropia or exotropia-
orbital floor. often of V-pattern.
• Hypotropia due to inferior rectus 2. Secondary overaction of the inferior oblique
thickening in thyroid ophthalmopathy. muscle is caused by paralysis or paresis of either
its antagonist muscle (ipsilateral superior
• Hypotropia in monocular elevation
oblique muscle) or its yoke muscle (contralateral
deficiency (MED) caused by fibrotic
superior rectus muscle). Another cause of
inferior rectus muscle.
secondary overaction of the inferior oblique,
ii. Restrictive vertical deviation due to structural unrelated to paralysis is non-parallelism of the
adhesions (induced adhesive syndromes), e.g.: plane of superior and inferior oblique muscles.
• Acquired Brown’s syndrome due to (The term desagittalization has been suggested
scarring around the trochlea. to describe this dysfunction.)
 258 Theory and Practice of Squint and Orthoptics

Clinical features (32%). The associated horizontal deviation is

Clinical features of primary inferior oblique usually of V-pattern. It may be a part of infantile
overaction (PIOO) versus secondary inferior esotropia syndrome (page 218). In contradiction
oblique overaction (SIOO) are as follows: to it, SIOO is usually not associated with any form
of concomitant deviation.
1. Age of onset. PIOO usually occurs after one
year of age, while SIOO can occur at any age. 5. Associated vertical deviation in primary
SIOO occurs either spontaneously or few weeks position is either absent or less than 5PD in
to months following paresis of the ipsilateral PIOO. While a vertical deviation of 10–22PD is
superior oblique muscle or contralateral characteristic of SIOO, a vertical deviation of
superior rectus muscle. more than 22PD usually suggests a coexisting
vertical rectus muscle weakness. Bilateral
2. Bilaterality. PIOO is frequently bilateral
inferior oblique overaction produces a left
(72%) and often asymmetric while SIOO is
hypertropia in right gaze, a right hypertropia in
occasionally bilateral.
left gaze and little or no vertical deviation in
3. Upshoot or over-elevation of the eye in
primary position. Further, primary IOOA is
adduction is the clinical characteristic of primary
commonly associated with DVD also.
as well as secondary inferior oblique overaction.
6. Head tilt is typically present in patients with
That is, with the eyes in lateral gaze and the
SIOO, when the onset is after 6 years of age,
abducting eye fixing, the adducted eye is over
while it is usually absent in patients with PIOO.
elevated. When the eyes are in lateral gaze and
Distinguishing PIOOA from SIOOA is largely
the adducting eye is made to fix, the abducted
dependent on the head tilt test. If the head tilt
eye will be depressed and manifest a hypotropia
test is negative this indicates a PIOOA, whereas
on alternate cover testing. (c.f. DVD page 267). a positive head tilt test (increasing hypertropia
Clinically, the upshoot of the eye can be graded with tilt to the side of IOOA ) indicates a superior
on a scale of +1 to +4 (page 115). In a +4 oblique paresis or secondary inferior oblique
overaction, the cornea nearly disappears overaction.
(Fig. 11.1). 7. Associated excyclodeviation. Objective
4. Associated horizontal deviation in primary excyclodeviation, as evidenced by a disturbed
position. PIOO is commonly (but not universally) relationship of the optic disc with foveola
associated with some horizontal deviation such (normally the foveola is aligned approximately
as infantile esotropia (72%), accommodative with the junction of the middle and lower third
esotropia (34%) and intermittent exotropia of disc), can be demonstrated both in patients

Fig. 11.1 A patient with bilateral primary inferior oblique overaction. A, no vertical deviation in primary position; B, +4 inferior
oblique overaction in left eye on dextroversion; and C, +4 inferior oblique overaction in right eye on levoversion.
 Vertical Strabismus and Cyclodeviations 259

Fig. 11.2 Fundus photograph showing relation of optic disc with foveola in a normal subject (A) and in a patient with inferior
oblique overaction (B) direct view; (C) indirect ophthalmoscopic view.

with primary as well as secondary inferior procedure on the inferior oblique muscle is
oblique muscle overaction (Fig. 11.2). However, indicated. Mostly, surgery is done for the
subjective excyclodeviation (as demonstrated by functional reasons, that is, when the hypertropia
double Maddox rod test, Hess screen test, the produced by the overacting inferior oblique
major amblyoscope or the Lancaster red-green muscle presents an obstacle to fusion in
test) is typically present in patients with SIOO lateral gaze or a V-pattern exists that prevents
(and extorsion is maximum in downgaze), when fusion in upward (V-exotropia) or downward
the onset is after 6 years of age, but is absent in (V-esotropia) gaze.
patients with PIOO. Absence of subjective
Inferior oblique weakening procedures that have
excyclodeviation in PIOO might be due to the
been employed are as follows:
development of some sensory adaptation in
such patients owing to early onset of the 1. Disinsertion, i.e. cutting of the muscle from
condition. the globe near its insertion is an effective and
easy technique. However, because of the
8. Forced duction test is usually positive in
unpredictable results and high rate of
primary as well as secondary inferior oblique
recurrences (>50%), this procedure is not much
overaction, indicating there by that both
popular.
mechanical and innervational factors contribute
2. Myectomy involves excision of approximately
to most inferior oblique overaction, including
8 mm of inferior oblique muscle. This procedure
primary form.
is generally performed between the temporal
border of inferior rectus muscle and insertion
Differential diagnosis
of the inferior oblique. It can also be performed
1. Dissociated vertical deviation. Inferior oblique nasally between the origin of inferior oblique
overaction should be differentiated from and the nasal border of inferior rectus muscle.
dissociated vertical deviation (see pages 218 and This procedure is also becoming unpopular
219). because of unpredictable results, a high rate of
2. SIOOA. Primary inferior oblique overaction recurrences (especially nasally performed
can be differentiated from the secondary inferior myectomy) and occurrence of postoperative
oblique overaction as described in clinical adhesive syndrome in some cases. However,
features. temporally performed myectomy is still
3. Pseudo ‘V’ pattern due to DVD, Duanes preferred by many surgeons especially for
syndrome and large intermittent exotropia also treating recurrent inferior oblique overaction. A
needs to be differentiated from PIOOA. unilateral myectomy will correct 5 to 20 PD of
hypertropia.
Treatment 3. Denervation and extirpation, i.e. excision of
When hyperdeviation of the adducted eye whole of the muscle with its Tenon's capsule
becomes clinically significant, a weakening covering after cauterisation of the neurovascular
 260 Theory and Practice of Squint and Orthoptics

bundle has been reported to be effective with from the insertion, the inferior oblique is
least incidence of recurrence. The procedure is reattached to the sclera near the lateral end of
reported to be effective in severe overaction (+4) the inferior rectus insertion. This technique is
or in residual overaction when recession or even easier than the simple recession.
anteriorization has already been perfomed. Anteriorizing the inferior oblique muscle
However, it is not being considered necessary insertion, anterior to eyeball’s equator changes
to sacrifice the muscle. the IO muscle from an elevator to more of a
4. Recession of the inferior oblique is being depressor. The more IO is anteriorized the more
preferred by many surgeons especially for it becomes a depressor and this enhances its
secondary inferior oblique overaction and for recession effect. Compared with recession,
cases with mild to moderate primary inferior anteriorization decreases ocular elevation by
oblique overaction. Recession of 6–10 mm can approximately 15 PD more.
be performed as indicated. In this procedure, Graded IO anteriorization (as described below)
inferior oblique is detached from its insertion and works extremely well for both PIOOA and
is reattached to the sclera (along the lateral margin SIOOA:
of the inferior rectus) at a site selected depending • +4 overaction: Full anteriorization, up to the
upon the amount of recession to be done. For insertion line of temporal border of inferior
example: rectus.
• For a maximum recession of 12–14 mm, anterior • +3 overaction: 1 mm posterior to IR insertion
border of inferior oblique muscle is placed line
4 mm behind the insertion line of inferior • +2 overaction: 3 to 4 mm posterior to IR
rectus muscle. insertion
• For 10 mm recession, the anterior suture is • +1 overaction: 4 mm posterior and 1 mm lateral
placed 6 mm posterior to the lateral border of to IR insertion.
the inferior rectus muscle insertion and 4 mm It is important to note that full anteriorization
temporal to the lateral border of the inferior of the IO can be performed with a ‘J’ deformity
rectus (approximately at the vortex vein), in of the new insertion, which can limit ocular
this there occurs anteriorization of IO by elevation and produce a postoperative
1–1.5 mm, that is why based on long-term hypotropia worse in upgaze. The ‘J’ deformity
follow ups it is said to be more powerful than is created when the posterior fibres of IO are
12–14 mm recession. anteriorized parallel or in front of inferior rectus
A recurrence rate to the tune of 15% is reported insertion. This is seen mainly with U/L anterior
with simple recession. The major advantage of the transposition, so should be reserved for patients
recession is that it allows the weakening with appreciable hypertropia in upgaze or
procedure to be titrated according to the severity patients with superior oblique paresis with
of inferior oblique overaction as below: hypertropia >25 PD.
• For 1+ or 2+ overaction, the inferior oblique An anterior and nasal transposition procedure
muscle is recessed by 12–14 mm. converts inferior oblique from an extorter to
• For 3+ overaction, inferior oblique muscle is intorter and from an elevator to depressor,
recessed by 10 mm. significantly improving large ‘V’ patterns.
• For 4+ overaction, 10 mm recession or full Note. For surgical techniques of IO recession,
anterior transposition of IO is preferred. see page 431.
5. Recession with anterior transposition is being
considered more effective than simple recession. Some observations about inferior oblique weakening
Therefore, it is recommended that this 1. Bilateral asymmetry. If there is no superior
procedure may be preferable for large over- oblique muscle paresis but there is a marked
actions, particulary those associated with V- asymmetry of the overactions of the inferior
pattern due to primary inferior oblique oblique muscles, unilateral surgery on the
overaction. In this procedure, after detaching muscle with the most marked overaction will
 Vertical Strabismus and Cyclodeviations 261

often be followed by a significant degree of 5. Associated dissociated vertical deviation.

overaction in the unoperated eye. Therefore, When DVD is associated with inferior oblique
bilateral inferior oblique weakening procedures overaction, the procedure inferior oblique
are indicated for bilateral overaction, even if recession with anterior transposition should be
asymmetrical. However, inferior oblique done. Since in most cases, this procedure alone
muscles that are not overacting should not have can correct DVD, so any other surgical treatment
a surgical weakening procedure. Further, for DVD should be deferred until later.
recession of the inferior oblique can be graded 6. Effect on head tilt test and excyclodeviation.
depending upon the asymmetry. Weakening of inferior oblique in primary
2. Graded response. The response to any surgical overaction does not produce a positive head-tilt
weakening procedure on the inferior oblique is test or any effect on subjective cyclodeviation.
graded one, i.e. the larger overactions will show However, the objective excyclodeviation (as
a greater response to the same amount of surgery. evidenced by indirect ophthalmoscopy) is either
3. Effect on associated horizontal deviation. fully corrected or greatly reduced.
When PIOO is associated with the V-pattern 7. Recurrence of overaction is a common compli-
horizontal deviation, weakening of the one or cation after weakening procedure. Its likelihood
even both inferior oblique muscles has little to with a particular procedure in order of
no effect on the horizontal alignment in the increasing frequency is as follows: Extirpation
primary position. Therefore, there is no need to (least common), recession with anterior
make any adjustment in the amount of transposition, simple recession (15%), temporally
horizontal rectus surgery. Further, the performed myectomy, disinsertion, and nasally
weakening of the inferior oblique should be performed myectomy (33%).
performed along with the horizontal muscle
surgery and that there is no need to do two SUPERIOR OBLIQUE OVERACTION
separate procedures. It has been observed that Superior oblique overaction, also referred to as
unilateral weakening of overacting inferior strabismus deorsoadductorius, is now termed as over-
oblique in V-pattern strabismus can be expected depression in adduction (ODA). It is characterized
to cause 5–10 prism dioptres and bilateral by a downshoot of the eye in adduction.
weakening 15–25 prism dioptres of eso shift in
upgaze (decrease of an exodeviation or increase Etiology
of an esodeviation) but almost little or no effect 1. Primary overaction of the superior oblique
in primary gaze and downgaze. muscle is of unknown etiology. Perhaps it may
4. Effect on associated vertical deviation. In be due to mechanical or innervational causes or
cases with overaction of inferior oblique, a combination of the two. It may occur as an
secondary to paresis of ipsilateral superior isolated phenomenon or in association with
oblique muscle, a weakening of that inferior exotropia or esotropia—often of A-pattern.
oblique muscle could be expected to correct up 2. Secondary overaction of the superior oblique
to 15 prism dioptres of vertical deviation in muscle is caused by a paralysis or paresis of
primary position. The amount of vertical either its antagonist muscle (ipsilateral inferior
correction is roughly proportional to the degree oblique muscle) or its yoke muscle (contralateral
of preoperative overaction. If the deviation is inferior rectus muscle). Rarely, it can also occur
more than 15 PD, then additionally, the yoke in cases with contracture of the contralateral
contralateral inferior rectus should also be superior rectus muscle, occasionally seen in
recessed (1 mm for every 3 PD deviation). conjunction with long-standing paralysis of the
However, in cases with primary inferior contralateral superior oblique muscle. Other
oblique overaction neither unilateral nor causes of secondary overaction of the superior
bilateral weakening of this muscle has any oblique, unrelated to paralysis are, Brown's
significant effect on the vertical deviation in syndrome and Duane's syndrome with co-
primary position. contraction of the horizontal rectus muscles.
 262 Theory and Practice of Squint and Orthoptics

Clinical features
It is important to note that unlike superior
oblique muscle, isolated palsy of inferior oblique
is not much known and so is the secondary
overaction of the superior oblique muscle. So
much so that all bilateral superior oblique
muscle overaction can be considered 'primary'.
Anyhow, clinical features of primary superior
oblique overaction (PSOO) versus secondary
superior oblique overaction (SSOO) are as
follows:
1. Age of onset. PSOO usually occurs by the age
of 2–3 years while SSOO can occur at any age.
SSOO occurs either spontaneously or few weeks
to months following paresis of the ipsilateral
inferior oblique muscle or contralateral inferior
rectus muscle.
2. Bilaterality. PSOO is frequently bilateral Fig. 11.3 A patient with +4 overaction of superior oblique
although it can be asymmetric or rarely muscles. A, central downgaze; B, dextrodepression and;
unilateral; while SSOO is occasionally bilateral. C, levodepression.
3. Downshoot of the eye in adduction is the
clinical characteristic of primary as well as other. However, reversing hypotropias in side
secondary superior oblique overaction. That is gaze are typical, with a right hypotropia in left
with the eyes in lateral gaze and the abducting gaze and a left hypotropia in right gaze.
eye fixing the adducted eye downshoots or an 6. Head tilt in the direction of action of paralysed
exaggerated rotation of the eye occurs in the field inferior oblique is typically present in patients
of action of the superior oblique, i.e. the eye is with SSOO, when the onset is after 6 years of
over-depressed in adduction. Where the eyes are age (after visual maturity); while it is usually
in lateral gaze and the adducting eye is made to absent in patients with PSOO. Head tilting will
fix, the abducted eye will be elevated and not cause any difference in the amount of
manifest as hypertropia on alternate cover test. deviation in primary SOOA.
Clinically, overaction of superior oblique can be 7. Associated incyclodeviations. Objective
graded on a scale of +1 to +4. In a +4 overaction, incyclodeviation, as evidenced by a disturbed
the cornea is directed straight down rather than relationship of the optic disc with foveola, can
down and in (Fig. 11.3). be demonstrated in both patients with primary
4. Associated horizontal deviation in primary as well as secondary superior oblique over-
position. PSOO is commonly associated with action. However, subjective incyclodeviation (as
either comitant exotropia (more common) or demostrated by double Maddox rod test, Hess
esotropia, usually of A-pattern (<10 PD in screen test, the major amblyoscope or the
unilateral and >20 PD in bilateral SOOA). By Lancaster red-green test) is typically present in
contrast, SSOO is usually not associated with patients with SSOO, when the onset is after
any form of concomitant deviation. 6 years of age, but is absent in patients with
5. Associated vertical deviation in primary PSOO.
position is usually absent in PSOO. While 8. Forced duction test is usually positive in both
vertical deviation (hypotropia) is characteristic primary as well as secondary superior oblique
of SSOO. Bilateral SOOA is associated with a overaction. An 'edge' of resistance is felt as the
small or no hypotropia in primary position, since eye is passively rotated with forceps up and in
the bilateral vertical deviations cancel each (into the field of antagonist inferior oblique).
 Vertical Strabismus and Cyclodeviations 263

Differential diagnosis surgery can correct moderate A-pattern up to

1. Brown syndrome can be differentiated from 20 PD with SOOA.
primary SOOA, on a clinical basis as below: 3. Superior oblique lengthening by insertion of
• An ability to elevate the paretic eye in a silicone expander or a non-absorbable suture
adduction effectively rules out Brown is also an effective procedure. In fact several
syndrome. surgeons have proposed more controlled
• In contrast to SOOA where hypotropia is weakening by this procedure (rather than
maximum in downgaze, Brown syndrome is tenotomy) in patients with bifixation and normal
associated with a restriction of elevation thus stereopsis to eliminate the complications like
causing a hypotropia that is greatest in torsional diplopia or symptoms of secondary
upgaze. superior oblique palsy with head tilt observed
• ‘A’ pattern in SOOA, while there is‘V’ pattern with simple tenotomy.
in Brown syndrome. FDT +ve in Brown The weakening effect is graded by varying the
syndrome. length of the silicone that bridges the gap
2. Inferior oblique paresis. There is ipsilateral between the cut ends of the tendons. The spacer
SSOOA in which unlike PSOOA, there is lengthens the tendon without altering the
positive head tilt test, hypotropia is greatest muscle mechanics at its insertion . This corrects
when patient looks up and in. The deviation ‘A’ pattern from 20 to 55 PD and also the SOOA
worses in the gaze away from paretic eye. In IO associated with skew deviation.
palsy, the deviation worses with tilt toward the Recommended length of spacer according to the
paretic eye because intorsion is impaired. magnitude of SOOA is as below.
• +1 overaction: 4 mm
Treatment
• +2 overaction: 5 mm
In a patient with clinically significant ocular • +3 overaction: 6 mm
deviation or A-pattern associated with bilateral
• +4 overaction: 7 mm
superior oblique overaction, a bilateral superior
oblique weakening procedure is indicated, Note. For surgical techniques of SO weakening,
which can be accomplished as follows: see page 433.
1. Superior oblique tenotomy and tenectomy: It
is indicated for treating‘A’ pattern strabismus Some observations about
superior oblique weakening
and can correct 20 PD or more of A-pattern in
the primary position and up to 45 PD in 1. Graded response. The response to superior
downgaze. The amount of correction achieved oblique tenotomy is graded one, i.e. the larger
depends mostly on the size of preoperative overaction will show a greater response to the
pattern and the amount of SOOA. This can be same amount of surgery.
done either nasally or temporally to the superior 2. Effect on associated horizontal deviation. It
rectus muscle. Effectivity of superior oblique has been observed by several surgeons that
tenotomy increases, if performed closer to the superior oblique tenotomy decreases the
trochlea; therefore, nasally performed operation exotropia and increases the esotropia as shown
gives more effect. This usually causes post- in Table 11.1.
operative SO palsy and tortional diplopia in Since superior oblique tenotomy also influ-
patients with fusion. ences the deviation in primary gaze; therefore,
2. Posterior tenectomy of superior oblique by experience, the surgeons must make
(PTSO): The anterior fibres of SO tendon are adjustment for horizontal rectus muscle surgery
responsible for torsion, whereas the posterior when being contemplated simulta-neously.
fibres are implicated in downward rotation and Alternatively, the tenotomies may be performed
abduction. This procedure is preferred over first and the horizontal muscle surgery can be
tenotomy in patients with bifoveal fusion taken later on. Of course, this will require two
because negligible extorsion is induced. This general anaesthesias.
 264 Theory and Practice of Squint and Orthoptics

Table 11.1 Effect of superior oblique tenotomy on the The term dissociated vertical deviation is
associated horizontal deviation preferred because in it the two eyes are more
Procedure Decrease in exotropia or increase independent of each other (dissociated
in esotropia in prism dioptres movements) and do not follow the Hering's law
Upgaze Primary Down- of ocular motility, i.e. in contrast to a true vertical
gaze gaze deviation, in DVD, the fellow eye does not
Unilateral None 8–10 20–25 exhibit refixation movement in the opposite
tenotomy direction.
Bilateral tenotomy None 15 up to 70 Since the upward drifting of the non-fixing
eye is often associated with lateral deviation and
3. Effect on associated vertical deviation. excyclotorsion, the term ‘Dissociated strabismus
Secondary superior oblique overaction is almost Complex (DSC)’, has been suggested to denote
always associated with vertical deviation in all the components, i.e. dissociated vertical
primary gaze and in its field of action. Superior deviation (DVD), dissociated horizontal
oblique tenotomy is expected to correct almost deviation (DHD) and dissociated torsional
full vertical deviation in the field of action of deviation (DTD).
the muscle and of up to 15 PD in the primary
gaze. ETIOLOGY
4. Tortional diplopia, a positive head-tilt test Exact etiology of the DVD is not known.
and abnormal head posture have been reported Numerous theories have been put forward to
following bilateral superior oblique tenotomies explain the occurrence of DVD. A few such
performed for secondary superior oblique theories are mentioned below:
overaction in adults with a potential for single 1. Bielschowsky's theory of positive and
binocular vision. However, such problems are negative subcortical vertical divergence centres.
of no concern, if there is no potential for single Bielschowsky theorized that DVD occurs due to
binocular vision. alternating and intermittent excitation of both
subcortical vertical divergence centres.
DISSOCIATED VERTICAL DEVIATIONS However, this theory has not been further
substantiated.
Dissociated vertical deviation (DVD) is a 2. Theory of imbalance of binocular stimulation.
comparatively ill-understood form of strabismus. Spielmann postulated that DVD is caused by an
That is why, it has been described under different imbalance of binocular stimulation. This theory
names by the different workers. A few of the other explains the frequent occurrence of DVD in
names by which this condition has been described essential infantile esotropia and the occasional
are: Alternating sursumduction, alternating occurrence with sensory heterotropia. However,
hyperphoria, alternating hypertropia, alternating this theory does not explain the occurrence of
sursumvergence, occlusion hyperphoria, DVD in patients with otherwise normal
occlusion hypertropia, double hypertropia, binocular functions.
dissociated vertical divergence, dissociated
3. Brodsky theory. According to this theory,
hyperdeviation and so on.
DVD is a vestigial remnant of the dorsal light
Dissociated vertical deviations are basically reflex of lower animals.
characterized by a hyperdeviation in one eye
4. Other theories which have been put forward
that is present while the other eye is fixing. The
include:
non-fixing eye is also extorted and slightly
abducted. Thus, dissociated vertical devation • Theory of bilateral paralysis of the depressor
(DVD) can be defined as an intermittent muscles.
anomaly of the non-fixing eye consisting of • Theory of defective mid-brain stimuli.
upward excursion, excyclotorsion and lateral • Theory of two monocular conjugate mechan-
deviation. isms plus a binocular mechanism.
 Vertical Strabismus and Cyclodeviations 265

• Theory of defective monocular nasal retinal

quadrant stimuli.
• Guyton’s theory.
Thus many theories have been proposed to
explain occurrence of DVD. However, findings
of most of the theories could not be reproduced.
Therefore, the exact etiology is still illusive. The
lack of precise aetiologic information is further
reflected by the plethora of terms which have
been mentioned above to denote this condition.

CLINICAL FEATURES
1. Deviation. DVD is characterized by spont-
aneous occurrence of vertical deviation in either
eye, when the patient is fatigued, or day
dreaming (manifest DVD) or when fusion is
interrupted by artificial means (latent DVD)
(Fig.11.4). DVD is characterised by a slow
upward drifting of the non-fixing eye. The
vertically deviated eye is also extorted and
slightly abducted.
2. Associations of DVD: DVD may also occur
as an isolated phenomenon in patients with
apparently normal binocular function. In
general DVD may be associated with any con-
genital or acquired strabismus with motor fusion
deficiency. Its common associations include:
Infantile esotropia is associated with DVD in
over 75% cases. However, inspite of careful
search, the condition is rarely diagnosed in
infancy. It is usually diagnosed between 2 to 5
years of age. Mostly it becomes evident after the
surgical alignment for horizontal squint has
been done.
Infantile exotropia is also commonly associated
with DVD. It has been described to occur as a
part of a syndrome consisting of an A-pattern
exotropia with overaction of the superior
obliques and underaction of the inferior obliques
(Fig. 11.5).
Excycloduction and latent nystagmus are
frequently associated with DVD.
3. Head posture. Approximately, one-third of
Fig. 11.4 Dissociated vertical deviation. A, no initial
patients with DVD have a spontaneous
deviation; B, left hypertropia with right eye fixing immediately
abnormal head posture. after removal of the cover from the left eye; C, the left eye is
4. Laterality. DVD is frequently bilateral drifting down; D, right hypertropia with left eye fixing
(alternating sursumduction) usually assymetric. immediately after removal of the cover from the right eye;
Rarely it may be monocular. E, right eye is drifting back to its original position.
 266 Theory and Practice of Squint and Orthoptics

(double hyperdeviation). Not only does elevation

takes place, but each eye tends to extort on
occlusion and slowly depress and intort, when
occlusion is removed. This deviation appears to
be related to retinal illumination. It is important
to note that in DVD when the hypertropic eye
fixates, there is no corres-ponding hypotropia of
the fellow eye (i.e. the eyes do not follow Hering's
law of equal innervation). While in hypertropia,
when the hypertropic eye fixates a target, the
other eye moves downwards into a hypotropic
position of equal magnitude.
Symmetric versus asymmetric DVD. Bilateral
DVD may be symmetric or asymmetric. Degree
of asymmetry of dissociated strabismus is
determined by the magnitude of the deviation
in primary position compared between the two
eyes.
5. Comitance. DVD may be comitant or
incomitant. The difference in the deviation of
non-fixing eye between the primary and in all
other gaze positions determines the degree of
incomitance.
Comitant DVD. DVD that measures roughly the
same (within 7 PD) in primary position,
adduction and abduction is called commitant
DVD. This is the most common presentation.
Incomitant DVD refers to measurable disparity
in the magnitude of DVD (>7 PD) between the
primary position, abduction, and adduction.
Incomitant DVD is usually associated with
muscle dysfunction as below:
1. DVD larger in the field of inferior oblique of the
Fig. 11.5 Dissociated vertical deviation with associated non-fixing eye is characterized by following
underaction of both inferior obliques (A and B) and overaction features:
of both superior obliques (C and D). • In primary position, only DVD is seen.
• In adduction, both true hypertropia and DVD
Monocular dissociated hyperdeviation may be are present.
seen in the presence of an intermittent exotropia. • In abduction, DVD is <5 PD.
It is frequently found that when fusion is broken • ‘V’ pattern horizontal deviation may be
and the eye is deviated, the deviated eye observed.
develops a small hyperdeviation. This deviation 2. DVD larger in adducted position only, but DVD
is not associated with underaction of yoke in abduction is >5 PD.
muscles and is not present, when the deviated 3. DVD more in abduction due to associated
eye is not in abduction. It generally disappears SOOA and ‘A’ pattern. As in the adducted
following horizontal surgical correction. position, SOOA causes more depression than the
Bilateral DVD or alternating sursumduction is a elevation occurring due to DVD so DVD
situation in which either eye elevates under cover increases in the abducted position.
 Vertical Strabismus and Cyclodeviations 267

Superior rectus contracture syndrome in DVD. hypertropic DVD, it should be considered a

Cases with marked asymmetric DVD, especially unique form of the dissociated strabismus
those with reduced visual acuity in one eye, can complex. The downward drifts are not
coexist with a superior rectus overaction/ associated with any torsional movements and
contracture syndrome in the non-fixing eye. In no nystagmus is observed.
these cases, DVD is usually markedly decompen-
sated and there occurs almost a permanent DIAGNOSIS
elevated position of the non-fixing eye which 1. Cover-uncover test
leads to shortening and contracture of the
superior rectus. The presence of a co-existing • In patients with unilateral manifest DVD, when
superior rectus contracture syndrome in patients the fixating eye is covered, there will occur a
with marked asymmetric DVD should be downward movement of the uncovered eye
suspected when the following clinical (but there will be no corresponding hypotropia
characteristics are present: of the covered eye).
• Hypertropia in the non-dominant eye in all • In patients with alternate DVD, either eye
upgaze positions that is larger in supra- elevates under cover (double hyperdeviation)
abduction. while the uncovered eye will move down to
• Manifest hypertropia in the non-dominant eye take fixation.
in primary position. • In patients with latent DVD, there occurs no
• Positive Beilschowsky head tilt test towards the movement of the uncovered eye, but the eye
affected side. under cover is elevated and moves down-
• Hypertropia of the normal eye when the non- wards after it is uncovered (i.e. DVD is present
dominant eye is fixing. only when the eyes have been dissociated).
• Larger hypertropia when the non-dominant eye 2. Spielmann’s translucent occluder test is better
is rotated down and out. than the simple cover-uncover test for DVD. In
a patient with latent DVD, through the
Note. Patients with superior rectus contracture
translucent occluder, the eyeball is seen to
syndrome and DVD might need very large
upshift and there occurs characteristic slow
recessions of the superior rectus (up to 14 mm).
drifting recovery on removing the occluder
It can be done in both eyes when symmetric DVD
(Figs 11.6A and B). While in hypertropia
is seen but mostly asymmetric SR recession is
associated with inferior oblique overaction,
required.
there occurs a refixation movement which is a
6. Binocular vision and sensory adaptations fast saccade.
• Suppression usually develops in patients with 3. Head tilt test. Most patients with DVD show
spontaneous DVD and thus eliminates an increase in the size of DVD on contralateral
diplopia. head tilt (e.g. DVD in right eye increases with
• Peripheral fusion is often present in patients with left head tilt). However, a few patients show
DVD having a manifest deviation of 4D or less. converse response.
• An absolute facultative central scotoma in one 4. Red glass (filter) test. Red glass (filter)
eye, while both eyes are being used together dissociates the two eyes and the eye behind the
for peripheral vision, is present even in filter drifts up in a patient with DVD and the
patients having a latent DVD. patient appreciates diplopia with red image
being lower. The fact that a patient will localize
Hypotropic DVD the red image below the fixation light, regardless
Hypotropic DVD is a rare condition. It is mostly of whether the red glass is held before the right
unilateral and commonly associated with or left eye, clearly differentiates a DVD from
monocular visual deficits or high myopia. other forms of cyclovertical deviations in which
Although the nature of this intermittent slow the red image is localized below or above the
downward ocular deviation is similar to that of fixation light, depending on which eye fixates.
 268 Theory and Practice of Squint and Orthoptics

5. Demonstration of Bielschowsky phenomenon. with base-down are placed in front of the

In this test, one eye is covered with Spielmann deviating eye under the occluder and patient is
translucent occluder (and is elevated undercover) asked to focus on the fixation target at 6 m
while the other eye fixates (Fig. 11.6A). Then a distance. The occluder is then quickly shifted to
photometric graded density neutral filter bar is the fixating eye allowing the previously
placed before the fixing eye. In patients with DVD, dissociated and elevated eye to take up fixation.
as the filter with gradually increasing density is The alternate cover test is continued and base-
moved in front of the fixing eye, the elevated eye down prism is added until no downward
under cover will start moving downwards movement of the deviating eye is observed on
(Fig. 11.6C) and may go even below the primary switching occlusion. In the case of bilateral DVD,
position. When the filter bar is moved from
each eye is tested separately.
positions of greater to lesser density the eye behind
the cover will again elevate (Fig. 11.6D). Since Grading of DVD, based on the degree, is as
Bielschowsky was first to note this intriguing below:
observation, hence it is known by his name. • 1+ DVD: A small deviation (up to 9 PD)
6. Measurement of DVD
i. Prism base-down under the occluder test can be • 2+ DVD: A moderate deviation (10 to 19 PD)
useful in measuring DVD. In this test, prisms • 3+ DVD: A large deviation ( >20 PD)

Fig. 11.6 Detection of DVD using Spielmann's translucent occluder (A and B) and dimostration of Bielschowsky
phenomenon (A, C and D).
 Vertical Strabismus and Cyclodeviations 269

For a combined dissociated and a non-dissociated Table.11.2 Dissociated vertical deviation versus inferior
vertical deviation, i.e. in patients with DVD and oblique overaction
true vertical deviation, measure the non- Sr. Feature Dissociated Inferior
dissociated component by adding base-up prism no. vertical deviation
oblique over-
over the non-DVD eye until the hypodeviation action
is neutralized during the alternate cover test. The 1. Hyper- Present in Maximum in
deviation primary position adduction,
next step is to measure the vertical deviation as in adduction and never in
previously described for dissociated vertical in abduction abduction
deviation. The actual dissociated vertical 2. Incyclo- Present Absent
deviation measured is the difference between duction on
the two steps. refixation
ii. Modified form of Krimsky test may be used to 3. Speed of up- Slow Rapid
measure DVD in patients who cannot fix with ward movement (2–200°/sec) (200–400°/sec)
in deviating
deviating eye.
eye during
For grading the full deviation on cover test, refixation
the occluder should be turned obliquely so that with non-
the eye remains occluded and at the same time deviating eye
examiner can look behind the occluder to grade 4. Bielschowsky Present Absent
deviation (Fig. 11.7). Alaternatively, a translucent phenomenon
occluder may be used to disrupt fusion while at 5. V-pattern Absent Present
the same time observing DVD undercover. 6. Superior May Usually
iii. An approximate grading of DVD may be oblique overact underaction
action
done on cover test as follows:
7. Pseudoparesis Absent Present
• 1 + DVD: A small deviation
of contra-
• 2 + DVD: A moderate deviation lateral
• 3 + DVD: A large deviation superior
rectus
DIFFERENTIAL DIAGNOSIS 8. Latent Often present Absent
DVD must be differentiated from inferior nystagmus
oblique overaction as shown in Table 11.2. 9. External Absent Present
rotation of
fundus on
indirect
ophthalmo-
scopy (shown
by an altered
relation
between fove-
ola and
optic disc)

TREATMENT
A. Non-surgical treatment is not so effective in
DVD. However, following measures may be
useful:
I. Conservative therapy to strengthen the
fusional mechanisms should be provided as
below:
• Optimal spectacle correction should be
Fig. 11.7 Technique of grading dissociated vertical deviation prescribed. As the blurred vision may cause a
on cover test using occluder in tilted positon. latent DVD to become a manifest more
 270 Theory and Practice of Squint and Orthoptics

frequently providing a clear images to both as several years after an initial satisfactory result
eyes encourages fusion are reported by many workers. Botulinum toxin
• Correcting an associated horizontal deviation A injection into SR has been suggested as an
either surgically or with prisms, promotes the alternative.
possibility of peripheral fusion. 2. Large recession of superior rectus muscle.
• Treating amblyopia by occlusion therapy also Unconventionally, large recession (7 to 10 mm)
promotes peripheral fusion of superior rectus muscle has been suggested
• Treating heterophorias by orthoptics also as effective treatment for DVD. Bilateral
promotes peripheral fusion. recession is indicated in bilateral cases of DVD.
II. Conservative therapy in the form of changing The asymmetric amount of surgery may be
the fixation pattern by patching or optical performed in the two eyes, when the deviation
means may be useful, especially in patients with is asymmetric. Graded recessions, recommended
asymmetric involvement or those accustomed based on the degree of DVD, are as below:
to wearing glasses. For example, if a patient is • Grade I (up to 9 PD): 5–7 mm
having significant DVD of left eye while fixing • Grade II (10–19 PD): 7–9 mm
with right eye and an insignificant DVD of right • Grade III (>20 PD): 9–14 mm.
eye while fixing with left eye; a slight optical 3. Resection of the inferior rectus muscle has also
blurr induced in the right eye by addition of been suggested by some workers. Preferably,
+2.0D lens will shift the fixation preference to this procedure should be performed, if
the left and DVD may no longer be a cosmetic recurrences occur even after the large recession
problem. of superior rectus muscle. Recommended
B. Surgical treatment is indicated, when DVD amount of resection of the inferior rectus muscle
causes a significant cosmetic problem, i.e.: for DVD is 4 mm for small deviation, 6 mm for
• When large DVD manifests frequently or intermediate deviation and 8 mm for large
• Anomalous head posture in present to control angles. Resection of inferior rectus muscle may
the DVD. also elevate the lower eyelid.
However, before contemplating surgery, it 4. Recess-resect procedure. A 4–5 mm recession
is important to differentiate DVD from over- of superior rectus muscle and 6 mm resection
action of the inferior oblique muscle (Table 11.2), of inferior rectus muscle have also been
since the surgical approach to these two recommended for treating large angle DVD.
conditions is different. However, this procedure should be limited to
those relatively infrequent patients who have a
Note. Further, it should also be borne in mind
that even the surgical treatment is also predominantly monocular vertical deviation
palliative, since currently no such surgical with a hypertropia in primary position.
procedure is available that will completely cure II. Surgical procedures for incomitant DVD
this disorder. 1. When DVD is larger in the field of inferior
Following surgical procedures have been tried oblique of the non-fixing eye, i.e in adducted
by different workers: position only, then the inferior oblique
I. Surgical procedures for comitant DVD weakening procedure, like IOAT is preferred.
1. Faden operation with superior rectus As suggested by Scott, anterior transposition of
recession. In this procedure, a 3–5 mm recession the inferior oblique muscle has been an effective
of the superior rectus muscle is combined with treatment for DVD with IOOA. It is
anchoring this muscle to the globe with a non- hypothesized that the IOAT procedure creates
absorbable suture 12–15 mm posterior to its a vector for depression, and this antielevating
insertion (posterior fixation suture, or force helps in controlling DVD. This procedure
retroequatorial myopexy, or Faden operation). can create a limitation of elevation of the
This procedure is more effective than SR operated eye and may cause hypotropia in
recession alone, but recurrence occurring as late primary position when performed unilaterally.
 Vertical Strabismus and Cyclodeviations 271

It can also cause a restriction of elevation in the contralateral eye with this test can be
abduction associated with Y or V pattern when ignored.
performed bilaterally, particularly if the • DHD may be distinguished from other horizontal
posterolateral fibres of the inferior oblique strabismus by lack of a corresponding
muscle are spread out laterally at the time of exodeviation of the contralateral eye on
resuturing to the sclera. This complication was alternate cover testing.
first called as ‘antielevation syndrome’ (AES) by
Kushner. IO transpostition more than 1 mm Treatment
anterior to the inferior rectus muscle insertion DHD, depending upon the amount, is treated
as well as lateral spreading of the posterior fibres with LR recession of 3–8 mm on the affected side.
>2 mm at new insertion point are the risk factors
to develop AES.
CYCLODEVIATIONS
2. When DVD is larger in adducted position but
significant in abduction (>5 PD) also. In these Cyclodeviation (torsional strabismus) refers to
patients, both SR recession and IO weakening a misalignment of the eyes around the antero-
procedures are preferred. posterior axes.
3. When DVD is more in abduction due to
associated SOOA and ‘A’ pattern, following CLASSIFICATION
procedures are required: A. Depending upon the constancy of deviation
• Bilateral SR recession should be considered in 1. Cyclophoria
patients with small degree of A pattern (up to 2. Cyclotropia
12 PD). Theoretically, the cyclodeviations have been
• Additional SO weakening procedure like classified into cyclophoria (latent cyclodeviation)
posterior SO tenectomy is required with and cyclotropia (manifest cyclodeviation);
bilateral SR recession in patients with larger however, practically in most cases, no
amounts of A-pattern (12–25 PD). distinction can be made between a cyclophoria
and a cyclotropia by means of the cover tests.
DISSOCIATED HORIZONTAL DEVIATION Therefore, practically the cyclodeviations cannot
Dissociated horizontal deviation (DHD) is be subdivided into latent and manifest forms.
defined as a change in the horizontal ocular B. Depending upon character of deviation
alignment, unrelated to the accommodation, that
is brought about solely by a change in the 1. Excyclophoria and excyclotropia. The devia-
balance of visual input from the two eyes. ted eye is extorted, i.e. the eye is rotated around
its anteroposterior axes in such a way that the
Characteristic features superior portion of the vertical meridian
(12 O'clock meridian) is torted temporally and
• DHD usually manifests as spontaneous
the inferior portion of the vertical meridian
unilateral exodeviation or an exodeviation of
(6 O'clock meridian) is torted nasally.
greater magnitude in one eye during prism
and alternate cover testing. 2. Incyclophoria and incyclotropia. The
• Unlike in other forms of intermittent deviated eye is intorted, i.e. the eye is rotated
exotropia, the observed exodeviation is slow, around its anteroposterior axes in such a way
variable and asymmetrical in the two eyes. that the superior portion of the vertical meridian
(12 O'clock meridian) is torted nasally and the
Assessment inferior portion of the vertical meridian
• To assess the horizontal dissociated deviation (6 O'clock meridian) is torted temporally.
(DHD), the same procedure as described for
DVD is performed with base-in prism over the ETIOLOGY
eye with DHD until no further inward In general, cyclodeviations result from an
movement of that eye is seen. Movement of imbalance in the relationship between intorters
 272 Theory and Practice of Squint and Orthoptics

(superior oblique and superior rectus) and • Physiologic adaptation

extorters (inferior oblique and inferior rectus) • Psychologic adaptation
of the eyeball under following situations: 1. Cyclofusion. It has been reported that cyclo-
1. Paresis or paralysis of any cyclovertical disparity caused by cyclodeviations is mostly
muscle, particularly oblique muscles (having compensated by sensory cyclofusion which is
larger torsional effect than vertical recti) is very well developed in humans. Normal
commonly associated with cyclodeviation. individuals can fuse up to 10°–15° of cyclodisparity
Cyclodeviation in such conditions is usually in either direction, by vertical and cyclofusional
associated with a vertical deviation, but may also movements. But only a few degrees of this
occur alone. disparity are offset by motor fusion.
Bilateral excyclotropia secondary to closed head The effectivity of sensory cyclofusion is well
injury occurs due to bilateral superior oblique illustrated by comparing the testing of
paralysis resulting from rupture of the anterior cyclodeviation by two different methods as below:
medullary velum, which is that area in the
When tested with Double Maddox rod test, a
midbrain where the superior oblique fibres from
patient shows cyclotropia. Same patients with
both superior oblique nuclei decussate.
well-developed cyclofusion, when tested with
2. Dissociated vertical deviations are frequently Bagolini lenses (after correcting the co-existing
associated with cyclodeviations. vertical and horizontal deviations, if any, with
3. A- and V-pattern of horizontal strabismus are prisms), show that Maddox rod test which
also associated with cyclodeviations without an disrupts fusion reveals the exact amount of
obvious paretic component. cyclotropia that can be measured exactly
4. Horizontal deviations and simple hyper- whereas the Bagolini lenses which do not
phorias may also be associated with cyclodevi- disrupt fusion, test one's ability of cyclofusion.
ations, though rarely.
5. Surgery on vertical recti or oblique muscles 2. Suppression and anomalous retinal corres-
may sometimes be complicated by cyclo- pondence. Some patients do not experience
deviations. torsional diplopia due to development of
6. Uncorrected oblique astigmatism may also be suppression or anomalous retinal corres-
associated with cyclodeviation. pondence.
7. Other conditions which have been reported Note. It has been observed that even after
to be associated with cyclodeviations include— occlusion of the non-paralysed eye, the patient
endocrine ophthalmopathy, myasthenia gravis does not see the visual environment tilted
and plagiocephaly. inspite of the fact that the affected eye is rotated
around the anteroposterior axes. The cyclofusion
CLINICAL CHARACTERISTICS and suppression or ARC also cannot explain this
Theoretically, one would expect that patients finding. Such a unique happening has been
with cyclodeviations should have symptoms explained by the occurrence of physiological and
such as torsional diplopia, dizziness and psychological adaptation in patients with cyclo-
difficulties in negotiating stair way, steps and tropia. These phenomena are described below.
streetcurves. However, in practice, most of the 3. Physiologic adaptation. It has been reported
patients are asymptomatic, with the exception that to compensate for the image tilt in some
of paretic conditions of very recent onset, patients with congenital or early acquired
particularly traumatic unilateral or bilateral cyclodeviation, there occurs a physiologic
superior oblique paralysis. The reasons (factors) adaptation in the form of spatial reorientation
which have been put forward for most of the of the horizontal and vertical retinal meridians
patients with cyclodeviations being asymp- (Fig. 11.8). Perhaps such a physiologic adaptation
tomatic are: has a neurophysiologic basis in the form of a
• Cyclofusion change in orientation tuning of the striate
• Suppression and ARC cortical neurons. Further, it has also been
 Vertical Strabismus and Cyclodeviations 273

DIAGNOSTIC TESTS
The deviated eye does not realign itself on
covering the fixating eye, its position remains
unchanged under monocular and binocular
conditions. Therefore, cyclodeviations cannot be
detected by routine objective examination.
Therefore, tests for their diagnosis are specially
done under following circumstances:
• When a patient complains of tilting of images
(which is not very often).
• When the examiner notices a torsional
movement of the eye on alternate cover test
(only a very experienced and observent
examiner can notice it).
• When there is a palsy of a vertical muscle,
especially one of the two obliques. Associated
vertical deviation is also noted.
• When it is detected while performing diplopia
fields with Franceschetti method.
Diagnostic tests for cyclodeviations can be
divided into subjective and objective tests.

Subjective diagnostic tests

The subjective tests can only be used for cyclo-
deviations beginning after visual maturation.
Fig. 11.8 Physiologic adaptation in the form of spatial Subjective tests include the following:
reorientation of the horizontal and vertical retinal meridia in • Maddox double rod test
left eye with excyclodeviation. Note, image of a cross is no
longer formed on the normal vertical (V1–V2) and horizontal
• Maddox double prism test
(H1–H2) retinal meridia; but on the new vertical (Va1–Va2) • Moddox wing test
and new horizontal (Ha1–Ha2) meridia. • Major amblyoscope test
• Bagolini's striated lenses test
reported that this physiologic adaptation is • Lancaster red-green test
reversible and after surgical treatment of 1. Maddox double rod test . Maddox double rod
cyclotropia, patient may get normal orientation test is a quantitative test to subjectively measure
after a brief period of seeing tilted environment. the cyclodeviation; but it cannot differentiate
4. Psychologic adaptation. A sort of psychologic between cyclophoria and cyclotropia. It can be
adaptation due to empirical spatial clues may performed at distance and near.
also help some patients with cyclotropia to be Procedure. To perform this test, patient is asked
unaware of the tilted environment. The to fixate a spotlight in a dark room. By means of
empirical spatial clues are based on the past a trial frame, a white Maddox rod is placed
experience of seeing the orientation of the before the left eye, and a red Maddox rod before
familiar objects such as doors, windows, trees, the right eye with their axes set at 90°
etc. in the physical space. This fact is supported (Fig. 11.9A). If no vertical deviation is present
by the observation that patients with cyclotropia (which is not very often the case), a 6 D prism is
(diagnosed on Maddox rod test) who do not see placed with its base-down in front of one eye to
the environment tilted may see a faintly assist the patient in observing the two lines
illuminated horizontal line as tilted, when no easily (Fig. 11.9B). The results are interpreted as
other familiar visual clues are available. below:
 274 Theory and Practice of Squint and Orthoptics

Fig. 11.9 Double Maddox rod test (for explanation, see text).

• If the two horizontal lines, one red and other double Maddox rod test, even though it is the
white, observed by the patient are parallel, no normal eye.
cyclodeviation is present (Fig. 11.9C). Drawback of the double Maddox rod test
• If the red line is tilted outward(Fig. 11.9D),
Double Maddox rod test is of value in
incyclodeviation is present and if the red line
substantiating and measuring cyclotropia since
is tilted inward (Fig.11.9E), excylodeviation is
it disrupts the fusion. But, as it does not permit
present in the right eye. The examiner than
cyclofusion so it may be clinically insignificant
rotates the red Maddox rod inward or outward,
under casual viewing which allows cyclofusion.
respectively until the red and white lines are
The small frame size of the maddox rods,
parallel. The amount of deviation is read in
available in most of the refraction trial boxes,
degrees on the trial frame. For example, if the
makes it difficult, if not impossible, to examine
red line which is tilted inward becomes parallel
for cyclodeviations in the peripheral field of
at the 100° position, indicates that patient has a
vision. The cyclophorometer designed by Burian
10° right excyclodeviation.
overcomes this problem but is not commercially
• Similarly, if cyclodeviation is present in left available.
eye the white line will be tilted depending 2. Maddox double prism test. Maddox double
upon the type and degree of cyclodeviation. prism consists of two 4D prisms mounted base
• In the presence of bilateral cyclodeviation, e.g. to base in a frame. It displaces the image in
in bilateral excyclodeviation in a patient with vertically opposite directions. To perform this
bilateral superior oblique palsy following test, patient is asked to fixate on a horizontal
closed head injury both the white and red lines line drawn on a sheet of paper with the eye to
will be tilted inward. be tested (e.g. say right eye) and the double
Note. It is important to note that with this test prism is placed before the left eye. Patient sees
cyclodeviated eye will always be the non-fixing three lines, central with the right eye and upper
eye even when the patient fixes with the paretic and lower with the left eye. The results of this
eye. For example, if a patient with a left superior test are interpreted as below:
oblique palsy fixes with the left eye, the right • No cyclodeviation is present, when all the three
eye will show the excyclodeviation on the lines are parallel.
 Vertical Strabismus and Cyclodeviations 275

• Right incyclodeviation is present, if the central • It gives information about the pattern of
line is tilted outward. cyclodeviation in each direction of gaze, and
• Right excyclodeviation is present, if the central • Measures cyclodeviation simultaneously
line is tilted inward. along with the horizontal as well as vertical
deviations.
Limitations
• This test can be performed only for near. 5. Synaptophore test is also very useful for
detecting and measuring the cyclodeviations.
• Being a qualitative test, it cannot measure the
cyclodeviation and cannot differentiate Objective diagnostic tests
between cyclophoria and cyclotropia.
The objective diagnostic tests are useful for
3. Bagolini's striated lenses test. Bagolini's revealing any cyclodeviation, regardless of the age
striated lenses permit testing for cyclotropia of onset (c.f. subjective tests). These tests include:
under casual viewing conditions where • Indirect ophthalmoscopy and fundus
cyclofusion takes part. To perform this test, photography.
patient is asked to fixate a spotlight in a dark • Monocular visual field charting
room. By means of a trial frame, the Bagolini's
striated lenses are placed before both eyes 1. Ophthalmoscopy and fundus photography
with the axes of striations pointing towards Presence of cyclodeviation can be objectively
the 90° mark. These lenses will produce an evidenced by a disturbed relationship of the
image of streak of light, perpendicular to the optic disc with foveola on indirect ophthal-
axes of striations without obstructing the moscopy and fundus photography. Normally,
surrounding fusible visual details (c.f. the foveola is aligned approximately with the
Maddox rods). The results of this test are junction of the middle and lower third of disc
interpreted as below: (Fig.11.10A). In excyclodeviation, fundus is
rotated externally and the foveola appears to be
• If the patient is able to fuse the two vertical situated below a line extending horizontally
lines that indicate that cyclotropia is fully from just below the lower pole of optic disc
compensated by cyclofusion. (Fig.11.10B). In incyclodeviation, fundus is
• If the patient is unable to fuse the two vertical rotated internally and foveola appears to be
lines, the lenses are turned until fusion occurs situated above a line extending horizontally
and the amount and direction of the from the centre of the optic disc (Fig. 11.10C).
cyclotropia is read on the trial frames. 2. Monocular visual field testing. Evidence of
4. Lancaster red green test. This is the most objective cyclodeviation can also be found on
complete test and has some advantages over monocular visual field charting which will show
other tests: inward rotation of the blind spot in excyclo-
• It measures the torsional amplitude of each deviation and outward rotation of the blind spot
eye in nine different directions of gaze. in incyclodeviation.

Fig. 11.10 Fundus photograph showing relation of optic disc with foveola in a normal person (A), in a patient with
excyclodeviation (B) and, in a patient with incyclodeviation (C).
 276 Theory and Practice of Squint and Orthoptics

Interpretation of results of objective and iii. A patient having bilateral superior oblique
subjective tests for cyclodeviation palsy producing V-pattern esotropia and an
1. Objective tests' results will always be positive excyclodeviation, needs bilateral tucking of the
in the affected (cyclodeviated) eye. superior oblique to fully correct the entill
2. Subjective tests' results are affected by various problem.
factors such as cyclofusion, sensory adaptation,
physiologic adaptation and psychologic 2. Treatment of subjective cyclodeviation
adaptation. In patients with positive objective without associated vertical deviation
tests for cyclodeviation, subjective tests may be Such a proposition is of rare occurrence but often
negative or reverse positive as follows: more difficult to handle; since a conventional
i. Negative subjective tests. Subjective tests which weakening or strengthening procedure on
do not produce dissociation of fusion such as cyclovertical muscle may correct the cyclo-
Bagolini lenses test may be negative in patients deviation but it may produce an unwanted
with positive objective tests for cyclo-deviation. vertical deviation. Therefore, such surgical
It indicates complete compensation of deviation procedures should be performed which will
by the cyclofusion. exclusively affect the cyclodeviation. Following
ii. Reverse positive subjective tests. In contrast to that procedures have been described:
observed on objective tests for cyclodeviation; on i. Harada-Ito procedure (anterolateral advance-
the subjective tests, the cyclodeviated eye is ment of superior oblique tendon).
always the non-fixing eye. This is true even when • This procedure, described in Japan, is becoming
the non-fixing eye is normal and the opposite quite popular for correction of monocular
fixing eye has a paretic cyclovertical muscle and aswellas binocular excyclodeviation due to
shows a cyclodeviation on objective tests. This palsy of superior oblique muscle.
has been explained by the assumption that • This procedure is based on the theoretical
perhaps a monocular sensorial adaptation takes assumption that only the anterior part of the
place in the paretic, fixing eye. superior oblique tendon insertion is responsible
TREATMENT
for incyclodeviation of eye.
• This procedure basically consists of anterolateral
Only symptomatic (subjective) cyclodeviation
advancement of the anterior part of the superior
(usually >5°) needs treatment, which is always
oblique tendon. This will result in shift of the
surgical. The choice of surgery is as follows:
line of pull in such a way that now on downgaze
1. Treatment of subjective cyclodeviation intorsion occurs which overcomes the extorsion
associated with vertical deviation without causing any vertical imbalance.
i. When a patient gets hyperdeviation and ii. Nasal transposition of inferior rectus muscle.
excyclodeviation due to unopposed action of the This procedure has been suggested as an
inferior oblique following paralysis of effective alternative for excyclodeviation in
homolateral superior oblique muscle, obviously downgaze, where Harda-Ito procedure is not
the treatment is to weaken the offending inferior possible; for example, in patients with congenital
oblique. This will correct both hyperdeviation absence of superior oblique tendon or in those
and excyclodeviation. where it has already been tenotomized.
ii. When a patient develops vertical deviation iii. Temporal transposition of the superior rectus
and cyclodeviation in the field of action of muscle may be added to nasal transposition of
paretic muscle (say superior oblique) without inferior rectus muscle for correcting the
any overaction of the antagonist (i.e. inferior excyclotropia present in the primary position
oblique); obviously the treatment of choice is (see Fig. 15.18).
tucking of the tendon of paretic muscle (superior iv. Temporal transposition of inferior rectus
oblique). Tucking of superior oblique will along with nasal transposition of the superior
eliminate both hyperdeviation and excyclo- rectus muscle has been found effective for
deviation occurring in its field of action. correcting incyclodeviation.
 Vertical Strabismus and Cyclodeviations 277

v. Other procedures which have been reported 13. Hooten, K, Myers E, Worall, R, and Stark, L:
to correct cyclodeviation without producing Cyclovergence: the motor response to
vertical or horizontal strabismus are: cyclodisparity, Graefes Arch. Ophthalmol.
210:65, 1979.
• Slanting of the insertion of all rectus muscles.
14. Kii, T, Ogasawara, K, Ohba, M, Hotsubo, M,
• Vertical transposition of the horizontal rectus
Sakai, N and Nakagawa, T: The effectiveness of
muscles (see Fig. 15.19).
the Faden operation on the superior rectus
• Transposition of the anterior aspects of the muscle combined with recession of the muscle
inferior and superior oblique tendons. for the treatment of dissociated vertical
deviation, Acta Soc. Opthalmol. Jpn. 98:98, 1994.
BIBLIOGRAPHY 15. Mumma, JV: Surgical procedure for congenital
absence of the superior oblique, Arch.
1. Anderson, JR: Ocular vertical deviations and
Ophthalmol. 92:221, 1974.
nystagmus, London, 1959. British Medical
Association. 16. Noorden, GK von, Brown, DJ, and Parks,M:
2. Bagolini, B, Campos, E, and Chiesi, C: Plagio- Clinical observations in cyclotropia. Presented
cephaly causing superior oblique deficiency and at the American Orthoptic Council - American
ocular torticollis, Arch. Ophthalmol. 100:1093, 1982. Association of Certified Orthoptists symposium
3. Bielschowsky, A: Die einseitigen und at the American Academy of Ophthalmology
gegensinnigen ("dissoziierten") Vertikalbewe- and Otolaryngology, Dallas, September 16, 1973.
gungen der Augen, Graefes Arch. Ophthalmol. 17. Noorden, GK von: Clinical observations in
125:493, 1931. cyclodeviations, Ophthalmology 86:1451, 1979.
4. Bielschowsky, A: Lectures on motor anomalies.
18. Noorden, GK von: Indications of the posterior
Hanover NH, 1956, Dartmouth Publishing Co.
fixation operation in strabismus, Ophthalmology
5. Burke, JP, Scott, WE, and Kutschke, PJ: Anterior
85:512, 1978.
transposition of the inferior oblique muscle for
dissociated vertical deviation. Ophthalmology 19. Noorden, GK von, and Chu, MW: Surgical
100:245, 1993. treatment options in cyclotropia, J Pediatr
6. Duncan, L, and Noorden, GK von: Surgical Opthalmol Strabismus 27:291, 1990.
results in dissociated vertical deviations, J 20. Ogle, KN, and Ellerbrock, VJ: Cyclofusional
Pediatr. Ophthalmol. Strabismus 21:25, 1984. movement, Arch. Ophthalmol 36:700, 1946.
7. Esswein. MB, Noorden, GK von, and Coburn,
21. Oliver, P, and Noorden, GK von: Excyclotropia of
A: Comparison of surgical methods in the
treatment of dissociated vertical deviation, Am the nonparetic eye in unilateral superior oblique
J Ophthalmol. 113:287, 1992. muscle paralysis, Am J Ophthalmol. 93:30, 1982.
8. Fink, WH, Surgery of the vertical muscles of the 22. Ruttum, M, and Nooden, GK von: Adaptation to
eye, ed. 2, Springfield, III., 1962, Charles C tilting of the visual environment in cyclotropia,
Thomas, Publisher, P. 369. Am J Ophthalmol 96:229, 1983.
9. Guyton, DL and Noorden, GKvon: Sensory 23. Spielmann, A: A translucent occluder for
adaptations to cyclodeviations. In Reinecke. studying eye position under unilateral or
RD, editor: Strabismus, New York, 1978, Grune bilateral cover test, Am. Orthopt J 36:65, 1986.
& Stratton, Inc.
10. Harada, M and Ito, Y: Surgical correction of 24. Spielmann, A: Les divergences verticales
cyclotropia, Jpn J Ophthalmol. 8:88, 1964. dissociees: exces de sursumversion lie a la
11. Helveston, EM: Dissociated vertical deviaton: a fixation, Ophthalmologie 1:457, 1987.
clinical and laboratory study, Trans. Am 25. Spielmann, A: The oblique Kestenbaum
Ophthalmol. Soc 78:734, 1980. procedure revisited (sloped recession of the recti).
12. Herzau, V and Joos-Kratsch, E: Objective and In Lenk-Schafer, M, editor: Orthoptic horizons,
subjective evaluation of xyclovergence and Transactions of the Sixth International Orthoptic
cyclofusion, Doc. Ophthalmol. 58:85, 1984. Congress, Harrogate, England, 1987, p. 433.
 12
Incomitant Strabismus

CLASSIFICATION RESTRICTIVE OCULAR MOTILITY DEFECTS

VERTICALLY INCOMITANT HORIZONTAL Restrictive strabismus due to misdirected
HETEROTROPIAS muscle forces
(A-, V-, X-, Y- and -PATTERN Congenital cranial dysinnervation disorders
HETEROTROPIAS) (CCDDs)
• Terminology • CCDDs primarily affecting horizontal ocular motility
• CCDDs primarily affecting vertical ocular motility
• Etiology • CCDDs primarily affecting facial muscles with
• Clinical characteristics associated ocular motility defects
• Treatment Restrictive strabismus due to mechanical
restriction
PARALYTIC SQUINT
Tight extaocular muscles
Etiology
• Brown’s syndrome.
Clinical features • Thyroid ophthalmopathy
Investigations • Orbital blowout fracture
Differential diagnosis
• Strabismus fixus
• Congenital tight inferior rectus muscle
Clinical varieties of ocular palsies Structural adhesions
• Isolated ocular muscle paralysis • Adherence syndrome
• Paralysis of 3rd cranial nerve • Tight lateral rectus syndrome
• External ophthalmoplegia • Contracture of extraocular muscles
• Adhesive syndrome
• Total ophthalmoplegia
• Postoperative scarring
• Internuclear ophthalmoplegia
• Orbital myositis

CLASSIFICATION ii. Paralytic exotropia

• Isolated medial rectus paresis
Incomitant squint is a type of heterotropia • Complete third nerve paralysis
(manifest squint) in which the amount of
• Paralysis of convergence
deviation varies in different directions of gaze.
Further, amount of deviation may also vary iii. Paralytic vertical deviation
depending on which eye is fixing. Incomitant • Single muscle paresis or paralysis
deviations include the following conditions: – Superior oblique paralysis or paresis
1. Vertically incomitant horizontal heterotropias
– Inferior oblique paralysis or paresis.
(A-, V-, X-, Y- and -pattern heterotropias)
– Superior rectus paralysis or paresis.
2. Paralytic strabismus
i. Paralytic esotropia – Inferior rectus paralysis or paresis.
• Lateral rectus paresis or paralysis • Part of complete third nerve paralysis.
• Divergence paralysis • Supranuclear lesions.
278
 Incomitant Strabismus 279

– Double elevator paralysis. A-pattern and V-pattern which are now

– Double depressor paralysis. accepted worldwide. In practice, the term
3. Restrictive ocular motility defects vertically incomitant horizontal heterotropias
A. Restrictive strabismus due to misdirected muscle has become synonymous with "A- and V-
forces. pattern". A few other patterns are also recognized
as follows.
1. Congenital cranial dysinnervation disorders
(CCDDs) A-pattern horizontal heterotropia
2. Congenital ectopic extraocular muscle An A-pattern designates a vertically incomitant
insertion and/or pulley location horizontal heterotropia with increasing conver-
3. Displaced extraocular muscle gence (decreasing divergence) in upgaze and
B. Restrictive strabismus due to mechanical increasing divergence in downgaze. An A-pat-
restrictions tern deviation is considered clinically significant,
1. Tight extaocular muscles when the difference between upgaze and
• Inelastic superior oblique in congenital downgaze is at least 10 prism dioptres or more.
Brown’s syndrome • A-esotropia: Esotropia will increase in
• Thyroid ophthalmopathy upgaze and decrease in downgaze (Fig. 12.1).
• Entraped inferior rectus muscle in blow- • A-exotropia: Exotropia will decrease in
out fracture of orbital floor. upgaze and increase in downgaze (Fig. 12.2).
• Monocular elevation deficiency (MED),
caused by fibrotic IR muscle.
• Strabismus fixus.
2. Structural adhesions
• Fat adherence to extraocular muscles or
sclera after strabismus surgery, retinal
detachment surgery or periocular trauma
• Congenital fibrotic bands
• Acquired Brown’s syndrome due to
scarring/inflammation around, the trochlea
• Conjuctival and Tenon’s capsule scarring
3. Orbital mass lesions
Fig. 12.1 A-pattern esotropia. Note left esotropia in primary
• Orbital tumours causing mass effect on gaze (A) which increases in upgaze (B) and decreases in
the globe movements. downgaze (C).
• Glaucoma explant with large bleb causing
mass effect.

VERTICALLY INCOMITANT HORIZONTAL

HETEROTROPIAS (A-, V-, X-,Y- AND  -
PATTARN HETEROTROPIAS)

TERMINOLOGY
The term vertically incomitant horizontal
heterotropias refers to those horizontal
deviations that change in magnitude with
upgaze and downgaze. Urist introduced this Fig. 12.2 A-pattern exotropia. Note left exotropia in primary
concept to American literature in 1951 and gaze (A) which decreases in upgaze (B) and increases in
Albert suggested the excellent descriptive terms downgaze (C).
 280 Theory and Practice of Squint and Orthoptics

V-pattern horizontal heterotropia

The V-pattern is present, when there is vertically
incomitant horizontal deviation with increasing
convergence (decreasing divergence) in
downgaze and increasing divergence in upgaze.
The V-pattern is considered clinically significant,
only when it measures 15 prism dioptres or more
difference between upgaze and downgaze.
• V-esotropia. The esotropia will increase in
downgaze and decrease in upgaze (Fig. 12.3).
• V-exotropia. The exotropia will increase in
upgaze and decrease in downgaze (Fig. 12.4).

Y-pattern horizontal heterotropia Fig. 12.5 Y-pattern exotropia. Note left exotropia in upgaze
Patients with Y-pattern have exotropia only in (A) and no deviation in primary gaze (B) and downgaze (C).
upgaze (Fig. 12.5).
 (lambda)-pattern horizontal heterotropia X-pattern horizontal heterotropia
These patients have exotropia in downgaze only. These patients essentially have no deviation or
only a small one in primary position, but a
significant exotropia is present in upgaze as well
as in downgaze (Fig. 12.6).
ETIOLOGY
Various theories have been put forward to
explain the occurrence of A- and V-patterns.
However, it has not been possible to explain the
occurrence of such patterns in every case by any
single aetiological factor. Perhaps different
factors might be responsible in different cases.
Each of the following conditions has been firmly
documented as a cause of A- and V-patterns:
Fig. 12.3 V-pattern esotropia. Note left esotropia (A) which
decreases in upgaze (B) and increases in downgaze (C).

Fig. 12.4 V-pattern exotropia: Diagrammatic depiction of left

exotropia (A) which increases in upgaze (B) and decreases in Fig. 12.6: X-pattern exotropia. Note no deviation in primary
downgaze (C). gaze (A) and exotropia in upgaze (B) and downgaze (C).
 Incomitant Strabismus 281

1. Oblique muscle dysfunction. Dysfunction of the 3. Vertical rectus muscle dysfunction. Brown
oblique muscles is the most common clinical (1953) suggested that A-V-patterns may be
finding and surgery on these muscles has been caused by vertical rectus muscle dysfunction.
eminently successfull in the elimination of these However, this concept never gained popularity
patterns. Following observations have been made: due to lack of any supporting evidence. Further,
• Inferior oblique overaction is frequently the horizontal transposition of the vertical recti
associated with V-patterns and surgical proposed to correct A-V-pattern had also not
weakening of this muscle is effective in been found very effective and thus not used
correcting the anomaly in most of such cases. today.
4. Orbital factors. It has been proposed that an
• Superior oblique overaction is often associated
apparent dysfunction of the oblique muscles
with A-patterns and surgical weakening of
unrelated to paresis of any cyclovertical muscle
these muscles is effective in correcting the
might be due to some structural orbital anomalies
anomaly in majority of such patients.
as evidenced by following observations of
Factors blamed for oblique muscle dysfunction are several workers:
as follows: • Patients with Alpert's syndrome or Crauzon's
• Innervational (primary or secondary syndrome frequently show a V-pattern
overaction). exotropia or esotropia with marked elevation
• Desagittalization of the muscle planes, i.e. of the adducting eye, which resemble the
disturbed parallelism of the superior and pattern caused by overacting inferior obliques.
inferior oblique muscles. • Patients with upward or downward slanting
• Anomalous insertion of oblique muscles. palpebral fissures may show A- and V-patterns.
• Ocular or orbital torsions. • Orbital factors might be responsible for
The cause of oblique muscle dysfunction desagittalization of the muscle planes which
seems to be of secondary importance with in turn might be producing A- and V-patterns
regard to the management of these conditions. in some cases.
The main point is to search for the overacting or 5. Pulley abnormalities in the form of heterotopia
underacting oblique muscles. Unfortunately, or laxity of the pulley have also been reported
there are some cases of A- and V-patterns that as a cause of ‘A’ and ‘V’ patterns. Diagnosis of
clearly do not show overaction of the obliques; pulley abnormalities is made on MRI orbital
and an alternative explanation and surgical imaging. In such cases, surgery could be
treatment for these patients is necessary. performed to stabilize or reposition the orbital
pulley.
2. Horizontal rectus muscle dysfunction. Urist
(1958) hypothesized that horizontal recti are CLINICAL CHARACTERISTICS
responsible for A- and V-patterns as follows:
Prevalence
• A-esotropia: Underacting lateral recti. Exact prevalence varies depending upon the
• A-exotropia: Underacting medial recti. criteria used to define the condition, degree of
• V-esotropia: Overacting medial recti. upgaze and downgaze used to test and the ethnic
group tested. However, in general, between 15%
• V-exotropia: Overacting lateral recti.
and 50% of all strabismus cases have been
But, no convincing evidence has been reported to have associated A- or V-pattern in
presented to explain A- and V-patterns different studies. Common patterns found in
exclusively on a dysfunction of the horizontal clinical practice are V-esotropia, V-exotropia, A-
recti. esotropia and A-exotropia.
However, surgically supraplacement and
infraplacement of the medial and lateral recti are Symptoms and signs
effective procedures for A-V-pattern, when not Since fusion may have to be maintained for a long
associated with overaction of the obliques. time in certain positions of gaze, so patients with
 282 Theory and Practice of Squint and Orthoptics

A- and V-patterns are more frequently troubled Clinical work-up

by: A detailed and thorough clinical work-up
• Intermittent transient diplopia, and should be done as in any case of strabismus
• Asthenopic symptoms. (page 100). However, points of special reference
• Abnormal head posture may be assumed to and interest are mentioned here.
get rid of the symptoms. Chin is moved a. Measurement of deviation is essential for
towards the apex of A- or V- in esotropia and diagnosis of A-V-pattern. Important points to
away from the apex in exotropia as follows: be kept in mind are:
– V-esotropia and A-exotropia chin may be • Prism and alternate cover test should be
depressed. employed for measurement.
– V-exotropia and A-esotropia chin may be • Measurements are done in extreme positions
elevated. of gaze, using the proper refractive correction.
• Knapp stressed that false measurement can be
Sensory adaptations and amblyopia best avoided by measuring the deviation at
• Abnormal retinal correspondence may also occur distance fixation.
in patients with A-V-pattern. However, since • von Noorden, after detailed observations, has
the angle of deviation varies in different gazes, recommended that the best way to measure
so will be the angle of anomaly. This is called the deviation is at 33 cm fixation distance, with
covariation of the angle of anomaly. the refractive error fully corrected, and the
• Strabismic amblyopia in A-V-pattern is as eyes in position of 25° elevation and 35°
frequent as in other forms of horizontal depression while fixing on an accommodative
strabismus. However, some patients with Y- target (Fig. 12.7).
and -patterns exotropia possess fusion • After measurement in upward gaze and
potential in primary gaze, and so they will have downward gaze, A-V-pattern should be
less chances of developing strabismic established, only if the difference in deviation
amblyopia. between upward and downward gazes is as
follows:

Fig. 12.7 von Noorden's technique of measuring deviation with eyes in primary position, 25° elevation and 35° depression
while fixing on an accommodative target to demonstrate A- and V-patterns.
 Incomitant Strabismus 283

– For V-pattern, 15 prism dioptres or more, and to personal experience. Alternatively, horizontal
– For A-pattern, 10 prism dioptres or more. rectus muscle surgery may be deferred for a later
date.
b. Version test should be performed to note
5. When both the superior as well as inferior
overactions and underactions of the oblique
obliques are overacting, weakening of any
muscles. This is most important factor for
oblique muscle is contraindicated.
planning the management of A-V-pattern.
6. Vertical transposition or slanting of insertion of
c. Compensatory head posture should also be
the horizontal recti to correct A-V-pattern should
properly noted.
be considered, only if the obliques are not
d. Binocular vision should be tested especially overacting.
in the physiologically important positions of 7. Horizontal transposition of the vertical rectus
gaze, i.e. in the primary and downward muscles is an ineffective procedure and so better
(reading) positions to stress on the clinical avoided.
significance of the vertical incomitance on the
degree of interference with normal binocular
Surgical procedures
function.
I. Weakening of oblique muscles
TREATMENT For technical facts about inferior oblique
weakening, see page 431 and for superior oblique
Indications
weakening, see page 433.
Only clinically significant A-V-pattern needs to
be treated for any of the following indications: II. Transposition of the horizontal rectus muscles
1. To eliminate motor obstacles in order to Indications
maintain, improve or regain comfortable
1. As discussed earlier, vertical transposition or
single binocular vision;
slanting of insertion of the horizontal rectus
2. To improve the cosmetic appearance of the patient; muscle for correction of A-V-pattern is to be
and considered, only if there is no associated
3. To eliminate abnormal head position: Chin overaction of the oblique muscle.
elevation or chin depression.
2. Occasionally, A-V-patterns may not be
completely corrected or recur following
Basic principles
weakening of the overacting oblique muscles,
1. While planning surgery, a special care should especially with inferior oblique recession. Such
be taken for the functionally most important cases should be treated by extirpation of the over-
positions of gaze, i.e. primary and downgaze acting muscle (see page 431). However, if still
(reading). there is residual A-V-pattern, then transposition
2. Overacting oblique muscles should be identified of the horizontal rectus muscles may be tried.
with special attention and when discovered
weakening of these muscles, surgery should be Principles
the first choice. 1. Medial rectus muscles are always moved
3. Inferior oblique weakening has no effect on towards the direction of vertical gaze where the
horizontal deviation in primary position, so convergence is greater, i.e. upward in A-pattern
horizontal muscle surgery should be selected and downwards in V-pattern. In other words,
independently. Further, this procedure tends to towards the apex of A- or V-pattern (Fig. 12.8).
be self-adjusting correcting all the V-patterns 2. Lateral rectus muscles are moved towards the
present without overcorrection. direction of vertical gaze where the divergence
4. Superior oblique weakening may have some is greater, i.e. upward in V-pattern and
influence on the horizontal deviation in primary downwards in A-pattern (Fig. 12.8). In other
position, so surgical procedure for the horizontal words, towards the open or splayed portion of
rectus muscle should be modified also according A or V.
 284 Theory and Practice of Squint and Orthoptics

apex of A or V, the leverage of the lateral recti

will be enhanced and the apical portion will
open. Thus A- or V-pattern will be corrected.

III. Horizontal transposition of the vertical

rectus muscles
Horizontal transposition of the vertical recti has
been proposed as an alternative treatment. The
superior recti are moved temporally and the
inferior rectiare moved nasally for an A-pattern
A B and opposite for V-pattern. While this procedure
is theoretically appealing, it is rarely performed
Fig. 12.8 Direction of displacement of medial and lateral today because of following reasons:
rectus muscles in weakening operations to treat A-pattern
(A) and V-pattern (B) deviations. i. Most surgeons have not found it very
effective.
To remember, a useful mnemonic is MALE: ii. Since most patients with A-V-pattern
Medial recti to the apex and lateral recti to the also require horizontal muscle surgery for
empty space. correction of the deviation in primary
Amount of transposition position; most surgeons prefer to transpose
the horizontal rectus muscles rather than risk
Most of the surgeons prefer to move the
anterior segment ischaemia by operating on
insertion of the medial and lateral recti by half
all four rectus muscles in the same session.
their width or 5 mm. Supraplacement or
infraplacement by 5 mm will produce an However, occasionally when vertical trans-
approximate 15 and 20 prism dioptres change position of the horizontal rectus muscles is
in the A- and V-patterns between upgaze and ineffective in correcting A-V-pattern, it may be
downgaze. worthwhile to try the horizontal transposition
of the vertical rectus muscles.
Mechanics of correction of A-V-pattern
1. When the medial recti are surgically Summary of treatment for the vertically
incomitant horizontal strabismus
transposed towards the apex of an A- or V-
pattern, the arc of contact and therefore, the 1. Treatment of V-pattern esotropia
effective leverage of the medial recti will be i. For correction of horizontal deviation in
reduced, when the eyes are moved in the primary position:
direction of the apex. Hence the apex of A- or • Bilateral medial rectus recession, or
V-pattern will open. Conversely, when the eyes
• Medial rectus recession and lateral rectus
are moved towards the open or splayed portion
resection.
of the A- or V-pattern, the arc of contact of the
ii. For correction of V-pattern:
medial recti will be increased, and the leverage
of these muscles will also be enhanced, and thus a. Inferior oblique (IO) muscle weakening, when
the open portion of the A- or V-pattern will close. overacting is done as below:
Consequently, A- or V-pattern will be corrected. • Mild overaction: 6 mm IO recession
2. When the lateral recti are transposed in the • Moderate overaction: 8 mm IO recession
direction of the open or splayed portion of the • Severe overaction: 10 mm IO recession
A- or V-pattern and if now the eyes are moved • Markedly severe overaction: IO recession
in the same direction, the lateral recti will be combined with anteropositioning (modified
less effective because of decreased leverage due Elliot and Nankin method).
to decrease in the arc of contact; consequently b. Infraplacement of medial recti or infra- placement
the open end of A- or V-pattern will close. of MR and supraplacement of LR; or slanting their
Conversely, if the eyes are moved towards the insertion.
 Incomitant Strabismus 285

2. Treatment of A-pattern esotropia 5. Treatment of Y-pattern exotropia

i. When superior oblique overaction is i. No treatment is required, when the patient
associated, bilateral tenotomy of superior fuses in primary position and downward gaze,
• Oblique muscles should be performed and has a normal head posture, and does not have
either bilateral medial rectus recession or double vision in upward gaze.
recess-resect procedure should be performed ii. When chin elevation is present and there is
for correction of horizontal deviation in the associated overaction of the inferior oblique;
primary gaze after making adjustment for 10– surgical weakening of both inferior oblique
15 prism dioptres which is corrected by muscles will close the Y-pattern.
bilateral superior oblique tenotomy. Alter- iii. If the inferior obliques are not overacting,
natively, horizontal rectus muscle surgery supraplace the lateral recti without recessing
may be deferred for a later date. or infraplace the medial recti without resecting.
• Transational recession of SO (Prieto-Diaz
procedure) may be performed for very severe 6. Treatment of  -pattern exotropia
overaction of SO muscle. Despite fusion in primary position, patients with
ii. When superior oblique overaction is not -pattern exotropia may have considerable
present, the treatment of choice is either bilateral difficulty with near work (i.e. in downgaze). No
medial rectus recession with supraplacement of horizontal surgery is indicated, but following
the insertions; or combined supraplacement of procedures may be performed:
the medial rectus and infraplacement of the i. If the superior obliques are overacting, per-
lateral rectus muscle with a recess-resect form bilateral superior oblique tenotomies.
procedure; or slanting their insertion. ii. If the superior obliques are not overacting,
supraplace the medial recti without resecting
3. Treatment of V-pattern exotropia or infraplace the lateral recti without
i. If inferior oblique muscle overaction is present, recessing.
these muscles should be recessed and either a
bilateral lateral rectus muscle recession or PARALYTIC SQUINT
recess–resect procedure should be performed for
the horizontal deviation in primary position. Paralytic squit refers to ocular deviation
ii. When inferior oblique overaction is not present, resulting from complete or incomplete paralysis
the treatment of choice is either bilateral lateral of one or more extraocular muscles. Complete
rectus muscle recession with supraplacement of paralysis is also called palsy and incomplete
the insertion or combined supraplacement of LR paralysis is called paresis.
and infraplacement of MR with a recess-resect
procedure; or slanting their insertion. ETIOLOGY
In many situations it may not be possible to
4. Treatment of A-pattern exotropia pinpoint the cause. The lesion may be
i. If superior oblique overaction is present, its neurogenic (supranuclear, internuclear, nuclear,
bilateral tenotomies should be performed. And or infranuclear), myogenic or at the level of
keeping in mind the effect of superior oblique neuromuscular junction. The common causative
tenotomies on the horizontal deviation in primary lesions can be grouped as follows:
position, either bilateral lateral rectus recession
or a recess–resect procedure should be planned. I. Neurogenic lesions
ii. When superior oblique overaction is not 1. Congenital lesions. Hypoplasia or absence of
present, the treatment of choice is either bilateral nucleus is a known cause of third and sixth
lateral rectus muscle recession with infraplace- cranial nerves palsy. Birth injuries may mimic
ment of the insertion or combined infra- congenital lesions.
placement of the LR and supraplacement of MR 2. Inflammatory lesions. These may be in the
with a recess-resect procedure. form of encephalitis, meningitis, neurosyphilis
 286 Theory and Practice of Squint and Orthoptics

or peripheral neuritis (commonly viral). Nerve CLINICAL FEATURES

trunks may also be involved in the infectious 1. Diplopia. It is the main symptom of paralytic
lesions of cavernous sinus and orbit. squint. It is more marked towards the action of
3. Neoplastic lesions. These include brain paralysed muscle. It may be crossed (in
tumours involving nuclei, nerve roots or divergent squint) or uncrossed (in convergent
intracranial part of the nerves; and intraorbital squint). It may be horizontal, vertical or oblique
tumours involving peripheral parts of the depending on the muscle paralysed. Diplopia
nerves. occurs due to formation of image on dissimilar
4. Vascular lesions. These are known in patients points of the two retinae (Fig. 12.9).
with hypertension, diabetes mellitus and 2. Confusion. It occurs due to formation of image
atherosclerosis. These may be in the form of of two different objects on the corresponding
haemorrhage, thrombosis, embolism, aneurysms points of two retinae following misalignment of
or vascular occlusions. Cerebrovascular acci- the visual axes of two eyes (Fig. 12.10).
dents are more common in elderly people. 3. Ocular deviation. In paralytic strabismus, the
primary ocular deviation is incomitant and
5. Traumatic lesions. These include head injury
differs from the secondary deviation. However,
and direct or indirect trauma to the nerve trunks.
with the passage of time there occurs spread of
6. Toxic lesions. These include carbon monoxide comitance.
poisoning, effects of diphtheria toxins (rarely),
Primary deviation. It is deviation of the affected
alcoholic and lead neuropathy.
eye, when the unaffected eye is used for fixation
7. Demyelinating lesions. Ocular palsy may and is away from the action of paralysed muscle,
occur in multiple sclerosis and diffuse sclerosis. e.g. if lateral rectus is paralysed, the eye is
convergent.
II. Myogenic lesions
Onset of paralytic ocular deviation may be of
1. Congenital lesions. These include absence, sudden as seen in trauma and vascular
hypoplasia, malinsertion, weakness and occlusions; or gradual as seen in tumours and
musculofacial anomalies of extraocular multiple sclerosis.
muscles.
Incomitance depending upon gaze. The angle of
2. Traumatic lesions. These may be in the form deviation changes with the direction of gaze. It
of laceration, disinsertion, haemorrhage into the
muscle substance or sheath and incarceration of
muscles in fractures of the orbital walls.
3. Inflammatory lesions. Myositis is usually
viral in origin and may occur in influenza,
measles and other viral fevers.
4. Myopathies. These include, thyroid myopathy,
carcinomatous myopathy and that associated
with certain drugs. Chronic progressive external
ophthalmoplegia (CPEO) is a bilateral myopathy
of extraocular muscles, which may be sporadic
or inherited as an autosomal dominant disorder.

III. Neuromuscular junction lesion

It includes myasthenia gravis. The disease is
characterised primarily by fatigue of muscle
groups usually starting with the small extra-
ocular muscles, before involving other large Fig. 12.9 Uncrossed diplopia in a patient with left convergent
muscles. squint.
 Incomitant Strabismus 287

Spread of comitance. A paralytic deviation

undergoes several stages. The first stage is
characterized by weakness of the paralyzed
muscle during which secondary deviation is
much more than the primary deviation as
described above. Almost immediately following
the paresis of an extraocular muscle, the direct
antagonist begins to overact. A contracture of the
antagonist muscle will develop within days to
weeks, if the patient fixes with the unaffected eye.
At this point, the deviation may become greater
in the field of action of antagonist than it is in the
field of action of weak agonist muscle. During
the next stage, the deviation will spread into all
fields of gaze and become increasingly comitant,
i.e. there occurs a gradual spread of comitance to
all fields of gaze. Ultimately, it may then no longer
be possible to determine the nature of original
deviation since the secondary deviation is often
much the same as the primary deviation.
In most cases, spread of comitance occurs
Fig. 12.10 Confusion due to formation of image of two within a few weeks, months or even one to two
different objects on the corresponding points of two retinae. years after the onset of paralysis. However, the
is greatest in the direction in which maximal spread of comitance is not a rule; in some cases
activity is required for the involved muscle, i.e. of paralytic squint, it may not occur at all.
in the diagnostic position of the muscle. For Spread of comitance is quite common in infants
example, in a patient with paresis of right lateral with sixth nerve palsy, and ultimately patient
rectus muscle during levoversion, no or little may present with a comitant esodeviation.
esotropia may be noted in right eye, while in 4. Ocular movements. Restriction of ocular
primary position, right esotropia is marked, movements occurs towards the direction of
which will become maximum in dextroversion, action of the paralysed muscle/muscles. When
i.e. deviation will be maximum in the field of the paralysis is of recent onset, a careful study
action of right lateral rectus muscle. of duction and version movements will make
Secondary deviation. It refers to deviation of the the diagnosis on the basis of incomplete
unaffected eye seen under cover, when the movements in the field of action of the paralysed
patient is made to fix with the affected eye. In a muscle. However, in long-standing cases,
recently acquired ocular palsy, the secondary development of muscle sequelae such as
deviation is much greater than the primary contracture of the direct antagonist muscle and
deviation. This is due to the fact that the strong secondary inhibitional palsy of the contralateral
impulse of innervation required to enable the antagonist muscle, present difficulties in the
eye with paralysed muscle to fix is also identification of the paralysed muscle. Under
transmitted to the yoke muscle of the sound eye these circumstances, other tests like head-tilt test
resulting in a greater amount of deviation. This and Hess screen charting, etc. may be helpful in
is based on Hering's law of equal innervation of diagnosing the paralysed muscle.
yoke muscles. 5. Past pointing. Past pointing also described as
In a long-standing ocular palsy, the secondary false projection or orientation occurs due to
deviation is often much the same as the primary increased innervational impulse conveyed to
deviation because completion of the muscle the paralysed muscle during movement in the
sequelae leads to comitance. direction of action of paralyzed muscle.
 288 Theory and Practice of Squint and Orthoptics

It can be demonstrated by asking the patient direct antagonist is more or less unopposed and
to close the sound eye and then to fix an object thus overact and is responsible for the primary
placed on the side of action of paralysed muscle. deviation. Within a few weeks, the overacting
Patient will locate it further away in the same muscle becomes spastic, contracting more and
direction. For example, a patient with paralysis more leading to a greater angle of deviation.
of right lateral rectus will point more towards Eventually, this leads to a contracture, an
right than the object actually is. organic change in the muscle in which muscle
6. Nausea, vertigo and dizziness. Nausea, vertigo fibres are replaced by fibrous tissue.
and dizziness result from diplopia, confusion iii. Secondary inhibitional palsy of the contra-
and false localization. These symptoms are more lateral antagonist muscle. It is a manifestation
prevalent in vertical and torsional diplopias than of Hering's law of equal innervation. Since the
in horizontal diplopia. They do not occur in direct antagonist of the paretic muscle is more
patients with congenital defects and disappear or less unopposed, so it will require less than
quickly in children. Adults adapt more slowly. normal innervation for a particular extent of a
7. Muscle sequelae. Muscle sequelae refer to movement. According to Hering's law, the same
changes that take place in the extraocular innervation will flow to its yoke muscle (which
muscles after some time of the paralysis or is contralateral antagonist of the paretic muscle).
paresis of one or more of the extraocular Consequently, the contralateral antagonist of the
muscles. The speed and extent to which they paretic muscle will exhibit a weakness; which
develop in different patients vary markedly. The has been called the secondary inhibitional palsy
exact reasons for this are unknown, but the of the contralateral antagonist muscle. Perhaps
speed and degree of their development depend the better term will be 'simulated weakness of the
partly on which eye the patient uses for fixation. yoke's antagonist' or PAY syndrome: pseudo-
Mostly, the patients use sound eye, however, weakness of the antagonist of a yoke. This
sometimes the paretic eye may be used for underaction of the yoke muscle of the antagonist
fixation if: (1) it has better visual acuity, (2) it occurs earlier and is more pronounced, when
is originally dominant eye, or (3) fixation with the paretic eye is used for fixation than when
paretic eye increases the separation of the double the sound eye is preferred for fixation.
picture (due to secondary deviation) which The muscle sequelae developing following
causes less problem. paresis of a particular muscle are shown in
Muscle sequelae occur to much lesser degrees Table 12.1.
in patients with congenital paralysis as Figures 12.11 and 12.12 show the muscle seq-
compared to the acquired paralysis. These occur uelae occurring in a patient with right lateral rectus
more in paralysis due to lesions of the nerves and left superior oblique muscles, respectively.
than the lesions of muscles and include the 8. Abnormal head posture. An abnormal head
following: posture is a common feature of the paralytic
i. Overaction of the contralateral synergistic strabismus. A compensatory head posture does
(yoke) muscle. Overaction of the yoke muscle not necessarily develop in every patient with a
develops quickly, when paretic eye is used for paresis or paralysis of extraocular muscles.
fixation and slowly, when the sound eye is used However, when present, it can aid in making
for fixation. This overaction of the yoke muscle the diagnosis.
is responsible for secondary deviation of the Reasons for abnormal head posture. Abnormal
sound eye. With the passage of time, this head posture may be adapted for any of the
overaction becomes habitual due to the following two reasons:
development of spasm and contracture and i. To achieve binocular single vision. Most
remains, even if the original paresis should frequently, an abnormal head posture is adapted
recover spontaneously. to achieve binocular single vision, i.e. to avoid
ii. Contracture of the direct antagonist. After the troubling diplopia and/or confusion. For
paralysis of a particular extraocular muscle, its this purpose, the head is turned into the field of
 Incomitant Strabismus 289

Table 12.1 Muscle sequelae following paresis of extraocular muscles

Paretic muscle Muscle sequelae
Overaction of Contracture of Secondary inhibitional
contralateral directant antagonist palsy of the contralateral
synergistic (yoke) muscle antagonist muscle
muscle
Right lateral rectus Left medial rectus Right medial rectus Left lateral rectus
Right medial rectus Left lateral rectus Right lateral rectus Left medial rectus
Right superior oblique Left inferior rectus Right inferior oblique Left superior rectus
Right inferior oblique Left superior rectus Right superior oblique Left inferior rectus
Right superior rectus Left inferior oblique Right inferior rectus Left superior oblique
Right inferior rectus Left superior oblique Right superior rectus Left inferior oblique

Fig. 12.11 Muscle sequelae following paralysis of right lateral rectus muscle.

action of the paralysed muscle, and that the eyes the double pictures as far as possible by turning
are directed by the doll's head phenomenon. This the head in the field of paretic muscle. In this field,
process allows the patient to move his/her the deviation of the involved eye will be maximal
limited field of single vision so that it coincides and, thus, the 'true' and the 'false' image of objects
with his/her egocentric (straight ahead) in front will be maximally separated.
position. In other words, the patient can see the Components of abnormal head posture
things in front of him/her as single. A. In horizontal rectus muscle palsy. If one of the
ii. To achieve wide separation of the two images. horizontally acting muscles (lateral or medial
Less frequently, patients with paralytic strabis- rectus) is involved, the abnormal head posture
mus develop abnormal head posture in order will consist of only one component, i.e. face turn
to increase the separation between diplopic towards the action of paretic muscle. For
images. This occurs in patients who have no example, in a paresis of right lateral rectus
useful field of bifoveal single vision but suffer muscle, there will be a face turn to the right side,
from the constant diplopia. Since they have no and in a paresis of right medial rectus muscle,
choice but to live with it, they attempt to separate there will be a face turn to the left side.
 290 Theory and Practice of Squint and Orthoptics

Fig. 12.12 Muscle sequelae following paralysis of left superior oblique muscle.

B. In palsy of cyclovertically acting muscles. The maximal vertical effect in abduction, the face is
superior and inferior recti and the superior and turned so that the involved eye is adducted,
inferior oblique muscles are cyclovertically acting when the patient looks straight ahead, i.e. in
muscles and contraction of any one of them alone paresis of the vertical recti, the face will be
would produce a combination of vertical, turned towards the affected eye. Since the
horizontal and torsional movements. If any one superior and inferior oblique muscles have their
of these muscles is paretic, there will be three greatest vertical effect in adduction, the opposite
components of abnormal head posture as follows: is true for them and the face is turned so that
• Chin elevation or depression the eye with the involved oblique is abducted,
• Face turn i.e. face is turned away from the affected eye
(towards left in paresis of oblique muscle of
• Head tilt
right eye).
1. Chin elevation or depression occurs in
3. Head tilt. The head tilt occurs to compensate
paralysis/paresis of elevators or depressors of
for the torsion or to help relieve the vertical
the eye, respectively. By doll's head pheno-
separation of the double images as follows:
menon, in chin elevation, the eyes move down
and in chin depression, the eyes move up. In • In paresis of a oblique muscle, head tilt occurs
this way, the involved eye is brought out of the to compensate the torsion caused by the direct
field of action of paretic elevator or depressor antagonist of the paralysed muscle. For
muscle. example, in paresis of right superior oblique,
2. Face turn. As mentioned above, face turn in the head will tilt towards left to compensate
the paresis of a horizontally acting muscle for the extorsion caused by right inferior
(medial or lateral rectus) is towards the action oblique muscle.
of the paretic muscle. However, in the paresis • In paresis of a vertical rectus muscle, the head
of one of the cyclovertically acting muscles, the tilt occurs to compensate the torsion caused by
face turn is such that the eyes are brought away the contralateral antagonist of the paralysed
from the field in which the muscle has its muscle. For example, in paresis of right
greatest vertical effect. Thus in the case of superior rectus, the head will tilt to the right to
superior and inferior recti which have their compensate for the extorsion of the left eye
 Incomitant Strabismus 291

caused by overacting left inferior oblique torticollis and because of it, patient's head may
muscle. not straighten with monocular occlusion.
Diagnostic importance of abnormal head Further, if secondary vertebral column changes
posture. The typical head posture adapted may have been allowed to develop, it may no longer
be a pointer towards the paretic muscle especially be possible to correct the abnormal head posture
in a fresh case of isolated muscle palsy. even by surgically aligning the eyes.
Abnormal head postures assumed in paresis Facial asymmetry due to atrophy of the lower
of extraocular muscle of right eye are as shown side of the face is a feature of both long-standing
in Table 12.2. ocular as well as congenital torticollis, so it
cannot be considered a differentiating feature.
As discussed above, it is not difficult to
understand the different abnormal head 9. Sensory adaptations. Sensory adaptations
postures that would theoretically occur as a such as suppression, abnormal retinal corres-
result of the deficient eye movements caused by pondence and amblyopia are, in general, less
paralysis of individual muscle. However, in known with paralytic squint vis-a-vis
practice, conditions are not so clear because of concomitant squint; perhaps, because of the
the following factors: following reasons:
• The paresis may be so slight that a partial • Patients with paralytic squint can assume
compensation is already sufficient and the abnormal head posture to achieve a single
typical head posture may never develop. binocular vision which may prevent the
• More than one muscle may be involved occurrence of suppression, ARC and amblyopia.
leading to a complex picture. • Patients with paralytic squint have variable
angle of deviation in various positions of
• Muscle sequelae and secondary changes
gaze; while sensory adaptations usually
may alter the original condition.
develop in patients who have stable and
Ocular torticollis. The abnormal head posture constant angle of deviation.
adopted by the individuals with congenital and
With the passage of time, the deviation
infantile paralytic squint is sometimes referred
becomes increasingly comitant, as discussed
to as acquired ocular torticollis, because it
under spread of comitance. Under such circum-
resembles an orthopaedic deformity called
stances, the patient is unable to maintain fusion
congenital torticollis. In the later condition, the
in any direction of gaze, and as a result,
head cannot be straightened due to organic
suppression, ARC and amblyopia become
changes in the neck musculature. In contrast, the
established.
ocular torticollis is merely a functional position
Occurrence of comitance in paralytic squint
and the head can be straightened passively. In
is common but not universal. If the strabismus
the early stages, if one eye is occluded, the
remains incomitant and onset is during
patient with ocular torticollis will straighten the
childhood, diplopia in the paretic field
head. However, in late stages, secondary
of fixation may be prevented by regional
scoliosis may occur as a consequence of ocular
suppression.
Table 12.2 Abnormal head posture in extraocular muscle Amblyopia occurs in only those patients of
paralysis paralytic squint who are unable to maintain
Components of abnormal head posture
simultaneous binocular vision in any direction
of gaze and in whom paralysis occurs in early
Muscle paralysed Chin Face Head
turn tilt life. Further, sometimes, presence of amblyopia
Right superior rectus Elevation Right Right may cause confusion in the diagnosis of
Right inferior rectus Depression Right Left paralytic squint. This is because some patients,
Right superior oblique Depression Left Left who prefer to fixate with the paretic eye
Right inferior oblique Elevation Left Right because of certain reasons mentioned earlier,
Right lateral rectus – Right – develop amblyopia in the non-paretic deviated
Right medial rectus – Left – eye.
 292 Theory and Practice of Squint and Orthoptics

INVESTIGATIONS OF INCOMITANT SQUINT Cover test

It should include: (1) evaluation from strabismic It should be carried out for near and distance;
point of view and (2) investigations to find out with and without abnormal head posture. The
the cause of incomintant squint, such as orbital cover test will detect:
ultrasonography, orbital and skull com- 1. Presence of any manifest or latent deviation.
puterized tomographic scanning and detailed 2. Type of deviation
neurological investigations (which are beyond 3. Incomitance—primary versus secondary
the scope of this chapter). deviation
A detailed work-up of a strabismic patient is 4. Normally fixing eye. Patient usually fixes with
described on page 100. However, the salient the nonaffected eye; but this may be influ-
points relevant to the paralytic strabismus are enced by visual acuity or dominant eye.
mentioned here in brief.
Ocular movements
History
Investigation of ocular movements is carried out
A detailed history should be taken with while the patient watches a fixation target, i.e.
reference to following points: moved from the primary position into each of
1. Subjective symptoms the cardinal positions of gaze.
• Diplopia. Enquiry should be made to ascertain: 1. Version movements. The examiner compares
Onset, constant/intermittent, distance at the movement of the two eyes in all positions of
which diplopia is noticed, relative position of gaze. Symmetric movement indicates that no
images, field where greatest separation of defect is present. Unequal movements are seen
images occurs, any change since onset, does in underactions, overactions and limitations.
diplopia disappear, when eye is occluded. 2. Duction movements. Monocular movements
• Confusion. It occurs due to formation of images are of value only in differentiating between a
of the different objects on the corresponding paresis and a total paralysis. Testing for ductions
points of two retina. also helps to detect mechanical limitation of
• Other subjective symptoms which a patient with movements.
paralytic strabismus may experience are: 3. Doll's head movements and command move-
Dificulty in focussing, headache, eye strain, ments testing is of particular use in supranuclear
general asthenopic symptoms and discomfort gaze palsies.
from abnormal head posture. Measurement of deviation
2. Objective symptoms 1. Synoptophore method. Major amblyoscope is
• Constant/intermittent deviation the best instrument for measurement of deviation
in paralytic squint; since measurements are taken
• Abnormal head posture
to compare the size of the deviation in each of
• Ptosis, exophthalmos the cardinal directions of gaze while each eye in
3. Any attributed cause turn is used for fixation. To make the comparison
4. Any previous ocular problems and treatment taken valid, it is extremely important that the fixation
5. General health object be moved an equal distance from the
primary position in each direction. For this,
6. Family history
synoptophore can be adjusted so that the
deviation can be measured while the patient is
Inspection
looking at an equal angle from the primary
1. Ocular posture position in all directions of gaze.
2. Abnormal head posture; note its exact 2. Prism and cover test. It is an easy method,
components while carried out with the help of a prism bar.
3. Facial asymmetry Measurements should be taken with and
4. Ptosis, exophthalmos without abnormal head posture for near and
 Incomitant Strabismus 293

distance fixation, fixing either eye. Measure- of action of the paralysed muscle. Shortly
ments can be made in all the cardinal directions, thereafter, an overaction in the field of the
but for comparison these are not considered very antagonist muscle will be noted. With time, this
accurate. Since it is not possible to measure at overaction will produce a contracture of the
an equal angle from the primary position in all antagonist. Thereafter, there will be spread of
directions; as is possible with the synoptophore. comitance, so that the amount of deviation will
3. Measurement of torsional deviation can be gradually increase and become approximately
made with special slides on major amblyoscope, the same in the all fields of gaze. At this point,
or on adapted Lees screen. based on analysis of duction and version
movements of the eye, the diagnosis of
Note. In a paresis or paralysis of an extraocular cyclovertical palsy becomes impossible. At this
muscle, the deviation will be greatest in the juncture, the Parks' modification over Bielschowsky's
direction of maximal singular action of the head tilt test can be quite useful. There are three
muscle while the affected eye is fixating. steps of this test, each of which eliminates half
of the remaining potential muscles, leaving only
Diplopia test one muscle to be blamed after the three steps.
For details, see page 121. Salient points are as
follows: Procedure of three-step test
• Tested with red/green goggles and a linear Step 1
light, or without dissociation aids. • Perform cover-uncover test in primary
position and determine which eye is hyper-
• Position of maximum vertical and horizontal
tropic. If the patient's presenting sign is a
separation of images and position of maximum
hypodeviation, consider it hyperdeviation of
torsion is noted.
the opposite eye. Step 1 reduces the number
• Distal image belongs to the affected eye. of affected muscles from 8 to 4.
• The results are recorded either by written • A right hypertropia (RHT) implies any of the
description or as diplopia chart. Diplopia following:
charts of paralysis of extraocular muscles of – Weakness of depressors of right eye (RIR,
right eye and left eye are shown in Figs 12.13 RSO), or
and 12.14, respectively. – Weakness of elevators of left eye (LIO, LSR).
• A left hypertropia (LHT) implies any of the
Bielschowsky three-step test (B3ST) following:
The classical head tilt test was proposed by – Weakness of depressors of left eye (LIR,
Bielschowsky to differentiate between superior LSO), or
oblique palsy in one eye and superior rectus – Weakness of elevators of right eye (RIO,
palsy in the contralateral side. However, RSR).
presently in practice is the three step test as • Let us assume, for example, the patient being
modified by Parks'. It is useful in diagnosing examined has LHT. Draw an oval (with red
the paresis of any cyclovertically acting muscle. lines) around the two possible muscle pairs
There are in total 8 cyclovertically acting responsible for LHT (Fig.12.15A).
muscles; 4 work as depressors of the eyes, and
Step 2
4 work as elevators. The two muscles on each
eye that are responsible for depression are the • Determine whether hypertropia (HT) is larger
inferior rectus and superior oblique, and the in right gaze or left gaze.
two muscles on each eye that are responsible • If the LHT is larger in right gaze, it implies
for elevation are the superior rectus and the weakness of any of the 4 vertically acting
inferior oblique. muscles in right gaze:
As expected, at the onset of a cyclovertical – RSR, RIR
muscle palsy, there will be limitation in the field – LIO, LSO
294 Theory and Practice of Squint and Orthoptics

• If the LHT is larger in left gaze, it implies – LSR, LIR

weakness of any of the 4 vertically acting – RIO, RSO
muscles in left gaze, i.e.

Fig. 12.13 Diplopia charts (patient's view) of paralysis of extraocular muscles of the right eye.
 Incomitant Strabismus 295

Fig. 12.14 Diplopia charts (patient's view) of paralysis of extraocular muscles of the left eye.

• Let us say, in the same patient having LHT, • Note that at this point, the paretic muscle must
the deviation is greater in right gaze. Draw be either the LSO or RSR muscle, since they
an oval (with green lines) around the two are the only muscles encircled twice
possible muscle pairs (Fig. 12.15B). (Fig. 12.15B).
 296 Theory and Practice of Squint and Orthoptics

Fig. 12.15 Bielschowsky's three-step test (B3ST) in a patient with left superior oblique paralysis. A, step 1; B, step 2;
C, step 3 (for explanation, see text).

Step 3
• Determine, if the HT is larger, when measured
during head tilt to the left or right. For proper
measurement, the base of the prism should be
held parallel to the floor of the orbit and not
parallel to the floor of the room. The Maddox
rod and correcting prism should be held so
that the line and base are parallel to the floor
of orbit (Fig. 12.16).
• If the LHT is larger, when the head is tilted to
the right, this implicates any of four muscles Fig. 12.16 Measurement of deviation using Maddox rod
that act vertically in right tilt position, i.e. and prism in a patient with right hypertropia. Note, head is
tilted to the right and base of the prism is held parallel to the
either intorters of right eye (RSR, RSO) or floor of the orbit. Maddox rod is held in such a way that the
extorters of left eye (LIR, LIO). red line seen is also parallel to the floor of the orbit.
• If the LHT is larger, when the head is tilted to
the left, this implicates any of the four muscles the head is tilted to the right. Draw an oval (with
that act vertically in left tilt position, i.e. either blue lines) around the muscle implicated, i.e.
intorters of left eye (LSR, LSO) or extorters of (LSR, LSO, RIR, RIO) (Fig. 12.15C).
right eye (RIR, RIO). • Note that at this point, the LSO is the only
• Now, for the same individual, suppose that the muscle, i.e. surrounded by three ovals and
vertical deviation is quite large, when the head is connected by the line that represents head
is tilted to the left and is almost absent, when tilt to the right (Fig. 12.15C).
 Incomitant Strabismus 297

Summary of the test shown in Fig. 12.15: about the paretic muscles and the pathological
Step 1: LHT (LIR, LSO, RSR or RIO) sequelae of the paralysis, viz. overaction,
Step 2: Worse in right gaze (RSR or LSO) contracture and secondary inhibitional palsy.
Step 3: Worse in left tilt (LSO) Commonly employed tests to have a graphic
record of the relative power of extraocular
Results of B3ST in paralysis of various
muscles in all directions of gaze are as follows:
cyclovertically acting muscles are summarized
• Hess screen test
in Table 12.3.
• Lees screen test
Limitations of Park's three-step test • Lancaster red and green test
This test is quite useful in general, but it is not These tests are based on haploscopic principle
always diagnostic and can be misleading, and described in detail on page 121.
especially during following conditions:
• In cases of long-standing paresis. Uses of haploscopic tests
These tests are repeatable and their uses are as
• When more than one muscle are paretic, e.g.
follows:
– Bilateral fourth nerve palsy
• Diagnose underactions and overactions of
– Multiple other muscle weakness
extraocular muscles and provide a good
• In cases with restrictions. pictorial representation of muscle actions.
– Superior rectus overaction • Diagnose A- and V-phenomena.
– Superior rectus contracture • Diagnose mechanical/neurological palsy
– Inferior restriction • Diagnose congenital/acquired palsy
• Dissociated vertical deviation (DVD) •Aid in plan of surgery—preoperatively
• Pulley heterotopia • Show effect of surgery—postoperatively.
• Superior rectus palsy • Provide an accurate and permanent record of
• Skew deviation change in state of ocular movements in
• Prior extraocular muscle surgery subsequent visits and thereby form part of
Quantitative measurement of extraocular serial record of progress of palsy.
muscle actions • Measure torsional movement with linear
The quantitative measurement of extraocular pointer (Dulley and Harden) or cyclotiltmeter
muscle action is most essential to comment (Brown).

Table 12.3 Results of Bielschowsky three-step head tilt Field of binocular fixation
test in paralysis of cyclovertically acting muscles It must be tested, wherever applicable, i.e. if
Paralysed Bielschowsky head tilt test patient has some field of binocular single vision.
muscle Step 1 Step 2 Step 3 It provides, useful and repeatable information.
(hypertropia (HT worse (HT worse The area of binocular single vision is opposite
in primary in left or on left or to the direction in which ocular motility is
gaze) right gaze) right tilt) impaired.
RSO RHT Left Right The aim of treatment of muscle paralysis is to
provide comfortable field of binocular fixation,
RIR RHT Right Left
i.e. the central field and lower quadrants.
LIO RHT Right Right For details of the test, see page 127.
LSR RHT Left Left
LSO LHT Right Left Other tests
LIR LHT Left Right Other tests which can be carried out, if
RIO LHT Left Left necessary, includes forced duction test, EMG,
EOG, orbital ultrasonography and computerized
RSR LHT Right Right tomographic scanning.
 298 Theory and Practice of Squint and Orthoptics

DIFFERENTIAL DIAGNOSIS OF long-standing paralysis, there occurs spread of

INCOMITANT SQUINT comitance and thus it becomes extremely diffi-
Differential diagnosis to be considered in cult and at times even impossible to differen-
patients with incomitant squint in general are tiate such a condition from the comitant squint.
as follows: Anyshow, for a ready reference, the differences
between paralytic and non-paralytic squint are
• Comitant (non-paralytic) versus incomitant
depicted in Table 12.4.
(paralytic) squint.
• Congenital versus acquired palsies. Congenital versus acquired ocular palsy
• Paralytic versus restrictive incomitant squint. Many a time, patients with congenital paralysis
of an extraocular muscle may remain asympto-
Comitant (non-paralytic) versus matic for decades because of either a strong fusion
incomitant (paralytic) squint mechanism or by an unnoticed slight abnormal
As mentioned earlier, by and large, there arises head posture. Such a patient, when reaches adult
no problem in differentiating comitant (non- life, the chances are that, unless the latent
paralytic) squint from the paralytic (incomitant) deviation is small one, decompensation will begin
squint of recent onset. However, in patients with to occur, especially between the ages of 30 and

Table 12.4 Differences between paralytic and non-paralytic squint

S. no. Feature Paralytic squint Non-paralytic squint
1. Age of onset Any age Usually in childhood
2. Type of onset Usually sudden, rarely may be slow Usually gradual
or since birth
3. History of head Common Uncommon
injury
4. Diplopia Usually present Usually absent
5. Ocular movements Limited in the direction of paralysed Usually full
muscle
6. False projection It is common in palsy of recent onset, False projection is negative
i.e. patient cannot correctly locate the
object in space, when asked to do so
in direction of paralyzed muscle. There
occurs past pointing.
7. Head posture A particular abnormal head posture may Normal
be present, depending upon the muscle
paralysed.
8. Nausea and vertigo Usually present, due to confusion, Absent
diplopia and false projection
9. Primary versus Secondary deviation is more than Secondary deviation is equal
secondary primary deviation to primary deviation
deviation
10. Sensory adaptations Uncommon Common
(ARC, suppression,
amblyopia)
11. Cyclotropia Common with cyclovertical paresis Uncommon, except in A- and
V-patterns
12. Muscle sequelae Present in old cases Absent
13. Neurological May be present Usually absent
findings or
systemic diseases
 Incomitant Strabismus 299

40 years. The patient may notice that he/she is extraocular muscles and are often confused
beginning to suffer from an intermittent diplopia, with one another by even experienced
especially if he/she is tired, overworked, or examiners. Though, these are two distinct
suffering from ill health. Some of these patients problems but mere measurement obtained with
may develop intermittent squint without diplopia prism and alternate cover tests with either eye
due to suppression of the image of the deviating fixing do not differentiate between them, since
eye. Such patients may experience difficulty in in both, the secondary deviation is typically
focussing or have a feeling of using only one eye. greater than the primary deviation. However,
Under these circumstances, one needs to a differentiation between the two is most
differentiate between cases of congenital important for the successful treatment of
paralysis with recent decompensation and those incomitant deviation. It is unequivocal to state
of acquired paralysis of recent onset. It is very that the restrictions must be relieved first,
essential, since in the former the treatment is before any other therapy, whether surgical or
invariably operative while the latter requires a non-surgical to be effective.
diligent search for its cause by a complete Commonly employed tests to differentiate
medical and neuro-ophthalmologic evaluation between palsies and restrictions are as follows:
and the appropriate treatment. Some of the chief
differences between the congenital and acquired 1. Passive forced duction test (traction test)
ocular palsies are summarized in Table. 12.5. As mentioned above, detection of associated
restriction is most important for the successful
Paralytic versus restrictive incomitant squint therapy of an incomitant squint. Therefore, it is
Incomitant ocular deviations are known both mandatory to carry out forced duction test (FDT)
due to palsies as well as restrictions of before any surgical therapy is undertaken.

Table 12.5 Differences between congenital and acquired ocular palsies

S.no. Feature Congenital ocular palsy Recent acquired ocular palsy
1. Onset of symptoms Usually indefinite and intermittent Usually definite and sudden
2. Diplopia Rare, intermittent diplopia in Almost invariably present, but may
decompensation be limited to paretic field
3. Primary deviation May be intermittent or constant Usually constant
angle of deviation may be large; Angle of deviation may be small and
but symptoms may be only slight yet the symptoms may be pronounced
4. Secondary deviation Only slightly greater than the Usually much greater than the
primary deviation (due to primary deviation
spread of comitance)
5. Past pointing Usually absent Present
6. Abnormal head posture May persist on covering paretic Disappears on covering paretic eye
eye because of secondary scolio-
sis and contracture of neck muscles
7. Facial asymmetry Common with torticollis of long Absent
standing
8. Amblyopia May be present Absent
9. Forced duction test May be positive due to Negative
contracture of antagonist
10. Abnormal head posture May be present Absent
in old photographs
 300 Theory and Practice of Squint and Orthoptics

Steps of the forced duction test (FDT) – Care should be taken not to push the globe
i. Anaesthesia. In adults and cooperative elder into the orbit posteriorly, since this may
children, FDT can be performed preoperatively conceal a restriction of the movement resulting
under topical anaesthesia with 4% xylocaine in a false negative FDT.
instilled every 4 minutes for 4 times. In small • To test the restrictions in the field of action
and uncooperative children, FDT is done under of recti, the globe should be rotated, up,
general anaesthesia during surgery, taking an down, medially or laterally.
account of following points: • To test the restrictions in the field of action
of oblique muscles, the globe should be
– To remove the effect of tonic innervational rotated both down and in, and up and in.
factors, the FDT should be performed, when
patient has reached stage 3 of anaesthesia. Note. FDT should be repeated at the time of
surgery and also after completion of the surgery.
– If succinylcholine is to be used, preferably the
FDT should be performed while the patient Interpretation of the results of FDT
has received an inhalation anaesthetic by
1. Forced duction test is labelled negative, if
mask, but before intubation. Otherwise one
no resistance is encountered during passive
will have to wait for at least 20 minutes till
rotation and the examiner can rotate the
the contraction of the extraocular muscles globe to its full extent. A negative FDT implies
caused by succinylcholine is over. that the motility defect is clearly caused by
– Pancuronium, a nondepolarizing muscle paralysis of the weak muscle.
relaxant, that does not alter the FDT, should 2. Positive FDT is labelled, if a resistance is
be preferred over succinylcholine. encountered during passive rotation of the
ii. Grasping of the globe. After proper anaesthesia, globe. With a feeling of resistance, if the
the globe should be grasped near the limbus examiner can rotate the globe no further than
with either a forceps without teeth or Pierse the patient voluntarily can, the motility defect
forceps to avoid tearing of the conjunctiva. is purely due to mechanical restriction.
Preferably, the globe should be held with the However, with a feeling of resistance, if the
help of two forceps at right angle to the axis in examiner can passively rotate the globe beyond
which restriction is to be tested. where the patient can voluntarily rotate it, but
not to its full extent, the motility defect is a
For example, in a patient with divergent
combination of mechanical restriction and
squint (Fig. 12.17A), to distinguish between
agonist muscle weakness.
lateral rectus paralysis and mechanical
The restriction noted in the positive FDT
restriction involving the medial aspect of the may be one of the following types:
globe, the forceps should be applied at 6 and 12
i. Leash restriction is caused by the mechanical
O'clock positions (Fig. 12.17B).
factors such as marked scarring of Tenon's
iii. Passive rotation of the globe. After grasping, capsule and conjunctiva, contracture of an
the globe should be rotated passively towards extraocular muscle and/or entrapment of
the direction of action of suspected weak muscle, muscle or its facial sheath on the side of globe
e.g. into abduction in patients with lateral rectus opposite the limited field of rotation.
weakness versus mechanical restriction involving The globe can be passively rotated
medial aspect of the globe (Fig. 12.17C), taking freely up to a point after which tethering
following precautions: effect of restriction does not allow the globe
– When FDT is being performed under topical to move further any more. Such a restriction
anaesthesia, patient should be instructed to is not only felt but can also be seen as a taut
look at his/her hand held in the direction in string of conjunctiva (String sign).
which the eye is to be rotated by the forceps. ii. Reverse leash restriction. The tethering effect
This will help in avoiding the effect of tonic of restriction is similar to leash restriction as
innervational factor. described above. However, the mechanical
 Incomitant Strabismus 301

Fig. 12.17 Technique of forced duction test (for explanation, see text).

factors responsible for tethering are marked restriction, there occurs a partial resistance
shortening of conjunctiva and Tenon's over the entire range of ocular movement
capsule, marked posterior scarring of orbital which can be overcome by an increased force.
tissues or a tight posterior fixation suture
used in Faden's operation on the same side 2. Exaggerated traction test
of globe in which rotation is limited. It is a modified forced duction test which is
iii. Elastic restriction is caused by an early performed to estimate the tightness in superior
contracture of a muscle following paresis of oblique (SO) and inferior oblique (IO) muscles.
its agonist, co-contraction of extraocular Procedure. For checking tightness of RSO, the
muscles due to effect of succinylcholine and eyeball is grasped near the limbus at 6 and 9
orbital cellulitis. In contrast to the leash and O'clock positions, as described in FDT. To
reverse leash restriction (in which globe can perform this test, the eyeball is first pushed in
be rotated to a point after which tethering the orbit and then elevated, adducted and rolled
effect of restriction does not allow the globe back and forth by extorting and intorting the
to move further any more), in elastic globe across the tendon. During this manoeuvre,
 302 Theory and Practice of Squint and Orthoptics

if the eyeball jumps across the tendon, tightness

of the superior oblique is indicated.
Tightness of the inferior oblique is also tested
in the similar manner, except that instead of
elevating and adducting, the eyeball is pushed
down and nasally.

3. Spring-back balance test

It is a continuation of the FDT, when performed
under general anaesthesia. It is of specific use
in patients who are suspected (after FDT) of
having mechanical restriction and not a weak
muscle. In this test, after holding near the
limbus, eyeball is rotated back and forth
vigorously for 2–3 times and then released Fig. 12.18 Technique of judging active force generated
suddenly. After settling, normally, the globe during ocular movement (muscle contraction). (For
comes to rest in straight ahead position. explanation, see text).
However, in the presence of a significant
mechanical restriction, the eyeball will be drawn • Lid fissure widening and a relative proptosis
towards the direction of the mechanical pull, e.g. is noted in paralytic squint as the patient
the eyeball will be adducted, if the cause of looks into the field of action of paretic rectus
mechanical restriction is located medially. muscle.

4. Active force generation test 6. Electro-oculographic measurement

of saccadic velocity
In this test, eyeball is stabilized with the forceps
applied at the limbus under topical anaesthesia Saccades are sudden, jerky conjugate eye
and patient is asked to move his/her both eyes movements, that occur as the gaze shifts from
in the direction of the muscle to be tested. For one object to another. These movements bring
example, if right lateral rectus muscle is to be the object of regard quickly on the fovea with
tested, patient is asked to move his/her eyes in an average velocity of 250°/second in the field
dextroversion. During this movement, the force of action of the muscle concerned. Measure-
generated by the contracting muscle of the eye ment of saccadic velocity with the help of
being tested (e.g. Rt LR) is transmitted through specially designed electro-oculographic (EOG)
the forceps to the examiner's fingers. From the recorder can help in differentiating muscle
feel of the transmitted force, examiner can judge restrictions from the muscle weakness. The
subjectively whether the contracting muscle is saccadic velocity is decreased in paretic muscle,
weak or normal (Fig.12.18). For objectively while it is near normal in mechanical muscle
quantifying this test, calibrated forceps are restrictions.
available which indicate the amount of force
generated in grams. A normally acting muscle 7. Positional tonometry
generates a force of 60–80 g in extreme gaze. This It has been reported that intraocular pressure
test is quite useful in diagnosing the weak rises from the compression of a non-relaxing stiff
muscle. However, it can only be performed in muscle, when attempts are made to move the
alert and co-operative patients. eye into the field of its antagonist. Perkin's hand-
held applanation tonometer or Digilab Pneumo-
5. Lid fissure changes on eye movements tonometer can be used to measure the IOP in
• Narrowing of lid fissure along with globe different gaze positions. A pressure increase of
retraction is seen in restrictive squints, as in over 5 mm Hg in a particular field of gaze is
Duane’s retraction syndrome. indicative of a restriction.
 Incomitant Strabismus 303

CLINICAL VARIETIES OF OCULAR PALSIES syndrome where the trochlear nerve is absent
1. Isolated ocular muscle paralysis. and results in secondary atrophy of the
2. Paralysis of 3rd cranial nerve superior oblique muscle.
3. External ophthalmoplegia • The second type has a normal trochlear nerve
4. Total ophthalmoplegia and size of the superior oblique muscle, but
5. Internuclear ophthalmoplegia has an abnormal laxity of superior oblique
tendon.
ISOLATED OCULAR MUSCLE PALSIES Note. It has been reported that sometimes a
Superior oblique (4th nerve palsy) and lateral spontaneous manifestation of fourth cranial
rectus (6th nerve palsy) are the most common nerve palsy in adult age might be due to
muscles to be paralysed singly, as they have decompensation of fusion mechanism in a
separate nerve supply. Isolated paralysis of the patient with congenital palsy.
remaining four extraocular muscles is less known. 2. Trauma is another frequent cause of fourth
nerve paralysis (about 34% cases). Because of
PARALYSIS OF FOURTH CRANIAL NERVE
the position of the trochlear nerves with respect
(SUPERIOR OBLIQUE MUSCLE PARESIS)
to the tentorial edge, closed head injury (even
The fourth cranial nerve (trochlear) is entirely minor) can result in fourth nerve palsy. Due to
motor in function and supplies only the superior an impact in the area of anterior medullary
oblique muscle of the eyeball. It differs from velum, where the two nerves decussate, bilateral
other cranial nerves in being: trochlear nerve palsies are quite common in
• The only cranial nerve to arise from the dorsal head injury. Iatrogenic trauma, occurring after
aspect of the brain (midbrain); SO tenectomy and ethmoid sinus surgery, now
• The only cranial nerve to cross completely on has become rare due to refinment in the surgical
the other side (i.e. the trochlear nerve arises techniques.
from the contralateral nucleus; and 3. Idiopathic. In about 20% cases of 4th nerve
• The longest and thinnest of all cranial nerves. palsy, cause could not be ascertained.
The fourth cranial nerve palsy (superior
4. Vascular and neurogenic causes account for
oblique muscle paralysis) is the most common
isolated cyclovertical muscle palsy encountered about 3 to 5% cases, seen in elderly age group,
by the ophthalmologists. Fourth cranial nerve having acute onset and small angle hypertropia
palsy may be unilateral or bilateral. Bilateral (<6 prism diopters).
palsies are almost always acquired. Unilateral • Aneurysms and tumours (trochlear Schwnomes
palsy (more common than bilateral) may be and brain tumours) are rare causes.
congenital or acquired. • Ocular myasthenia gravis may present as an
Note. The applied anatomy of fourth cranial isolated unilateral superior oblique paralysis
nerve (Page 12) should be reviewed before with an insidious course. Therefore, as a
proceeding further. general rule, a patient who presents with an
unexplained diplopia of any type should
Etiology undergo a tensilon test.
In order of frequency, following are the causes • Diabetic neuropathy may occasionally involve
of fourth cranial nerve paralysis: the trochlear nerve. Therefore, in an undiag-
1. Congenital paralysis is quite frequent (about nosed case of 4th nerve paralysis, a glucose
40% cases). Congenital paralysis may result from tolerance test should also be done to rule out
a defect in the nucleus or the motor portion of diabetic cranial mononeuropathy.
the nerve (hypoplasia or even oplasia rarely). • Herpes zoster can also be considered a
High definition magnetic resonance imaging potential etiologic agent.
(MRI) studies have identified two groups of • Hydrocephalus may be a cause of acquired
congenital SOP: unilateral or bilateral fourth nerve palsies.
• The most frequent type, present in 73% of • Idiopathic intracranial hypertension can also lead
cases, is a congenital cranial dysinnervation to fourth nerve palsies.
 304 Theory and Practice of Squint and Orthoptics

5. Cavernous sinus and superior orbital fissure 3. Facial asymmetry. Another important clinical
syndrome may be considered a cause of 4th sign is facial asymmetry, which is a characteristic
nerve paralysis in association with 3rd and 6th finding of congenital palsy. There occurs typical
cranial nerves (see pages 312 and 328). shallowing of mid-facial region between lateral
canthus and the angle of the mouth on the side
Clinical features of head tilt. This is indication of long-standing
SO palsy.
Clinical features in a patient with 4th nerve
4. Diplopia is seldom noticed, if onset is during
paralysis (Fig.12.19) are as follows:
visual immaturity. However, when onset is after
1. Cyclovertical deviation. visual maturity, i.e. adult patients will
When the patient fixates with normal eye, experience homonymous vertical, diagonal or
usually the involved eye is elevated, slightly torsional diplopia. Image seen by the involved
adducted and extorted following weakness of eye is lower, uncrossed and intorted. If principal
the superior oblique muscle (Fig. 12.19K). complaint is of torsional diplopia then bilateral
The hyperdeviation becomes more obvious, palsy should be suspected. Vertical separation
when the head is tilted towards ipsilateral increases while looking down, therefore,
shoulder (Bielschowsky head tilt test) (Fig. diplopia is particularly noticed by the patient
while coming down the stairs. Further, such a
12.19M). Depending upon severity, deviation
patient may not have much problem as long as
seen is as below:
the eyes look above the horizontal plane.
• Mild cases show hypertropia in down and 5. Ocular movements. Three abnormalies may
adducted position. be observed:
• Modirate cases hypertropia in adduction only • Ipsilateral SO under action is seen in patients
(without depression). with marked paresis or lax SO tendons.
• Severe cases show hypertropia in primary • Ipsilateral IOOA is present in most of the
position. cases.
When the patient fixes with the paretic eye, the • Contralateral SO overaction or pseudo SO
normal eye is hypotropic (depressed), adducted overaction which are clinically indistin-
and extorted more than the primary deviation. guishable due to decreased infraduction in
Such a condition has been labelled as fallen eye abduction of the involved eye, there is
syndrome. apparent over depression of the fellow eye.
In congenital cases, typically, parents may Note. Long-standing hypertropia can cause
notice that one eye of this infant is higher than contracture of SR muscle which causes
the other and that there is abnormal head posture. restrictions in depression and can be tested by
2. Abnormal head posture occurs towards the forced duction test.
action of paralysed superior oblique, i.e. chin is Ocular movements in a patient with superior oblique
depressed, face is slightly turned towards the paralysis, e.g. of right eye, are affected as below:
opposite side and the head is tilted towards the • Movements of left eye are limited, when
opposite shoulder (Fig. 12.19A). The degree of looking down and to right (Fig. 12.19H) (angle
abnormal head posture is not always of deviation is also greatest in this direction).
proportional to the size of the hypertropia. • Overaction of left eye as looking up and
Compensatory head posture is the most to right (Fig. 12.19B).
common presenting sign of SO palsy. • Overaction of right eye on looking down and
• In congenital SO palsy, there is a large head tilt to right (Fig. 12.19H).
which is confirmed on the family album • Underaction of right eye on looking up and
photography or FAT scan. to right (Fig. 12.19B).
• In bilateral acquired palsies, there is a chin down • In a long-standing palsy with the paretic eye
posture to compensate for V esotropia. fixing, the inferior rectus of the hypotropic
Note. Rarely there may be head tilt towards non-paretic eye can undergo hypertrophy,
affected side to increase separation of images then contracture, resulting in limited elevation
and hence to ignore the second image. of the non-paretic eye on both ductions and
 Incomitant Strabismus 305

Fig. 12.19 Left superior oblique palsy. A, Abnormal head posture, note head is tilted to the right shoulder, face is slightly
turned to the right and chin is slightly depressed. B to J, Eyeballs in nine positions of gaze, note left hypertropia (F) which
increases on right gaze (E). Also note left hypertropia (K) which increases on tilting the head to the left shoulder (M) and
no change in hypertropia on tilting the head to the right shoulder (L). (Courtesy: Dr Kanwar Mohan)
 306 Theory and Practice of Squint and Orthoptics

versions. The effect can simulate a double • Acquired SO palsy is usually associated with
elevator palsy in the non-paretic eye, but this complaints of subjective torsion.
can be ruled out with the forced duction and • Cyclotorsion of <10° is seen in unilateral
head tilt tests. palsy and >10° in bilateral palsy.
Diagnosis 3. Hess screen (Fig. 12.20) or Lancaster red/
In the diagnosis of superior oblique palsy, one green test is useful for a meticulous follow-up
needs to consider the following: of the patients.
• Differentiation of superior oblique paresis 4. Diplopia charting (Fig. 12.21) is also
from other cyclovertical deviations. important as in all cases of acquired strabismus
• Unilateral versus bilateral superior oblique and should be done in all cases of SO palsy
paresis. presenting with complain of vertical and
diagonal diplopia.
• Congenital versus acquired superior oblique
paresis. 5. Measurement of ocular deviation in all 9 diag-
nostic gaze positions, as well as in right and left
Diagnosis of superior oblique paralysis head tilt is important in diagnosing and planning
Differential diagnosis is made by performing treatment for superior oblique palsy.
following tests: 6. Force duction test should be done to look for
1. Three-step test The key to diagnose SO palsy SR contracture.
is 3-step test which requires motility measure- 7. Oblique traction test. Intraoperative testing
ment in primary gaze, right and left lateral gaze of SO is essential for evaluating patients with
and right and left head tilts (for details see page SO palsy, especially in young children where
293). precise orthoptic measurement cannot be taken.
2. Torsion is usually measured objectively by This not only helps to identify the lax tendon
indirect ophthalmoscope and subjectively by that should be tucked but also importantly
double Maddox rod test. identifies tendon of normal length which should
The following features are observed: not undergo this procedure for the fear of brown
• Congenital SO palsy usually has no torsion. syndrome.

Fig. 12.20 Hess chart of a patient with right superior oblique palsy.
 Incomitant Strabismus 307

Table 12.6 Modified Knapp and Moore's classification

of superior oblique palsy into eight classes
Class Pattern of deviation (e.g. in paralysis of LSO)
Description Diagrammatic
depiction

I HT is greatest when eye

is elevated and adducted

II HT greatest when eye is

depressed and adducted

III HT of equal magnitude in

the entire paralysed field
IV HT of equal magnitude in
the entire paralysed field
and the entire inferior field
(L-shaped or reverse L-
Fig. 12.21 Diplopia chart of a patient with right superior pattern)
oblique palsy.
V HT is greatest in the entire
8. Neuroimaging. Most patients with isolated SO inferior field
palsy do not need neurological workup. VI Underaction of both SO,
Indications of neuroimaging are: overaction of both IO,
• Fresh trauma V-pattern esotropia,
• Non-isolated, multiple palsies. bilateral positive
• Acquired palsy in absence of trauma. Bielschowsky head tilt test
VII Canine tooth syndrome
• Presence of associated neurological sign.
characterized by under-
• Younger patients presenting with acute
action of inferior oblique
symptoms (acquired Brown's) and
Modified Knapp and Moore's classification of under-action of superior
superior oblique palsy. Depending upon the oblique. It usually occurs
amount of hypertropia in different diagnostic due to trauma in the area
positions of gaze, Knapp and Moore have of the trochlea
classified common manifestations of superior VIII Comitant hypertropia but with positive head
tilt test
oblique paralysis into 7 classes which has been
modified by other workers by adding class VIII unilateral cases, actions may be normal or
(Table 12.6). diminished.
4. Head tilt test is positive for the involved eye
Unilateral versus bilateral superior oblique palsy
in unilateral cases, i.e. hypertropia increases on
1. Esotropia in downgaze is usually little in tilting the head towards ipsilateral shoulder.
unilateral cases while in bilateral cases usually While, in bilateral palsies, tilting to either side
there is a V-pattern esotropia with chin down will increase the hypertropia, i.e. right hyper-
head posture. tropia on right tilt and left hypertropia on left tilt.
2. Torsion, when measured by the double Even after spread of comitance, the head-tilt test
Maddox rod test, usually shows an excyclo- should be positive in fourth cranial nerve palsy.
deviation of less than 10° in unilateral cases and
more than 10° in bilateral cases. Congenital versus acquired superior oblique palsy
3. Ductions of the superior oblique muscles are Most patients are congenital, so facial asymmetry
usually diminished in bilateral cases while in with old photographs showing head tilt, or
 308 Theory and Practice of Squint and Orthoptics

asthenopia symptoms of long duration are • On imaging, enlargement of muscle belly is

sufficient to rule out acquired palsy. Following seen.
points are useful in differentiating congenital 2. Brown syndrome. In it, there is underaction
from acquired SO palsy: of ipsilateral IO whereas in SO palsy, there is
1. Amblyopia is uncommon in acquired paresis ipsilateral IOOA.
but may be present in congenital ones. 3. Primary IOOA. It is usually seen with
2. Excyclodeviation and complaint of apparent infantile esotropia. There is absence of
tilting of objects are common in acquired cases. hyperdeviation in the primary position, lack of
torsion and negative head tilt test.
3. Abnormal head posture may be traced to
childhood from the old family photographs in 4. Skew deviation is an acquired, acute, hyper-
patients with congenital palsy while not in those deviation which may or may not be comitant.
Presence of neurologic signs refers to brainstem
with acquired palsy.
or cerebellum involvement.
4. Presence of increased vertical vergence may be
the only suggestion of a previous palsy in some Treatment
adult patients who develop spontaneous
I. General principles of therapy are same as
manifestations of 4th nerve palsy due to
described in therapy of paralysis of sixth (page
decompensation of fusion mechanism in old
313) and third (page 332) nerves.
case of congenital palsy.
5. Long, redundant or floppy superior oblique tendon II. Non-surgical treatment
seen during surgery may indicate a congenital 1. Amblyopia, when present, should be treated
palsy. first; if the patient is a child, surgery is usually
Table 12.7 summarizes the differences between indicated.
congenital and acquired superior oblique palsy. 2. Segmental membrane prisms or segmental
occlusion of the lower third of the spectacle lens
Differential diagnosis before the paretic eye with semiopaque Scotch
1. Thyroid ophthalmopathy is a chronic tape may be tried till surgery is undertaken.
restrictive disease in which: 3. Alternate occlusion or occlusion of the sound
• IR muscle, which is most commonly involved eye may be performed to create visual comfort
(positive on FDT). in patients in which binocular single vision
cannot possibly be restored by any means.
• Signs of proptosis, lid lag, lid retraction,
lagophthalmos. 4. Prisms may be used to overcome diplopia in
small, symptomatic, comitant or nearly comitant
• Signs of active disease, i.e. chemosis, orbital
deviations that do not have a symptomatic
congestion are seen.

Table 12.7 Differences between congenital and acquired superior oblique palsy.
Features Congenital SO palsy Acquired SO palsy
Diplopia No complain of diplopia, only intermittent Usually complain of vertical,
vertical diplopia in decompensated palsy diagonal or torsional diplopia
with incomitant hypertropia
Torsion There is no measurable subjective torsion Excyclotorsion usually seen
more so in bilateral palsy
Head tilt Present since infancy (old photographs) Anytime later following the onset
Traction test Lax SO tendon confirmed by traction test SO traction test is normal
intraoperatively
Facial asymmetry Facial asymmetry is usually present There is no facial asymmetry
Fusional amplitudes Significantly increased (16–30 prism diopters) Normal (2–3 prism diopters)
Amblyopia in the May be present Usually absent
involved eye
 Incomitant Strabismus 309

torsional component. If this alleviates the • To reduce or eliminate the head tilt in
symptoms, surgery is not warranted. congenital SO palsy that presents in early
III. Surgical treatment. Surgical treatment is childhood.
indicated for a: 2. Anterior temporal displacemet of the anterior
• Significant abnormal head posture, half of the SO muscle tendon (Harada-Ito
• Vertical deviation, or procedure) is indicated as an alternative to SO
• Diplopia. tuck, when the deviation is primarily torsional.
A significant head tilt is the main indication This procedure does not correct any vertical
for surgery in children younger than age 5, as it deviation in primary position, but corrects the
is thought that the uncorrected torticollis will excyclo-deviation and the abducting weakness
lead to progressive facial asymmetry. of the SO muscle in downgaze.
• Intraoperative adjustment of Harada–Ito procedure
Surgical approach employed should take into is possible by visualizing objective torsion of
consideration: the fundus using indirect ophthalmoscope.
• Presence of SR contracture, • Postoperative adjustment of Harada–Ito procedure
• SO laxity, and has also been described using adjustable
• Degree of torsion. sutures.
3. Fell’s modification of Harada-Ito procedure
Various surgical schemes have been recom- involves disinserting the anterior fibres of the
mended. Knapp and von Noorden's modified superior oblique tendon and transposing them
approach depending upon the class of paresis 8 mm posterior to the superior insertion of the
is summarized in Table 12.8. lateral rectus muscle.
Based on the degree of deviation (hypertropia) 4. Bilateral Harada-Ito procedures are indicated
and status of muscle sequelae following in some cases of bilateral fourth nerve palsy.
paresis of SO muscle, the line of surgical
management is summarized below: LATERAL RECTUS PARALYSIS
Hypertropia is <20 PD The abducent (sixth cranial) nerve is a small
One muscle surgery as below: and pure motor nerve that supplies the lateral
• IO weakening—if IOOA rectus muscle. Isolated lateral rectus muscle
• SR recession—if contracture of SR positive paralysis (sixth cranial nerve paralysis) is next
common to isolated paralysis of superior
• SO tuck—if SO tendon laxity
oblique muscle.
• Contralateral IR recession—if no SR contracture
and no SO tendon laxity Note. Applied anatomy of sixth cranial nerve
(page 16) should be reviewed before proceeding
Hypertropia >20 PD further.
Two muscle surgery, i.e. ipsilateral IO weakening
+ any of the following: Etiology
• SR recession—if contracture of SR positive A. Congenital sixth nerve palsy due to hypo-
• SO tuck—if SO tendon laxity plasia of its nucleus or developmental anomaly
• Contralateral IR recession—if no SR in the motor nerve fibres is quite rare.
contracture and no SO tendon laxity. • Congenital absence of the 6th nerve nucleus and
aplasia of the nerve is associated with Duane’s
Other recommendations are: syndrome (congenital cranial dysinnervation
1. Superior oblique muscle tuck is a hard disorder).
procedure to quantitate. It is best employed: • Congenital horizontal gaze palsy due to
• When the deviation is greatest in opposite involvement of gaze centre may be confused
downgaze and so muscle is moderately to with congenital 6th nerve palsy.
markedly underacting. • However, sixth cranial nerve paresis occurring
• For acquired bilateral SO palsy or weakness. shortly after birth had been reported and
310 Theory and Practice of Squint and Orthoptics

Table 12.8 Knapp's and von Noorden's surgical schemes for left superior oblique muscle paresis
Modified knapp’s Pattern of deviation Muscle sequelae associated with Recommended surgical
class of paresis LSO palsy treatment
Class I Maximum HT in Overaction of ipsilateral Weakening of ipsilateral
dextroelevation IO (LIO) IO (LIO)
Class II Maximum HT in Underaction of paretic
dextrodepression LSO
a. If laxity of SO tendon Tuck of ipsilateral SO (LSO)
(grade III and IV laxity) or recession of contralateral
b. If no laxity of SO tendon inferior rectus (RIR)
and IO overaction present Weakening of ipsilateral
IO (LIO)
Class III HT equal in entire Weakness of paretic SO (LSO)
paralysed field (all with overaction of ipsilateral
dextroversion position) IO (LIO)
a. If HT <20 PD Weakening of ipsilateral
b. If HT >20 PD with IO (LIO)
• SO tendon laxity LIO weakening + LSO tuck
• No SO tendon laxity LIO weakening + RIR
recession
Class IV HT equal in entire Contracture of ipsilateral SR
paralysed field and (LSR)
inferior field (i.e. a. If deviation <20 PD LSR recession + LIO
dextroposition and weakening
downgaze positions b. If deviation >20 PD with LSR recession + LIO
(L-shaped) • SO tendon laxity weakening + LSO tuck
• No SO tendon laxity LSR recession + LIO
weakening + RIR recession
Class V HT maximum in all Long-standing SO palsy with
downgaze positions spread of comitance
a. If SO tendon laxity LSR recession + LSO tuck
or RIR recession
b. If with IO overaction LSR recession + LIO
weakening
Class VI V-pattern esotropia Bilateral SO palsy (underaction)
with reversing with bilateral IO overaction
Bielschowsky head tilt a. If torsion <15° Bilateral IO weakening
test b. If torsion >15° Bilateral IO weakening +
B/L Harada-Ito procedure
or B/L IR recession
Class VII HT in all downgaze Underaction of inferior
position, primary oblique and superior oblique
position and in (acquired Brown’s syndrome)
dextroversion Usually occur due to trauma
in trochlear area (canine tooth
syndrome)
• If aligned (no deviation) in No procedure
primary position
• If ipsilateral hypertropia Recess contralateral IR (RIR)
• If ipsilateral hypotropia Explore trochlea to release
restriction
Class VIII Comitant hypertropia Spread of comitance
but with positive head • If IO overaction LIO weakening
tilt test • If tight SR LSR recession
• If lax SO tendon SO tuck or RIR recession
 Incomitant Strabismus 311

usually resolves spontaneously. It is thought and occasionally recurrent form of 6th nerve
to be caused by the increased intracranial palsy occurs in children, usually following
pressure associated with labour and delivery. upper respiratory infections, other forms of mild
• Congenital bilateral abducent paralysis viral illness, including immunization. The
associated with facial diplegia and microglossia spontaneous benign lesions usually resolve
constitutes the Mobius syndrome. over several months.
B. Acquired sixth nerve palsy can occur due to b. Vascular disorders such as hypertension and
lesions at various levels as follows: diabetic microangiopathy can also cause 6th
nerve palsy specially in elderly due to
1. Nuclear lesions. Nuclear lesions never cause
ischaemic infarction.
isolated sixth nerve palsy. A lesion in and
around the sixth nerve nucleus causes the c. Multiple sclerosis is an important cause of
following: 6th nerve palsy in patients below 40 years of
age.
• Ipsilateral sixth nerve palsy.
d. Idiopathic sixth cranial nerve palsy has been
• Ipsilateral seventh nerve palsy of upper motor
labelled as a major group in most of the series
neuron type due to concomitant involvement
of isolated sixth nerve palsy. Some of such
of facial fasciculus.
cases might be due to undiagnosed vascular
• Loss of conjugate movements to the same side ischaemic infarcts.
resulting from involvement of horizontal gaze
centre in the pontine paramedian reticular 4. Lesions of the basilar part of sixth nerve. The
formation (PPRF). important causes which may damage the basilar
part of the sixth nerve include:
2. Fascicular lesions may cause:
i. Foville's syndrome. It results due to lesions of i. Acoustic neuroma. It should be emphasized that
dorsal pons involving sixth nerve fasciculus as the first symptom of acoustic neuroma is hearing
it passes through PPRF and is characterized by loss and the first sign is diminished corneal
the following: sensations. Therefore, hearing and corneal
• Ipsilateral sixth nerve palsy sensations should be tested in all patients with
• Loss of conjugate movement to the same side sixth nerve palsy.
• Ipsilateral facial nerve palsy ii. Nasopharyngeal tumours
• Facial analgesia from involvement of the iii. Fracture base of the skull, and
sensory portion of the fifth nerve. iv. The involvement of petrous bone from otitis
• Deafness media may cause Gradenigo syndrome,
ii. Raymond's syndrome. It results due to lesions characterized by the following:
of the ventral pons involving fasciculus as it • Ipsilateral 6th nerve palsy
passes through pyramidal tracts and is • Deafness
characterized by: • Neuralgias in the distribution of first division
• Ipsilateral 6th nerve palsy, of trigeminal nerve.
• Contralateral hemiplegia, and • Facial weakness.
• Variable number of signs of dorsal pontine
v. Involvement in raised intracranial pressure.
lesions.
Sixth nerve paralysis is one of the commonest
iii. Millard-Gubler syndrome. It results due to
false localizing sign in cases with raised
lesions similar to Raymond's syndrome and is
intracranial pressure. Its susceptibility to such
characterised by the same features with addition
damage is due to its long course in the cisterna
of ipsilateral 7th nerve palsy.
pontis, to its sharp bend over the superior border
3. Lesions involving the 6th cranial nerve trunk of the petrous temporal bone and the downward
anywhere are as follows: shift of the brainstem (towards the foramen
a. Infections and immunological processes may magnum) produced by raised intracranial
involve the sixth cranial nerve trunk. A benign pressure (Fig.12.22).
 312 Theory and Practice of Squint and Orthoptics

• Vascular disorders, e.g. hypertensive and

diabetic microangiopathy causing diabetic
mononeuropathy
• Idiopathic, i.e. cause not known

Clinical features
Clinical features in an isolated 6th nerve palsy
are as follows:
1. Incomitant esotropia Initialy esotropia occurs
in the involved eye (Fig. 12.23A) due to
unopposed action of the medial rectus muscle
and is proportional to the loss of LR function.
The esotropia increases in gaze towards the
paretic lateral rectus muscle (Fig. 12.23B) and
patient becomes orthotropic in the opposite gaze
(Fig. 12.23C). When tested with cover test,
Fig. 12.22 Mechanism of sixth nerve palsy resulting from secondary deviation in the uninvolved eye is
raised intracranial pressure.
larger than the primary deviation.
Later on due to secondary MR contracture, the
5. Lesions of the intracavernous part of 6th weaken abduction function is further limited and
nerve. Since the sixth nerve runs through the esotropia occurs in contralateral gaze too. Ocular
middle of the cavernous sinus, it is more prone deviation should be measured with face turn with
to damage than the other nerves from the forced primary position and in lateral gaze. V-
intracavernous lesions such as aneurysms, pattern may result from lessened abduction and
meningioma, carotid cavernous fistulae and
Tolosa-Hunt syndrome (granulomatous inflam-
mation). In contrast to third nerve, aneurysms
rarely cause a sixth nerve palsy. Vascular causes
such as diabetes and hypertension are, however,
common causes of sixth nerve palsy.
Since in its intracavernous part, the sixth nerve
is joined by the sympathetic branch from
paracarotid plexus; so occasionally sixth nerve
palsy may be accompanied by Horner's
syndrome.
6. Lesions of the intraorbital part of sixth nerve.
Isolated sixth nerve palsy due to intraorbital
lesions is not so common. However, it is
involved in the lesions producing orbital apex
syndrome and superior orbital fissure syndrome.
Causes of acquired 6th nerve palsy in
descending order of frequency are:
• Tumours (45%)
• Raised intracranial pressure (such as from
idiopathic intracranial hypertension or
hydrocephalus)
Fig. 12.23 A patient with left lateral rectus palsy having left
• Trauma esotropia in primary gaze (A) which increases in left lateral
• Inflammations gaze (B) and becomes orthotropic in right gaze (C).
 Incomitant Strabismus 313

unopposed action of MR in downgaze. There may Differential diagnosis

be presence of vertical deviation less than 3 PD
Sixth cranial nerve palsy should be differentiated
due to removal of inhibitory effect of LR on
from:
vertical movements. If hyperdeviation is larger
than 5 PD, rule out 4th nerve palsy too. i. Duane's retraction syndrome (page 334).
2. Abnormal head posture is frequently associated ii. Congenital esotropia with crossed fixation—
with incomitant esotropia. Face is turned towards pseudoabducens paralysis (page 219), and
the action of the paralysed lateral rectus. iii. Nystagmus blockage syndrome.
3. Diplopia may not be a problem in visually
Management
immature children. However, visually mature
old patients have an uncrossed horizontal I. Clinicoinvestigative work-up and treatment of
diplopia with maximal image separation at the cause
distance and to the side of involvement The diagnosis of 6th cranial nerve palsy as such
(Fig. 12.24). does not pose any problem. However, aetio-
Initially with near fixation, equilibrium in logical diagnosis especially in cases of isolated
convergence can occur without diplopia but lateral rectus muscle palsy may not be possible
later on with greater and older deviations and in many a cases.
with secondary contractures permanent diplopia • Careful history should be taken to define
occurs for distance and near. antecedent infections, head trauma, or other
4. Ocular movements in a patient with lateral possible inciting factors for sixth nerve paresis.
rectus muscle paralysis (e.g. of right eye) are • In children less than 16 years with no neurological
affected as below: signs and symptoms, no work up is required.
• Abduction in right eye is limited due to Close follow up every 2 weeks initially and
paralysis of lateral rectus muscle. then monthly is required. Persistent palsy
• Overaction of right eye on levoversion. after 3 months requires evaluation.
• Overaction of left eye on dextroversion. • In young adults, 16 to 40 years, risk factors
• Underaction of left eye on levoversion. should be ruled out.
• In older patients more than 40 years, look for
vascular causes such as hypertension, diabetes,
atherosclerosis.
• If more than 55 years, giant cell arteritis should
be ruled out.
• Haematological investigations like fasting blood
glucose, complete blood count, ESR, ANA
titres, RPR test, FTA-ABS, TFT should be
carried out.
• Lumbar puncture is required in cases of raised
intracranial tension.
• Orbital ultrasound should be performed to rule
out enlarged extraocular muscles.
• Binocular diplopia free fields to be evaluated
by Goldmann perimeter using III 4e target
size.
• Neurological evaluation with computerized
tomography or magnetic resonance imaging is
indicated:
Fig. 12.24 Diplopia chart of a patient with right lateral rectus – When neurological signs or symptoms are
palsy. present.
 314 Theory and Practice of Squint and Orthoptics

– In the presence of multiple cranial nerve eye is advised to stimulate fixation of affected
palsies, and eye in abduction and relaxation of ipsilateral
– In younger patients without muscular causes. MR, to prevent contracture of medial rectus.
Note. After an exhaustive investigative work- • In cases with severe palsy alternate occlusion
up, if some cause is found and when treatable, is preffered as it alternates fixation, relieves
appropriate measures should be taken; which diplopia prevents medial rectus contracture,
by and large are the domain of neurophysicians and prevents amblyopia.
and/or neurosurgeons. ii. Fresnel press-on prisms are useful to correct
the diplopia in primary position.
II. Conservative measures iii. Botulinum toxin injection into the antagonist
A wait and watch for a minimum period of 6–8 medial rectus causes its paralysis and is thus
months is must, when self-improvement is useful for a temporary alignment of the eyes in
expected, especially in idiopathic cases and cases the primary position. Such injections are also
with benign palsies. Following conservative useful in preventing the contracture of the
measures may be useful during this period: medial rectus muscle, while the patient is
1. Measures to expedite recovery from palsy observed for several months prior to surgical
intervention. The effect lasts for 2–3 months.
i. Vitamin B-complex may be used as a neurotonic. May be repeated if necessary.
ii. Systemic steroids may hasten the recovery in 3. Wait and watch Spontaneous resolution of
patients with non-specific inflammations. the sixth nerve paresis, sometimes occurs,
2. Measures to prevent amblyopia and relieve making the surgery unneccessary.
diplopia. The main aim of conservative • Hess screen charting (Fig. 12.25) is useful for a
measures while waiting for spontaneous meticulous follow-up of the patients during
recovery is to provide relief from diplopia and this wating period.
additionally in children to prevent amblyopia
and preserve binocular vision. Following III. Surgical treatment
measures may be useful: Surgery is indicated, when spontaneous
i. Patching or occlusion is advised as below: resolution does not take place after 6 months or
• In cases with mild esodeviation and residual more of follow-up and after exclusion of the
LR function intact, occlusion of the unaffected intracranial lesions.

Fig. 12.25 Hess chart of a patient with paralysis of right lateral rectus muscle.
 Incomitant Strabismus 315

Aim of surgical treatment is to correct the tendons are disinserted and sutured with the
incomitant esotropia, improve abduction, tendon of lateral rectus at its insertion. This
provide a useful field of binocular single vision procedure combined with a recession of the
and to eliminate the abnormal head posture. medial rectus is recommended by von Noorden
Recommended surgical measures are as below: in children with complete paralysis of sixth
I. Recess-resect operation. A supra maximal cranial nerve.
(12–16 mm) recession of the antagonist medial 4. Carlson and Jampolsky transposition
rectus with about 8–10 mm resection of the procedure. In this procedure, medial rectus
lateral rectus muscle is often a successful first muscle is spared. After splitting the vertical
operation in most patients with incomplete recti, their temporal halves are inserted under
paralysis. This procedure often provides a useful the lateral rectus. It is essential to separate the
field of binocular single vision and eliminates halves of the vertical recti to the deepest point
the abnormal head posture. toward the apex of the orbit, allowing the
• In case a mild paresis is still present, weakening transposed parts of the vertical recti to slide
of the contralateral medial rectus muscle with or toward the LR, which also minimises vertical
without Faden procedure may be considered deviations. This procedure reduces the angle
as a second operation. of deviation below 10PD, improves abduction,
• Adjustable suture surgery may be helpful for prevents relapses, and also prevents anterior
final adjustments in co-operative patients with segment ischemia.
paralytic squint. 5. Superior rectus transposition with MR
recession have also shown good results in
II. Muscle transposition procedures recommended literature. This procedure is successfully tried in
for a complete paralysis of lateral rectus muscles. lateral rectus palsy and also in abduction deficit
Medial rectus recession should also be combined conditions like Duane’s syndrome and Mobius
especially, when there is medial rectus syndrome, especially if hypertropia is also
contracture. Forced duction test is useful in associated. It may induce vertical deviation also.
discovering MR contracture.
III. Contralateral medial rectus muscle
1. Jensen's procedure combined with the medial recession with or without Faden operation can
rectus recession is useful by balancing the be considered an option for the residual lateral
partially active forces. In the Jensen's rectus abduction deficit and/or residual
procedure, the superior rectus, inferior rectus esotropia, often the above surgical measure.
muscles and the paralysed lateral rectus muscle
are split for 8–10 mm from their insertion
MEDIAL RECTUS PARALYSIS
backwards with the help of a muscle hook.
Then the superior half of the lateral rectus is Isolated medial rectus paralysis is extremely
united with the lateral half of the superior rare, yet do occur, often without any satisfactory
rectus and the inferior half of the lateral rectus explanation.
with the lateral half of inferior rectus with the
help of a non-absorable suture (e.g. 5–0 Etiology
Mersilene). The knot should be tied near the Exact etiology of this rare entity is not known.
equator (Fig. 15.22). However, trauma and vascular disorders may
2. Hummelscheim operation. In this procedure, be implicated in occasional cases.
after spliting the superior and inferior recti, their
lateral halves are disinserted and sutured to the Clinical features
tendon of lateral rectus muscle. This operation 1. Incomitant exotropia occurs in the primary
is rarely done nowadays and is thus mainly of position in the involved eye (Fig. 12.26A) due
historical interest. to unopposed action of the lateral rectus. The
3. Berens and Girard procedure. In this exotropia increases in gaze towards the
procedure, the full inferior and superior rectus paralysed medial rectus (Fig. 12.26B). The
 316 Theory and Practice of Squint and Orthoptics

Fig. 12.27 Diplopia chart of a patient with right medial

Fig. 12.26 A patient with right medial rectus paralysis rectus palsy.
having right exotropia in primary gaze (A) which increases
in left gaze (B) and the patient becomes orthotropic in right Treatment
gaze (C).
1. Conservative treatment is similar to that of
patient becomes orthotropic in the opposite gaze lateral rectus palsy (page 314).
(Fig. 12.26C). 2. Surgical treatment, when indicated, consists
• Secondary deviation in the uninvolved eye, of:
when the patient fixates with the paretic eye • Recession of the antagonist lateral rectus
is larger than the primary deviation. combined with resection of the involved
2. Abnormal head posture is frequently associated medial rectus. The amount of surgery depends
with incomitant exotropia. Face is turned towards upon the size of deviation.
the action of the paralysed medial rectus. • Vertical rectus transposition to the medial rectus
3. Diplopia. A crossed horizontal diplopia with muscle is also recommended as an alternative
maximal image separation while looking procedure.
towards opposite side and during near fixation
is appreciated (Fig. 12.27). INFERIOR RECTUS MUSCLE PALSY
4. Ocular movements in a patient with paralysis An isolated paralysis of the inferior rectus
of medial rectus (e.g. of right eye) affected are muscle is extremely rare condition.
as below:
• Adduction in right eye is limited. Etiology
• Overaction of right eye on dextroversion 1. Congenital. Inferior rectus palsy, a rare entity,
• Overaction of left eye on levoversion is often congenital.
• Underaction of left eye on dextroversion. 2. Acquired. Occasionally, inferior rectus palsy
5. Hess screen charting is as shown in Fig. 12.28. may be due to an injury of the orbit (either at
the time of injury or at the time of repair of the
Differential diagnosis orbital floor fracture), myasthenia gravis,
Isolated medial rectus palsy should be differen- vascular diseases and following a peribulbar or
tiated from internuclear ophthalmoplegia (see retrobulbar anaesthesia for any intraocular
page 333). surgery.
 Incomitant Strabismus 317

Fig. 12.28 Hess chart of a patient with right medial rectus paralysis.

Clinical features 6. Ocular movements in a patient with inferior

1. Deviation. In primary position, the involved rectus palsy (e.g. of right eye) are affected as
eye is hypertropic and slightly intorted due to below (Fig. 12.29 I to IX):
the unopposed action of the antagonistic • Movements of right eye are limited, when
superior rectus muscle (Fig. 12.29A). When looking down and right (Fig. 12.29 VII).
patient fixates with the paretic eye, secondary • Overaction of right eye on looking up and to
deviation in the opposite eye consists of right (Fig. 12.29 I).
depression and intorsion more than the primary • Overaction of left eye on looking down and to
deviation. right (Fig. 12.29 VII).
2. Pseudoptosis can be appreciated in the • Underaction of left eye on looking up and to
normal eye, when patient fixates with the right (Fig. 12.29 I).
paretic eye. This occurs because of the drooping 7. Hess screen pattern seen in right inferior
of the upper lid associated with moving down rectus palsy is shown in Fig. 12.31.
of the globe.
Treatment
3. Widening of palpebral fissure may be seen in
1. Conservative treatment is similar to as
the paretic eye, when patient fixates with the
described for lateral rectus palsy (page 314).
normal eye. This occurs due to upward
2. Surgical treatment, when indicated should be
movement of the upper eyelid associated with
planned depending upon the size of the
the elevation of the globe.
deviation and results of forced duction test
4. Abnormal head posture is often present and (FDT) as below:
consists of slight chin depression, face turn and i. Recession of the superior rectus should be
head tilt towards the affected side (Fig. 12.29B). performed, if FDT shows restriction of this
5. Diplopia (Fig.12.30). Image seen by the muscle.
involved eye is lower, crossed and extorted; ii. Resection of inferior rectus is preferred, when
vertical separation increases, when looking FDT is negative and deviation is small.
down and to the same side; extorsion increases, iii. Resection of inferior rectus and recession of the
when looking towards the opposite side. superior rectus should be combined, if the
 318 Theory and Practice of Squint and Orthoptics

Fig. 12.29 A patient with right inferior rectus paralysis: A, Right hypertropia with left eye fixating; B, Abnormal head posture;
and I to IX, Ocular movement in nine positions of gaze (Courtesy: Dr Subhash Dadeya).

deviation is large. This surgery can correct

up to 30–40D of vertical deviation in the
primary position.
iv. Other options include IR plication, reverse
Knapp, IO anterior transposition.

SUPERIOR RECTUS PARALYSIS

An isolated paralysis of superior rectus muscle
is a rare entity. Since the superior division of
the third cranial nerve innervates both superior
rectus and levator muscle, so weakness of
superior rectus may be associated with
weakness of levator and thus a ptosis in the
affected eye.

Etiology
Fig. 12.30 Diplopia chart of a patient with right inferior 1. Congenital. Isolated superior rectus muscle
rectus paralysis. palsy is usually congenital.
 Incomitant Strabismus 319

Fig. 12.31 Hess chart of a patient with right inferior rectus paralysis.

2. Acquired. SR palsy may be secondary to

trauma (e.g. after a bridle suture during cataract
surgery) or occasionally be associated with
vascular disorders and viral infections.

Clinical features
1. Deviation. When the normal eye is fixing, the A
paretic eye is hypotropic in primary position
(Fig. 12.32A). Usually, there is no vertical
deviation in adduction. The inferior rectus
overacts and consequently objective excyclodevia-
tion develops which can be detected by indirect
ophthalmoscopy or by plotting the blind spot in
the visual field. Subjective cyclodeviation is
typically absent, since in most cases the condition B
is congenital. When patient fixates with the
Fig. 12.32 A patient with right superior rectus paralysis and
paretic eye the normal eye is hypertropic, slightly pseudoptosis and hypotropia in the affected eye while
abducted and slightly extorted; more than the fixating with the left nonaffected eye (A). When fixating with
primary deviation (Fig. 12.32B). the paretic right eye, pseudoptosis disappears and the
nonparetic left eye becomes hypertropic (B).
2. Ptosis may be associated in the affected eye,
if LPS is also weak. in some recent cases, chin may be elevated, face
3. Pseudoptosis may be associated with the turned to the same side and head tilted towards
hypotropic globe in primary position (Fig. 12.32A). the normal side. In most patients, after
It must be differentiated from the true ptosis. sometimes, head is tilted towards the same side
Pseudoptosis disappears, when the patient due to overaction of the yoke muscle (opposite
fixates with the paretic eye. inferior oblique).
4. Abnormal head posture is not always present 5. Diplopia may be present in recent cases only.
in patients with superior rectus palsy. However, For example, in a patient with paralysis of right
 320 Theory and Practice of Squint and Orthoptics

• Movements of right eye are limited, when

looking towards up and right.
• Overaction of right eye on looking down and
to right.
• Overaction of left eye on looking up and to
the right.
• Underaction of left eye on looking down and
to right.
7. Hess screen charting pattern seen in right
superior rectus palsy is shown in Fig. 12.34.

Differential diagnosis
1. Primary superior rectus palsy needs to be
differentiated from the inhibitional palsy of the
superior rectus (contralateral antagonist)
secondary to weakness of the superior oblique
muscle of the opposite eye.
Fig. 12.33 Diplopia chart of a patient with right superior
rectus palsy. 2. Paretic weakness of the superior rectus
should be differentiated from the restrictive
superior rectus, image seen by the involved eye limitation of movements following entrapment
is higher, crossed and intorted (Fig. 12.33). of inferior rectus and/or soft tissue in fracture
Vertical separation increases, when looking up floor of the orbit and contracture of inferior
and to the right; intorsion increases, when rectus following myositis, after cataract surgery,
looking to left. ocular epibulbar anaesthesia.
6. Ocular movements in a patient with right sup- Forced duction test, active force generation
erior rectus muscle paralysis are affected as test and saccadic velocity test are quite useful
below: in differentiating paretic limitation from
restrictive limitation of movements (page 300).

Fig. 12.34 Hess chart of a patient with right superior rectus palsy.
 Incomitant Strabismus 321

Treatment
1. Conservative treatment is on the general lines
(page 314).
2. Surgical treatment of isolated superior rectus
muscle paralysis, depending upon the size of
deviation, is as follows:
i. Recession of inferior rectus by 4 mm is sufficient
for a deviation up to 15.
ii. Recession of inferior rectus by 4 mm combined
with 4 mm resection of superior rectus may
correct a vertical deviation of 30–40 in
primary position.
iii. Weakening procedure on the inferior oblique of
the non-paretic eye may be required in
addition to the recess-resect procedure, when
the deviation is more than 40.

INFERIOR OBLIQUE PALSY Fig. 12.35 Right inferior oblique palsy. Note: A, right hypo-
tropia in primary gaze; B, underaction of right inferior oblique
Isolated inferior oblique palsy is the least on levoelevation and C, secondary overaction of right superior
common of all extraocular muscle palsies. oblique on levodepression.

vertical separation increases, when looking up

Etiology
and to left; extorsion increases, when looking to
Inferior oblique palsy may be congenital or right (Fig. 12.36).
acquired. Acquired disorders like stroke, syphilis
5. Ocular movements in a patient with paralysis
and myasthenia gravis and trauma can also
of inferior oblique muscle, say of right side, will
cause IO palsy.
be affected as below:
• Underaction of right inferior oblique, i.e.
Clinical features movements of right eye are limited, when
1. Deviation. When fixing with the normal eye, looking up and to left (Fig. 12.35B).
patient will have hypotropia of the involved eye
(Fig. 12.35A) and when fixing with the paretic
eye, patient will have hypertropia of the normal
eye in the primary position. The greatest
deviation occurs, when the patient attempts to
elevate the adducted paretic eye (Fig. 12.35B).
The deviation worsens with horizontal gaze into
the field of action of affected IO or with head
tilt towards the side opposite the paretic eye.
2. Abnormal head posture consists of chin
elevation, head tilt towards the paralyzed side,
and the face is turned towards the uninvolved
side, i.e. away from the side of palsy.
3. Bielschowsky head tilt test is positive on
tilting the head towards the normal side.
4. Diplopia is usually not a complaint, since in
most of the cases, onset is congenital. However,
in a recent acquired case image seen by the Fig. 12.36 Diplopia chart of a patient with right inferior
paretic eye is higher, uncrossed and extorted; oblique palsy.
 322 Theory and Practice of Squint and Orthoptics

• Secondary overaction of right superior oblique, Treatment

i.e. overaction of right eye on looking down 1. Conservative treatment, when required, is on
and to left (Fig. 12.35C). the general lines (page 314).
• Overaction of left eye on looking up and to 2. Surgical treatment is frequently indicated
left. since in most cases, onset is congenital. Aims of
• Underaction of left eye on looking down and surgery are to correct abnormal head posture,
to left may occur due to inhibitional palsy of to correct deviation in primary position and to
contralateral IR. treat diplopia. The general principle of strengthe-
6. An objective incyclodeviation is evidenced ning the weak muscle and weakening the strong
on indirect ophthalmoscopy. muscle is not applicable in cases with inferior
7. Hess screen charting in a patient with inferior oblique palsy; since most workers have reported
oblique palsy is shown in Fig. 12.37. that inferior oblique resection or advancement
is usually unsatisfactory. Surgery has to be
Differential diagnosis individualized approach depending on amount
Brown's superior oblique sheath syndrome can of vertical deviation, spread of comitance and
be differentiated from inferior oblique palsy as degree of increase in deviation in ipsilateral
below: gaze. Therefore, recommended treatment is as
below:
• In Brown's syndrome the involved eye is
frequently depressed more severely in i. Superior oblique tenotomy should be
adduction than it is with inferior oblique performed in the involved eye, when
paralysis. superior oblique overaction is marked, i.e.
weakening of ipsilateral antagonist.
• Overaction of superior oblique is minimal
ii. Recession of the superior rectus (i.e. weakening
or absent in Brown's syndrome.
of contralateral yoke muscle, and resection of the
• Forced duction test is positive in Brown's
inferior rectus (i.e. strengthening of contra-
syndrome.
lateral antagonist) of the normal eye is reco-
• 'A' pattern is typically present with inferior
mmended, when there is not much overaction
oblique palsy, whereas a 'V' pattern in typical
of the superior oblique muscle in the
of Brown syndrome.

Fig. 12.37 Hess chart of a patient with right inferior oblique palsy.
 Incomitant Strabismus 323

involved eye. Though, this procedure will i. One view is that basically weakness of the
not improve the action of the inferior oblique elevation is caused by a superior rectus palsy
muscle, it will restore comitance in the paretic of long-standing and that due to spread of
field of gaze. comitance the field of action of inferior
oblique is involved.
DOUBLE ELEVATOR PALSY (MONOCULAR ii. Other workers have suggested it to be a
ELEVATION DEFICIENCY) supranuclear elevation insufficiency.
The term 'double elevator palsy', literally means Supranuclear MED is characterized by intact
paralysis of both elevators (superior rectus and or mildly reduced vertical saccadic velocity
inferior oblique) of the same eye; resulting in a below midline and absent velocity above
clinical condition characterized by elevation midline with Bell’s phenomenon being
deficiency in the entire range of upward gaze, usually present. While in SR palsy the
i.e. in primary gaze, in adduction as well as in vertical saccades are slowed both above and
abduction. In the literature, many cases have below the midline and Bell’s phenomenon is
been reported with similar clinical characteristics absent.
(i.e. limitation of elevation in entire upgaze) b. Acquired elevator weakness is usually
occurring due to mechanical restrictions sudden in onset and nearly muscular in
involving the inferior aspect of the globe origin. It has been suggested by some workers
(without any associated palsy of elevators). Even that vascular insufficiency in a penetrating
some cases with mixed etiology have also been vessel to the pretectum is the likely cause of a
reported. Keeping in view the above, it will be postulated unilateral supranuclear lesion just
better to use the term 'Monocular elevation rostral to the oculomotor nuclear complex.
deficiency (MED) syndrome', for the patients 2. Mechanical restriction to elevation of globe
having such an elevation deficiency. is another cause of monocular elevation
deficiency. This group is characterized by:
Etiological types of monocular elevation
• Positive forced duction to elevation,
deficiency
• Normal active force generation (no muscle
Monocular elevation deficiency may be either paralysis), and
congenital or acquired. Depending upon the • Normal saccades of the superior rectus.
underlying factors responsible, the monocular
elevation deficiency can be classified into Etiology. Its reported causes are:
following three groups: • Congenital or acquired fibrosis involving the soft
1. Double elevator weakness. This entity is tissue in the inferior aspect of the globe.
characterized by: • Anomalous insertion of the inferior rectus
i. Negative forced duction test, muscle.
ii. Reduced force generation of elevators Note. Other forms of mechanical restrictions
(evidence of paralysis), and such as those associated with fractures of the
orbital floor and endocrine myopathy also cause
iii. Reduced saccadic velocities in upgaze
elevation deficiency, but cannot be included as
movements of affected eye.
the cause of restrictive form of 'monocular
Etiology of the combined weakness (paresis) of deficiency syndrome'. In fact, these are well
superior rectus and inferior oblique of the same established separate entities and need to be
eye is not known exactly and is difficult to differentiated for management point of view.
explain even anatomically. Possible explanations 3. Combination of restriction and weakness.
given for the two types (congenital and Monocular elevation deficiency in this group is
acquired) of double elevator weakness are as caused by inferior rectus restriction and also
follows: weak elevators. This group is characterized by:
a. Congenital elevator weakness. There are two • Positive forced duction in elevation (evidence
views about it: of restriction).
 324 Theory and Practice of Squint and Orthoptics

• Reduced force generation of elevators of i. Hypotropia of the affected eye, and

involved eye (evidence of paralysis). ii. Vertical diplopia
• Reduced upward vertical saccadic velocities
2. Fixation with uninvolved eye. These patients
in involved eye (evidence of paralysis).
have following features:
i. Hypotropia in involved eye (Fig. 12.38E)
Clinical features
ii. Pseudoptosis accompanying hypotropia
I. Vertical deviation, head posture, diplopia and
(Fig. 12.38E). (The ptosis disappears, when
ptosis are the cardinal features of monocular
the patient fixates with the paretic eye
elevation deficiency. Manifestation of these
(Fig. 12.38F).
features depends upon the presenting pattern.
Depending upon the fixation pattern adapted iii. True ptosis due to associated LPS weakness,
by a particular patient (because of many known may be present in 50% of cases. True ptosis
and unknown factors), following three presen- does not resolve when patient fixes with
tation patterns are known in patients with paretic eye. Pseudoptosis and true levator
monocular elevation deficiency: weakness may also occur simultaneously.
iv. Amblyopia of the affected eye with a visual
1. Binocular fixation. Some patients prefer to acuity of 6/60 or less.
keep their head tipped backward and maintain
v. Diplopia is absent.
a binocular fusion. In fact, the posture adopted
by these patients moves the eyes, by means of 3. Fixation with involved eye . Because of certain
the ''dolls head phenomenon'', into the field of predispositions, some patients prefer to fix with
action of the depressors of the globe. Visual the involved eye. These patients present with
acuity is usually normal in these patients. following features:
However, when these patients hold their head i. Hypertropia of normal eye which is greater
erect in primary position, there may occur: due to secondary deviation (Fig. 12.38F).

Fig. 12.38 Monocular elevation deficiency of the left eye. Note: limitation of elevation in upgaze (B), on levoelevation (C),
and dextroelevation (A). Note left ptosis in primary gaze when fixing with normal eye (E) and marked hypertropia due to
secondary deviation in normal right eye when fixing with the paretic right eye (F).
 Incomitant Strabismus 325

ii. Visual acuity in the normal eye may be Surgical procedures recommended are as
reduced. follows:
iii. True ptosis due to associated weakness of LPS 1. Knapp's procedure is useful in patients with
may be present. elevator weakness without any restrictive
II. Limitation of elevation (in adduction as well involvement. In this procedure, medial rectus
as in abduction, both on versions and ductions) and lateral rectus muscles of the involved eye
beyond midline, is the main clinical characteristic are transposed to a position near the insertion
of this condition (Fig. 12.38 A to C). of the superior rectus muscle (Fig. 15.21).
2. Recession of the inferior rectus muscle is
III. Other clinical characteristics recommended in the presence of inferior
i. An extra or deep lower lid fold on the affected restriction for a vertical deviation up to 18–20D.
side may be seen in patients with inferior 3. Recession of the inferior rectus muscle plus
rectus muscle restriction. resection of the superior rectus is indicated, when
ii. Bell's phenomenon is usually very poor or in the presence of inferior restriction, the
absent in the eye with inferior rectus muscle deviation is more than 18 to 20D.
restriction as compared to the eye without 4. Recession of inferior rectus with Knapp’s procedure
inferior rectus muscle restriction. may be required in patients with MED due to
iii. Positional tonometry performed with applana- combined restriction as well as weakness of
tion tonometer shows a rise in IOP of more elevation. It is best to perform the operation in
than 3 mm Hg in 15o upward gaze as compared two stages. In the first stage, IR recession should
to that in primary gaze in patients with be done and at a later stage, if required, Knapp’s
restrictive limitations. No such significant procedure should be done. The second procedure
increase in IOP occurs in paretic weakness. should be done after 4 months to prevent anterior
segment ischemia. However, if both procedures
Differential diagnosis are performed in same sitting then ciliary vessel
Congenital elevator weakness should be sparing procedure should be preferred.
differentiated from: Note. Knapp’s procedure usually corrects 20–
• Brown's syndrome 35 PD of hypotropia in primary position. Knapp
• Congenital fibrosis of inferior rectus muscle. procedure after IR recession usually has greater
• Vertical Duane's syndrome effect.
• Congenital absence of SR 5. Augmented transposition using posterior
• Congenital palsy of superior division of 3rd fixation suture should be done for large
nerve. hypotropia >35 PD, with no IR restriction.
6. Modified Knapp’s. If horizontal deviation is also
Acquired elevator weakness should be
present then recess-resect and also transpose the
differentiated from:
MR and LR muscles.
• Blow-out fracture of orbital floor with
incarcination of inferior rectus muscle. DOUBLE DEPRESSOR PALSY
• Thyroid ophthalmopathy with inferior rectus The term 'double depressor palsy' literally
myopathy. means paralysis of both depressors (inferior
• Acquired fibrosis of IR rectus and superior oblique) of the same eye
• Anomalous insertion of inferior rectus muscle. resulting in a clinical condition characterized by
• Abnormal accessory muscle between annulus of depression deficiency in the entire range of
Zinn and the posterior part of the globe. downward gaze, i.e. in primary gaze, in
Treatment adduction as well as in abduction.
Indications of treatment include: Etiology
• Large vertical deviation in primary position The exact etiology of this rare congenital
with or without ptosis, and condition is not known. Further, even anato-
• Significant abnormal head posture. mically it is not possible to explain the
 326 Theory and Practice of Squint and Orthoptics

involvement of two muscles innervated from 2. Ocular movements. Ductions are limited in
different nuclei. It has been suggested that the the entire lower field of gaze (Fig. 12.39A II to
condition double depressor palsy might result IV and BVII to IX) and normal in all other gaze
from any of the following situations: positions.
1. A long-standing inferior rectus palsy with
spread of comitance to the field of the Differential diagnosis
ipsilateral superior oblique muscle. Double depressor palsy should be differentiated
2. A long-standing inferior rectus palsy and from mechanical restriction. Forced duction test
secondary superior rectus contracture. and saccadic velocity measurements are helpful
3. A long-standing superior oblique palsy with in differentiating paresis from mechanical
spread of comitance to the ipsilateral inferior
restrictions.
rectus muscle.
Clinical features Treatment
1. Deviation. The paretic eye is hypertropic in 1. Inverse Knapp's procedure is useful in
primary position, when the normal eye is fixing patients having a definite weakness. In this
(Fig. 12.39A I and B V). procedure, lateral and medial recti are

I II III

IV V VI

VII VIII IX

Fig. 12.39 Double depressor palsy right eye: A, Diagrammatic representation; and B, clinical photograph in nine positions
of gaze (Courtsey Dr Shubhang Bhave). Note right hypertropia in primary position (A I and B V) and limitation of depression
in central downgaze (A II and B VIII), dextrodepression (A III and BVII) and levodepression (A IV and B IX).
 Incomitant Strabismus 327

disinserted and reattached near the insertion 1. Nuclear lesions

of inferior rectus. • Lesions involving purely the third nerve
2. Recession of the superior rectus muscle may nucleus complex are relatively uncommon.
be performed in patients with superior • Common causes include vascular diseases,
mechanical restrictions. demyelination, primary tumours and
metastasis.
• Lesions involving entire nucleus cause an
TOTAL THIRD NERVE PARALYSIS
ipsilateral third nerve palsy with ipsilateral
The third cranial (oculomotor) nerve is entirely sparing and contralateral weakness of
motor in function. It supplies all the extraocular elevation.
muscles except lateral rectus and superior • Lesions involving paired medial rectus sub-
oblique. It also supplies the intraocular muscles nuclei (ventromedial nucleus) cause a wall-
namely—sphincter pupillae and ciliary muscle. eyed bilateral internuclear ophthal-moplegia
Total paralysis of third cranial nerve may be (WEBINO), characterised by defective
congenital or acquired. While acquired paralysis convergence and adduction.
of third nerve is of common occurrence, the 2. Fascicular lesions
congenital palsies are uncommon. • Causes are similar to nuclear lesions.
Note. Applied anatomy of third nerve and its • Benedikt's syndrome characterised by
nucleus should be reviewed before proceeding ipsilateral third nerve palsy associated with
further (see page 12). tremors and jerky movements of the contra-
lateral side, occurs due to lesions at the
ETIOLOGY intermediate level of the midbrain. Third
nerve paralysis occurs due to involvement of
A. Congenital third cranial nerve palsy fasciculus as it passes through the red nucleus.
It has been reported to occur due to following • Weber's syndrome. In it fascicular part of the
causes: nerve is involved, while passing through
1. Developmental aplasia or hypoplasia of the the cerebral peduncle. The syndrome is
third nerve nucleus or the nerve is extremely characterised by ipsilateral third nerve palsy,
rare. contralateral hemiplegia and facial palsy of
2. Intrauterine trauma has been mentioned as a upper motor neuron type.
cause of congenital third nerve palsy. 3. Lesions involving basilar part of the nerve
• As the nerve runs in the subarachnoid space
3. Birth trauma. It is believed that most
at the base of skull unaccompanied by any
congenital third nerve palsies are caused by
other cranial nerve, isolated third nerve
deformation of the soft skull of the fetus by the
palsies are frequently basilar.
moulding forces during labour. Probably, • Causes: (1) Aneurysms at the posterior
compression of the third nerve occurs at some communicating artery cause isolated third
point between the brainstem and the entry of nerve palsy with involvement of pupil.
the nerve into the cavernous sinus. (2) Extradural haematoma which may cause
4. As a part of cerebral palsy. Third nerve palsy tentorial pressure cone with downward
may occur as one of the components of cerebral herniation of the temporal lobe this compresses
palsy. the third nerve as it passes over the tentorial edge
5. Infantile intracranial infections such as (Fig. 12.40). Initially there occurs fixed dilated
encephalitis and meningitis have also been pupil, which is followed by a total third nerve
implicated as possible causes. palsy. (3) Diabetes causes isolated 3rd nerve palsy
with sparing of the pupillary reflexes.
B. Acquired third nerve palsy 4. Lesions involving intracavernous part of the
The causes which can involve the third nerve at nerve
various levels with their peculiar features are • Because of its close proximity to other cranial
as follows: nerves, intracavernous third nerve palsies
 328 Theory and Practice of Squint and Orthoptics

• Vascular causes of third nerve palsy include

diabetes and hypertension. Diabetic cranial
mononeuropathy is a well-known cause of
third cranial nerve palsy. It is characterized
by rapid onset, periocular pain, pupil sparing
with complete resolution within 2–3 months
without development of aberrant regeneration.
• Herpes zoster ophthalmicus can involve the
third nerve by means of contiguous spread
from the ophthalmic division of the fifth
cranial nerve to other cranial nerves or they
pass through superior orbital fissure.
• Ophthalmoplegic migraine may cause partial
or complete 3rd nerve palsy especially in
children under 10 years. It is characterized
by periocular pains, nausea and vomiting
followed by ophthalmoplegia.
Fig. 12.40 Mechanism of third nerve palsy in extradural
haematoma.
• First syndrome, characterized by a bilateral,
progressive and painless ophthalmoplegia,
are usually associated with involvement of is another neuropathic cause of third nerve
the fourth, the sixth nerve, and the first palsy. It is a variant of Gullian-Barre
division of the trigeminal nerve. syndrome. It may be associated in the
• In intracavernous 3rd nerve palsy, pupil is bilateral peripheral facial weakness.
spared. Sometimes, pupil may be constricted
Clinical groups of causes of third nerve palsy
owing to involvement of sympathetics.
• Causes: (1) Pituitary apoplexy may cause a Causes of isolated third nerve palsy can be
third nerve palsy as a result of haemorrhagic summarized as below:
infarction of a pituitary adenoma (e.g. after 1. Idiopathic. In about 25% cases, cause is not
child birth), with lateral extension into the known.
cavernous sinus. (2) Intracavernous lesions 2. Vascular diseases. Diabetes and hypertension
which may cause a third nerve palsy include are the common medical causes of pupil sparing
aneurysms, meningiomas, carotid cavernous isolated third nerve palsy.
fistulae and Tolosa-Hunt syndrome (granulo- 3. Trauma is also an important cause of isolated
matous inflammation). third nerve paralysis. Mechanism of third nerve
5. Lesions of the intraorbital part of the nerve involvement by an extradural haematoma is
• May cause isolated extraocular muscle shown in Fig. 12.40.
palsies or may involve either superior division 4. Aneurysm at the junction of the posterior
or inferior division or both divisions. communicating artery with the internal carotid
• Involvement of superior divison of 3rd nerve artery is an important cause of isolated, painful
is commonly associated with other cranial third nerve palsy with involvement of the pupil.
nerve palsies, whereas involvement of Other causes of painful third nerve palsy are
inferior divison of 3rd nerve is frequently migraine, Tolosa-Hunt syndrome and diabetes.
isolated. 5. Miscellaneous. Other rare causes include
• Causes: Orbital tumours, pseudotumours, tumour, vasculitis associated with collagen
trauma, viral illness and vascular diseases. disorder, syphilis and tuberculosis.
6. Neuropathic processes which can involve the Causes of third nerve palsy in children
third nerve after it leaves the midbrain are as • Congenital (40–50%)
follows: • Traumatic
 Incomitant Strabismus 329

• Inflammatory
• Post-viral syndromes
• Migraine
• Neoplasms (rarely)

Causes of third nerve palsy in adults

• Aneurysm
• Diabetes
• Neuritis
• Trauma
• Infection
• Tumour (rare causes)

Causes of pupil sparing third nerve palsy.

Vascular causes such as diabetes and hyper-
tension which affect the vasa nervorum often
cause pupil sparing third nerve palsy. On the
other hand, compressive lesions usually cause Fig. 12.41 A patient with third cranial nerve paralysis showing
pupil dilatation due to compression of pial ptosis (A) and divergent squint (B).
vessels.
4. Pupil is fixed and dilated due to paralysis of the
CLINICAL FEATURES sphincter pupillae muscle. However, in pupil
Clinical features of complete third nerve sparing lesions, it is normal.
paralysis can be described as below: 5. Accommodation is completely lost due to
paralysis of the ciliary muscle. However, in
• General features
pupil sparing lesions, it is normal.
• Congenital versus acquired paralysis
6. Diplopia. A crossed horizontal diplopia with
• Features due to aberrant regeneration. slight vertical misalignment elicited on
• Cyclic oculomotor paralysis. manually raising the eyelid occurs due to
paralytic divergence squint with associated
General clinical features slight intorsion and downward rotation.
Depending upon the anatomical basis, in 7. Abnormal head posture. If the pupillary area is
general, clinical features of a case of total third uncovered, to avoid diplopia, head is moved in
nerve palsy (Fig. 12.41A and B) are as follows: a position consistent with the direction of action
1. Ptosis. It occurs due to paralysis of the levator of paralyzed muscles, i.e. face is turned on the
palpebrae superioris muscle. opposite side, head is tilted towards the affected
side and chin is slightly elevated.
2. Ocular deviation. Eyeball is turned down, out
and slightly intorted due to actions of the lateral Clinical features: congenital versus acquired
rectus and superior oblique muscles. third nerve palsy
3. Ocular movements (Fig. 12.42) are restricted due 1. Age. Congenital third nerve palsy is more
to paralysis of the various muscles as below: frequently seen in children, while acquired is
more common in adults.
• Adduction—due to medial rectus
2. Intraocular muscles are usually not involved
• Elevation—due to superior rectus and inferior in congenital third nerve palsy.
oblique 3. Ptosis is variable in congenital third nerve
• Depression—due to inferior rectus palsy, but, some degree of lid drooping is
• Extorsion—due to inferior rectus and inferior usually present while, in most acquired cases
oblique. there is complete drooping of the upper lid.
 330 Theory and Practice of Squint and Orthoptics

Fig. 12.42 Ocular movements and nine positions of gaze in a patient with right third nerve paralysis.

4. Suppression and amblyopia are quite common in a compressive injury of the oculomotor nerve,
in congenital paralysis. However, it is often e.g. those associated with birth trauma.
surprising to note good visual acuity in the Features due to aberrant regeneration of third
affected eye, because it is expected that the patient nerve (all of which may not necessarily be
always uses the normal eye and the affected eye present in a given patient) are as follows:
will have deep amblyopia. Probably visual acuity
1. Pseudo-Graefe's sign. It refers to elevation of the
is maintained because the patient uses the paretic
upper lid on attempted downgaze. It perhaps
eye in the down and out position, a position in
occurs due to miswiring of the nerve fibres
which nose precludes the use of normal eye. As
originally meant for inferior rectus with the nerve
a result, most patients with congenital third nerve
fibres going into levator palpebrae superioris.
palsies have only a small degree of amblyopia,
approximately 6/18 to 6/24. 2. Widening of the palpebral fissure on adduction
and narrowing of the fissure on abduction.
5. Aberrant regeneration is more frequently seen
in congenital palsy. 3. Pseudo-Argyl Robertson pupil. It refers to a
dilated fixed pupil that does not react to direct
Features due to aberrant or consensual light stimulation but does react
regeneration of third nerve slightly on convergence and also on adduction.
Aberrant regeneration can occur following 4. Retraction of the upperlid occasionally may be
congenital or acquired third nerve palsy. It may accompanied by contraction of the pupil.
occur even without a preceding oculomotor 5. Eyeball may be retracted and adducted on
paralysis in patients with a slowly growing attempted upgaze.
intracavernous meningioma or with a carotid
aneurysm. Aberrant regeneration is thought to Cyclic oculomotor paralysis
result from a miswiring of axons from the It is the rarest but an interesting form of third
proximal portion of the nerve into the peripheral nerve paralysis which is usually congenital in
segment of the nerve. The misdirection syndrome origin. As the name implies, it is characterized
(aberrant regeneration) follows more commonly by an alternate paresis and spastic contraction
 Incomitant Strabismus 331

of the extraocular and intraocular muscles taken care of. Further, diabetic mononeuro-
supplied by the third cranial nerve. The spastic pathy should always be ruled out by glucose
phase is shorter than the paretic phase. The two tolerance test in patients with pupil sparing
phases continue to alternate even in sleep but third nerve palsy.
disappear in deeper stages of anaesthesia. All such patients should be observed
1. Paretic phase is characterized by occurrence frequently and if pupil is involved or signs and
of ptosis, dilatation of pupil, impairment of symptoms of subarachnoid haemorrhage
accommodation, weakness of adduction, develop immediately, angiography is required
weakness of vertical movements and an to rule out aneurysm.
outward turning of the globe. 2. Tensilon test. One should remember the
2. Spastic phase is characterized by contraction dictum that any unexplained cause of diplopia
of the muscles—beginning with adduction and or ptosis requires a Tensilon test to exclude
elevation of the lid. It is followed by pupillary myasthenia gravis.
constriction and improvement in accommo- 3. ESR. In patients of more than 55 years of age
dation. Ultimately, the eye may return to with symptoms of polymyalgia rheumatica, ESR
primary position to be followed, shortly by the estimation should be done. If ESR is found high,
paretic phase. then temporal artery biopsy should be per-
formed to rule out temporal arteritis.
MANAGEMENT
Treatment of the cause
Management of third cranial nerve palsy remains
the most difficult, incomplete and least satisfying. If on investigation, a definite cause of third nerve
In general, the management includes investi- palsy such as intracranial aneurysm, diabetes,
gations, treatment of the cause, conservative myasthenia gravis, etc. is found, patient should
treatment and surgical treatment. be referred to neurosurgeon or neurophysician
depending upon the indication. However, if no
Investigations surgical cause is found, patients should be
Third nerve palsy of acute onset, especially if managed conservatively followed by extra-
non-pupil sparing, should be subjected to ocular muscle surgery, if required.
through neuro-ophthalmic evaluation and be Conservative treatment
investigated with prompt and appropriate
neurologic studies. 1. Observations. Like any other paralytic squint,
wait and watch for the self recovery should be
1. Magnetic resonance imaging (MRI) and
done at least for 6–8 months. During this period,
carotid angiography. Probably, it is best to
patient should be followed every 6 weeks, and
perform MRI in such cases and then proceed
at each follow-up visit, following examinations
further as below:
should be done.
a. In children below 10 years of age regardless of
• Measurement of exotropia and hypotropia
the state of pupil, if MRI is normal, carotid
with prism for cover test.
angiography is not essential because of the
less likelihood of aneurysm. • Diplopia charting.
b. In patients above 10 years of age with pupil • Hess charting.
involvement, if MRI shows a mass compatible 2. Amblyopia is frequently associated with third
with an aneurysm or even if MRI is normal, nerve paresis in paediatric patients and must be
perform carotid angiography to rule out sought and treated aggressively. Therefore,
aneurysm. surgical treatment to raise the ptotic lid is
c. In patients above 10 years of age with pupil needed urgently in children. Alternate patching
sparing, if MRI is normal, a thorough medical should be done to prevent occurrence of
evaluation should be conducted. In patients amblyopias.
of vasculopathy age group (>40 years), 3. Diplopia is difficult to treat with prisms,
hypertension and atherosclerosis should be because of its variable nature.
 332 Theory and Practice of Squint and Orthoptics

• Complete ptosis is useful in preventing diplopia oblique (SO) overaction, and lateral rectus (LR)
in visually mature patient. Therefore, ptosis contracture may further complicate the matter.
surgery should be deferred until after the eye Further, it should be explained to the patient or
has been straightened. parents that several operations will most likely
• Alternate patching is required to prevent be necessary to straighten the eyes and both
diplopia in visually mature patients with patience and understanding are important
incomplete ptosis. Opaque contact lens or throughout the course of treatment. It should
blurred spectacles can be used as alternative also be emphasized to the patient that inspite of
to patching. multiple operations, one can achieve functional
4. Botulinum toxin. Use of botulinum toxin is and cosmetic correction only in primary position
another nonsurgical option in the acute phase and not in different gazes.
of partial third nerve paresis. This is, especially
useful in cases of isolated involvement of MR Goals of surgery
muscle. It paralyses the antagonist LR • To improve alignment in primary gaze.
temporarily and thus neutralizes horizontal • To produce or enlarge some degree of
deviation in the primary position. It also binocular single vision.
prevents contracture of LR muscle. After
recovery of the injected muscle, the remaining Surgical procedures
vertical deviation may need to be corrected by Surgery should be contemplated, only if the
prisms or surgical therapy. Some patients may strabismus measurement and diplopia remain
not need surgery later on. Use of botulinum stable for 3 months (i.e. partial recovery has
toxin for vertical muscle imbalance is rarely stabilized). It usually occurs after 6–8 months
indicated, as SR should not be injected as ptosis of paralysis.
can occur if toxin is placed into the levator—SR Aim of surgery is to give alignment in the two
complex. important positions, i.e. primary and downgaze.
5. Vitamin B-complex may be used as neurotonic. Planning for the appropriate surgical procedure
6. Systemic steroids may hasten the recovery in must be dictated by the severity of the weakness
patients with non-specific inflammation. of the muscles as follows:
Further, in patients with temporal arteritis or
1. Surgery for exotropia (lateral rectus recession
rheumatological disorder, high doses of steroids
and medial rectus resection)
are recommended.
• In an incomplete palsy, recess-resect procedure
should be planned as done for comitant
Surgical treatment
exotropia.
General principles • In complete paralysis, Helveston and many
1. Wait and watch approach. Like any other others have advised supramaximal recession
paralytic squint at least 6–8 months should of lateral rectus (14–16 mm) and resection of
elapse before performing any surgical treatment medial rectus (8–14 mm), to align the eye in
to straighten the eye. primary position. But this has limited success
2. Surgery should be undertaken continuously in as overtime chronic contracture of LR and
patients with complete palsy and good binocular elongation of resected muscle, causes
visual function, since elevation of the lid and exotropic drift again.
incomplete realignment without useful single Myectomy of LR muscle to accomplish a super-
binocular fields may produce incapaci-tating maximal weakening effect of abduction in
diplopia. patients with complete third nerve palsy has
3. Surgery for third nerve paralysis is challenging also been recommended. However, this often
and the outcome must be discussed with the patient. results in recurrence of exotropia.
Associated factors such as the presence of ptosis, • Adjustable sutures during recess-resect
pupillary involvement, amblyopia, aberrant procedure are quite useful in co-operative
regeneration, poor Bell’s phenomenon, superior patients.
 Incomitant Strabismus 333

2. Surgery for hypotropia include: 6. Anchoring of the lateral rectus to periosteum

• Supraplacement of horizontal recti during recess- of lateral orbital wall by non-absorbable. 5-0
resect procedure is preferred by some surgeons. mersilene suture along with 8-0 mm of medial
• Superior oblique tenotomy is preferred by some rectus resection has also been recommended for
surgeons over the supraplacement of alignment of globe in the primary position.
horizontal recti. 7. Surgery for ptosis. Once the paralytic strabis-
• Inferior rectus recession with resection of superior mus is treated maximally, the paralytic ptosis is
rectus is preferred by some surgeons to correct treated with frontalis sling, taking care that the
hypotropia is ease with preserved patial globe is not overly jeopardized because of
function of vertical recti. But a caution is impaired or absent Bell's phenomenon. Ptosis
required about the possibility of anterior should be corrected only to cover half of the
segment ischaemia. cornea with relaxed brow. Protective measures
• Faden recession of contralateral vertical recti is also to avoid exposure keratopathy must be taken.
helpful in correcting the vertical misalignment INTERNUCLEAR OPHTHALMOPLEGIA
in primary position or downgaze.
Internuclear ophthalmoplegia results from a
3. Transposition of superior oblique tendon. If lesion of the medial longitudinal fasciculus
eye remains still exotropic after 3 months of (MLF) and that is why, is also known as MLF
above procedures, in this situation, a further syndrome. It is a supranuclear disorder
adduction effect can be obtained by transposition described on page 372.
of the insertion of the superior oblique tendon to
a point 2 to 3 mm anterior to the medial side of EXTERNAL OPHTHALMOPLEGIA
superior rectus insertion. Transposition External ophthalmoplegia refers to paralysis of
procedure is meant to create a tonic adducting all extraocular muscles sparing the intraocular
force to the globe to keep it in primary position muscles.
rather than produce any true adductive force
TOTAL OPHTHALMOPLEGIA
during horizontal gaze. This is a difficult
procedure. Total or complete ophthalmoplegia refers to
paralysis of all extraocular muscles including
Thus, transposition of superior oblique
LPS and intraocular muscles, viz., sphincter
tendon may be considered in a patient with
pupillae and ciliary muscle. It results from
acquired third nerve palsy, only if the following
combined paralysis of third, fourth and sixth
conditions are met:
cranial nerves. It is a common feature of orbital
• Palsy is complete apex syndrome and cavernous sinus syndrome.
• Involved eye is fixing eye.
• Maximum recess-resect surgeries on the RESTRICTIVE OCULAR MOTILITY
horizontal recti have failed to restore the globe DEFECTS
to primary position.
Restrictive strabismus is a type of incomitant
4. In case of palsy of inferior division of 3rd nerve. squint characterized by limitation of movements
Transposition of lateral rectus muscle to the site due to causes other than paralytic. In such cases
of insertion of inferior rectus muscle and limitation of ocular movements is out of
transposition of superior rectus muscle to medial proportion to the amount of deviation in the
rectus area, combined with tenotomy of superior primary position.
oblique to align the eye in primary position
Causes of restrictive strabismus can be arranged
should be carried out. This also helps to correct
in two groups:
intorsion and provides a vector force in the
A. Restrictive strabismus due to misdirected
direction of palsied muscle.
muscles forces.
5. In case of palsy of superior division. Knapp's B. Restrictive strabismus due to mechanical
procedure should be carried out (see page 440). restriction.
 334 Theory and Practice of Squint and Orthoptics

A. RESTRICTIVE STRABISMUS DUE TO • Duane’s retraction syndrome (DRS), and

MISDIRECTED MUSCLE FORCES • Horizontal gaze palsy with progressive
scoliosis (HGPPS).
Misdirected muscle foreces that work against
the normal agonist muscle function as seen in DUANE'S RETRACTION SYNDROME
following conditions: Duane's retraction syndrome is a common
1. Congenital cranial dysinnervation disorders entity. Stilling (1887) was probably the first
(CCDDs), e.g. Duane’s retraction syndrome. person to describe this condition followed by
2. Congenital ectopic extraocular muscle insertion Turk (1890). In 1905, Alexander Duane defined
and/or pulley location. the retraction syndrome to consist of six
3. Displaced extraocular muscle, e.g. characteristic features and since then his name
• Superior displacement of medial rectus as has been attached with this syndrome. However,
seen in craniosynostosis. European literature prefers to refer to it as
• Slippage of lateral rectus muscle as seen 'Stilling-Turk-Duane' retraction syndrome. The
in some patients of high myopia with large syndrome in its classic form is characterized by
posterior staphylloma. the following features:
• Iatrogenic displacement of muscle as • Limitation of abduction and/or adduction
reported after inferior oblique anterioriza- • Retraction of globe on adduction
tion. • Narrowing of palpebral fissure on adduction
and widening on abduction.
CONGENITAL CRANIAL • Frequently, upshoot or downshoot of eye on
DYSINNERVATION DISORDERS attempted adduction.
Congenital cranial dysinnervation disorders
Etiology
(CCDDs) encompass a group of disorders that
result from developmental errors in innervation Currently, it is believed that Duane's syndrome
of the ocular and facial muscles and not from is a congenital cranial dysinnervation disorder
primary dysfunction of the muscles themselves. (CCDD) of brainstem origin rather than
The CCDDs can be classified as below: occurring due to structural anomalies of the
muscles as thought earlier.
I. CCDDs primarily affecting horizontal ocular
motility Paradoxical innervation of the horizontal rectus
muscles is the main etiological factor.
• Duane’s retraction syndrome (DRS)
Electromyographic (EMG) studies have shown
• Horizontal gaze palsy with progressive
that most cases of DRS have paradoxical
scoliosis (HGPPS)
innervation of the horizontal rectus muscles as
II. CCDDs primarily affecting vertical ocular shown in Fig. 12.43 and explained under the
motility pathogenesis of ocular features individually
• Congenital fibrosis of extraocular muscles (page 336–338).
(CFEOMs) type 1, 2 and 3
Paradoxical innervation is there for both LR and MR
• Congenital ptosis
rectus muscles, so both co-contract during
III. CCDDs primarily affecting facial muscles with abduction and adduction resulting in:
associated ocular motility defects • Abduction—limitation
• Congenital facial weakness (CFP) • Adduction—limitation
• Moebius syndrome
• Palpebral aperture—narrowing in primary
I. CCDDs PRIMARILY AFFECTING position as well as in adduction and abduction.
HORIZONTAL OCULAR MOTILITY • Globe retraction.
These disorders resut from developmental Probable cause of dysinnervation is:
anomalies of sixth cranial nerve and/or its • Embryopathy, i.e. developmental disturbance
nucleus, and include: is responsible for dysinnervation. This fact is
 Incomitant Strabismus 335

with dominant inheritence pattern of the

syndrome.

Classification of Duane's syndrome

Duane's retraction syndrome (DRS) has been
classified by various workers (Brown 1950, 1958;
Lyle and Bridgeman 1959, Huber, 1974).
However, to avoid confusion only the most
commonly accepted Huber's classification is
given here.
Huber's classification is not only most useful
one clinically, but is well supported by
electromyographic documentation. Huber
classified Duane's syndrome into three types I,
II, III and recently type IV has also been added
(all associated with paradoxical innervation) as
given below:
Fig. 12.43 Right eye illustration of potential innervation Duane's type I is the most common (78%)
patterns in different types of Duane syndrome. VI indicates followed by type III (15%) and type II is the least
fibres of the sixth cranial nerve. III indicates fibres of the common variety. A manifest ocular deviation in
third cranial nerve. Dashed lines indicate hypoplastic or
absent nerve fibres. Thin lines indicate relative decreased
primary position may or may not be present in
innervation. Duane's retraction syndrome. Huber's classifica-
tion though most useful clinically, does not tell
corobarated by the type of associated systemic about the associated type of deviation. Khurana
anomalies as well as the frequent association of (1988) has modified Huber's classification by
Duane’s syndrome in well documented cases of dividing each type into three subtypes
thalidomide embryopathy. depending on the deviation in primary position,
• Hereditary basis for the anomaly has also been viz. a, b and c for esotropia, exotropia and
suggested on the basis of familial occurrence orthotropia, respectively (Table 12.9). This

Table 12.9 Khurana's modification over Huber's classification of Duane's retraction syndrome
Type Subtype Eye in primary position Other features
Ia Esotropic Marked limitation of abduction
Almost normal adduction
I Ib Exotropic Narrowing of palpebral fissure on adduction
Ic Orthotropic Retraction of globe on adduction
IIa Esotropic Marked limitation of adduction,
almost normal abduction
II IIb Exotropic Narrowing of palpebral fissure on
attempted adduction
IIc Orthotropic Retraction of globe on attempted
adduction
IIIa Esotropic Marked limitation of abduction
III IIIb Exotropic Marked limitation of adduction
Narrowing of palpebral fissure on
attempted adduction and abduction
IIIc Orthotropic Retraction of globe on attempted
adduction and abduction
 336 Theory and Practice of Squint and Orthoptics

minor modification has made the Huber's lack of innervation to the LR causing marked
classification complete and more specific. limitation of abduction and on attempted
adduction, along with MR, the LR also gets
Clinical features innervation (paradoxical).
Clinical features of Duane's syndrome can be Characteristic features of type I DRS (Fig. 12.44),
discussed as: (I) General features, (II) Features thus, are:
related to ocular motility defect, (III) Associated
ocular abnormalities, and (IV) Associated In primary position eye may be orthophoric,
systemic abnormalities. esotropic (more common) or exotropic.

I. General features On attempted abduction since LR muscle does

1. Females are more frequently involved than not receive innervation, so:
males. • Abduction is limited markedly,
2. Left eye is more commonly affected (75%) • Palpebral fissure is normal or slightly widened,
than the right. and
3. Bilateral involvement is less frequent (20%) • Globe is slightly protused. This happens as a
than unilateral occurrence. result of relaxation of both LR and MR muscles
4. Sporadic cases are most common, but familial during abduction.
occurrence with dominant inheritance
On attempted adduction, since along with MR,
pattern has also been reported.
LR also gets anomalous innervation, so due to
II. Characteristic of different types of Duane's contraction of MR and LR:
syndrome • Adduction is present but limited, due to co-
Characteristic ocular features of DRS types I, contraction of MR and LR
II, III have been described along with the • Palpebral fissure becomes narrow, and
pathogenesis (see page 336–338) • Globe is retracted,
Duane's retraction syndrome type I • Upshoot or downshoot of the globe may occur
due to slippage caused by co-contraction of
Pathogenesis. Electromyographic studies have
MR and LR
revealed that on attempted abduction, there is

Fig. 12.44 Type I Duane's retraction syndrome left eye with mechanical upshoot.
 Incomitant Strabismus 337

Duane's retraction syndrome type II On attempted adduction due to co-contraction

Pathogenesis. Electromyographic studies have of MR and LR:
demonstrated that: • Adduction is limited
• On abduction, the LR receives normal or • Palpebral aperture becomes narrow, and
subnormal innervation. • Globe is retracted.
• On adduction, LR also gets innervation Duane's retraction syndrome type III
(paradoxical) along with MR. Adduction and
Pathogenesis. Paradoxical innervation is present
other features similar.
for both LR and MR muscles.
Characteristic features of type II DRS (Fig. 12.45)
Characteristic features. There occurs co-
are:
contraction of LR and MR both during attempted
In primary position, eyeball may be orthophoric, adduction and abduction resulting in:
esotropic or exotropic (more common). • Marked limitation of adduction as well as
On attempted abduction abduction along with associated
• Abduction is normal or there may be slight • Narrowing of palpebral aperture, and
limitation due to subnormal innervation and/ • Marked retraction of the globe
or associated MR contracture. • Upshoots and downshoots are frequently
• Palpebral aperture remains normal or slightly present (Fig. 12.46).
widens.

Fig. 12.45 Type II Duane's retraction syndrome left eye (Courtesy: Dr Kanwar Mohan).

Fig. 12.46 Type III Duane's retraction syndrome left eye with innervational upshoot.
 338 Theory and Practice of Squint and Orthoptics

Type IV Duane syndrome Inverse Duane’s syndrome

Type IV Duane syndrome has also been called Inverse Duane’s syndrome has the following
as simultaneous abduction, synergistic features:
divergence, the “splits”, and perversion of the On abduction there occur
extraocular muscles by various workers from • Abduction limitation either due to co-contraction
time to time. (Wilcox et al, 1981 and Wagner et of MR along with LR muscle (congenital
al 1987). Schliesser et al (2016) have recommended inverse Duane’s syndrome) or due to some
that synergistic divergence (Fig. 12.47), a rare fibrosis or entrapment of MR along the medial
entity with features similar to those of Duane orbital wall following trauma (acquired
syndrome, should be classified as Type IV inverse Duane’s syndrome).
Duane syndrome as it has unique findings and
• Palpebral aperture narrowing, and
an innervation pattern different from the other
• Globe retraction.
three types.
On adduction, the LR relaxes normally so
Pathogenesis. In the case of simultaneous following features are noted:
abduction, the oculomotor nerve sends nerve
• Adduction may be normal or slightly limited
fibres to the lateral rectus and the signal causes
especially in acquired cases due to fibrotic bands.
the eye to abduct and co-contract when it should
• Palpebral aperture usually remains normal, and
simply adduct.
Globe also remains within normal position.
Characteristic features of Type IV Duane
syndrome include (Fig. 12.47): III. Other ocular features
• Exotropia in primary gaze, 1. Horizontal deviation. The eyeball in primary
• Face turn opposite the involved eye, position may be esotropic, orthotropic or
• Essentially full abduction of the involved eye, exotropic.
• One series has reported orthotropia in 31%
• Absent adduction of involved eye with
cases, esotropia in 53% cases and exotropia
simultaneous abduction in gaze opposite the
in 16% cases.
involved eye, and
• Esotropia has been reported to occur most
• Narrowing of the palpebral fissure.
frequently with type I, followed by type III
Associations of type IV Duane reported include: and type II.
• Abnormal MRI—midbrain finding, • Exotropia is associated more commonly with
• Goldenhar syndrome, type II than types I and III.
• Cerebral palsy, • Orthotropia is more frequent with type III
• Nystagmus, and than types I and II.
• Anisometropia Deviation should be measured in both
habitual head position and forced straight head
Treatment Weakening by recession of the tight primary position both for distance and near and
lateral rectus is the key treatment in these patients. also in up, down and lateral gazes. The poor

Fig. 12.47 Photograph of patient with type IV Duane syndrome.

 Incomitant Strabismus 339

abducting saccade in affected eye undercover binocularity in a preferred fusing position by

makes the test less accurate, so the prisms should means of compensatory head posture despite
be placed in front of the affected eye and fellow the incomitance. The face turn is towards the
eye should be observed under partial cover. The affected eye in eso Duane and away from
deviation in esotropic patients is usually less affected eye in exo Duane.
than 30 PD in unilateral cases with the non- 4. Diplopia is complained very infrequently
affected eye fixing. However, in bilateral non- despite the fact that prevalence of deviation in
fusing Duane’s or unilateral Duane’s fixing with primary position is quite common. This may be
the affected eye there may be large degree of because of either face turn to achieve fusion or
esodeviation. occurrence of suppression.
Patterns: A large incidence of increased 5. Sensory adaptations. Patients who suppress
anomalous innervation of LR in elevation or also develop abnormal retinal correspondence
depression explains the frequent observation of and amblyopia.
V, A or X pattern in these patients.
IV. Associated ocular abnormalities
Y or lambda patterns are seen in patients with
anomalous LR recruitment only in upgaze or • Anisometropia with hyperopia is a frequent
downgaze, respectively. Bilateral Duane's with association and may even be the cause of
fusion often displays A-pattern. In DRS, the amblyopia in some cases. Therefore, refraction
patterns are not due to oblique dysfunction but is very important especially in children.
by co-contraction of lateral and vertical muscles. However, more recent studies have shown that
2. Vertical deviations are often present with anisometropia in Duane's syndrome is no higher
characteristic upshoot or downshoot, when the than the normal population.
affected eye is adducted. These occur more • Other ocular abnormalities which have been
frequently in severe anomalous lateral rectus associated with Duane's syndrome include
recruitment. Two types of upshoots and optic nerve hypoplasia, morning glory
downshoots have been described; syndrome, congenital ptosis, nystagmoid
a. Mechnical upshoots and downshoots are reported movements, congenital cataract, heterochromia
to result from a tight lateral rectus muscle iridis, persistent hyaloid arteries, choroidal
(leash effect). In this condition, the affected eye colobomas, districhiasis, pupillary anomalies,
begins to adduct normally and then suddenly keratoconus, microphthalmos and many
upshoots or downshoots. others.
b.Innervational upshoots and downshoots are V. Associated systemic abnormalities
reported to result from misinnervation of the
Some of the systemic abnormalities which have
vertical recti muscles. In this condition, there
been reported to be associated with Duane's
occurs a progressively increasing vertical
retraction syndrome are as follows: Klipple-Feil
deviation of the affected eye as it starts
syndrome, Goldenhar's syndrome, facial hemi-
adducting.
atrophy, cervical spina bifida, umbilical hernia,
Electromyographic studies have also demons-
polydactly, Chiari I malformations, sensori-
trated an abnormal synergistic innervation
neural hearing deficits and Wildervank
between the medial rectus and the superior and
syndrome or cervico-oculoacoustic syndrome
inferior oblique muscles in some cases; and this
(combination of Duane's syndrome, sensori-
may also explain the upshoot or downshoot in
neural hearing loss and Klippel-Fiel anomaly of
adduction, i.e. frequently seen in this syndrome.
the spine).
Gradual upshoots may be due to superior rectus
contracture or inferior oblique overaction Diagnosis
(IOOA). • Duane's retraction syndrome is usually
3. Abnormal head posture. Unilateral cases are diagnosed on version tests by its typical
frequently accompanied by a head turn for signs. Carefully look for anomalous vertical
fusion. So DRS patients usually have normal movements also, as this is important in
 340 Theory and Practice of Squint and Orthoptics

determining the surgical strategy. There is a syndrome (Pseudo-Duane's syndrome) may

curved outward rotation pattern uniquely occur as a result of scarring following muscle
characteristics of Duane's which shows complete surgery or other surgery involving the
absence of abduction (–4) in horizontal gaze, conjunctiva and Tenon's capsule and thus
while more outward rotation in upgazes and need to be differentiated from true Duane's
downgazes due to anomalous innervation in syndrome.
upgaze and downgaze.
Management
• Adduction saccadic velocity can be reduced
in the affected eye in all types of Duane's Non-surgical measures Refractive error and/
syndrome. or amblyopia, when present, should be treated
first, on general lines (see page190)
• Abduction saccadic velocity is reduced only
in type I and III. Surgical treatment in Duane's retraction
• Measuring the eyelid fissure in abduction and Indications include:
adduction to determine the degree of globe • An abnormal head posture,
retraction. This represents an indirect sign of • A significant deviation in the primary position,
amount of anomalous innervation to LR muscle. • Marked globe retraction, or
A greater degree of anomalous innervations
• Marked upshoots and downshoots.
is correlated with an increased amount of globe
retraction. General observations. In general, disappointing
• Force duction and force augmentation tests. results of surgery have been reported for this
The two components responsible for abduction condition. Therefore, better one should not
limitation are: amount of normal innervation to operate, when binocular single vision is present
LR and degree of contracture of MR. These can in primary position of gaze or if it can be
be differentiated by force duction test and force maintained with a slight head turn.
augmentation test which compares the • Further, no set rules exist for surgical treatment
voluntary abduction end point with possible of Duane's syndrome and that planning in each
extension of this end point by forcing further case should be made individually taking into
abduction manually with forceps. This proves consideration the coexisting horizontal and
the paralysis of agonist and no contracture of vertical deviations.
antagonist. • Rough guidelines, recommended, are described
Force generation test also helps to determine the briefly.
active force generated by LR. Adduction
limitations which are usually seen in patients I. Management of Eso-DRS
with severe anomalous innervation to LR that Esotropia is classically more common in DRS
prevents the eye from moving into adduction. type I where LR innervation is normal in
This can be determine by force degeneration test abduction and anomalous in adduction.
described by Romero Apis. Sometime esotropia may also occur in DRS type
In this test, the patient is asked to look halfway II and type III. Depending upon the findings eso-
between the primary position and full adduction DRS can be managed as below.
and then, eye is moved further into adduction
while the patent is asked to further adduct the Management of esotropia
eye. Resistance caused by co-contracting LR in 1. MR recession of affected eye may be effective
adduction can be appreciated at this point. Then in small angle esotropia (<20 PD) in primary
the patient is asked to abduct the eye and position in patients with mild DRS (cases with
grasping at temporal limbus, the examiner minimum anomalous LR activity and normal LR
attempts to adduct the eye. Then note that if in adduction).
resistance has disappeared as now in abduction, 2. Assymetric bilateral MR recession with more
LR does not receive abnormal innervation. recession of the fellow good eye (even up to
• Rarely, an acquired form of Duane's retraction 9 mm depending upon the amount of deviation)
 Incomitant Strabismus 341

is indicated in cases with large esotropia (>20 II. Management of exo-DRS

PD). Patients with exo-DRS, usually have anomalous
Advantage of asymmetric MR recession is that it LR innervation in adduction and normal or
produces a fixation dures without fear of subnormal LR innervations in abduction. There
adduction deficiency in the good eye. In addition may be associated globe retraction and upwards
to correcting esotropia in primary position, it will and downshoot. Depending upon the situation,
also decrease the likelihood of MR contracture following measures used:
of the affected eye through the fixation dures of
1. LR recession (supramaximal) of the affected
the good eye which leads to a continuous
eye may be useful for correcting exotropia amd
inhibition of the MR of the affected eye.
globe retraction.

Management of associated globe retraction 2. LR periosteal fixation has been reported to

be effective in correcting exotropia, and
LR recession of the affected eye along with
anomalous head posture, and improving
asymmetric MR recession may be helpful in cases
adduction (as PF wards off the anomalous
of esotropia with severe globe retraction, due to
activity of LR). Further, it can be combined with
anomalous LR.
partial VRT to improve abduction in patients
with subnormal LR innervation.
Management to improve abduction
Superior rectus transposition (SRT) or vertical 3. LR recession with Y-split is an option for
rectus transposition (VRT) to the lateral rectus exotropia with upshoot and downshoot in cases
is useful to achieve abduction force in cases with normal LR activity.
where there is no severe anomalous LR
recruitment and there is marked abduction III. Management of ortho-DRS
deficit (–3 to –4). These procedures help to
These patients are usually orthotropic in
improve the abduction and also less MR
primary position but there is severe globe
recession is required to correct the exotropia.
retraction with upshoots and downshoots.
SRT has decreased risk of anterior segment
ishaemia, but may induce vertical incomitance. Symmetrical recession of MR and LR of the
Balanced partial VRT, is therefore, preferred over affected eye with adjustable sutures on one
SRT by many surgeons. muscle and Y-split of LR is a good option in such
cases.
Management of upshoots and downshoots
Management of Y and lambda patterns in DRS
1. Faden operation (posterior fixation suture)
with or without recession of the lateral rectus Such patients usually have:
muscle 14 mm posterior to its insertion has • Less deficient abduction,
been advocated in patients with marked • Anomalous LR innervation only in vertical
upshoots or downshoots of mechanical type. gazes
(Leash phenomenon). • No globe retraction, and
2. Y-splitting of the lateral rectus muscle. In • Adduction in the involved eye is worse in
case of upshoots and downshoots, Y-split with upgaze.
LR recession of the affected eye can be done in
addition to treating esotropia. The LR is split Bilateral LR recession with elevation of tendons
from its insertion as far posteriorly as possible, in Y-pattern and downshift in lambda pattern
spreading the muscle halves 10 mm up and is the procedure of choice.
10 mm down (20 mm between them). This is also
followed by LR recession 5–10 mm to negate the Bilateral SR recession in Y-pattern and bilateral
resection effect created by splitting the muscle IR recession in lambda pattern can also be
halves. considered.
 342 Theory and Practice of Squint and Orthoptics

HORIZONTAL GAZE PALSY WITH PROGRESSIVE Primary defect. In this condition is of superior
SCOLIOSIS division of oculomotor nerve.
Etiology and genetics Clinical features include bilateral (Fig. 12.48A).:
Horizontal gaze palsy with progressive scoliosis • Congenital ptosis,
(HGPPS) is a rare autosomal recessive disorder • Globe infraducted in primary position
with the locus on chromosome 11 (11q 23–25). • Gaze restriction is noticed in upgaze as well as
The condition is thought to result from agenesis horizontal gaze.
of the abducens nucleus including both alpha
• Misdirected eye movements in the form of marked
motar neurons and interneurons.
synergistic convergence on attempted upgaze.
Clinical features • Forced duction test is often positive in upgaze.
• Congenital horizontal gaze palsy with • Marcus Gunn jaw winking phenomenon is
progressive scoliosis (HGPPS), as the name noticed in 30–40% cases.
indicates, is characterized by complete
absence of conjugate horizontal gaze and CFEOM 2 phenotype
childhood onset progressive scoliosis. Genetics. Autosomal recessive disorder with
• Vertical eye movements and convergence are main locus, FEOM 2, on chromosome 11.
preserved. Primary defect is in the development of both the
• There are no associated ptosis and other oculomotor and trochlear nuclei.
somatic abnormalities.
• Some patients may have nystagmus, esotropia, Clinical features (Fig. 12.48B).
and/or retraction on adduction. • Ptosis is often severe
• Exotropia is usually of large angle
MRI scan of brain in patients with HGPPS • Ocular movements, horizontal as well as vertical
shows hypoplasia of pons, absence of facial are severely restricted.
colliculi, butterfly configuration of medulla and
deep midline pontine cleft (split pons sign).

II. CCDDs PRIMARILY AFFECTING VERTICAL

OCULAR MOTILITY
These disorders result from the developmental
anomalies of third and fourth cranial nerves or
their nuclei. These include:
• Congenital fibrosis of extraocular muscles
(CFEOMs), and
A
• Congenital ptosis.

CONGENITAL FIBROSIS OF EXTRAOCULAR MUSCLES

Congenital fibrosis of extraocular muscles
(CFEOMs) refers to a group of disorders that
result from primary dysinnervation of oculomotor
and/or trochlear innervated extra-ocular
muscles. The genetic loci for the CFEOMs
phenotypes are known as FEOM. Till date, three
CFEOMs and four FEOM loci have been defined.

CFEOM 1 phenotype B
Genetics. It is an outosomal dominant disorder Fig. 12.48 Congential fibrosis of extraocular muscles
with main locus FEOM 1, on chromosome 12. (CFEOM): A, Type I and B, Type II
 Incomitant Strabismus 343

CFEOM 3 phenotype
Genetics. Inheritance is autosomal dominant
with incomplete peneterance. The main locus is
the FEOM 3 on chromosome 16.
Primary defect in the development of
oculomotor nucleus is variable.
Clinical features are variable:
• Forced duction test is usually positive
• Misdirected eye movements or globe retraction
are rarely seen.
Fig. 12.49 A child with mobius syndrome (Courtesy: Dr.
Differential diagnosis Kalpana and Dr. Sandra).
CFEOMs should be differentiated from: • Deviation. Usually eyes are straight in primary
• Monocular elevation deficiency, position, a few patients may have esotropia
• Brown’s syndrome, • Facial weakness is characterised by:
• Congenital progressive external ophthal-
– Mask-like facies with mouth constantly held
moplegia (CPEO), and
open.
• Duane’s retraction syndrome.
– Eyelids cannot be closed completely.
III. CCDDS PRIMARILY AFFECTING FACIAL Associations include:
MUSCLES WITH ASSOCIATED OCULAR • Paralysis or hypoplasia of tongue due to involve-
MOTILITY DEFECTS ment of hypoglossal nerve with speech and
These disorders result from the abnormalities swallowing difficulties are extremely common.
in the development of the facial nerve and/or • Other cranial nerves which can be involved are
nucleus. These include: 3rd, 4th, 9th, and 10th.
• Congenital facial weakness, and • Craniofacial anomalies which may be associated
• Möbius syndrome are epicanthal folds, microstomia, micrognathia
and external ear defects.
MÖBIUS SYNDROME • Limbs abnormalities include webbed fingers
The eponym Möbius syndrome refers to and toes, suppernumerary digits, club foot
congenital bilateral abducent paralysis and syndactyly.
associated with congenital facial palsy with • Deafness and mental retardation are also reported.
variable other associations. • Congenital heart defects such a ventricular septal
defect are also reported.
Etiology and genetics
• Respiratory defects with tachypnoea and other
Möbius syndrome is being considered a hetero-
respiratory difficulties occur in some patients.
geneous group of congenital disorders caused
by developmental defects related to a variety of
Management
insults such as ischaemia, toxic effects of
prenatal used drugs such as misoprostol, • Prevention of exposure keratitis due to facial
benzodiazepines. weakness may be done by tarsorrhaphy.
To date, two phenotypes with responsible • Esotropic patients, though rare, may be managed
genotypes reported are: by MR recessions with or without LR resection.
• MBS 1 phenotype with the locus 13q12.2–13, and
• MBS 4 phenotype with the locus 1p22. B. RESTRICTIVE STRABISMUS DUE TO
Clinical features (Fig. 12.49) MECHANICAL RESTRICTIONS
• Abduction is usually limited in both eyes. Restrictive strabismus due to mechanical
• Gaze palsy may be there restrictions may be caused by:
 344 Theory and Practice of Squint and Orthoptics

I. Tight extaocular muscles, as occurs in: which has not been proved by subsequent
• Inelastic superior oblique in congenital workers. In fact, Parks observed that the superior
Brown’s syndrome oblique tendon sheath does not exist at all and
• Thyroid ophthalmopathy that the term 'superior oblique tendon sheath
syndrome' introduced by Brown is a misnomer.
• Entrapped inferior rectus muscle in blow-out
fracture of orbital floor. 2. Acquired Brown's syndrome is presently
• Monocular elevation deficiency (MED), thought to be caused by an acquired taut
caused by fibrotic IR muscle. superior oblique tendon, secondary to following
conditions:
• Strabismus fixus.
• Tenosynovitis of the superior oblique
II. Structural adhesions, e.g. as seen in: trochlear apparatus.
• Fat adherence to extraocular muscles or sclera • Trauma to the trochlear region in any form
after strabismus surgery, retinal detachment may cause this abnormality. The 'canine
surgery or periocular trauma tooth syndrome' of Knapp also falls into this
• Congenital fibrotic bands category. Surgical trauma has emerged
• Acquired Brown’s syndrome due to scarring/ as another cause of acquired Brown's
inflammation around, the trochlea syndrome (traumatic Brown's syndrome).
• Conjunctival and Tenon’s capsule scarring • Rheumatoid nodules on the superior oblique
tendon posterior to the trochlea. Association
III. Orbital mass lesions, e.g. with other autoimmune diseases like SLE,
• Orbital tumours causing mass effect on the Sjögren syndrome and Graves' ophthal-
globe movements. mopthy is also reported.
• Glaucoma explant with large bleb causing • Retrotrochlear thickening of the tendon or
mass effect. anomalies of the trochlea itself may lead to
Note. A few of the conditions are described here. impaired slippage of the tendon through the
trochlea.
TIGHT EXTRAOCULAR MUSCLES • Idiopathic

BROWN'S SYNDROME
Clinical features
Brown syndrome refers to mechanical restriction
Congenital cases (also known as true or primary
(a significant limitation) of elevation in
syndrome) are constant and unilateral in 90%
adduction caused by an overly taut superior
percent of patients. Most acquired cases are
oblique tendon of the same eye. On version
intermittent and more likely to improve
testing, the condition mimics an inferior oblique
palsy except that a V-pattern is present in spontaneously. The syndrome is rarely seen in
contrast to A-pattern associated with inferior adults. The clinical features of this syndrome can
oblique palsy. be divided into main consistent features and less
important variable features.
Etiology Main consistent clinical features include the
The condition may be congenital or acquired. following (Fig. 12.50):
Originally, Brown divided the syndrome into 1. Elevation is limited significantly, characteris-
true sheath syndrome (now congenital) and tically in adduction and present in abduction.
simulated sheath syndrome (now acquired). There may or may not be mild limitation of
1. Congenital Brown's syndrome is presently elevation in midline. Degree of limitation is
thought to be caused by a congenitally taut same on versions and ductions.
superior oblique tendon (short and inelastic 2. Overaction of superior oblique is characteris-
tendon). Originally, Brown thought that these tically absent which normally would be found
cases occur due to congenitally shortened anterior with a paretic inferior oblique muscle.
sheath of the superior oblique tendon; the theory 3. Divergence in upgaze producing a V-pattern.
 Incomitant Strabismus 345

Grading of Brown's syndrome

Eutis et al have graded Brown's syndrome into
mild, moderate and severe (Table 12.10).
Brown plus syndrome, is the term used by
Jampalesky for the cases having vertical deviation
in the primary position or in adduction, with or
without compensatory head posture.

Table 12.10 Grading of Brown's syndrome (Eutis et al)

Grade Restriction of Downshoot Hypotropia
elevation in in adduction in primary
adduction position
1–Mild + – –
2–Moderate + + –
3–Severe + + +

Differential diagnosis
1. Inferior oblique paralysis versus Brown's
syndrome
• Limitation to elevation in adduction is greater on
testing for ductions than versions in inferior
Fig. 12.50 Brown's syndrome right eye: (A), Note limitation
of elevation in right eye which is marked in adducted
oblique paralysis, while in Brown's syndrome,
position (B) (Courtesy: Dr Kalpana an Dr Sandra). it is equal.
• Overaction of the superior oblique muscle is
4. Forced duction test is positive on attempts to typically absent in Brown's syndrome, while
elevate the adducted eye; but is negative on it is present in inferior oblique palsy.
attempts to elevate the abducted eye. • Forced duction test is positive in Brown's
syndrome, while it is negative in inferior
Less important and variable clinical features oblique palsy.
include: • Park's 3-step test is positive in inferior oblique
1. Downshoot in adduction. palsy.
2. Widening of the palpebral fissure on adduction. 2. Other conditions with restriction of elevation
3. Straight eyes in primary position are present which need to be differentiated from the Brown's
in most patients but a few may have syndrome are double elevator palsy, fracture of
hypotropia. the orbital floor, Graves' ophthalmopathy and
4. Compensatory head posture (chin up) may be congenital fibrosis of the inferior rectus muscle.
present in patients with hypotropia. In all these conditions, elevation is equally
5. An audible click may be produced when such restricted in adduction, primary gaze and in
patients are able to elevate their adducted abduction; while in Brown's syndrome,
eye. Some observers have even used the term elevation is restricted only in adduction.
'superior oblique click syndrome' for such
patients. Management
6. Inflammatory signs like superonasal orbital 1. Conservative treatment. Acquired cases
pain, and tenderness may be present in should be observed, since spontaneous
acquired cases. improvement has been reported. These cases
Bilateral Brown's syndrome, when present, depending upon the situation may need:
exihibits 'V' pattern exotropia. Rest features • Range of eye motility exercises (elevation and
being similar to unilateral cases. adduction exercises).
 346 Theory and Practice of Squint and Orthoptics

• Steroids either orally or by injection near the • Chicken suture in nasal half of tendon or
trochlea in inflammatory cases. • Loop suture at the insertion may also be tried.
• Correction of the underlying cause, when
possible such as trauma to the trochlea. THYROID OPHTHALMOPATHY
2. Surgical treatment. Results of the surgery for This term is coined to denote typical ocular
this entity are controversial and, therefore, surgery changes which include lid retraction, lid lag, and
should only be undertaken in severe (grade 3) proptosis. These changes have also been labelled
cases of Brown's syndrome, i.e. in the presence as: Endocrine exophthalmos, malignant
of: exophthalmos, dysthyroid ophthalmopathy and
• A significant cosmetically disfigurement head ocular Graves' disease (OGD).
tilt; or
Etiopathogenesis
• Severe and constant congenital Brown
syndrome that threatens binocularly and It may be a part of Graves' disease (the syndrome
development of amblyopia consisting of hyperthyroidism, goitre and eye
• A large hypotropia, in primary position or signs) or may be associated with hypothyroidism
or even euthyroidism. Thus, a direct causative
• Unacceptable downshoot in adduction.
connection between the thyroid dysfunction and
Surgical procedures recommended are as the ocular changes remains elusive. There is an
follows: increasing evidence to suggest that Graves'
1. Superior oblique tenotomy. It is a simple, safe ophthalmopathy has an autoimmune etiology.
and effective procedure in treating Brown's Most data presently support the postulate that
syndrome. However, about 50% cases develop an autoantigen is coexpressed in the thyroid
symptoms of superior oblique paralysis which gland and orbital fibroblast. This antigen is
can be managed by recession of either ipsilateral recognised by the circulating T cell lymphocytes.
inferior oblique or contralateral inferior rectus. Activating the T cells (CD4 cell) trigering an
2. Superior oblique tenectomy. To avoid the risk immune response. The activated T cells secrete
of superior oblique palsy, Parks recommends various cytokines, interferon, interleukin L-
performing a 6 mm superior oblique tenectomy alpha and tumour necrosis factor (TNF), which
within the intermuscular septum along the nasal cause proliferation of fibroblasts in the orbit and
border of superior rectus. production of glycosaminoglycans (GAGs).
Presence of mucopolysaccharides, predo-
3. Superior oblique weakening with a silicone
minantly hyaluronic acid, together with
expander has also been advocated by Wright in
interstitial oedema and inflammatory cells
1991 to prevent superior oblique palsy following
accounts for the proptosis, and swelling of
tenotomy. This procedure involves expanding
extraocular muscles.
the length of the tendon using a silicone spacer
usually silicone retinal 240 band. The weakening
effect is graded by varying the length of the Clinical features
silicone that bridges the gap between the cut 1. Lid signs. These are: (i) retraction of the upper
ends of the tendon. In a Brown syndrome lids producing the characteristic staring and
6–7 mm expander is recommended. A chicken frightened appearance (Dalrymple's sign),
suture can be placed in lieu of an expander to (ii) Lid lag (von Graefe's sign), i.e. when globe is
retain the cut-ends of superior oblique tendon moved downwards, the upper lid lags behind,
together. However, this procedure is difficult (iii) fullness of eyelids due to puffy oedematous
and cumbersome. Such a procedure may swelling (Enroth's sign), (iv) difficulty in
actually be more relevant in cases with superior eversion of upper lid (Gifford's sign), (v)
oblique overaction (Brown plus). infrequent blinking (Stellwag's sign).
4. Other superior oblique weakening or lengthening 2. Conjunctival signs. These include deep
procedures such as: injection and chemosis.
 Incomitant Strabismus 347

3. Pupillary signs. These are of less importance retraction, exophthalmos, lagophthalmos, inability
and may be evident as inequality of dilatation to elevate the eyes and a decreased blink rate.
of pupils. 7. Optic neuropathy. It occurs due to direct
4. Ocular motility defects. These range from compression of the nerve or its blood supply by
convergence weakness (Mobius's sign) to partial the enlarged rectus muscles at the orbital apex.
or complete immobility of one or all of the It may manifest as papilloedema or optic
extrinsic ocular muscles. Severe restrictive atrophy with associated slowly progressive
myopathy occurs due to lymphocytic infiltration impairment of vision.
of the extraocular muscles and varying amounts American Thyroid Association (ATA)
of oedema, inflammation and fibrosis. classification
• The most common ocular mobility defect is a ATA has classified Graves' ophthalmopathy,
unilateral elevator palsy, Fig. 12.51 caused by irrespective of the hormonal status into following
involvement of the inferior rectus muscle classes characterised by the acronym 'NOSPECS'
followed by failure of aduction due to Class 0 : No signs and symptoms.
involvement of medial rectus muscle.
Class 1 : Only signs, no symptoms (signs are
• Thus, thyroid (endocrine) myopathy is a
limited to lid retraction, with or
common cause of acquired vertical deviation in
without lid lag and mild proptosis).
adults, especially females. It is a rare cause of
Class 2 : Soft tissue involvement with signs
acquired vertical deviation in children.
(as described in class-1) and symptoms
5. Exophthalmos. It is a common and classical including lacrimation, photophobia,
sign of the disease. As a rule, both eyes are lid or conjunctival swelling).
affected; but it is frequent to find one eye being Class 3 : Proptosis is well established.
more prominent than the other. Even unilateral
Class 4 : Extraocular muscle involvement
proptosis is not uncommon. In majority of cases,
(limitation of movement and diplopia).
it is self-limiting.
Class 5 : Corneal involvement (exposure
6. Exposure keratitis and symptoms of ocular
keratitis).
surface discomfort. These include sandy or gritty
Class 6 : Sight loss due to optic nerve involve-
sensation, lacrimation and photophobia. Corneal
ment with disc pallor or papilloedema
exposure has been attributed to upper lid
and visual field defects.

Fig. 12.51 A patient with Graves' ophthalmopathy depicting limitation of left superior rectus muscle due to involvement of
left inferior rectus muscle.
 348 Theory and Practice of Squint and Orthoptics

For practical purposes, it has been described 6. Lateral tarsorrhaphy should be performed
as 'early' (which include ATA class 1 and 2) and in patients with exposure keratopathy (with
'Late Graves' ophthalmopathy' (class 3 to 6). mild to moderate proptosis) not responding to
topical artificial tears.
Investigations 7. Prismatic glasses may help to relieve the mild
1. Thyroid function tests. These should include: diplopia in primary position or rending gaze.
serum T3, T4, TSH and estimation of radioactive But since the deviation is usually very
iodine uptake. incomitant, prism often does not alleviate the
2. Positional tonometry. An increase in intraocular diplopia in all positions of gaze.
pressure in upgaze helps in diagnosis of 8. Surgical orbital decompression: It should be
subclinical cases. performed, only when systemic steroids and
radiotherapy have proved ineffective in patients
3. Ultrasonography. It can detect changes in
with marked proptosis associated with severe
extraocular muscles even in class 0 and class 1
exposure keratopathy and/or optic neuropathy
cases and thus helps in early diagnosis. In
with imminent danger of permanent visual loss.
addition to the increase in muscle thickness,
The most commonly employed technique is
erosion of temporal wall of orbit, accentuation
'two wall decompression' in which part of the
of retrobulbar fat and perineural inflammation
orbital floor and medial wall are removed.
of optic nerve can also be demonstrated in some
9. Extraocular muscle surgery. It should be
early cases.
carried out for left out diplopia in primary gaze,
4. Computerised tomographic scanning. It may after the congestive phase of disease is over
show proptosis, muscle thickness, thickening of and the angle of deviation is constant for the
optic nerve and anterior prolapse of the orbital last 6 months.
septum (due to excessive orbital fat and/or Recession of the affected muscle (inferior rectus,
muscle swelling). medial rectus or superior rectus as the case may
be) is the primary surgical treatment. Strengthe-
Management of Graves' ophthalmopathy ning procedure should be avoided. Extraocular
It is in addition to and independent of the muscle surgery may eliminate diplopia in
therapy for the associated thyroid dysfunction; primary gaze but rarely restores normal motility
as the later usually does not alter the course or because of the restrictive myopathy that typifies
ophthalmic features. The treatment modalities Graves' ophthalmopathy.
employed are as follows: Adjustable suture surgery may help optimize the
alignment and rotations in these difficult cases.
1. Topical artificial tear drops in the day time
Since late overcorrection frequently occurs,
and ointment at bedtime are useful for relief of
especially with large inferior rectus recessions,
foreign body sensation and other symptoms of
slight undercorrection (fusion with a slight chin-
ocular surface drying.
up position) is desirable at the time of surgery.
2. Guanethidine 5% eyedrops may decrease the 10. Cosmetic surgery for persistent lid
lid retraction caused by overaction of Muller's retraction. It consists of levator and Muller's
muscle. muscle recession. Recently, implantation of
3. Systemic steroids may be indicated in acutely scleral grafts has become a popular technique.
inflamed orbit with rapidly progressive 11. Blepharoplasty. It may be performed by
chemosis and proptosis with or without optic removal of excess fatty tissue and redundant
neuropathy. skin from around the eyelids.
4. Immunosuppressive drugs may be required
to control acute inflammation when steroids are ORBITAL BLOW-OUT FRACTURE
not effective or contraindicated. These are isolated communited fractures which
5. Radiotherapy (2000 rads given over 10 days occur, when the orbital walls are pressed
period). It may help in reducing orbital oedema indirectly. Blow-out fractures mainly involve
in patients where steroids are contraindicated. orbital floor and medial wall.
 Incomitant Strabismus 349

Etiology degree may also be present initially because

These generally result from trauma to the orbit of the associated orbital oedema and
by a relatively large, often rounded objects, such haemorrhage.
as tennis ball, cricket ball, human fist (Fig. 12.52) • Enophthalmos. After about 10 days, as the
or part of an automobile. The force of the blow oedema decreases, the eyeball sinks backward
causes a backward displacement of the eye and and somewhat inferiorly resulting in
an increase in intraorbital pressure with a enophthalmos. Three factors responsible for
resultant fracture of the weakest point of the producing enophthalmos are: (a) escape of
orbital wall, usually this point is the orbital floor, orbital fat into the maxillary sinus; (b)
but this may be the medial wall also. backward traction on the globe by entrapped
inferior rectus muscle and (c) enlargement of
Classification the orbital cavity from displacement of
1. Pure blow-out fractures. These are not fragments.
associated with involvement of the orbital rim. • Diplopia also becomes evident after decrease
2. Impure blow-out fractures. These are associated in oedema. It typically occurs in both up- and
with other fractures about the middle third downgaze (double diplopia) due to entrapment
of the facial skeleton. of soft tissue structures in the area of the blow-
out fracture (floor more commonly than
Clinical features medial wall).
The presence of muscle restriction can be
• Periorbital oedema and blood extravasation in and
confirmed by a positive 'forced duction test'.
around the orbit (e.g. subconjunctival
ecchymosis) are initial lesions. This may mask • Restricted elevation, restricted depression may
certain signs and symptoms seen later. occur in fracture floor of the orbit.
• Emphysema of the eyelids occurs more • Pseduo-Duane's refraction syndrome (or acquired
frequently with medial wall than floor inverse-Duane's syndrome), presentation may
fractures. It may be made worse by blowing occur in fracture of medial wall of the orbit.
of nose. Paraesthesia and anaesthesia in • Saccadic eye movement testing is sometimes
infraorbital nerve distribution (lower lid, helpful to determine whether ocular
cheek, side of nose, upper lip and upper teeth) movement limitation is because of a restrictive
are very common. process or a paretic process.
• Ipsilateral epistaxis as a result of bleeding from • Associated severe ocular damage is rare. This is
maxillary sinus into the nose is frequently because a 'blow-out fracture' is nature's way of
noted in early stages. Proptosis of variable protecting the globe from injury. Nevertheless
the eye should be carefully examined to exclude
the possibility of intraocular damage.

Radiological examination
1. Plain X-rays. The most useful projection for
detecting an orbital floor fracture is a nose-chin
(Water's) view. The common radiological
findings are—fragmentation and irregularity of
the orbital floor; depression of bony fragments
and 'hanging drop' opacity of the superior
maxillary antrum from orbital contents
herniating through the floor (Fig. 12.53).
2. Computerised tomography scanning and
Fig. 12.52 Mechanism of blow-out fracture of the orbital magnetic resonance imaging. These are of
floor. greater value for detailed visualisation of soft
 350 Theory and Practice of Squint and Orthoptics

extreme position. It may be unilateral or

bilateral. It occurs in two forms—convergent
and divergent.
Strabismus fixus convergence is more common.

Etiology. Strabismus fixus convergence (SFC)

can be congenital or acquired.
• Congenital cases are more common. In such
cases, lateral rectus palsy with medial rectus
fibrosis has been described as the cause.
• Acquired cases though rare, are reported to be
due to myopic myositis and amyloidosis of the
lateral rectus muscle.

Fig. 12.53 Plain X-ray orbit (AP view) showing herniated

Clinical features Patient's eyes are fixed in
orbital contents (arrow) with blow-out fracture of the orbital extreme convergent position and he/she cannot
floor. abduct either eye past the midline (Fig. 12.54).
It can be differentiated by forced duction test
tissues. Coronal sections are particularly useful from bilateral sixth nerve palsy.
in evaluating the extent of the fracture.
Treatment of strabismus fixus convergence
Management includes:
Surgical repair to restore continuity of the orbital • Medial rectus recession. Supramaximal
floor may be made with or without implants. recession with silicon expanders have been
It may not be required in many cases. reported to give good functional and cosmetic
• Optimal time for surgery, when indicated, is results.
after 10–14 days of injury. • Loop myopaxy of LR and SR to the sclera in
• Indications of surgical intervention include: superotemporal part with non-absorbable
– Diplopia not resolving significantly in the suture or silicon sling is also reported to give
early days after trauma reasonable good results in cases with tight
– A fracture with a large herniation of tissues MR.
into the antrum • Disinsertion of MR and resection of LR has also
– Incarceration of tissues in the fracture with been described.
resulting globe retraction and increased In addition, recession of the medial conjunctiva
applanation tension on attempted upward and Tenon's capsule may be needed to bring the
gaze; and eyes in the centre in primary position.
– Enophthalmos greater than 3 mm. Strabismus fixus divergence is comparatively
Any of these factors, alone or combined, could rare condition characterized by fixation of eyes
indicate that early orbital repair is necessary. in extreme divergence.
Surgery for divergent strabismus fixus is just
Residual strabismus after 3–6 months of injury
reverse of the strabismus fixus convergence.
can generally be corrected by using standard
eye muscle surgical techniques: CONGENITAL TIGHT INFERIOR RECTUS MUSCLE
• Recession of inferior is done first
• In some children, a congenital hypotropia
• Resection of superior rectus may also be done, occurs accompanied by marked limitation of
if required. upgaze.
• The condition may be unilateral or bilateral
STRABISMUS FIXUS
and in some cases there may be fibrosis of the
In strabismus fixus, fibrosis involves the levator muscle as well. Probably, the condition
horizontal recti and the involved eye is fixed in is a variant of generalized fibrosis.
 Incomitant Strabismus 351

Fig. 12.54 Strabismus fixus convergence

• Forced duction test reveals an inferior adhesions have been removed, after the surgery,
restriction. eye should be rotated medially for lateral
• Surgical exploration reveals a tight inferior adherence syndrome and inferiorly for superior
rectus which is adherent to the globe. adherence syndrome.
• Treatment consists of release of globe adhe-
sions and a maximal inferior rectus TIGHT LATERAL RECTUS SYNDROME
recession, to relieve the restriction in upward Causes This syndrome is probably seen most
rotation. commonly in association with a long-standing
large angle exotropia. Large bimedial recession
STRUCTURAL ADHESIONS followed by contracture of the lateral recti has
also been implicated as a cause.
ADHERENCE SYNDROME
Clinical features The tight lateral rectus
Clinical features. Two types of adherence
syndrome is characterized by bilateral restriction
syndrome, the lateral adherence syndrome and
of the eyes on attempted adduction and an
superior adherence syndrome, have been
apparent overaction of all the four obliques.
reported to occur due to developmental
• Forced duction test shows restriction of both the
abnormal fascial connections.
lateral recti, an observation which helps in
• In the lateral adherence syndrome, an abnormal differentiating it from bonafide oblique overaction.
fascial connection is seen between the muscle
Treatment consists of recessions of the lateral
capsule of the lateral rectus and inferior
recti combined with temporal conjunctival
oblique, which produces limitation of ocular
recession. Medial rectus, resection or advancement
rotation in the field of lateral rectus muscle.
may be required.
• In the superior adherence syndrome, an
abnormal fascial connection exists between CONTRACTURE OF EXTRAOCULAR MUSCLES
the superior rectus and tendon of the superior
• Contracture of antagonist extraocular muscle is
oblique, causing limitation of rotation in the
of common occurrence after paralysis of an
field of superior rectus muscle.
agonist extraocular muscle, that with time
Treatment consists of severing of all the produces a restriction.
adhesions after disinsertion of the lateral or • Treatment consists of recessing the antagonist
superior rectus muscle. To comfirm that, all the muscle.
 352 Theory and Practice of Squint and Orthoptics

ADHESIVE SYNDROME • Cysticercosis of extraocular muscles

• Adhesive syndrome or cicatricial strabismus • Mild grade orbital cellulitis.
refers to a restrictive type of strabismus Clinical features include:
which occurs following squint surgery, most • Ocular pain
commonly after inferior oblique myectomy
• Conjunctival congestion
done at the insertion end of the muscle.
• Proptosis
• It is thought to result from a fibrous and fatty
• Ptosis may also occur in some cases
proliferative inflammatory response following
• Deviation of the involved eye and diplopia
surgical entry of the portion of Tenon's capsule
and fat into the wound. • Restriction of eye movements (positive forced
duction test).
• Hypotropia is associated with an inferior
restriction or forced duction test. Investigations helpful in diagnosis include:
• Orbital ultrasonography
POSTOPERATIVE SCARRING • CT scan/MRI imaging of the orbit and head
Postoperative scarring of the conjunctiva and (to rule out suspected neurocysticercosis).
extraocular muscles may occur producing Treatment
restrictions. Treatment consists of recession of • Systemic steroids are useful in idiopathic orbital
the affected muscles and conjunctiva. inflammatory diseases and autoimmune
Postoperative scarring of the Tenon's capsule myositis.
has been reported to produce an L-deformity of • Oral albendazole, under cover of steroids is
inferior oblique, J-deformity of rectus muscle useful in cysticercosis.
and cicatricial advancement of a rectus muscle. • Antibiotics and anti-inflammatory drugs are
• L-deformity of the inferior oblique occurs needed in mild grade orbital cellulitis.
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 13
Supranuclear Control and
Disorders of Ocular Motility

SUPRANUCLEAR CONTROL OF EYE • Optokinetic system

MOVEMENTS • Position maintenance system
Supranuclear ocular motor neural SUPRANUCLEAR DISORDERS OF EYE
pathway MOVEMENTS
• Cortical control centres • Ocular motor apraxia
• Subcortical control centres • Horizontal conjugate gaze paralysis
• Internuclear ophthalmoplegia
Supranuclear eye movement systems
• One-and-a-half syndrome
• Saccadic system • Vertical conjugate gaze paralysis
• Smooth pursuit system • Skew deviation
• Vergence system • Cogwheeling
• Vestibular system • Ocular dysmetria, ocular flutters and opsoclonus

CORTICAL CONTROL CENTRES

SUPRANUCLEAR CONTROL OF
EYE MOVEMENTS The cortical control centres include:
• Frontal ocular motor area, and
There exists a highly accurate, still not fully • Parieto-occipitotemporal (POT) junction.
elucidated, supranuclear control of eye
movements which keeps the two eyes yoked I. Frontal ocular motor area
together so that the image of the object of The frontal ocular motor area is primarily
interest is simultaneously held on both foveas involved in the saccadic eye movement system.
despite the movements of the perceived object It is thought to control voluntary rapid conjugate
or the observer’s head and/or body. For the gaze, both vertically and horizontally. Recently
purpose of understanding, the neural control four main cortical areas involved in the
of eye movements can be discussed under two generation of saccades have been recognized.
parts: (1) supra-nuclear ocular motor neural These include: (1) frontal eye field (FEF),
pathway and (2) supra-nuclear eye movement (2) supplementary eye field (SEF), (3) dorso-
systems. lateral prefrontal cortex (DLPFC), and
(4) posterior eye field (PEF).
The frontal eye fields (FEFs) are located at
SUPRANUCLEAR OCULAR MOTOR Brodman’s area 8, the posterior end of the
NEURAL PATHWAY second frontal convolution.
The supranuclear pathway concerned with the Role of FEFs is as below:
control of various ocular movements include • Horizontal gaze movements result, when the
(Fig. 13.1): extraocular muscles receive signals from one
• Cortical control centres, and hemisphere (contralateral) only. Stimulation
• Subcortical control centres. of the frontal lobe area, on the right, for
355
 356 Theory and Practice of Squint and Orthoptics

Fig. 13.1 Showing supranuclear connections of ocular motor neural pathway.

instance, leads to conjugate movements of latter projections modulate visually directed

both eyes to the left (Fig. 13.2). saccadic eye movements. The POT junction
• Voluntary vertical conjugate gaze movements cortex plays the key supranuclear role in the
occur, when equal signals are transmitted visual ocular reflex by way of projections to the
simultaneously from the both frontal ocular PPRF and riMLF. This reflex keeps a moving
motor areas (Figs 13.3 and 13.4). image projecting on the fovea. There are specific
efferent fibres for horizontal, vertical, and
II. Perieto-occipitotemporal (POT) junction torsional movements.
The ipsilateral parieto-occipital ocular motor Damage to only one side of the MT cortex
area is primarily concerned with the fixation and slows ipsilateral slow pursuit, requiring catch-
pursuit movements. Following cortical areas up saccades. Such lesions also temporarily
have been identified in relation with the pursuit impair pursuit responses to fast targets in
movements: moving in either direction.
• Middle temporal (MT) visual area, and ii. Medial superior temporal (MST) visual area:
• Medial superior temporal (MST) visual area In human, it is considered to lie superior and a
little anterior to MT area within the inferior
i. Middle temporal (MT) visual area. The
parietal lobe.
cerebral cortex in the region of the POT junction
is important in the control of smooth pursuit eye SUBCORTICAL CONTROL CENTRES
movements and object tracking in space. This
area is known as the middle temporal (MT) area The brainstem control centres include:
in non-human primates. The area of the human • Paramedian pontine reticular formation
brain that is the equivalent of the MT cortex of (PPRF)
the non-human primate is Flechsig’s area 10. The • Rostral interstitial nucleus of medial longi-
MT receives visual information from the striate tudinal fasciculus(riMLF)
and prestriate cortex. It projects to the brainstem, • Convergence and divergence centre
cerebellum, superior colliculi, and the FEF. The • Posterior commissure
 Supranuclear Control and Disorders of Ocular Motility 357

Fig. 13.2 Pathway for horizontal gaze saccadic eye movements. The horizontal gaze centre (present in PPRF) is connected
with ipsilateral lateral rectus muscle (LR) and with abducens internuclear neurons whose axons cross the midline and travel
in the medial longitudinal fasciculus (MLF) of the opposite side to that part of the nucleus of IIIrd nerve which innervates the
medial rectus muscle.
358 Theory and Practice of Squint and Orthoptics

Fig. 13.3 Pathway for vertical gaze (downgaze) saccadic eye movements.
 Supranuclear Control and Disorders of Ocular Motility 359

Fig. 13.4 Pathway for vertical gaze (upgaze) saccadic eye movements.
 360 Theory and Practice of Squint and Orthoptics

• Superior colliculus contains neurons which appear to be involved

• Vestibular apparatus in vertical gaze holding and vertical pursuit.
• Cerebellum • Afferent connections. The vertical gaze centre
• Medial longitudinal fasciculus (riMLF) receives impulses from both the
frontal and occipital cortical ocular motor
1. Paramedian pontine reticular formation centres as well as from the superior colliculus.
Horizontal gaze centre. The paramedian pontine • Efferent connections. The riMLF nucleus
reticular formation (PPRF) is the primary centre projects through the posterior commissure to
responsible for generating horizontal conjugate its equivalent on the other side of the
gaze. The PPRF is positioned ventral to the mesencephalon as well as directly to the nuclei
medial longitudinal fasciculus (MLF). It extends of III and IV cranial nerves supplying the
from the level of the trochlear nerve nucleus to extraocular muscles concerned with the
the abducens nerve nucleus (Fig. 13.1). vertical movements (Figs 13.3, 13.4 and 13.6).
• Afferent connections. Most afferent connections
to the PPRF are through the vestibular nucleus.
3. Posterior commissure
It receives signals from both the frontal cortical
areas concerned with generation of saccades and Dorsal and rostral to the riMLF is the posterior
ipsilateral occipitoparietal cortical area concerned commissure, a fibre tract that contains some
with generation of pursuits directly (for voluntary scattered neuronal cell bodies. Lesions in this
movements) and through the superior colliculus region produce abnormalities of upward gaze.
(for involuntary movements). Vestibular input for It is likely that the fibres for upward gaze leave
horizontal eye movements comes from the the riMLF and pass through this region before
contralateral vestibular apparatus by way of the reaching the oculomotor and trochlear nuclei.
vestibular nuclei. An axon from the vestibular Involvement of the posterior commissure may
nucleus crosses to the opposite abducens nucleus, be part of the dorsal midbrain syndrome
where it innervates a motor neuron and an (Parinaud syndrome). In this syndrome, there
internuclear neuron for horizontal gaze in the is impairment of upwardly directed saccades or,
opposite direction (Fig. 13.2). in extreme cases, loss of all vertical movement.
• Efferent connections. The centre for horizontal Other signs include pupillary mydriasis and
gaze movements in turn is connected with the light-near pupillary dissociation, corectopia, and
homolateral abducent nucleus present next to convergence-retraction nystagmus.
it. The axons of the internuclear neurons of the
abducent cross the midline and travel through 4. Convergence and divergence centre
the medial longitudinal fasciculus (MLF) of the At present, nothing is known about the location
opposite side to that part of nucleus of the of a subcortical divergence centre. In fact, there
oculomotor nerve which innervates the medial is no evidence that such a centre exists at all.
rectus muscle (Figs 13.2 and 13.5). Therefore, Similarly, a subcortical centre for convergence
impulses from this centre produce contraction also may not exist at all and convergence as well
of the homolateral lateral rectus muscle and the as divergence may be purely cortical functions.
opposite medial rectus, hence binocular gaze However, clinical evidence points that the
movements to the side of the stimulated centre. pretectal area is probable site for the subcortical
convergence centre; since lesions in this area
2. Rostral interstitial nucleus of abolish convergence. The impulses to the so-
medial longitudinal fasciculus called convergence centre come from the frontal
Vertical gaze centre. The vertical gaze centre is and the occipital ocular motor centres.
the rostral interstitial nucleus of the medial long- Convergence occurs only upon simultaneous
itudinal fasciculus (riMLF) located at the level bilateral stimulation of either the frontal or the
of upper pole of the red nucleus. Slightly caudal occipital motor centres. These impulses are
to the riMLF and directly connected to it lies the relayed to the nuclei of both third nerves which
interstitial nucleus of Cajol (INC). This nucleus innervate the medial recti muscles (Fig. 13.8).
 Supranuclear Control and Disorders of Ocular Motility 361

Fig. 13.5 Neural pathway of horizontal pursuit eye movements.

5. Superior colliculus substantia nigra. There are efferent projections to

These structures in the dorsal midbrain play a role the brainstem premotor areas. The superior
in both ocular motor and sensory function. The colliculus can generate visually directed saccades
superior colliculus receives visual input directly independently and may play a role in the control
from branches of retinal ganglion cell axons. of pursuit eye movements. In primates, ablation
Visual input also comes indirectly from the visual of both FEFs and both superior colliculi is
cortex, the parietal and frontal lobes, and the necessary to produce permanent saccadic defects.
 362 Theory and Practice of Squint and Orthoptics

Fig. 13.6 Neural pathway of vertical persuit (downgaze) eye movements

6. Vestibular apparatus even in blind. The vestibular apparatus is a

Reflex eye movements that compensate for receptor specialized to sense changes of
changes in the position of head or body originate equilibrium and position. It is part of the inner
from the vestibulum. They are called statokinetic ear and is comprised of three semicircular
reflexes. If, for instance, the head is turned to canals, the sacculus and the utriculus, all of
the left, the eyes perform an involuntary which belong to the membranous labyrinth. The
compensatory movement to the right which apparatus derives its name from the vestibulum,
allows continuous fixation of the object. These which is the bony cavity housing the utriculus
reflexes are innate and unconditioned, occurring and the sacculus.
 Supranuclear Control and Disorders of Ocular Motility 363

Fig. 13.7 Neural pathway of vertical persuit (upgaze) eye movements.

Semicircular canals certain pull on the sensory hair of the cristae.

The semicircular canals contain receptor organs This stimulus excites the receptor cells and elicits
called cristae that sense movements of the head. a nerve impulse that is transmitted through the
Each of the canals is oriented perpendicular to vestibular nerve (part of nerve VIII) to the
the other two so that a three-dimensional vestibular nuclei in the brainstem.
structure is formed roughly coinciding with the
horizontal, vertical, and frontal planes. The Sacculus and utriculus
canals are filled with a watery fluid, the The sacculus and the utriculus each contains a
endolymph. When the head moves, the macula that functions in a manner similar to that
endolymph is subjected to inertia and exerts a of the cristae of the semicircular canals. Whereas
 364 Theory and Practice of Squint and Orthoptics

the vestibular nerve that supply the maculae. The

function of the sacculus is not known.
Vestibular nerve
The vestibular nerve (part of nerve VIII) enters
the brainstem at the level of the lower end of
the pons and terminates in the vestibular nuclei.
The vestibular nuclei, in turn, are connected
directly to the abducens nuclei and to the nuclei
of the ocular motor nerves through the medial
longitudinal fasciculi (Figs 13.3–13.7).
Stimulation of a labyrinth or of a vestibular
nerve causes conjugate deviation of the eyes to
the opposite side. Depending on the nature of
the stimulus, the eyes may remain in the deviate
position or a nystagmus may result. A
nystagmus is an oscillatory movement of the two
eyes. Both eyes turn in the same direction and
are suddenly pulled back into the primary
position by a fast, jerky movement, where upon
the cycle begins again with conjugate deviation.
Usually, the first phase of nystagmus, the
conjugate deviation, is distinctly slower than the
second phase in which the corrective movement
takes place. Diagnostically, vestibular nystagmus
can be produced to test the integrity of the
vestibular reflex mechanism. For this, the patient
is either submitted to rotation on a revolving
chair (rotatory vestibular nystagmus) or his/her
external auditory canals are irrigated with hot
or cold water (caloric vestibular nystagmus).

7. Medial longitudinal fasciculus

The medial longitudinal fasciculus (MLF) is a
fibre tract that extends from the spinal cord to
the oculomotor nerve nucleus. It contains
primarily ascending fibres, the majority of which
arise in the superior and medial vestibular
nuclei. The MLF is in close proximity to the
Fig. 13.8 Presumptive pathway of convergence. ocular motor nuclei and influences both
ipsilateral and contralateral nuclei.
the cristae respond to head movements Functions of MLF. The medial longitudinal
(statokinetic reaction), the macula of the utricle fasciculus plays an important role in the pathway
responds to gravity (static reaction). On top of of ocular movements. Its main functions can be
each macula rests a gelatinous plate into which summarized as follows (Fig. 13.9):
protrudes hair from the surface of the macula. • It connects the oculomotor nuclei with one
The plates, which contain mineral crystals, follow another.
the force of gravity and slide to which ever side • It transmits signals from the subcortical–
is dependent, thereby pulling on the hair. This horizontal gaze centre for the horizontal
leads to stimulation of the terminal branches of versions to the opposite medial rectus muscle.
 Supranuclear Control and Disorders of Ocular Motility 365

Fig. 13.9 Connections of medial longitudinal fasciculus (MLF).

• It transmits impulses originating from the Lesions of MLF. An abnormality of the MLF
vestibular nucleus (in response to statokinetic causes problems with horizontal and vertical
stimulation) to the ocular motor nuclei as well gaze co-ordination of the two eyes. The clinically
as to nucleus innervating muscles of head and most important connection passing through the
neck. MLF links the contralateral abducens nucleus
• It relays signals from the proprioceptors of the with the ipsilateral medial rectus subnucleus.
head and neck muscles to the ocular motor Abnormalities of this tract produce an
nuclei. internuclear ophthalmoplegia. Such a lesion
 366 Theory and Practice of Squint and Orthoptics

produces slowed or complete loss of adduction one object to another. Thus they are performed
of the ipsilateral eye and abducting nystagmus to bring the image of an object quickly on the
of the fellow eye. fovea. Though normally voluntary, saccades
may be involuntary aroused by peripheral,
8. Cerebellum visual or auditory stimuli. The saccades
include:
The cerebellum appears to be involved in the
• Horizontal saccades, and
immediate modulation of ongoing eye
movements, as well as in the long-term adaptive • Vertical saccades.
processes that compensate for ocular motor Detailed features of saccadic eye movements are
dysmetria. The cerebellum controls and adjusts described on page 34.
the size of saccades. The latter ability is essential
for maintaining accurate ocular motor Neural pathway
performance during growth and aging, during Cortical areas
and after ocular motor disease, or even while The pathway originates in the premotor cortex
using spectacles. For instance, the use of aniso- of the frontal motor area. From there, the fibres
metropic spectacles produces a varying for voluntary saccades pass directly and for
anisophoria in different directions of gaze, involuntary saccades through the superior
which must be compensated in each direction colliculus to the contralateral horizontal gaze
of gaze. centre in PPRF (Fig. 13.2).
Hemicerebellectomy produces ipsilateral
Pathways involved in the cortical generation
saccadic and contralateral pursuit defects,
of saccades
while total cerebellectomy creates persistent
It appears that there are three pathways
saccadic dysmetria and abolishes smooth
involved in the cortical generation of saccades:
pursuit. The cerebellum has numerous
connections to nuclear and supranuclear ocular i. Ventral pathway. The ventral pathway
motor centres. projects by way of the posterior portion of the
anterior limb of the internal capsule and the
medial part of cerebral peduncle to reach the
SUPRANUCLEAR EYE MOVEMENT pons, where there is a partial decussation and
SYSTEMS termination in the PPRF.
Following supranuclear eye movement systems ii. The dorsal pathway passes from the FEF
have been recognized: through the thalamus, the pulvinar, the pretectal
• Saccadic system nuclei, and the superior colliculus to reach the
brainstem.
• Smooth pursuit system
• Vergence system iii. The intermediate pathway extends from the
FEF to the rostral ocular motor nuclei and the
• Vestibular system
interstitial nucleus of Cajal.
• Optokinetic system
• Position maintenance system Brainstem pathway
All these systems perform specific functions Recent evidence suggests that the saccades
and each one is controlled by a different neural (horizontal as well as vertical) are generated by
system but share the same final common path, groups of neurons located in the brainstem and
i.e. the motor neurons that supply the are controlled by higher frontal system (for
extraocular muscles. voluntary saccades) and collicular system (for
involuntary saccades). The brainstem neurons
SACCADIC SYSTEM concerned with generation of saccades form the
Saccades are sudden, jerky conjugate eye final premotor circuits. These neurons are of
movements that occur as the gaze shifts from three types:
 Supranuclear Control and Disorders of Ocular Motility 367

• Excitatory burst neurons (EBN) nucleus for superior oblique muscles. riMLF
• Inhibitary burst neurons (IBN), and nuclei of both sides are connected by a
• Pause neurons (PN). commissure which projects into the interstitial
nucleus of Cajal (INC) and to the ipsilateral
Brainstem pathway for saccades is described
IIIrd nerve nucleus.
below.
• For upward saccades (Fig. 13.4), the activated
Pathway for horizontal saccades neurons in the riMLF send impulse through
For horizontal saccades, the excitatory neurons are the fibres that synapse upon the inferior
located in horizontal gaze centre in paramedian oblique subnucleus of the ipsilateral IIIrd
pontine reticular formation (PPRF) and project nerve; and through the fibres which pass via
to the ipsilateral abducens nucleus. The axons posterior commissure and synapse upon the
from these cells synapse in the abducens nucleus superior rectus subnucleus of the contra-
on motor neurons that innervate the ipsilateral lateral IIIrd nerve.
lateral rectus and on the interneurons that Activities of brainstem involved in generation
innervate contra-lateral medial rectus subnucleus of saccades neurons
by way of the contralateral MLF (Fig. 13.2).
As mentioned above, three types of neurons are
involved in generation of saccades: Excitatory
Pathway for vertical saccades
burst neurons (EBN), inhibitory burst neurons
For the vertical saccades, the excitatory neurons (IBN) and pause neurons (PN). These neurons
are located in the vertical gaze centre formed generate saccades by a ‘pulse-step’ innervation
by rostral interstitial nucleus of medial system (Fig. 13.10). The ‘pulse’ is created by
longitudinal fasciculus (riMLF) and other sudden firing of the neurons to the extraocular
neurons in the region of posterior commissure. muscles. After the eyeball is moved to the new
• For downward saccades (Fig. 13.3), the activated position; to keep it in the same position,
neurons in the riMLF send impulse directly sustained contraction of the muscle is required.
through the fibres that synapse upon the This is called a step and is affected by tonic
inferior rectus subnucleus of the ipsilateral contraction of muscles due to continuous
IIIrd nerve and contralateral IVth nerve discharge from neurons.

Fig. 13.10 Showing relation between the three sets of neurons (excitatory burst neurons, pause neurons and inhibitory
burst neurons) concerned with generation of saccades.
 368 Theory and Practice of Squint and Orthoptics

Excitatory burst neurons then possibly directly to the ocular motor nuclei.
The excitatory burst neurons (EBNs) discharge The right occipital lobe, therefore, controls
at high frequencies just prior to and during the pursuits to the right and the left occipital lobe
saccades and provide the eye velocity those to the left. The cerebellum is closely
commands known as the pulse (Fig. 13.10). Burst associated with normal pursuit movements. The
cells discharge, only when there is need for a FEF and the superior colliculi paly a modulating
fast eye movement and do not discharge during role in the production of pursuit eye movements
fixation, pursuit or vergence eye movements. by POT junction. Lesions in the POT area
The EBNs send impulses to the neurons of produce ipsilateral pursuit defects.
cranial nerve nuclei supplying to yoke muscles
for the gaze movements. VERGENCE MOVEMENT SYSTEMS
Inhibitory brust neurons (IBNs) Vergence movements allow focussing of an
The inhibitory burst neurons (IBNs) send impulses object which moves away from or towards the
through the medullary reticular formation to the observer or when visual fixation shifts from one
neurons of cranial nerve nuclei supplying to the object to another at a different distance.
antagonist muscles of the yoke muscles for the Vergence movements are very slow (about 20º/
concerned gaze movement and thus inhibit these sec) disjugate movements. They have a latency
muscles and allow the gaze movement to occur. of about 160 msec. Different types of vergence
Their firing rate is inversely proportional to movements are described on page 35.
the excitatory burst cells Neural pathway. The exact neuroanatomical
Pause neurons substrate is not known. Stimulation of parieto-
These neurons discharge tonically, except just occipital region (area 19 or 22) provokes
before and during saccades, when they pause. vergence movements in monkeys. Premotor
They appear to exert an inhibitory influence on signals are thought to originate in the
the burst neurons preventing extraneous mesenchephalic reticular formation with
saccades occurring during fixation. These cells separate population of cells for convergence and
inhibit the burst cells within the ipsilateral PPRF. divergence. The fibres travel then to the relevant
These cells are important during fixation and cranial nuclei (Fig. 13.8). This link seems to
smooth pursuit. Abnormalities of these cells lead course outside the MLF, since the MLF lesions
to opsoclonus and ocular flutter. usually spare convergence.

SMOOTH PURSUIT EYE MOVEMENT SYSTEM VESTIBULAR EYE MOVEMENT SYSTEM

Smooth pursuit movements are tracking Vestibular movements are usually effective in
movements of the eye as they follow moving compensating for the effects of head movements
objects. These occur voluntarily, when the eyes in disturbing visual fixation (vestibulo-ocular
track moving objects but take place invo- reflex—VOR). These movements operate
luntarily, if a repetitive visual pattern is through the vestibular system (see page 362).
displayed continuously. Their features are Most rotations of the head do not involve
described on page 34. When the velocity of the angular rotations as fast as 300º/sec and the
moving object is more, the smooth pursuit vestibular system can compensate for these.
movement is replaced by small saccades (catch- However, when the body is rotated at great
up saccades). speeds around a vertical axis (e.g. a skater
Neural pathway for pursuit movements performing a spin), eye movements show the
originates in the cortex of the perito-occipito- so-called oculovestibular nystagmus, with a
temporal (POT) junction. The fibres then slow motion of the eyes in the opposite direction
descend and terminate in the ipsilateral PPRF to that of rotation—this is initiated by the
for horizontal pursuits (Fig. 13.5) and the vestibular mechanism—followed by a quick
ipsilateral mesencephalic reticular formation for jerky binocular ‘return’ movement in the
the vertical pursuits (Figs 13.6 and 13.7); and direction of rotation. This sequence is repeated
 Supranuclear Control and Disorders of Ocular Motility 369

as long as the angular acceleration lasts. It is mesencephalon as occipitoparieto-mesencephalic

likely that the fast component of nystagmus is pathway.
mediated by mechanisms similar to those Supranuclear disorders thus result from the
responsible for saccades. lesions involving the cortical centres or the
lesions interrupting the frontomesencephalic
OPTOKINETIC SYSTEM
pathway, occipitoparietomesencephalic path-
This system helps to hold the images of the seen way, cerebellar pathways, centres for horizontal
world steady on the retinae during sustained and vertical conjugate gazes and medial
head rotation. This system becomes operative, longitudinal fasciculus. Thus, the supranuclear
when the vestibular reflex gets fatigued after 30 ocular motility disturbances result from
seconds. The optokinetic response is evoked by interruption of the neural pathway before they
rotation of the visual field before the eyes. It reach the eye movement generators.
consists of a movement following the moving
scene, succeeded by a rapid saccade in the Characteristic of supranuclear disorders
opposite direction. In fact, the transient head The basic characteristic of supranuclear lesions
rotation stimulates both the vestibulo-ocular (other than those interrupting vergence
reflex with a latency of only 10 ms and the mechanism and the medial longitudinal
optokinetic reflex with a latency of 70 ms.
fasciculus) are that
However, during sustained rotation with eyes
• Both eyes are affected equally and that the
open, the vestibulo-ocular reflex ceases while the
disturbance is one of deficient or defective
optokinetic system maintains a steady discharge
conjugate eye movements.
from the vestibular nuclei to sustain the
• They usually do not produce diplopia because
compensatory optokinetic nystagmus.
both eyes are equally involved.
POSITION MAINTENANCE SYSTEM List of common supranuclear disorders
This system helps to maintain a specific gaze Some of the common supranuclear disorders are
position by means of rapid micromovements as follows:
called ‘flicks’ and slow micromovements called • Oculormotor apraxia
‘drifts’. This system coordinates with other
• Horizontal conjugate gaze paralysis
systems.
• Internuclear ophthalmoplegia
Neural pathway for this system is believed to
• One-and-a-half syndrome
be the same as for saccades and smooth pursuits.
• Vertical conjugate gaze paralysis
• Skew deviation
SUPRANUCLEAR DISORDERS OF EYE • Cogwheeling
MOVEMENTS • Ocular dysmetria, ocular flutter and opsoclonus
INTRODUCTION • Double elevator paralysis
• Nystagmus, and
Anatomic background and site of lesions
• Comitant squint. (It is considered the most
As described above, the supranuclear pathways common supranuclear disorder. It is being
connect the frontal lobe, occipital lobe and presumed that the comitant squint represents
cerebellum with the mesencephalon, i.e. primarily a deficiency in the vergence
midbrain and pons (containing the nuclei movement mechanism).
controlling the extraocular muscles and the
centres for horizontal and vertical conjugate Differentiating supranuclear from
gazes). The pathway transmitting impulses from nuclear and infranuclear lesions
the frontal lobe centres to the mesencephalon In patients with gaze palsy, following tests can
may be referred to as frontomesencephalic pathway be used to know whether the eyes can be moved
and that connecting occipital lobe centres with reflexively in the direction of the paresis.
 370 Theory and Practice of Squint and Orthoptics

1. Oculocephalic (doll’s eye) reflex test. Clinical features

Patient’s head is tilted forward by 30 degrees Clinical features include:
and asked to fixate a distant target. Patient’s • An inability to generate normal, voluntary
head is then rotated in the direction opposite horizontal saccades. In early infancy,
to the gaze palsy and movements of the eyes blindness may be suspected because of the
are noted. Since this test uses direct projections inability to fixate or follow objects.
from the vestibular system to the oculomotor • Changes in horizontal fixation are made by a
nuclei, gaze palsies caused by lesion of the head thrust that overshoots the target
cerebral cortex can be typically overcome. followed by a rotation of the head back in the
However, in prenuclear nuclear or infranuclear opposite direction once fixation is established.
lesions, the doll’s eye reflex does not overcome • Vertical saccades and random eye movements
the palsy. are intact.
• Vestibular and optokinetic nystagmus are
2.Vestibulo-ocular reflex (VOR) testing. To
impaired.
perform VOR test, patient’s head is tilted by 60
• Reading can be difficult with this condition.
degrees and the external auditory meatus is
• Developmental delay, especially motoric, may
irrigated with either cold or warm water to
be present.
stimulate the horizontal semicircular canal. In
patients with supranuclear gaze palsies the Differential diagnosis
stimulation causes the eye to slowly deviate
Congenital ocular motor aparaxia (COMA) need
resulting in nystagmus with fast (corrective)
to be differentiated from ‘Familial horizontal
phase to the opposite side with cold stimulation gaze palsy with scoliosis’(HGPS). HGPS is an
and same side with warm stimulation (to autoimmune recessive disorder characterized by
remember the mneumonic COWS: Cold- paralysis of horizontal gaze since birth,
Opposite; Warm-same). progressive scoliosis, impaired OKN and VOR
reflexes, but intact convergence and vertical eye
OCULAR MOTOR APRAXIA movements.
A total loss of voluntary conjugate gaze in all
directions is known as oculomotor apraxia. In Acquired ocular motor apraxia
order to bring their eyes into the desired gaze Bilateral lesions of the frontoparietal cortex have
position, patients with oculomotor apraxia been reported to be associated with the acquired
characteristically have a head thrusting oculomotor apraxia. It needs to be differentiated
movement. Oculomotor apraxia can be con- from the conditions that affect the generation of
genital or acquired. voluntary saccades, including metabolic and
degenerative diseases such as Huntington's
Congenital ocular motor apraxia chorea.
Etiopathogenesis
HORIZONTAL CONJUGATE GAZE PARALYSIS
Congenital ocular motor apraxia (COMA) is a
Horizontal conjugate gaze paralysis refers to
rare disorder of unknown etiopathogenesis. It is
equal paralysis of same sided horizontal
more common in boys than girls and may be
movement in both eyes, i.e. either levoversion
familial.
or dextroversion is deficient or defective.
• Agenesis of the corpus callosum and hydro-
cephalous have been reported to be associated Site of lesion
with some cases. As described earlier, the frontal lobe is
• Mass lesions of cerebellum compressing the rostral connected with the mesencephalon through the
part of the brainstem have been reported in frontomesencephalic pathway. This pathway
many infants presenting with oculomotor descends into the internal capsule and is thought
apraxes. to cross completely at the level of fourth nerve
 Supranuclear Control and Disorders of Ocular Motility 371

nucleus (Figs 13.2–13.4). It is primarily involved Efferent fibres from the occipital lobe
in the saccadic eye movement system. connecting the mesencephalon from the
A destructive lesion in the cortical centre or occipitoparietomesencephalic pathway. This
frontomesencephalic pathway above decussation pathway also crosses to the opposite side at the
(Fig. 13.11A) produces defects in conjugate gaze level of 4th nerve nucleus. Lesions involving this
to the opposite side. Lesions below the pathway will also produce conjugate movements
decussation (Fig. 13.11B) produce defects in to the opposite side or same side depending upon
conjugate gaze to the same side as the lesion. the site of lesions similar to frontal lobe lesions.
Lesions involving 6th nerve nucleus (1 in However, the conjugate paralysis caused by
Fig. 13.14) and PPRF (2 in Fig. 13.14) can also lesions of occipitoparietal pathway is of lesser
produce horizontal gaze palsy on the same side. magnitude than that caused by lesions involving
It is important to note that since the area of the the frontal voluntary system.
frontal lobe concerned with horizontal eye
movements is so large, small lesions of the Causes of conjugate paralysis
frontal lobe rarely affect conjugate horizontal 1. Vascular lesions producing infarctions or
gaze. haemorrhages.
2. Inflammatory lesions
3. Tumours

Clinical features
As described above, when the frontomesence-
phalic tract is interrupted by a lesion above the
crossing, there is a deficit of rapid eye move-
ments to the opposite side; thus the right sided
lesion will affect:
1. Saccades to the left.
2. The fast phase of the optokinetic nystagmus,
when it is to the left.
3. The fast phase of caloric or vestibular
nystagmus when it is to the left.
4. On looking to the left, there may be a gaze
paretic nystagmus with the fast phase to the
left with varying amplitude and rhythm.
5. Gaze paralysis and conjugate deviations caused
by hemispheric lesions are usually transient,
probably due to compensation from the
contralateral uninvolved hemisphere. While,
paralysis resulting from lesions in the brainstem
are less marked but last as long as the lesion
exists since no compensation of function occurs.
6. Lesions causing gaze paralysis (e.g. infarction,
haemorrhages and tumours) also produce
severe neurologic signs such as hemiplegia
and thus the eye signs are usually over-
shadowed.

INTERNUCLEAR OPHTHALMOPLEGIA
Fig. 13.11 Site of lesions producing conjugate paralysis to Internuclear ophthalmoplegia (INO) results from
the opposite side (A) and the same side (B). a lesion of the medial longitudinal fasciculus
 372 Theory and Practice of Squint and Orthoptics

(MLF) that interrupts fibres passing from the • Tropia in the involved side.
subcortical centre for horizontal gaze of one side • Limitation of adduction on the involved side
to the nucleus of the third nerve on the other side (Fig. 13.13A) and
(Fig. 13.12A). It is the only supranuclear defect • Normal abduction of the opposite eye
that does not result in a gaze paralysis. (Fig. 13.13B).
Etiology • Nystagmus is associated with abduction of the
• Disseminated sclerosis is the most common opposite side. Nystagmus in INO is a
cause of bilateral INO. secondary response to the weakness of
• Cerebrovascular accident and brain tumour can adduction and not caused directly by the
also cause bilateral INO. central defect.
• Infarct of small branch of the basilar artery is • Convergence is normal in many patients and thus
usually associated with unilateral INO. both eyes adduct normally to fixate a near object.
• Skew deviation of some degree may be noted
Clinical features with the eye ipsilateral to the lesion slightly
Unilateral INO. As is clear from the Fig. 13.11, higher than the other.
patient with unilateral INO on version
movements will exhibit: Bilateral INO is more frequent than unilateral
INO. Depending upon the site of lesion, other
cranial nuclei and convergence mechanism may
also be involved.

Differential diagnosis
1. Isolated medial rectus palsy needs to be
differentiated from the INO.
2. Pseudointernuclear ophthalmoplegia should
be distinguished from the true INO. Pseudo-
INO, i.e. a condition characterised by limitation
of adduction and nystagmus of the abducting
eye caused by lesions other than lesions of MLF,
such as:
• That occurring due to myasthenia gravis
• That following recession and retroequa-torial
posterior fixation of both medial rectus
muscles.

Fig. 13.13 Diagrammatic depiction of right internuclear

ophthalmoplegia. Note right exotropia in primary position
Fig. 13.12 Site of lesion (A) producing internuclear (A) and limitation of adduction in the right eye with normal
ophthalmoplegia. abduction in the left eye (B).
 Supranuclear Control and Disorders of Ocular Motility 373

Treatment
Unfortunately, still there is no effective
treatment for INO.

ONE-AND-A-HALF SYNDROME
One-and-a-half syndrome (paralytic pontine
exotropia) consists of a unilateral internuclear
ophthalmoplegia and a contralateral-horizontal
gaze palsy.

Etiology
An extensive caudal lesion in the pons can affect
the horizontal gaze centre and the adjacent MLF,
resulting in a palsy of both medial rectus
muscles and one lateral rectus (i.e. gaze palsy
plus INO) (Fig. 13.12B and 4 in Fig. 13.14).
Causes include demyelination, vascular,
tumour and inflammation.

Features
The main clinical and diagnostic features are as
follows: The only remaining horizontal movement
is abduction by the unaffected lateral rectus,
which is associated with the typical abducting
nystagmus. When the patient attempts to fixate
with this eye in the primary position, the
nystagmus will reduce or cease. There is,
therefore, a palsy of conjugate gaze on one side
and an INO on looking to the other side. A
marked compensatory head posture may be Fig. 13.14 Site of lesions producing supranuclear disorders.
adopted to achieve fixation with the preferred Horizontal gaze palsy may result from lesion involving 6th
nerve nucleus (1) and PPRF (2); Internuclear ophthalmo-
eye. Although complete ‘one-and-a-half’
plegia results from a lesion of medial longitudinal fasciculus
syndromes are rare, partial or incomplete (3), one-and-a-half syndrome results from a lesion involving
syndromes are more common. They can be both the MLF and PPRF (4); and vertical gaze palsy may
diagnosed on clinical assessment. A Hess chart result from a lesion involving riMLF (5).
is useful in monitoring the condition. However,
care must be taken in its interpretation, as gaze or both upward and downward gazes
synergist muscles are affected in patients with equally in both the eyes.
lesions involving the horizontal gaze centre as
well as the MLF (partial one-and-a-half Site of lesion, causes and clinical features
syndrome). Since the basis of the Hess chart is a As mentioned earlier (page 360), the vertical
comparison of action of synergistic muscles, the gaze centres (riMLF) are located at the level of
test is comparing abnormal with abnormal and, upper pole of red nucleus and that a vertical
if viewed in isolation, the gaze palsy element version occurs only upon simultaneous
may be missed. stimulation from both the hemispheres.
Therefore, vertical gaze paralysis will occur, if
VERTICAL CONJUGATE GAZE PARALYSIS there is a lesion of similar extent in each
Vertical conjugate gaze paralysis refers to hemisphere involving riMLF (5 in Fig. 13.14).
paralysis of either upward gaze or downward Further, the fact that paresis of upward gaze
 374 Theory and Practice of Squint and Orthoptics

may occur while the downward gaze is inferior rectus muscle or a unilateral superior
unaffected and vice versa, indicates that there rectus underaction. Incyclotorsion of the
must be some separation of the two centres hypertropic eye. This is similar to one-half cycle
anatomically. of see-saw nystagmus and reflects the common
occurrence of both disorders with lesions
The upgaze palsy is typically caused by lesions
involving the interstitial nucleus of Cajal. Other
involving the posterior commissure and
signs of central nervous system disease, usually,
characteristically occurs in Parinaud's dorsal
involving the brainstem and cerebellum. The
midbrain syndrome. The most common cause
strabismus can be monitored using a Hess chart.
of a Parinaud's syndrome is a pinealoma in
which deficiency of upward gaze is associated
COGWHEELING
with pupillary abnormalities.
Defects in slow pursuit tracking to the opposite
The downgaze palsy is less common and occurs, side may be produced by lesions affecting one
when both sides of the midbrain tegmentum occipital lobe. These pursuit movements may be
posterior to the red nucleus are damaged. interrupted by saccadic movements; this sign is
Downgaze palsy is frequently associated with known as cogwheeling. A lesion interrupting the
convergence paresis and thus it can be assumed occipitoparieto-mesencephalic pathway above
that perhaps the convergence centre and the the decussation in the midbrain will cause a
pathway mediating convergence are in close breakdown in tracking or following objects to
approximation to the centre for downgaze. the opposite field. When the lesion is below the
crossing of fibres, the defect is to the same side.
SKEW DEVIATION
Skew deviation is a vertical strabismus OCULAR DYSMETRIA, OCULAR FLUTTERS
resulting from a disruption of input into the AND OPSOCLONUS
oculomotor and trochlear nuclei. Cyclotorsion Ocular dysmetria. It is a defect in the saccadic
is often a feature and other symptoms and signs eye movement mechanism believed to be caused
of central nervous system disease are usually by lesions of the cerebellum and cerebello-
present. mesencephalic pathway. Normally, when a
patient is asked to make saccadic movement
Etiology from one target to another, there is a small
Skew deviation arises from a peripheral or undershoot and a precise end point is reached.
central imbalance of otolith inputs to the In ocular dysmetria, there is tendency of both
oculomotor and trochlear nuclei. eyes to overshoot the object of regard, and small
Structures involved in the pathogenesis of oscillations occur until a stable endpoint is
skew deviation include: The middle ear and the reached.
vestibular nerve; the vestibular nuclei in the Ocular flutter. It refers to rapid horizontal
brainstem; the cerebellum; the medial oscillations of the eyes in primary position. It is
longitudinal fasciculus; the interstitial nucleus thought to be related to fixation difficulties.
of Cajal in the midbrain.
Opsoclonus. It refers to a sequence of saccadic
Features eye movements that may be in either direction
but are predominantly horizontal. These
The features of skew deviation are: A vertical
movements are spontaneous and may show a
strabismus, which can be transient or perma-
rhythm to their amplitude and frequency.
nent. Transient deviations are common in
unilateral internuclear ophthalmoplegia. A
deviation, which may be concomitant or incomi-
tant. Incomitant skew deviation must be BIBLIOGRAPHY
differentiated from a cyclovertical muscle palsy, 1. Bender MB: The oculomotor decussation. Am J
and typically resembles a unilateral or bilateral Ophthalmol 54:591, 1962.
 Supranuclear Control and Disorders of Ocular Motility 375

2. Bizzi E: Discharge of frontal eye field neurons 5. Daroff RB: Physiologic, anatomic and patho-
during eye movements in unanesthetized physiologic considerations of eye movements.
monkey. Science 157:1588, 1967. Trans Ophthalmol Soc UK 90:410, 1970.
3. Bizzi E: Discharge of frontal eye fields neurons 6. Gay AJ, Newman NM, Keltner JL., Stroud MH:
during saccadic and following eye movements Eye Movement Disorders. St Louis. CV Mosby
in unanesthetized monkey. Exp Brain Res Co. 1974.
6:69,1968. 7. Weber R. Daroff RB: The metrics of horizontal
4. Daroff RB: Control of ocular movement. Br. J saccadic eye movements in normal humans.
Ophthalmol 58:217, 1974. Vision Res 2:921, 1971.
 14
Nystagmus and Related Oscillations

NYSTAGMUS • Nystagmus due to abnormalities of the

Definition and features mechanism for holding eccentric gaze
• Definition Clinical evaluation and recording of eye
movements
• Features
Pathophysiology of nystagmus
• Clinical evaluation
• Recording of eye movements
Classification
Treatment of nystagmus
Physiological nystagmus
• Optical
• Optokinetic nystagmus • Medical
• End-point nystagmus • Surgical
• Physiological vestibular NYSTAGMUS LIKE OSCILLATIONS
nystagmus
Inappropriate Saccades
Pathological nystagmus Saccadic intrusions
Early onset (childhood) nystagmus • Square wave jerks
• Infantile nystagmus syndrome • Square wave pulses
• Fusion maldevelopment Saccadic oscillations
nystagmus syndrome • Macro-saccadic oscillations
• Spasmus nutans syndrome • Ocular flutter
• Opsoclonus
Acquired nystagmus • Superior oblique myokymia
• Nystagmus associated with • Ocular bobbing
diseases of the visual system Voluntary saccadic oscillations
• Vestibular nystagmus imbalance Roving eye movements

NYSTAGMUS foveating and those that move the fovea away

from the object are called defoveating. In
DEFINITION AND FEATURES nystagmus, each cycle of movement is usually
Definition initiated by an involuntary, defoveating drift of
Nystagmus comes from the Greek word the eye away from the object of interest,
nystagmos (to nod) and may be defined as followed by a return movement. Based on the
repetitive, to and fro involuntary movement of characteristics of defoveating and foveating
one or both eyes that is initiated by a slow phase movements, the nystagmus may be of pendular
(drift). The movements which are not regular or jerky type.
and rhythmic are called nystagmoid movements. • Pendular nystagmus, in which both the
defoveating and refoveating movements are
Features of nystagmus movements slow (non-saccadic), and of equal velocity in
1. Type of waveform Eye movements that point each direction (Fig. 14.1A). It may be horizontal,
the retinal fovea at an object of interest are called vertical, oblique, rotatory or mixed.
376
 Nystagmus and Related Oscillations 377

• Jerk nystagmus is characterized by the slow simplicity of recording, the direction of

defoveating movement in one direction and nystagmus in case notes the following method
fast refoveating movement (saccadic) may be used:
component in the other direction; the later
a. Pendular nystagmus
being a recovery phenomenon aimed at
refixation. The direction of jerk nystagmus is • Horizontal
defined by the direction of fast component • Vertical
(phase). For instance, if the fast phase beat to • Rotatory
the right, this is called right beating nystagmus. • Oblique
Likewise the jerk nystagmus may be left, up,
b. Jerk nystagmus with quick phase
down or rotatory.
Waveform can further be characterized and • To the right
documented by the nature of the slow phase as • To the left
shown in Fig. 14.1B to E. • Up
2. Direction. Direction or plane of nystagmus can • Down
be horizontal, vertical, oblique, torsional or
mixed. For a jerk nystagmus, the direction is • Right rotatory (anti-clockwise)
described according to the fast phase. For • Left rotatory (clockwise)
• Oblique up to right or left
• Oblique down to right or left
3. Conjugacy. Nystagmus may be of conjugate,
disconjugate or dissociative type.
• Conjugate nystagmus. Binocular nystagmus
with symmetric direction, amplitude and rate
in both eyes.
• Disconjugate nystagmus. Monocular or
binocular with different directions and
frequencies in two eyes.
• Dissociative nystagmus refers to unidirectional
but assymetrical nystagmus in two eyes. Such
type of nystagmus is seen in internuclear
ophthalmoplegia.
4. Amplitude. The amplitude is measured in
degrees and represents the extent of movement
between the start of drift away from fixation and
the start of corrective movement in the opposite
direction. The amount of movement should be
approximately equal. In majority of cases, the
amplitude increases, when the patient looks in
the direction of the fast phase (Alexender’s law).
Fig. 14.1 Waveform characteristics of pendular nystagmus When the patient looks in the opposite direction,
(A) and, jerk nystagmus (B to E). (B) Jerk nystagmus with the amplitude reduces, the oscillation ceasesor
slow components of constant velocity; (C) Jerk nystagmus the direction of the nystagmus may reverse.
with slow components of exponentially increasing velocity; There are very few exceptions to this rule, but
(D) Jerk nystagmus with slow components of exponentially
increasing velocity with extended foveation periods that
in some cases of vertical nystagmus, the
follow slow movements; (E) Jerk nystagmus with slow amplitude increases on looking in the direction
components of exponentially decreasing velocity. of the slow phase.
 378 Theory and Practice of Squint and Orthoptics

Grading of amplitude. The amplitude is graded

as small, medium or large as below:
• Fine (small)—excursions less than 3°
• Medium—excursions between 5° and 15°
• Coarse (large)—excursions more than 15°
A dissociated nystagmus has different
amplitudes in both eyes. Subtle forms of
nystagmus, due to low amplitude or inconsistent
presence, require prolonged observation over 2–
3 minutes. Low amplitude nystagmus may be
detected only by viewing the patient’s retina with
an ophthalmoscope. In case records amplitude
is recorded by the thickness of arrows or
increasing lines in arrows, e.g.
• Small or
• Medium or
• Large or
5. Frequency. Frequency of nystagmus refers to
number of beats, i.e. to-and-fro movements per
second. Fig. 14.2 Foveation period in: A, Jerk nystagmus;
B, Pendular nystagmus.
Grading and recording of frequency. It is done into
low (slow), moderate and high (fast): position of gaze and increases with the decrease
• Low (Lo): 1–2 Hz in intensity of nystagmus.
• Moderate (M): 3–4 Hz 8. Null zone and neutral zone
• High (H): > 5 Hz • Null zone refers to the position of eyes where
a jerk nystagmus is absent. Some patients may
6. Intensity. Intensity of nystagmus is the
assume a head posture to bring the eyes in
product of amplitude and frequency.
the null zone.
Grading of intensity. Nystagmus of each type may • Neutral zone is the point from where fast
be seen in the primary position or it may be component of nystagmus changes its direction.
made apparent, only when the visual axes are It may be different than the null zone or may
deviated. On these grounds, nystagmus may be be the same.
graded as to its intensity into:
9. Trajectories of nystagmus
Recording of frequency Not infrequently patients with nystagmus show a
• First degree nystagmus. In it, the movements waveform that has vertical, horizontal and rotary
occur only in that direction of the gaze in components. When a horizontal and a vertical
which the quick phase occurs. component coexist and are superimposed on each
other, three characteristic nystagmus trajectories
• Second degree nystagmus. In it, the movements
can be observed (Fig. 14.3):
are also present in the primary position.
• Third degree nystagmus. In it, movements are
also present in the direction of the gaze to the
side opposite to that of the quick phase.
7. Foveation period. It is the period in the
waveform where eye velocity is at minimum
and thus visual acuity is maximum (Fig.14.2). Fig. 14.3 Trajactories of nystagmus: A, Oblique; B, Circular:
The foveation period is maximum in the null and C, Elipticle.
 Nystagmus and Related Oscillations 379

a. Oblique trajectory occurs when vertical and other words, nystagmus occurs due to
horizontal components are of equal frequency disturbances in the sensory or motor systems
and amplitude and in phase with each other. responsible for neuromuscular coordination of
b. Circular trajectory occurs when vertical and the extraocular muscles, which in turn provides
horizontal components are of equal frequency a steady fixation under normal circumstances.
and amplitude and are 90°, out of phase with • Sensory system consists of the retina, the
each other. vestibular system and the proprioceptive
c. Eliptical trajectory occurs when vertical and endorgans of the cervical musculature.
horizontal components are of equal frequency • Motor system is formed by the extrinsic ocular
but unequal amplitudes and are 90° out of muscles innervated by the oculomotor cranial
phase with each other. nerves. The cerebellum controls the muscle tone
and facilitates the smooth operation of the
PATHOPHYSIOLOGY OF NYSTAGMUS reflex responses involved.
Nystagmus and other related conditions in
essence disrupt steady fixation and thereby CLASSIFICATION
degrade vision. To understand the patho- Many classifications have been proposed for the
physiology of nystagmus, we must, therefore, nystagmus, and so, the reader will find different
first look at what are the mechanisms that classifications in different books. It is important
maintain steady fixation. to note here that the terms motor and sensory
nystagmus are no longer being considered.
Mechanisms that work together to National Eye Institute Workshop has developed
maintain a steady gaze a new classification in 2001 called the
1. Fixation, which has two distinct components: classification of eye movement abnormalities
a. The visual system’s ability to detect retinal and strabismus (CEMAS). In this volume, a
image drift and program corrective eye simple classification has been adopted by
movements; and slightly modifying CEMAS as below:
b. The suppression of unwanted saccades that A. Physiological nystagmus
take the eye off target. 1. Optokinetic nystagmus
2. Vestibulo-ocular reflex (VOR), by which eye 2. Endpoint nystagmus (eccentric gaze
movements compensate for head movements at nystagmus)
short latency during natural activities, especially 3. Physiological vestibular (caloric or rotational)
locomotion. The proprioceptors of the vestibular nystagmus
system are the semicircular canals of the inner 4. Voluntary nystagmus
ear, which respond to changes in angular B. Pathological nystagmus
acceleration due to head rotation4. I. Early onset (childhood) nystagmus
3. Neural integrator, ability of the brain to hold 1. Infantile nystagmus syndrome (includes old
the eye at an eccentric position in the orbit names such as ‘congenital’, ‘motor’, ‘sensory’,
against the elastic pull of the suspensory idiopathic and nystagmus blockage)
ligaments and extraocular muscles. A gaze- 2. Fusion maldevelopment nystagmus syndrome
holding network called the neural integrator (old names “latent, manifest latent,”
generates this signal. The cerebellum, ascending nystagmus blockage)
vestibular pathways, and oculomotor nuclei are
3. Spasmus nutans syndrome
important components of the neural integrator.
For effective working of the performance, • Without optic pathway glioma
three gaze-holding mechanisms are tuned by • With optic pathway glioma
adaptive mechanisms (recalibration) that monitor II. Acquired nystagmus. This group includes
the visual consequences of eye movements. various forms of nystagmus acquired after
A nystagmus is caused by defect in any of infancy, which can be further classified as
above mechanisms or their adaptive tuning. In follows:
 380 Theory and Practice of Squint and Orthoptics

1. Nystagmus associated with diseases of visual 2. End-point nystagmus. It is a transient fine jerk
system horizontal nystagmus seen in normal persons
• Ocular jerk nystagmus on extreme right or left gaze.
• Vertical nystagmus 3. Physiological vestibular nystagmus. It is a jerk
• See-saw nystagmus nystagmus which can be elicited by stimulating
• Acquired pendular nystagmus the tympanic membrane with hot or cold water.
It forms the basis of caloric test. If cold water is
2. Vestibular nystagmus
poured into right ear, the patient develops left
i. Peripheral vestibular nystagmus, e.g. jerk nystagmus (rapid phase towards left) while
Menière and drug toxicity nystagmus the reverse happens with warm water, i.e. patient
ii. Central vestibular nystagmus develops right jerk nystagmus. It can be
• Downbeat remembered by the mnemonic 'COWS’ (Cold-
• Upbeat Opposite, Warm-Same).
• Torsional Physiological vestibular nystagmus can also be
• Horizontal elicited with head rotation (which is utilized for
vision assessment in young infants). Dampening
iii. Periodic alternating nystagmus
of physiological nystagmus should occur in 5–
3. Nystagmus due to disorders of gaze holding 10 seconds after stopping the rotation. If it does
• Gaze-evoked nystagmus not, it means that there exists significant visual
• Dissociated nystagmus (ataxic nystagmus) impairment.
• Bruns’ nystagmus
• Convergence-retraction nystagmus PATHOLOGICAL NYSTAGMUS
• Centripetal and rebound nystagmus A. EARLY ONSET (CHILDHOOD) NYSTAGMUS
Note. Only a very brief account of the various The three most common forms of nystagmus
types of nystagmus is given here just as a seen in childhood begin in infancy and are,
passing reference. For detailed accounts, the therefore, not congenital. These include:
readers should consult certain standard 1. Infantile nystagmus syndrome
textbooks on neuro-ophthalmology. 2. Fusion maldevelopment nystagmus syndrome
3. Spasmus nutans syndrome
PHYSIOLOGICAL NYSTAGMUS 1. Infantile nystagmus syndrome
1. Optokinetic nystagmus. It is a physiological Infantile nystagmus syndrome (INS) is the new
jerk nystagmus induced by presenting to gaze name given in the CEMAS system for the old
the objects moving serially in one direction; such ‘congenital nystagmus, motor and sensory
as strips of a spinning optokinetic drum. The nystagmus.’
eyes will follow a fixed strip momentarily and
Etiology. It may be: (I) idiopathic or (II) associated
then jerk back to reposition centrally to fix up a
with sensory deprivation due to any of the
new strip. Similar condition occurs while
following causes:
looking at outside things from a moving train.
• Retinal diseases, such as retinoblastism,
OKN is used for: retinopathy of prematurity (ROP), persistant
• Assessment of vision in young infants and hyperplastic primary vitreous (PHPV)
uncooperative adults. • Ocular albinism, characterized by iris transillu-
• Reversal of OKN is seen in infantile esotropia mination defects and foveal hypoplasia.
syndrome and congenital nystagmus (now • Aniridia, i.e. bilateral near total congenital iris
called infantile nystagmus syndrome). absence.
• Asymmetry/absent vertical OKN is diagnostic • Leber's congenital amaurosis, characterized by
of neurological/neurometabolic lesions and markedly abnormal or flat electroretinogram
warrants neuroimaging in children with bilateral congenital toxoplasmosis and bilateral
nystagmus. macular hypoplasia.
 Nystagmus and Related Oscillations 381

• Other causes include, bilateral congenital • With or without normal visual acuity due to
cataract, achromatopsia, congenital stationary associated sensory system deficits (e.g.
night blindness, bilateral optic nerve hypo- albinism, achromatopsia) associated
plasia. strabismus or refractive error
Characteristic features. It is characterized by • Null and neutral zones present,
erratic waveform with or without roving eye • Associated head posture or head shaking may
movement associated with reduced visual exhibit a “latent” component, “reversal” with
acuity due to above mentioned conditions. OKN stimulus or (a) periodicity to the
oscillation.
CEMAS criteria for INS is summarized below:
• May decrease with induced convergence,
• Infantile onset increased fusion, extraocular muscle surgery,
• Ocular motor recordings show diagnostic contact lenses, and sedation.
(accelerating) slow phases
• Wave forms may change in early infancy, 2. Fusion maldevelopment nystagmus syndrome
• Head posture usually evident by 4 years of Fusion maldevelopment nystagmus syndrome
age. (FMNS) is the new name for the old term—
• Vision prognosis dependent on integrity of latent/latent manifest nystagmus as described in
sensory system. CEMAS.
Symbolic recording of an idiopathic case of INS is Characteristic features CEMAS criteria for
shown in Fig. 14.4. FMNS are summarized below:
Common associated findings • Infantile onset
• Conjugate, horizontal-torsional, increases • High frequency, low-amplitude pendular
with fixation attempt nystagmus (dual-jerk waveform), jerk in
• Progression from pendular to jerk direction of fixing eye
• Family history often positive • Intensity decreases with age.
• Ocular motor recordings show two types of
slow phases linear and decelerating.
• Nystagmus is not present, when both eyes are
open. It appears when one eye is covered. It is
a jerk nystagmus with rapid phase towards
the uncovered eye.
• While testing visual acuity in such patients,
one eye should be fogged (by adding plus
lenses in front) rather than occluding to
minimize induction of latent nystagmus.
• May be associated with congenital esotropia
and dissociated vertical deviation (DVD).
• Becomes manifest under monocular viewing
conditions, i.e. in the presence of decreased
vision in one eye as in anisometropic
amblyopia, strabismic amblyopia, etc.
Symbolic recording of a case of FMNS is shown
in Fig. 14.5.
Fig. 14.4 Symbolic recording of a case of idiopathic infantile Common associated findings
nystagmus syndrome (INS) depecting left beating horizontal
Conjugate, horizontal, uniplanar; usually no
jerk nystagmus of medium amplitude and moderate
frequency in primary position. Intensity of nystagmus associated sensory system deficits (e.g. albinism,
increases in left gaze and decreases in right gaze. There is achromatopsia), may change with exaggerated
null zone in right gaze with pendular waveform. convergence (“blockage”), head posture
 382 Theory and Practice of Squint and Orthoptics

Common associated findings are: Dysconjugate,

asymmetric, multiplanar, family history of
strabismus, may be greater in one (abducting)
eye, constant, head posture/oscillation
(horizontal or vertical), usually no associated
sensory system deficits may have associated
strabismus and amblyopia, may increase with
convergence, head bobbing, head posture may
be compensatory; normal fundus exam;
decreases with increased fusion (binocular
function).

B. ACQUIRED NYSTAGMUS
I. Nystagmus associated with diseases of the
visual system
Pathogenesis
As mentioned in pathogenesis, fixation
Fig. 14.5 Symbolic recording of a case of fusion disorders can lead to nystagmus. The smooth
maldevelopment nystagmus syndrome (FMNS) depicting visual fixation mechanism stops the eyes from
right beating horizontal jerk nystagmus of medium amplitude drifting away from a stationary object of regard.
and moderate frequency. Intensity of the nystagmus
This fixation mechanism depends upon the
increases in the right gaze and decreases in left gaze.
motion detection (magnocellular) portion of the
associated with fixing eye in adduction, no head visual system which is inherently slow, with a
shaking, may exhibit “reversal” with OKN response time of about 100 milliseconds that
stimulus, no (a) periodicity to the oscillation. encumbers all visually mediated eye move-
Dissociated strabismus may be present. ments, including fixation, smooth pursuit, and
Decreases with increased fusion (binocular optokinetic responses.4 If the response time is
function). delayed further by disease of the visual system,
then the attempts by the brain to correct eye
3. Spasmus nutans syndrome drifts leads to ocular oscillations.
Spasmus nutans syndrome (SNS), old name Vision is also needed for recalibrating and
spasmus nutans (SN) is the 3rd most common optimizing all types of eye movements. These
nystagmus seen in infancy. functions depend on visual projections to the
cerebellum. Thus, signals go from secondary
Characteristic features CEMAS criteria for SNS
visual areas concerned with motion-vision
are as below:
project via the pontine nuclei and middle
• Infantile onset cerebellar peduncle to the cerebellum. For
• Variable conjugacy, small-frequency, low- calibration of the ocular motor system, visual
amplitude oscillation signals are compared with eye movement
• Abnormal head posture and head oscillation, commands. At present, it is not certain how or
improves (disappears) during childhood where this function is performed. A group of
• Normal MRI/CT scan of visual pathways cells in the paramedian tracts (PMT) in the
• Ocular motility recordings—high-frequency lower pons have been suggested as a probable
(>10 Hz), asymmetric, variable conjugacy, centre.
pendular oscillations Diseases affecting any part of the visual
• Usually spontaneously remits clinically in 2 to system, from retina to cortical visual areas, or
8 years, remains present with eye movement interrupting visual projections to pons and
recordings cerebellum, may be associated with nystagmus.
 Nystagmus and Related Oscillations 383

Clinical types of nystagmus with lesions affect the chiasm are associated with see-saw
affecting the visual pathways nystagmus. See-saw nystagmus has two types—
Ocular jerk nystagmus pendular and jerk. Pendular see-saw is seen in
It is seen in disease of the retina. Congenital or lesions of optic chiasm. It is because of the fact
acquired retinal disorders causing blindness, that, crossed visual inputs are important for
such as Leber’s congenital amaurosis, lead to optimizing vertical-torsional eye movements
continuous jerk nystagmus with components in and if interrupted, lead to see-saw oscillations.
all three planes, which changes direction over
Acquired pendular nystagmus
the course of seconds or minutes. This is due to
inability to calibrate the ocular motor system. Acquired pendular nystagmus is one of the most
This nystagmus often shows the increasing- common forms of nystagmus associated with
velocity waveform earlier thought to be specific disease affecting the visual system or its
for congenital nystagmus. brainstem-cerebellar projections and is asso-
ciated with distressing visual symptoms. Its
Vertical nystagmus pathogenesis is not entirely clear with more than
It is seen in vertical nystagmus disease affecting one mechanism responsible.
the optic nerves. Optic nerve disease is associated
Features Acquired pendular nystagmus has
with vertical pendular nystagmus. With unilateral
horizontal, vertical, and torsional components
disease of the optic nerve, nystagmus affects the
with the same frequency, although one
abnormal eye. The nystagmus has vertical, low-
component may predominate. (In congenital
frequency, bidirectional drifts (pendular),
pendular nystagmus, the oscillation usually is
unidirectional horizontal drifts with corrective
predominantly horizontal.) Depending on the
quick-phases (jerk) are less common. When disease
phase difference between various directions, a
affects both optic nerves, the amplitude of
pendular nystagmus may be oblique(If the
nystagmus is greater in the eye with poorer vision.
horizontal and vertical oscillatory components
Monocular nystagmus of childhood are in phase), elliptical. (If the horizontal and
vertical oscillatory components are out of phase)
Causes Benign to sight threatening causes of
or circular (phase difference of 90° and equal
poor vision, i.e. from amblyopia to optic
amplitude of the horizontal and vertical
neuropathy
components). Further, the nystagmus may be
Characteristic features of monocular nystagmus conjugate, or may appear convergent-divergent.
of childhood, a rare but important form of The frequency of oscillations ranges from
nystagmus, are: 1–8 Hz, with an average value of 3.5 Hz and remains
• Onset, almost always in early childhood constant for a given patient. Acquired pendular
• Nystagmus movements occur in the same eye nystagmus may be suppressed or brought out by
at all times and may be vertical or elliptical eyelid closure or evoked by convergence.
(of small amplitude)
Symbolic recording of a case of acquired
• Heimann-Bielschowsky phenomenon, i.e., pendular nystagmus is shown in Fig. 14.6.
monocular vertical nystagmus in an eye with
long-standing poor vision, may be seen. Conditions associated with acquired pendular
nystagmus are:
• Neuroimaging showed always be performed in
an infant presenting with monocular vertical • Visual loss (including unilateral disease of the
nystagmus associated with afferent pupillary optic nerve)
defect and optic atrophy; as it is suggestive of • Disorders of central myelin, such as:
an optic nerve or chaismal tumour (glioma). – Multiple sclerosis
– Pelizaeus-Merzbacher disease
Pendular see-saw nystagmus – Peroxisomal assembly disorders
It is seen in disease affecting the optic chiasm. – Cockayne’s syndrome
Parasellar lesions such as pituitary tumours that – Toluene abuse
 384 Theory and Practice of Squint and Orthoptics

is prevented. This is because in presence of

normal visual functions, the calibration
mechanisms discussed above prevent the
drifting of eyes.
Vestibular nystagmus is categorized as follows:
• First degree—if it is present only on looking in
the direction of the quick phases.
• Second degree—if it is also present in the central
position.
• Third degree—if it is present on looking in all
directions of gaze.
Pure vertical or pure torsional nystagmus.
almost never occur with peripheral vestibular
disease
Diagnosis of specific types of peripheral
vestibular nystagmus can be aided by observing
the effect of head position on nystagmus as
Fig. 14.6 Symbolic recording of a case of horizontal acquired below:
pendular nystagmus of a low amplitude and moderate • Complete unilateral labyrinthine destruction
frequency.
leads to a mixed horizontal-torsional nystagmus
• In benign paroxysmal positional vertigo
• Oculopalatal myoclonus
(BPPV), a mixed upbeat-torsional nystagmus
• Acute brainstem stroke reflecting posterior semicircular canal
• Whipple’s disease stimulation is seen.
• Spinocerebellar degenerations • Patients with dehiscence of the superior semi-
• Congenital nystagmus circular canal develop vestibular symptoms
and nystagmus, when exposed to certain
II. Vestibular nystagmus sounds (Tullio’s phenomenon).
Nystagmus related to imbalance in the Peripheral vestibular nystagmus induced by
vestibular pathway can be caused by damage caloric or galvanic stimulation
to peripheral or central structures. During caloric stimulation, a temperature
gradient across the temporal bone induces a
Peripheral vestibular nystagmus convection current in the endolymph of a
Disease affecting the peripheral vestibular semicircular canal, if it is orientated vertical to
pathway (i.e. the labyrinth, vestibular nerve, and the earth:
its root entry zone) causes nystagmus with linear • The subject is placed supine and the neck is
slow phases, which reflect an imbalance in the flexed at 30°.
level of tonic neural activity in the vestibular • A cold stimulus (30ºC) induces horizontal
nuclei. The slow phase of the nystagmus is slow phase components directed towards the
towards the side of the lesion. Gaze in the stimulated ear (quick phases in the opposite
direction of the fast component increases the direction).
nystagmus intensity, and gaze in the direction • A warm stimulus (44°C) and the same head
of the slow component decreases the intensity orientation, quick phases are towards the
(Alexander’s law). An imbalance of vestibular stimulated ear (mnemonic, COWS: cold-
tone also causes vertigo and a tendency to fall opposite, warm-same).
towards the side of the lesion. The nystagmus
is suppressed by visual fixation and sometimes Symbolic recording of a case of peripheral
may only become apparent, when visual fixation vestibular nystagmus is depicted in Fig. 14.7.
 Nystagmus and Related Oscillations 385

• Lithium intoxication
• Alcohol
• Wernicke’s encephalopathy
• Magnesium depletion
• Vitamin B12 deficiency
• Toluene abuse
• Congenital
• Transient finding in otherwise normal infants

Clinical features
• Present with the eyes in central position.
• Has a small amplitude (viewing the fundus
with an ophthalmoscope may be necessary).
It may occur intermittently.
• Generally, Alexander’s law is obeyed:
Nystagmus intensity is greatest in downgaze
and least in upgaze.
Fig. 14.7 Symbolic recording of a case of peripheral • The waveform is linear, but it may be
vertibular nystagmus depicting left beating mixed horizontal increasing in velocity.
jerk and rotary waveform caused by a right sided lesion.
Intensity of the nystagmus increases to the left (i.e. away
• Downbeat nystagmus may be evoked by
from the site of lesion) and decreases to the right (i.e. placing the patient in a head-hanging position.
towards the site of lesion). • Convergence may influence the amplitude
and frequency of the nystagmus or convert it
Central vestibular nystagmus to upbeat nystagmus.
Imbalance of central vestibular connections • Other ocular motor abnormalities accompany
commonly leads to downbeat, upbeat, and downbeat nystagmus and reflect coincident
torsional nystagmus. cerebellar involvement.
• Vertical smooth pursuit and the vertical VOR
Downbeat nystagmus are abnormal because of impaired ability to
Causes generate smooth downward eye movements.
• Downbeat nystagmus is usually associated • Impairment of eccentric horizontal gaze-
with lesions that effect the excitatory pro- holding, smooth pursuit, and combined eye-
jections from posterior semicircular canals, head tracking.
sites include vestibulocerebellum, flocculus, • The visual consequences of downbeat
paraflocculus, nodulus and uvula and the nystagmus are oscillopsia and postural
underlying medulla. instability.
• Cerebellar degeneration, including familial Symbolic recording of a case of downbeat
episodic ataxia, and paraneoplastic degeneration. nystagmus is shown in Fig. 14.8.
• Craniocervical anomalies, including Arnold-
Chiari malformation Upbeat nystagmus
• Infarction of brainstem or cerebellum Causes
• Dolichoectasia of the vertebrobasilar artery • Upbeat nystagmus is most commonly seen in
• Multiple sclerosis patients with lesions affecting excitatory
connections from the anterior semicircular
• Cerebellar tumour
canals. These include:
• Syringobulbia – The perihypoglossal nuclei and adjacent
• Head trauma medial vestibular nucleus (structures
• Anticonvulsant medication important for gaze-holding).
 386 Theory and Practice of Squint and Orthoptics

Clinical features
• It is present with the eyes close to central
position.
• Nystagmus intensity is usually greatest in
upgaze.
• It does not increase on right or left gaze.
• Removal of visual fixation has little influence
on slow-phase velocity.
• Convergence can enhance, suppress, or
convert upbeat nystagmus to downbeat.
• Placing the patient in a head-hanging position
increases the nystagmus in some individuals.
• There are asymmetries of vertical vestibular
and smooth pursuit eye movements, as well as
associated cerebellar eye movement findings.

Torsional nystagmus
Fig. 14.8 Symbolic recording of a case of downbeat Causes
nystagmus in primary position with a superimposed
horizontal gaze-evoked nystagmus. Intensity of downbeat
• Syringobulbia
nystagmus increases in downgaze and laterally. Oblique • Brainstem stroke (Wallenberg’s syndrome) or
trajectory shown in lateral gaze is due to superimposed arteriovenous malformation
horizontal gaze-evolved nystagmus. • Brainstem tumour
• Multiple sclerosis
– Ventral tegmentum (containing projections
from the vestibular nuclei that receive inputs • Oculopalatal myoclonus
from the anterior semicircular canals). • Head trauma
– Caudal medulla • Congenital
– Anterior vermis of the cerebellum • Associated with the ocular tilt reaction
– Brachium conjunctivum and midbrain Clinical features
• Cerebellar degenerations, including familial • Least common form of central vestibular
episodic ataxia nystagmus.
• Leber’s congenital amaurosis or other • It is difficult to detect and requires careful
congenital disorder of the anterior visual observation of conjunctival vessels or noting
pathways the direction of retinal movement on either
• Infarction of medulla, midbrain, or cerebellum side of the fovea.
• Tumours of the medulla, midbrain, or • Modulation by head rotations is similar to
cerebellum upbeat and downbeat nystagmus.
• Wernicke’s encephalopathy • There are variable slow-phase waveforms
• Brainstem encephalitis • Convergence suppresses the nystagmus.
• Behcet’s syndrome
• Meningitis Periodic alternating nystagmus
• Multiple sclerosis Causes
• Thalamic arteriovenous malformation Periodic alternating nystagmus (PAN) does not
• Organophosphate poisoning fall in a single pathological category. It occurs
• Tobacco in lesions affecting the cerebellum most notably
• Associated with middle ear disease nodulus and uvula. These areas determine the
• Congenital eye velocity of nystagmus seen after rotation
• Transient finding in otherwise normal infants (both VOR and OKN). Their destruction leads
 Nystagmus and Related Oscillations 387

to un-inhibited, prolonged vestibular impulses • With a right sided lesion, the reaction consists
which leads to nystagmus. of a left head tilt, a skew deviation with a right
hypertropia, tonic intorsion of the right eye
Clinical features and extorsion of the left eye, and misper-
• Spontaneous horizontal nystagmus, present in ception that earth-vertical is tilted to the left.
central gaze.
• Reverses direction approximately every 90– III. Nystagmus due to abnormalities of the
120 seconds. The changing direction of the mechanism for holding eccentric gaze
waveforms is caused by an actively shifting Gaze-evoked nystagmus
null zone.
Nystagmus that is induced by turning the eye
• Acquired PAN has the same characteristics in to an eccentric position in the orbit is called gaze-
light or in darkness. evoked nystagmus. It is the most common form
• Smooth pursuit and optokinetic nystagmus of nystagmus encountered in clinical practice.
are usually impaired. Gaze-evoked nystagmus is a general term that
includes both physiologic and pathologic
Jerk see-saw and hemi see-saw nystagmus nystagmus. When the nystagmus is physiologic,
Causes the term end-point nystagmus should be used.
Jerk see-saw nystagmus (hemi see-saw When the nystagmus is associated with a paresis
nystagmus) occurs in patients with lesions in the of gaze, e.g. ocular motor nerve palsies or
region of the interstitial nucleus of Cajal (INC). weakness of the extraocular muscles, the term
The associated ocular tilt reaction is due to an gaze-paretic nystagmus is appropriate.
imbalance of central otolithic projections from • Gaze-evoked nystagmus usually occurs on
vestibular nuclei to the INC. lateral (Fig. 14.10) or upward gaze, seldom on
looking down.
Clinical features • Waveform is dependent on effect of fixation.
• One-half-cycle consists of elevation and • If fixation is impaired, the slow phases consist
intorsion of one eye and synchronous of exponentially decaying waveform.
depression and extorsion of the other eye. In
the next half-cycle, the vertical and torsional
movements reverse (Fig. 14.9).
• The waveform may be pendular or jerk. Some
authorities label the pendular form as see saw
and jerk form as hemi see-saw.
• See-saw nystagmus may be congenital, or
Acquired.
• Patients often have a contralateral ocular tilt
reaction.

Fig. 14.10 Symbolic recording of a case of bilateral gaze-

evoked nystagmus. Note horizontal jerk nystagmus of small
Fig. 14.9 Symbolic recording of a case of see-saw nystag- amplitude and low-frequency with fast phase in the direction
mus (for explaination see text). of gaze.
 388 Theory and Practice of Squint and Orthoptics

• If visual fixation is possible, however, the slow nystagmus includes a series of saccades
phase has a linear profile. followed by postsaccadic drift that occurs, when
To hold gaze at an eccentric position, the elastic the patient attempts to look laterally away from
force of the fascia and ligaments has to be the side of the lesion. Since the saccades initiate
overcome. This is achieved by a tonic contraction the oscillations, this is not a true nystagmus. It
of the extraocular muscles. The neural signal represents an attempt by the brain to adaptively
involved in this contraction has been termed as correct hypometric saccades due to the weak
‘step’, that is generated by the gaze-holding medial rectus muscle, which because of Hering’s
network, also called the neural integrator. This law of equal innervation leads increase in the
network includes the vestibulo-cerebellum, the innervation to the normal, abducting eye,
medial vestibular nucleus and adjacent nucleus thereby resulting in overshooting saccades and
prepositus hypoglossy in the medulla, and the postsaccadic drift of the normal eye if the patient
interstitial nucleus of Cajal (INC) in the midbrain. attempts to fixate with the diseased eye. Thus,
Etiology. Gaze-evoked nystagmus is caused by
whenever a patient habitually prefers to fixate
a deficient step, so that the eyes cannot be with a paretic eye, the normal eye, will show a
maintained at an eccentric orbital position and dissociated nystagmus while looking in the
are pulled back toward central position by the direction of action of the paretic muscle,
elastic forces of the orbital fascia. Corrective regardless of the pathogenesis of the weakness.
quick phases then move the eyes back towards Other causes of dissociated nystagmus are:
the desired position in the orbit. • Previous extraocular muscle surgery
Additionally, lesions that produce gaze- • Myasthenia gravis
evoked nystagmus also impair visual fixation • Miller Fisher syndrome
and smooth pursuit. Bruns’ nystagmus
Causes of gaze-evoked nystagmus include: Tumours in the cerebellopontine angle, produce
• Medications, including alcohol, anticonvulsants a low-frequency, large-amplitude nystagmus,
and sedatives. when the patient looks toward the side of the
• Structural lesions that damage the gaze- lesion, and a high-frequency, small-amplitude
holding neural network. nystagmus, when the patient looks toward the
• Nucleus prepositus hypoglossi/medial side opposite the lesion. The nystagmus that
vestibular nucleus region occurs on gaze towards the side of the lesion is
• Interstitial nucleus of Cajal gaze-evoked nystagmus caused by defective
• Rarely, cerebellar lesions. gaze holding, whereas the nystagmus that
• Familial episodic ataxia type 2 (EA-2), which occurs during gaze towards the side opposite
also has attack of ataxia and vertigo. the lesion is caused by vestibular imbalance.
This is called Bruns’ nystagmus (Fig. 14.11).
End-point nystagmus
It is a gaze-evoked nystagmus encountered in Convergence-retraction nystagmus
normal subjects. It typically occurs on looking It is characterized by quick phases that converge
far laterally and is poorly sustained. The or retract the eyes on attempts to look up.
nystagmus is primarily horizontal. It is usually Affected patients usually have impaired or
symmetric. Differentiating features from a absent upward gaze for both pursuit and
pathological nystagmus are that this nystagmus saccadic eye movements. It is a saccadic disorder
has lower intensity (i.e. slower drift) and is not rather than nystagmus because the primary
accompanied by other ocular motor abnormalities. adductive movements are asynchronous
saccades. Causes include:
Dissociated nystagmus (ataxic nystagmus) • Lesions of the mesencephalon that damage the
It is a special type of pathologic gaze-evoked posterior commissure, e.g. pineal tumours.
nystagmus, most commonly associated with an • Chiari malformation
internuclear ophthalmoplegia (INO). Dissociated • Epileptic seizures
 Nystagmus and Related Oscillations 389

History
History should include:
• Duration of nystagmus
• Whether it interferes with vision and causes
oscillopsia
• Accompanying neurological symptoms
• Whether nystagmus and other visual
symptoms are worse with viewing far or near
objects, or with patient motion, or with
different gaze angles.
• If abnormal head posture is present, whether
or not these features are evident on old
photographs.

Examination of a patient with nystagmus

Comprehensive examination of the visual
system
Fig. 14.11 Symbolic recording of a case of Bruns’ nystagmus • Visual acuity assessment with and without
depicting a high frequency, small amplitude, right-beating
posture, for both near and distance and both
horizontal and rotary waveform.
binocularly and uniocularly. Binocular acuity
should be recorded before occlusion. Methods
Centripetal and rebound nystagmus of measuring monocular vision while
If a patient with gaze-evoked nystagmus avoiding total occlusion include fogging the
attempts to look eccentrically for a sustained other eye with plus spheres, polarizing lenses,
period, the nystagmus begins to decrease in central field occlusion and the red-green
amplitude and may even reverse direction, this duochrome slide test.
is called centripetal nystagmus. If the eyes are • Anterior and posture segment examination to rule
then returned to the central position, a short- out cause of low vision. Especially look for
lived nystagmus with slow drifts in the direction • Measurement of head posture. In most patients
of the prior eccentric gaze occurs. This is called with infantile nystagmus, the head position
rebound nystagmus. Both centripetal and corresponds roughly to the null zone.
rebound nystagmus reflect an attempt by However, an anomalous head posture may be
brainstem or cerebellar mechanisms to correct present in patients with INS for reasons other
for the drift of gaze-evoked nystagmus. than nystagmus, e.g. uncorrected astigmatism,
Rebound nystagmus occurs in patients with: incomitant squint, muscular torticollis.
• Cerebellar disease Nevertheless, presence of AHP in a patient
• In normal subjects with typical gaze-evoked with INS has better visual prognosis than no
nystagmus AHP. All the components of head posture, i.e.
• Lateral medullary infarction face turn, chin elevation or depression and
• Tumour confined to the flocculus. head tilt should be noted. Face turn can be
measured using the Goniometer (Fig. 14.12)
CLINICAL EVALUATION AND ELECTRO- or by simply using a scale and a protactor.
PHYSIOLOGICAL RECORDING AND Systematic examination of each functional class
NEUROIMAGING of eye movements (vestibular, optokinetic,
A. CLINICAL EVALUATION smooth-pursuit, saccades, vergence) and their
It is often possible to diagnose the cause of effect on nystagmus.
nystagmus through careful history and Examination of nystagmus in a systematic
systematic examination of the patient. manner. It is essential to have a mental checklist
 390 Theory and Practice of Squint and Orthoptics

during clinical examination. ABCDEF is a which exists between the cornea (+ve) and back
suggested pneumonic for systematic examination of the retina (–ve).
of nystagmus where: Technique of recording is shown in Fig. 14.13A and
A is amplitude B. Electrodes are placed over the orbital margin
B is basic shape or waveform near the medial and lateral canthi serve as active
C is conjugacy electrodes (E1–E4 in Fig. 14.13A). A forehead
electrode serves as a ground electrode or indif-
D is direction
ferent electrodes (E5 in Fig. 14.13A).
E is effect of gaze position and fixation, e.g.
• The stability of fixation (with the eyes close to Salient features.
primary position) viewing near and far • Horizontal range of measurement of 1 to 40°
targets, and at eccentric gaze angles. with a resolution of 1°.
• In patients with head turn or tilt, the eye • Useful for horizontal and some vertical
should be observed in various directions of movements.
gaze, when the head is in that position as well • A bitnoisy–1°.
as when the head is held straight. • Best clinical all rounder with good electrodes.
• During fixation, occlude each eye in turn to
check for latent nystagmus. 2. Electronystagmography
• The effect of removal of fixation (with Electronystagmography (ENG) is an adaption
Frenzelor high-plus spherical lenses). of electro-oculography (EOG). For ENG like
F is frequency. EOG (Fig. 14.13), a ground electrode is attached
to the forehead and three recording electrodes
Note. Details of the features of nystagmus and
are placed one each to the side, above and below
method of their clinical documentation has been
each eye which measure the eye movement.
described earlier (see pages 376–379).
Tests performed with ENG include:
B. ELECTROPHYSIOLOGICAL RECORDING OF • Oculomotor tests. ENG is used to record
EYE MOVEMENTS nystagmus during oculomotor tests such as
Electrophysiological recording of ocular motility saccades, pursuit and gaze testing and
has provided a new basis for eye movement optokinetics. Abnormal oculomotor test
abnormality classification, etiology and treat- results may indicate either systemic or central
ment. Only salient features of some of the pathology as opposed to peripheral (vesti-
bular) pathology.
techniques available for ocular motility
• Positional testing is performed to see the effect
recordings are mentioned here.
of head or body movements on the eye
1. Electro-oculography movements.
Electro-oculography (EOG) is based on the • Caloric test is performed to assess the
measurement of resting potential of the eye vestibular system.
3. Binocular infrared reflectance oculography
(BIRO)
• Useful for horizontal and some vertical
movements.
• Has a restricted range.
• Noise–0.1°

4. Electromagnetic scleral search

coil method
• Useful for horizontal, vertical and torsional
Fig. 14.12 Goniometer: An orthopaedic instrument which movements.
can be used to measure face turn. • Good resolution and frequency response.
 Nystagmus and Related Oscillations 391

Fig. 14.13 Technique of recording electro-oculogram: A, Position of electrodes; B, Ocular movements during recording;
C, Record of EOG.

• Requires mind bending mathematical analysis.

• Eye has to be anaesthetized and a thick silicone
lens is needed.
• Expensive.

5. Videonystagmography
Videonystagmography (VNG) is a sophisticated
technique in which nystagmus is recorded with
the help of the infrared video camera which is
incorporated in the specially designed infrared
camrea goggles (worm by the patient during the Fig. 14.14 Technique of videonystagmography with infrared
recording technique (Fig. 14.14). A very sensi- goggles worn by the patient.
tive head movement sensors are also incor-
ported in it. Advantages of VNG over ENG
• Less combursome and less time consuming as
Tests performed with VNG. Similar to ENG, electrodes are not required.
there are three parts of VNG testing: • Direct observation of video images of eye
• Ocular and optokinetic testing movements available.
• Positional nystagmus testing and • Simultaneous comparision of waveform can be
• Caloric testing performed.
 392 Theory and Practice of Squint and Orthoptics

• Computerised record allows storage, processing C. NEUROIMAGING

and analysis. Neuroimaging is indicated to find out associated
• Provides more information than ENG. In addition CNS abnormalities especially in patients with
to information about amplitude, frequency acquired nystagmus, periodic alternating
and intensity obtained from ENG; the VNG nystagmus, see saw nystagmus, spasmus nutans
also provides information about slow phase syndrome and infantile nystagmus syndrome
velocity and foveation window with the help with pallor disc and poor vision.
of intrinsic software.
Hertle’s criteria for neurological workupin
Clinical uses of eye movement recording patients with nystagmus are as follow:
Important clinical uses of eye movement I. History of:
recording are as follows: • Onset of nystagmus after 6–9 months of age.
• Identifies congenital type of nystagmus on the • History of prematurity or LBW or develop-
basis of waveform. About 40–60% cases of mental delay.
nystagmus have associated squint. About 35% • Abnormal pregnancy/delivery.
of these patients have FMNS. The best method • Exposure to toxins/drugs.
to differentiate INS form FMNS is eye II. Ocular features:
movement recordings. • Photophobia, delayed visual behaviour.
• Allows classification of acquired nystagmus • Structural abnormalities like foveal or optic
with greater certainty. nerve dysplasia.
• VNG helps to evaluate the evolution of nystagmus. • Nystagmus pattern vertical, asymmetric,
Many INS waveforms begin as pendular dysconjugate or associated with other ocular
nystagmus. Growth and development of the motor disorders (decrease pursuit, abnormal
visual sensory system evoke evolution of saccades, paretic gaze).
waveforms during early infancy from pendular III. General features:
to jerk type nystagmus by development of
• Patient having manifest hard, soft, focal or
corrective fast phases as well as breaking
diffuse neurologic signs.
saccades in slow phases producing the so called
‘mature’ waveforms associated with better • Localising signs of acquired nystagmus.
vision.
D. OTHER TESTS
• Measures slowness of saccades which can be
OCT is indicated in retinal dystrophies,
diagnostic, e.g. internuclear ophthalmoplegia.
degenerations, foveal hypoplasia, schisis cavity,
• Allows observation of motility in darkness retinal thinning, choroidal thinning.
(vestibular nystagmus).
Auto fluorescence can be used for diagnosing
• Allows temporal resolution of very fast eye accumulation of lipofuschin in macular
movements such as flutter, opsoclonus, dystrophies.
convergence nystagmus, dysmetria which are
difficult to evaluate with the naked eye. ERG is useful in sensory nystagmus associated
with conditions like achromatopsia, CSNB, LCA
• Trains one to interpret what is’ seen’.
and other atypical retinal dystrophies.
• Helps in objective assessment of visual functions
of a patient with nystagmus by calculating
TREATMENT OF NYSTAGMUS
foveation time, foveation eye position, and eye
velocity criteria. Aims of treatment
• Tells about the potential for visual improvement • To improve visual acuity by stabilizing the eyes
with treatment. • To shift the null zone, if any, in the primary
• Useful in objective documentation of response to position, i.e. to reduce abnormal head posture.
treatment. • To correct the associated strabismus
• Helps in null point evaluation. • To decrease any oscillopsia wherever possible.
 Nystagmus and Related Oscillations 393

Treatment modalities 5. Prismotherapy in nystagmus may be useful

Treatment modalities for nystagmus include: as follows:
• Optical, i. Base-out prisms may stimulate fusional
• Medical, and convergence (especially in patients with
• Surgical. congenital motor nystagmus) and thus
improve the visual acuity by dampening the
I. OPTICAL TREATMENT nystagmus.
1. Correction of refractive error may sometimes This results in improvement of visual acuity
significantly decrease nystagmus, especially in both for distance and near. The prism power
patients with bilateral aphakia. Although is usually split between the two eyes. Both
refraction is difficult in the presence of nystagmus ground in and membrane style prisms may
but every effort should be made to correct any be used. This option is best reserved for
significant refractive error. Retinoscopy may be patients with good vision, normal fusion and
performed in null zone, whenever it is present. modest nystagmus.
Further, contact lenses are more useful than the ii. Prisms with base opposite to preferred direction
spectacles (especially in high myopes), since of gaze may be helpful in correcting the head
these move with the movement of eye and so posture. Prisms minimize a head turn by
the visual axes always coincide with their optical reorienting the visual axis towards primary
axes. In addition to optical advantage, at actile gaze. Often the preferred eye is held in
feedback from the contact lenses have also been adduction. Equal power prisms are placed
reported to decrease the nystagmus. before each eye and oriented in the same
Clear or tinted contact lenses can be prescribed. direction so that the apex is in the direction
• Accommodation may be deficient in patients with of preferred gaze, and the base in the
congenital nystagmus. Dynamic retinoscopy direction of head turn. For example, in a
should be performed before dilation. If patient with head turn to the left, the null
accommodation is deficient, a bifocal zone is in dextroversion and a prism base-in
correction should be provided. before the right eye and base-out before the
2. Treatment of amblyopia. Amblyopia if left eye will be helpful in correcting the
present, should be treated with standard abnormal headposture (Fig. 14.15).
treatment. Penalization with plus lenses or Similarly, the appropriate prisms can also be
atropinization is preferred over occlusion by used to correct the vertical and oblique head
some workers. turns. Because of optical disadvantages, the
long-term use of prisms incorrecting the head
3. Stimulating accommodative convergence by
posture is discouraged. However, on the basis
over correcting minus lenses may improve
of patient’s response to prismo therapy, the
visual acuity at distance fixation by dampening
results of surgery for head turn can be predicted.
the nystagmus.
6. Galilean arrangement of contact lenses and
Patients chosen to have this treatment must
glasses (optical device by Rushton and Cox)
show evidence of good accommodative facility.
can be used to stabilize the retinal images in
4. Partial field occlusion. An unusual method
of decreasing the intensity of congenital
nystagmus, based on partial field occlusion was
suggested by Sasso. “Porthole” glasses that had
a clear 10 degree central field but an occluded
periphery were prescribed. For some patients
having torticollis and nystagmus, occlusion of
some parts of peripheral field alleviated
anomalous head posture. However, the data
was inadequate for evaluating this concept Fig. 14.15 Use of prism to correct head posture (for explan-
thoroughly. ation see text).
 394 Theory and Practice of Squint and Orthoptics

patients with acquired nystagmus and 6. Propranolol is reported to be effective for

oscillopsia. This device reduces the magnitude opsoclonus.
of image movement by limiting image slip 7. Brinzolamide (1%) eye drops used thrice a day
along the retina. As a result the visual acuity is noted to improve foveation by 50%, with
may improve through an increase in the broadening of the null zone. Its effect may be
foveation time. There occurs a decrease in equivalent to systemic acetazolamide or eye
oscillopsia also because of the reduced image muscle surgery but intermediate between those
motion. This device consists of a high minus of soft contact lenses or convergence. 80%
contact lens combined with a high plus Patients on the brinzolamide eyedrops may
spectacle lens. Rigid contact lenses of –58D or experience improvement in the best corrected
–28D are paired with spectacle lenses of +32D vision corresponding to one line on the Snellen
or +20D irrespectively. Stabilization of retinal chart. Visible reduction in nystagmus takes place
image up to 90% is possible using this system. in 27% and reduced AHP in 22% patients and
The prismatic effect of the large plus lens plays nearly 30% would experience no change.
key role in stabilization of retinal image. This The effect comes within one week and lasts as
lens images all objects at centre of rotation of long as the drops are continued. There may be
the eye, irrespective of exact direction of the additive effect of the drops when used after the
visual axis. Thus if eyeball rotates, light rays tenotomy and reattachment procedure. Topical
from the object will remain focussed at the brinzolamide may be contraindicated in
same central point within the eye. The minus congenital or acquired pathologies of corneal
contact lens refocuses the image on retina. As endothelium.
the contact lens moves with the eyes, there 8. Gabapentine (300 mg/qid) and memantine (10
should be no prismatic effect from the high mg/qid) are reported to be useful in patients
power plus lens. This has the added benefit of with upbeat nystagmus, acquired pendular
magnifying central 30° field. However the nystagmus and alternating periodic nystagmus.
peripheral field is not stabilised, leading to ring These drugs can also be tried in patients with
scotoma. congenital nystagmus.

II. MEDICAL TREATMENT III. SURGICAL TREATMENT

1. Cyclopentolate used as 1% eyedrops twice a INDICATIONS
day has been reported to reduce the amplitude,
• To eleminate abnormal head posture by
velocity and frequency of latent nystagmus in
shifting the null point to primary position.
about 60% of patients.
• To decrease the intensity of nystagmus in
2. Botulinum toxin injected into the retrobulbar patients having no abnormal head posture.
space (dose: 10–25 U in 0.1–1 ml) every 3–4 • Both of the above.
months has been reported to dampen nystagmus • To improve visual acuity (usually by 1–3 logmar
and improve visual acuity in patients with lines), contrast sensitivity and reaction time
acquired nystagmus and oscillopsia. (improves by 0.3 seconds) by improving
3. Baclofen has been observed to suppress the foveation.
acquired periodic alternating nystagmus. The • To correct strabismus and to restor binocular
initial recommended dose is 5 mg TDS, which fusion and stereopsis, where ever possible.
in case of no response can be increased stepwise
Important points to be considered while
every 3 days to a maximum of 80 mg/day.
planning surgery for nystagmus are as follows:
4. Clonazepam may be useful in patients with
1. Surgical intervention is useful in patients with
downbeat nystagmus and see-saw nystagmus.
congenital motor nystagmus and nystagmus
Clonazepam is used in dose of (0.5–1 mg/bd).
blockage syndrome.
5. Carbamazepine is useful in superior oblique 2. Surgery should be performed, only if the
myokymia. abnormal head posture causes a significant
 Nystagmus and Related Oscillations 395

cosmetic disturbance and/or visual difficulties, • Anderson procedure comprised of only

i.e. when head turn-or tilts more than 15°. recessions, e.g., for right face turn recession
3. Surgery should always be performed after the age of right MR and left LR muscle.
of 5–6 years; since spontaneous improvement • Modified Kestenbaum-Anderson procedures have
in abnormal head posture can occur in some been suggested by various workers thereafter.
cases up to this age. Moreover, by this age, it is Different modified surgical techniques are
possible to have more reliable examination now recommended for the face turn without
demonstrating head posture and ocular trabismus, for face turn with strabismus and for
alignment. For children with strabismus, earlier nystagmus blockage syndrome.
intervention may provide the best opportunity
for bifoveal fixation to develop. With an I. Surgical techniques for face turn
acquired nystagmus and torticollis, it is prudent 1. Surgical techniques for face turn without strabismus
to wait at least a year to be certain that the Modified Kestenbaum-Anderson procedure. It
abnormal eye movements are consistent and the consists of bilateral recess-resect operations.
head turn is stable. Up to 30° of face turn (e.g. in a patient with head
turn to the left) the modified Kestenbaum-
GENERAL PRINCIPLES OF SURGERY
Anderson procedure, as described by PARK, is
• Null point needs to be shifted in primary position. effective. It comprise lesser amount of recessions
For this, the eyes should always be moved in and also lesser amount of surgery on MR
the same direction as the abnormal head muscles as compared to LR muscles. He
posture. suggested the ‘5, 6, 7, 8’ guidelines for a bilateral
• Face turn will be corrected only if the surgery equal surgery as below (Fig. 14.16):
is done on the fixating eye. • Right lateral rectus (RLR) recession 7 mm
• In the presence of strabismus and an abnormal • Right medial rectus (RMR) resection 6 mm
head posture due to nystagmus, the head • Left medial rectus (LMR) recession 5 mm
position can be corrected only by operating • Left lateral rectus (LRL) resection 8 mm
on the fixing eye. Modified Kestenbaum-Anderson procedure, as
• Strabismus can be corrected by operating on described by PARK, is also called ‘classic
the fixating eye or nonfixating eye. maximum’ (Table 14.1)
• Surgery for face turn is performed first Up to 45° of face turn a 40% augmentation in the
followed by surgery for the strabismus. above surgery is recommended (Table 14.1).
• Surgical therapy should be based on the greatest Up to 60° of face turn a 60% augmentation in
amount of abnormal head position that is Park’s figures are suggested Table 14.1.
measured at distance.
2. Surgical techniques for face turn with
associated strabismus
(A) SURGICAL TECHNIQUES FOR ABNORMAL
HEAD POSTURE a. Face turn with esotropia
Patients with nystagmus who have null point i. Face turn ipsilateral to the fixing eye can be
(other than the primary position) assume an corrected by recess-resect procedure on the
abnormal head posture (AHP) to dampen the
nystagmus. A significant AHP needs to be
corrected.
Kestenbaum and Anderson were the first to
report on the surgical techniques for the
treatment of abnormal head posture in
congenital nystagmus.
• Kestenbaum procedure originally comprised of
Fig. 14.16 Diagrammatic depiction of position of eyes in a
recession-resection of all the four horizontal patient with left face turn and the suggested Kestenbaum
recti procedure to correct it.
 396 Theory and Practice of Squint and Orthoptics

Table 14.1 Modified Kestenbaum-Anderson procedures for nystagmus in a patient with left face turn
Type of surgery Dosage extraocular muscle surgery
Classic maximum 40% augmentation 60% augmentation
RMR resection 6 8.4 9.6
RLR recession 7 9.8 11.2
LMR recession 5 7 8
LLR resection 8 11.2 12.8

horizontal recti of the fixing adducted eye. For will also correct/reduce the associated
example, in a patient with left esotropia having exotropia. For example, in a patient with right
right face turn and fixating with right adducting exotropia having right face turn and fixating
eye, MR recession and LR resection of the right with left eye, surgery for the face turn should
eye should be performed. This procedure will be done on the left eye (MR resection and LR
correct face turn as well as an esotropia up to recession) to move it in the direction of right face
30PD. The residual esotropia, if any, can be turn. This procedure will correct right face turn
corrected later by surgery on the other eye. as well as the right exotropia. The residual
ii. Face turn ipsilateral to the deviating exotropia, if any, can be later corrected surgically
esotropic eye, should be first tackled with recess- by right MR resection and LR recession.
resect procedure on the opposite fixing eye. This
procedures will correct the face turn but will II. Surgical techniques in patients having head
increase the esotropia in the deviating eye, tip (chin elevation or depression) associated
which can be later corrected surgically. For with childhood nystagmus
example, in a patient with right face turn and Headtip occurs in patients having a null zone
right esotropia, surgery for the face turn should with eyes in depression or elevation. The basic
be first done on the left fixing eye (MR resection principle for surgically correcting the headtip is
and LR recession) to move it in the direction of like that for correcting face turns, i.e. the eyes
face turn. The resulting increased right esotropia should be moved in the direction of abnormal head
can be later corrected surgically by right MR posture. Symmetrical surgery on all the four recti
recession and LR resection. (vertical Kestenbaum) may be useful.
b. Face turn with exotropia Vertical Kestenbaum. Recommended procedures
i. Face turn ipsilateral to the fixing eye can be by Park are as follows:
corrected by recess-resect procedure on the
1. For headtip of more than 25°
horizontal recti of the fixing adducted eye. This
procedure will correct the face turn but will • For chin elevation. Bilateral 4 mm resection
increase the exotropia in the deviating nonfixing of the superior recti combined with bilateral
eye, which can be later corrected surgically. For 4 mm recession of the inferior recti.
example, in a patient with right face turn and • For chin depression. Bilateral 4 mm resection
left exotropia, surgery for the face turn should of the inferior recti combined with bilateral
be first done on the right fixing eye (MR 4 mm recession of the superior recti.
recession and LR resection) to move it in the
2. For headtip of less than 25°
direction of right face turn. The resulting
increased left exotropia can be later corrected The operation is limited to bilateral 4 mm
surgically by left MR resection and LR recession. recession of the depressors or elevators without
ii. Face turn ipsilateral to the deviating resection of their antagonists.
exotropic eye, should be first tackled with recess- However, recently most workers recommend
resect procedure on the fixing eye. This that the amount of surgery to be performed should
procedure, in addition to correcting face turn, be at least 5–6 mm to obtain satisfactory results.
 Nystagmus and Related Oscillations 397

III. Surgical techniques in patients having head- • Right medial rectus—transposed downwards.
tilt associated with childhood nystagmus • Right lateral rectus—transposed upwards.
Some patients with childhood nystagmus (CN)
have a combination of both horizontal and 4. Surgical slanting of the insertion of all four
rotatory components with latter being the major rectus muscles
component. Patients which have a significant Spielmann has recommended this surgery, for
headtilt have a torsional null position, and example, in a patient with head tilt towards right
shifting the null position by torsional the right eye is excycloducted by recession of the:
Kestenbaum is suggested in such cases. • Temporal part of the superior rectus
The basic principle to correct head tilt (not • Nasal part of the inferior rectus
related to paralysis of any cyclovertical muscle) • Inferior part of the lateral rectus, and
is to rotate the eyes in the direction of the
• Superior part of the medial rectus.
headtilt, which can be accomplished by any of
the following surgical techniques of torsional Note. A special care is required to preserve the
Kestenbaum: blood supply of the anterior segment of the
eyeball in this operation.
1. Surgery on two oblique muscles
In this technique, for example, in a patient with 5. Horizontal transpositioning of the vertical
head tilt towards right shoulder, the eyes can rectus muscles
be surgically rotated to the right by: von Noorden et al have recommended this sur-
• Weakening of right superior oblique muscle (either gery (as a preference over the other surgeries),
by a tenotomy or a recession of the anterior for example, in a patient with head tilt towards
fibres), and right as follows:
• Weakening of the left inferior oblique with a Right eye should be excyclotorted by transposing the
recession or some other weakening procedure. right superior rectus nasally and the right inferior
rectus temporally.
2. Surgery on four oblique muscles
Left eye should be incyclotorted by transposing the
On the basis of Kestenbaum’s principle, some left superior rectus temporally and left inferior
workers have proposed symmetrical surgery on rectus nasally.
all the four oblique muscles (torsional
They have recommended that transposition of
Kestenbaum). For instance, in a patient with head
each muscle should be by one full muscle width
tilt towards the right shoulder, the following
without changing their distance from the limbus.
surgery has been recommended:
Right eye is excycloducted by recessing the anterior
(B) SURGICAL TECHNIQUES FOR NYSTAGMUS
and retropositioning the posterior aspect of the BLOCKAGE (DAMPENING) SYNDROME
superior oblique tendon and advancing the
anterior and anteropositioning the posterior 1. Recession of the MR and resection of LR of
aspect of the inferior oblique tendon. the converging eye has been recommended by
Adelstein and Cuppers with good results.
Left eye is incycloducted by advancing the anterior
2. Bilateral medial rectus recession sometimes
portion and anteropositioning the posterior edge
combined with posterior fixation sutures has
of the superior oblique tendon and by recessing
been reported to be a more effective and thus a
the anterior portion and retro-placing the
better choice than the recess-resect procedure.
posterior position of the inferior oblique insertion.

3. Vertical transposition of the horizontal rectus (C) SURGICAL TECHNIQUES FOR DECREASING
muscles NYSTAGMUS INTENSITY
Decker has reported vertical transposition of the 1. Producing an artificial divergence (prismati-
horizontal rectus muscles, e.g. to cause cally and surgically) have been reported to be
excycloduction of the right eye as follows: of some use.
 398 Theory and Practice of Squint and Orthoptics

Surgical procedure consists of bilateral medial NYSTAGMUS-LIKE OCULAR MOTOR

rectus recession. OSCILLATIONS
Aim of the surgery is to induce a latent divergent
ocular position which the patient will then Nystagmus-like oscillations can be described as
overcome by exerting fusional convergence, below:
thereby damping the nystagmus. • Inappropriate saccades
• Voluntary saccadic oscillation ‘nystagmus’
For the success of procedure, the patients must
• Roving eye movements
have adequate fusional reserves to overcome the
induced deviation, so, preoperative testing with INAPPROPRIATE SACCADES
prisms is mandatory.
Involuntary inappropriate saccades include:
Indications include patient with INS and
convergence dampening with near vision > Saccadic intrusions
distance vision. These are basically transient breaks in fixation.
Benifits of the surgery include: These may occurs as:
• Dampening of nystagmus occurs by 70–80%. • Square wave jerks. These are small amplitude
conjugate saccades (<5°) which moves the eye
• Improvement in vision in magnificant.
away from fixation and back again (Fig. 14.17A).
2. Maximal recession of all the four horizontal • Square wave pulses also known as macro-
rectus muscles has been recommended for square wave jerk have a large ampitude (>5°)
decreasing the nystagmus intensity. For and a shorter intersaccadic latency (100–150 ms)
undisturbing the balance of forces, it has been (Fig. 14.17 B). They are usually and indication
recommended that a 10 mm of bilateral MR of neurological malfunction.
recession and 12 mm of bilateral LR recession is
the best procedure. However, before contempla- Saccadic oscillation
ting such a surgery, it should be made amply clear These movments oscillate across fixation and
to the patient that this operation will only decrease usually have amplitude large than 5°. Saccadic
its intensity and note liminate the nystagmus. oscillations are of following types:
Note. Large 4-muscle recessions or muscle • Macro-saccadic oscillations. They have a
extirpations are not preferred presently. similar intersaccadic latency to square wave
3. Tenotomy and reattachment. All the four jerk (Fig. 14.17C). They can reliably
horizontal recti are detached and resutured at distinguished from ocular flutter only by eye
the same insertion. movement recording.
Indications. Nystagmus with no null, primary • Ocular flutter occurs due to interruption of
position null or alternating null (periodic or cerebellar connection to brainstem. It is
aperiodic). characterized by horizontal oscillation and
Benefits of surgery include 25% reduced inability to fixate after change of gaze. Ocular
intensity of nystagmus, broadening of null zone, flutter represent back to back horizontal
improvement in vision by 1 to 3 logMar units in scaddes without any interscaddic latency
about 50% of patients and 40% increase in NAFX (Fig. 14.17D)
in 90% of patients. • Opsoclonus refers to combined horizontal,
4. Augmented Anderson procedure. It comprises: vertical and/or torsional oscillations asso-
• MR recession: 9 mm, and ciated with myoclonic movement of face, arms
and legs. It is seen in patient with encephalitis.
• LR recession: 12 to 13 mm.
Opsoclonus has a similar waveform to ocular
Indications. Patients with INS and face turn <25°. flutter but is multidirection and associated
Benefits of surgery include: with ossilopsia.
• Dampening of nystagmus by 90%. • Superior oblique myokymia is characterized
• Improvement in vision in magnification. by monocular, rapid, intermittent, torsional
 Nystagmus and Related Oscillations 399

ROVING EYE MOVEMENTS

Roving eye movements refers to conjugate large
amplitude low-frequency horizontal pendular-
like movements. The movements are apparent
soon after birth and are generally associated
with severe anterior visual pathway disease
such as Leber’s congenital amaurosis and severe
forms of bilateral optic nerve hypoplasia. With
time the amplitude becomes smaller and a
congenital nystagmus waveform may become
superimposed on the roving pattern or may
replace it.

BIBLIOGRAPHY
1. Boyle NJ, Dawson EL, Lee JP, Benefits of
Retroequatorial Four Horizontal Muscle
Recession Surgery in Congenital Idiopathic
Nystagmus in Adults, JAAPOS, 2006;10:404–8.
2. Dell’Osso LF, Tenotomy and congenital
Fig. 14.17 Inappropriate saccades: A, Square wave jerk; nystagmus: a failure to answer the wrong
B, Macro-square wave jerks; C, Macro-saccadic oscillations; question, Vision Res, 2004;44:3091–4.
and D, Ocular flutter. 3. Erbagci I, Gungor K, Bekir NA, Effectiveness of
retroequatorial recession surgery in congenital
vertical movements (which are best seen on nystagmus, Strabismus, 2004;12:35–40.
slit-lamp examination). 4. Flynn JT, Dell’Osso LF, The effects of congenital
• Ocular bobbing refers to rapid downward nystagmus surgery, Ophthalmology,
deviation of the eyes with slow updrift. It 1979;86:1414–27.
occurs due to pontine dysfunctions. 5. Hertle RW, Dell’Osso LF, Benefits of
retroequatorial four horizontal muscle recession
VOLUNTARY SACCADIC OSCILLATIONS ‘NYSTAGMUS’ surgery in congenital idiopathic nystagmus in
Voluntary saccadic oscillations ‘nystagmus’ adults, JPAAOS, 2007;11:313.
refers to the voluntary, poorly sustained 6. Schiavi C, Scorolli L, Campos EC, Surgical
management of anomalous head posture due to
conjugate oscillation of the eyes consisting
supranuclear gaze palsies and acquired
of rapidly alternating small-amplitude nystagmus. In: Spiritus M (ed.), Transactions of
saccades. the 23rd Meeting of the European Strabismological
Association, Nancy, 1996;229–32.
Characteristic features. The oscillations are 7. Sternberg-Raab A, Anderson–Kestenbaum
conjugate, usually horizontal and symmetrical, operation for asymmetrical gaze nystagmus, Br
and consist of back-to-back saccades. The J Ophthalmol, 1963;47: 339–45.
oscillations can be sustained only for a matter 8. Wang Z, Dell’Osso LF, Jacobs JB, et al., Effects
of seconds; convergence is usually associated of tenotomy on patients with infantile
with either the initiation or the maintenance of nystagmus syndrome, JAAPOS, 2006;10: 552–
the oscillation. The amplitude of the movement 60.
is small and the frequency high. There may be a 9. Wang ZI, Dell’Osso LF, Tomsak RL, Jacobs JB,
Combining recessions (nystagmus and
familial basis for the ability to initiate voluntary
strabismus) with tenotomy improved visual
‘nystagmus’ or it may be learned. Voluntary function and decreased oscillopsia and diplopia
‘nystagmus’ can be readily differentiated from in acquired downbeat nystagmus and in
acquired nystagmus and does not require horizontal infantile nystagmus syndrome,
further investigation. JAAPOS, 2007;11:135–41.
 15
Principles of Non-Surgical and
Surgical Management of Strabismus

NON-SURGICAL MANAGEMENT • Choice of operation and amount of

Optical treatment surgery
• Correction of refractive error • Anaesthesia for squint surgery
• Prismotherapy • Common operative steps in squint surgery
Pharmacologic treatment • Recession of rectus muscles
• Resection of rectus muscles
• Miotics • Adjustable sutures in rectus muscle surgery
• Atropine • Faden operation
• Botulinum toxin • Inferior oblique weakening procedures
• Other drugs • Inferior oblique strengthening procedures
Orthoptics • Superior oblique weakening procedures
• Goals of orthoptics • Superior oblique strengthening procedures
• Pre-requisites • Muscle transposition procedures
• Indications • Complications of extraocular muscle surgery
• Order of orthoptic treatment OUTLINES OF STRABISMUS MANAGEMENT
• Delivery of orthoptic treatment • Heterophoria
SURGICAL MANAGEMENT • Esotropia
• Exotropia
• Indications • Vertical and cyclodeviations
• Optimal time for surgery • Incomitant strabismus
• Types of surgical techniques

NON-SURGICAL MANAGEMENT • Optical treatment,

• Pharmacologic treatment,
Goals of Treatment of Strabismus • Orthoptics, and
Goals of treatment of strabismus are: • Surgical treatment.
1. To restore good visual acuity in each eye. Non-surgical treatment is essential in all most
all strabismus cases and in many cases may be
2. To achieve satisfactory cosmetic appearance.
sufficient to successfully treat the strabismus
3. To restore, if possible, normal single without surgery.
binocular vision.
A. OPTICAL TREATMENT
To achieve these goals, treatment of squint
varies for its different clinico-aetiological varieties I. CORRECTION OF REFRACTIVE ERROR
and has been described along with the each Spectacles for correction of refractive error
clinical entity. However, the principles, indi- should be prescribed in every case. These will
cations and the techniques of different treatment improve the visual acuity and provide sharp
modalities available for the squint have been retinal image that in young children is essential
described here in general. The commonly as a stimulus for the use of eyes and to achieve
practised treatment modalities include: fusion. Refractive correction also provides a
400
 Principles of Non-Surgical and Surgical Management of Strabismus 401

proper balance between accommodation and cutive exotropia. However, this should not be
convergence and thus at times may correct the at the cost of asthenopic symptoms.
squint partially or completely (as in accommo- 2. Use of over-minus glasses has been suggested
dative esotropia). Some important points by Jampolsky for controlling the intermittent
regarding refractive correction in patients with exotropia by stimulating accommodation and
strabismus are as follows: convergence in under 5 ear children.
General principles for prescribing glasses. 3. Inverse bifocal with a minus add for near has been
1. In general, full cycloplegic correction should be suggested for convergence insufficiency type of
prescribed (without making any tonus exotropia.
allowance for the cycloplegic used),
especially in young children from infancy to II. PRISMOTHERAPY
preschool age, neglecting the effect of glasses Prismotherapy for strabismus has become
on the patient's vision. popular after the introduction of Fresnel press-
2. In school going children, the refractive on prisms.
correction prescribed should be such that Advantages of Fresnel press-on membrane prisms
would provide an optimal distant vision. include: Light weight, cosmetically acceptable,
3. An overcorrection of +1.0DS to +3.0DS of easy to apply on the back of patient's glasses,
the non-amblyopic eye has been advocated availability in powers from 0.5D to 30D.
by some workers as penalization treatment.
Indications
Role of glasses in esotropia
The indications for prismotherapy in strabismus
1. Refractive accommodative esotropia. Full
are as follows:
cycloplegic correction should be made to correct
the esotropia. I. Role of prisms to assess the effect of surgery
2. Non-refractive accommodative esotropia with (Diagnostic Prism)
high AC/A ratio need to be treated with bifocal 1. Prism adaptation test (PAT). This test has
glasses giving full hyperopic correction in the been advocated by some workers to be
distance segment and an add of +1.00 D to performed before the surgical treatment is
+3.5 D in the near segment. The minimum add performed in patients with esotropia; while
required is tested in steps of 0.5D till the others do not agree on its utility. In PAT, base-
convergence excess for near is controlled. out prisms are given to the patient, so that
3. Esotropic patients having associated myopic esotropia is slightly overcorrected, i.e. to the
should be prescribed minimum myopic lenses point of slight exotropia. The results of the PAT
that give best corrected visual acuity. are interpreted as below:
4. Esotropic patients having associated myopia and
Favourable response or good fusional prognostic sign
high AC/A ratio also require bifocals.
is labelled, when a patient accepts the prism and
5. Residual esotropia of small amount (<15 PD) demonstrates fusion, when allowed to wear
should be prescribed the maximum hyper- prisms for several days before surgery. Such
metropic correction. If this is insufficient, patients are likely to respond well to slight
additional plus lenses may be tried to ascertain, surgical overcorrection of esotropia and develop
if binocular single vision can be achieved. spontaneous restoration of normal retinal
6. Consecutive esotropia of small amount (<15PD), correspondence.
persisting after 3 weeks of surgery for inter-
Unfavourable response or poor fusional prognostic
mittent exotropia can be treated by prescribing
sign is considered, if a patient overconverges in
full hyperopic correction. Bifocals can be
response to slight overcorrection with the prisms
prescribed, when esotropia is for near only.
in PAT and, thus returns with a marked
Role of glasses in exotropia esotropia in spite of its neutralization with
1. Undercorrection of hypermetropic error is prism. This overconvergence can occur within
recommended to reduce the degree of conse- minutes or hours or days or weeks after the
 402 Theory and Practice of Squint and Orthoptics

prisms are worn. A further increase in and thyroid ophthalmopathy, prisms may be
convergence is reported after addition of prisms employed in the early management till scenario
and, the patient is said to eat up the prism. for surgical treatment is clear.
The overconvergence in response to PAT has
been assumed to occur, most likely, in the III. Maintenance of binocular single vision by
presence of sensory abnormal retinal neutralizing the deviation
correspondence (ARC) and thus represents a 1. Vertical deviations. Prismotherapy is quite
peripheral motor fusional convergence. Such useful in patients with small (less than 12D)
patients are likely to respond in a way similar comitant vertical deviations. However, larger
to conventional surgical treatment as well and degrees of vertical deviations associated with
thus will have a recurrence of esotropia. greater degrees of incomitance almost always
However, in clinical practice, it has been require surgery.
observed that in many cases, ARC disappears 2. Horizontal deviations in which therapeutic
following surgical alignment without over- use of prisms for relief of diplopia may be quite
correction. Because of this controversy, PAT has successful are:
not become much popular and so most of the • Late onset deviations in visually mature patients
strabismologists no more practice prismotherapy such as those associated with muscle paresis
for sensorial anomalies. and divergence insufficiency.
2. To know the response of associated vertical • Primary intermittent exotropia. Some workers
deviation to surgical treatment for the hori- have recommended prismotherapy for the
zontal deviation. It has been reported that in treatment of primary intermittent exotropia,
some patients, associated vertical deviation also where surgical treatment is not yet warranted.
disappears following prismotherapy for the However, other workers have reported that
associated horizontal strabismus. It is assumed part time occlusion therapy is perhaps more
that in such cases surgical treatment for the effective and useful in such cases rather than
horizontal strabismus alone will be sufficient to the prismotherapy.
correct the associated vertical deviation as well. 3. Surgically overcorrected exotropia. It is
However, like PAT results of this test are also generally agreed that a slight overcorrection
based on the assumption that the response to (consecutive esotropia) for 1 to 3 weeks
surgical intervention will be similar to the postoperatively is desirable in patients with
response to prismotherapy. While in clinical intermittent exotropia. Fusional divergence
practice, it may not be cent percent true. usually develops spontaneously to allow the
3. To plan the amount of vertical muscle surgery eyes to straighten. If a stable esotropia persists
required in congenital or long-standing vertical over one month postoperatively, it needs to be
muscle palsies. corrected by base-out prisms. For a larger
overcorrection requiring resurgery, the base-out
II. Role of prisms in managing dilopia and prisms may be prescribed to prevent diplopia
abnormal head posture (relieving prisms) until surgical treatment can be undertaken.
Prism may be employed in the early management 4. Surgically undercorrected exotropia. It has
till scenario for surgical treatment is clear. been reported that in undercorrected exotropia,
1. Paralytic strabismus. During recovery phase the use of overcorrecting base-in prisms (10D
of paralytic squint, prisms are quite useful in greater than the deviation) in immediate
preventing contracture of the antagonist muscle, postoperative period may be successful in
e.g. as in: establishing a good fusional result. Hardesty has
described the convergence response to such
• Superior oblique palsy, and prism therapy. However, it has also been
• Sixth nerve palsy. reported that prisms used in this manner,
2. As a temporary measure in acquired ocular probably will not be effective, if given later than
restrictive defects such as blow-out fractures three months postoperatively.
 Principles of Non-Surgical and Surgical Management of Strabismus 403

5. Surgically undercorrected esotropia. In many • Patients with paralytic strabismus who have been
cases of esotropia with ARC in its peripheral deprived of binocular single vision for many
convergence response, a small residual esotropia months may not be able to fuse the images
with monofixation will be present postope- immediately prisms are introduced. Although
ratively. Prismotherapy in such a deviation is it may not be possible to demonstrate! fusion
not indicated, since it will not convert such cases in the clinic, it is worthwhile fitting trial
to bifoveal fixation. prisms for a period to see whether binocular
6. Surgically overcorrected convergence single vision can be obtained with more time.
insufficiency may spontaneously lead to • Patients with very incomitant deviations are
postoperative diplopia. During this period, thought to be unsuitable for prism therapy
prismotherapy may be useful in alleviating because the area of binocular single vision
the diplopia. achieved will be too small; however, even a
very small field of binocular fixation is
IV. Management of convergence insufficiency welcomed by some patients, while others find
Convergence insufficiency of hypoaccommo- the intermittent diplopia too distracting. It is
dative type and convergence paralysis are also often worth a short period of trial prisms in
relieved symptomatically by base-in prisms. these cases.

V. Management of heterophorias Methods of using prisms

Patients with marked asthenopic symptoms, Prisms can be used by following methods:
particularly those with vertical phorias respond • As Fresnel prism
well to prismotherapy. • In spectacle frames
• As clip-on prism
VI. Role of prisms in nystagmus (see page 393)
• Fresnel prisms are the method of choice for
Guidelines for prescribing prisms temporary use. Up to 30" can be applied to
In principle, the patient should be given the either eye but high-powered prisms may not
lowest powered prism which makes him/her be tolerated because of their adverse effect on
comfortable. The guideline generally applied is visual acuity; usually up to 20" can be worn
to correct two-thirds of the deviation, but the comfortably, at least by children.
variation found in different conditions and • In spectacle frames. If the patient has a high
among patients with the same condition make refractive error, the spectacle lenses can be
this rule of little value. Useful guidelines are: decentred to give the desired effect, provided
that the prism strength is not too great.
• Patients with acquired vertical muscle palsies Patients with lower refractive errors and those
usually require a prism equal or nearly equal who are emmetropic can be prescribed
to the angle of deviation. additional prisms. These methods are used,
• Patient with congenital or long-standing palsies when permanent or long-term use of prisms
and most patients with dysthyroid eye disease is envisaged.
involving vertical eye movement, may • Clip-on prisms: These are generally used, only
require as little as half the amount measured. when the patient cannot tolerate Fresnel
Patients with long-standing vertical prisms. The strength is limited to 10–12  in
strabismus caused by retinal detachment front of each eye because of the weight
surgery also behave in a similar way. involved. These are no longer commercially
• Prism correction. Patients with esodeviations, available.
especially those which increase in the distance
B. PHARMACOLOGIC TREATMENT
(divergence weakness type), are harder to
control and, therefore, require a larger prism I. Miotics (also see pages 193, 227 and 230)
in relation to the angle of deviation than do Mechanism of action. Though pharmacologic
those with exodeviations. actions of the miotics (parasympathomimetics)
 404 Theory and Practice of Squint and Orthoptics

include miosis and spasm of accommodation, Indications. Botulinum toxin may be useful in
their utility in strabismus is through their effect the short-term treatment of: Infantile esotropia,
on accommodation. paralytic strabismus especially acute 6th nerve
palsy, surgical overcorrections, Graves'
Miotics commonly used in strabismus manage-
ophthalmopathy and nystagmus.
ment include long-acting cholinesterase
inhibitors such as DFP (0.025% ointment and Dosage. Botulinum toxin is injected into the
0.1% solution), echothiopate (0.03%, 0.06%, muscle to be weakened under electromy-
0.125% and 0.25% solution), and demecarium ographic (EMG) control after local or general
bromide (0.125% and 0.25% solution). anaesthesia. When general anaesthesia is used,
ketamine hydrochloride rather than barbiturates
Indications for use of miotics include:
or halothane is used to preserve the EMG signal.
i. Diagnostic trial to differentiate between
Vertical and horizontal deviations less than 20D
accommodative (refractive and non-refractive)
are treated initially with 1.25 to 2.5 units and
and non-accommodative esotropia.
horizontal deviations greater than 20D with 2.5
ii. Therapy of accommodative esotropia especially
to 5 units initially in volumes of 0.05–0.15 ml.
non-refractive type (see page 229).
The saline reconstituted lyophylized powder
iii. Postoperative miotic therapy may be useful in
must not be shaken otherwise the protein will
patients with residual esotropia as well as in
denaturate. Reinjections may be titrated down
consecutive esotropia (after surgery for an
depending on the effect achieved from the
intermittent exotropia).
original injection.
iv. Amblyopia. Use of miotics in the amblyopic
eye and atropine in the sound eye has been Complications reported following injection
advocated as penalization treatment for of botulinum toxin are as follows:
amblyopia (see page 193). i. Diplopia due to transient overcorrection is very
common, but resolves in a few weeks.
2. Atropine ii. Blepharoptosis has been reported to occur in
Common uses of atropine in the management 25% children and 16% of adults after horizontal
of strabismus are as follows: muscle injection, due to spillage in the orbit,
i. Cycloplegic refraction. Atropine is most useful which resolved within a few months.
for cycloplegic refraction especially in children iii. Vertical deviations after horizontal muscle
with strabismus. injection have been reported to occur in 17%
ii. Therapy of accommodative esotropia. A few cases, which persisted only in 20% cases.
workers have recommended atropine in iv. Perforation of the globe and retrobulbar or
combination with overcorrection of hypermetropic subconjunctival haemorrhage are the other very
refractive error for treatment of accommodative rare complications noted.
esotropia.
iii. Amblyopia. Use of atropine in sound eye with 4. Other drugs
or without a miotic in the amblyopic eye has i. Chlordiazepoxide hydrochloride has been
been recommended as penalization treatment reported to decrease the deviation and/or
for amblyopia. improve the fusional amplitude in patients with
esodeviations.
3. Botulinum toxin ii. Phenytoin (dilantin) has been reported to
Mechanism of action. Botulinum toxin, when reduce the near point of accommodation and
injected into an extraocular muscle blocks AC/A ratio and thus affect accommodative and
release of acetylcholine and thus causes chemical partially accommodative esotropia.
denervation and thus paralysis of the muscle for iii. Levadopa/carbidopa combination has been
several weeks. As a result, the antagonist muscle used as an adjunct to occlusion therapy in
becomes comparatively strong and thus amblyopia, with no clear role. Some workers
neutralizes the deviation. have reported its role in early initiation of the
 Principles of Non-Surgical and Surgical Management of Strabismus 405

effect of occlusion in cases of moderate to severe to understand and their attention span may only
amblyopia. be 1 or 2 minutes. There are some exceptional
iv. Citicholine (CDP choline) has also been 4 years old who understand instructions well
reported to have a role in amblyopic patients. It but whose attention span may be too short to
is an essential intermediate for phosphatidyl make treatment worthwhile. The age of 5 years
choline synthesis. It increases cerebral blood is usually the youngest treatable age. The
flow and also shows neural restorative effect via average age to begin treatment is 6 years.
its action on dopaminergic pathway for central 2. At the parents' level. Since parents play the
nervous system. key role in their child's treatment, they must:
C. ORTHOPTICS • Realize that the reeducation process takes a
long time,
Literally, the word orthoptics means 'straight
eyes'. However, with regard to therapy • Have good understanding of the purpose of
orthoptics refers to teaching an individual to the treatment, methods and procedures used,
obtain the best possible use of both eyes together • Have good rapport between child and
in the form of a comfortable binocular single orthoptist.
vision. Practically orthoptics training is used to 3. At the orthoptist level. Since orthoptist is the
treat convergence insufficiency, to combat main person concerned with the treatment, he/
suppression, amblyopia and abnormal retinal she should:
correspondence and to improve fusional • Have a friendly but firm attitude towards the
amplitudes and stereopsis. child,
Goals of orthoptic treatment • Have a good rapport and understanding with
The ultimate goals of orthoptic treatment are: the treating ophthalmologist, and
1. Visual acuity levels in each eye should be best • Win the confidence of child and parents.
possible. 4. At the ophthalmologist level. Since it is the
2. Eyes should be straight—with or without ophthalmologist who refers the child for the
surgical help. orthoptic treatment, therefore, he should:
3. Binocular single vision. • Ensure that the other modes of treatment
4. Fusion with good amplitudes and reserves. required, i.e. refraction and prescription of
5. Reduction of refractive glasses (when glasses, prisms, miotics, etc. have been taken
involved). care of
• Ensure that symptoms must be attributable to
Pre-requisites for a successful orthoptic treatment the deviation and not to other causes.
1. At the patient level. Since orthoptics is mainly a • Must exclued pathological causes for strabismus
training process chiefly concerned with helping • Have frequent conferences with the orthoptist
to establish a new sensory pathway that will effect about the patient's treatment, and
the accurate usage of the two eyes since this takes
• Take decision about the surgical treatment
place at the cortical level, therefore, vital pre-
whenever required.
requisites at the patient level are:
• Reasonable intelligence, Indications of orthoptics
• Physical and psychological maturity, 1. Diagnostic indications. A complete orthoptic
• Good attention span and, work-up is required in each and every patient
• Confidence and co-operation. suspected of having a neuromuscular anomaly
• Able to attend regularly. of the eye.
Keeping in view the above points, the age of 2. Therapeutic indications. Orthoptic exercises
patient is very important. It is impossible to treat and training may be required in patients with
orthoptically 2–3 years old. They are too young phorias and tropias, both pre- and post-
 406 Theory and Practice of Squint and Orthoptics

operatively. The scope of orthoptic treatment Indications

includes: • To compensate a latent strabismus
• Elimination of convergence insufficiency • To improve control of an intermittent
• Fusion training, to increase fusion amplitude strabismus
• Antisuppression exercises • As a pre- and postoperative measure in constant
• Treatment of anomalous retinal correspondence strabismus
• Treatment of amblyopia 3. Orthoptic treatment of suppression includes
• Control of deviation the following:
• Diplopia exercises
Order of orthoptic treatment • Vergence control in heterophoria and surgical
The natural course of events, when a hetero- alignment of eyes in large tropias.
phoria slowly becomes a heterotropia and is left • Differential stimulation
untreated, in order of occurrence include: • Macular massage
• Deterioration of amplitudes • Occlusion therapy
• Occurrence of diplopia (for details, see page 190)
• Development of suppression and development
of amblyopia (in a monocular deviation).
However, the orthoptic treatment in a patient SURGICAL MANAGEMENT
with heterotropia proceeds in the opposite Extraocular muscle surgery is only a part of the
direction as follows: therapeutic management of a strabismic patient.
• Amblyopia is treated first, to be followed by Of course, the squint surgery is aimed to
• Antisuppression therapy, produce and maintain a condition in which the
• Diplopia training, and visual axes of the two eyes are directed , without
• Amplitude improvement. conscious effort to the object of fixation whatever
its position. However, in some cases, this may
Delivery of orthoptic treatment require more than one operation and in others
The details of the orthoptic treatment required it may even not be possible at all. All these facts
for various neuromuscular anomalies of the eye should be made amply clear to the patient and/
have been described along with the concerned or the parents.
anomaly. However, for a quick review, they are
listed below. INDICATIONS FOR SQUINT SURGERY
1. Treatment of convergence insufficiency. 1. To correct squint cosmetically as well as
Orthoptic treatment of convergence insufficiency functionally. This is possible in cases in which
is quite effective (for details, see page 157). It the visual acuity of the two eyes is equal or has
includes the following: been made nearly equal by appropriate
• Pencil convergence exercises occlusion treatment (so that alternation occurs
• Physiological diplopia exercises easily) and in which binocular function has been
improved (where possible) by appropriate
• Training for increasing fusional convergence
orthoptic treatment, if indicated.
with base-out prisms or on synoptophore.
2. To correct the squint only cosmetically. This
2. Exercises for increasing fusional amplitudes. is indicated in old children and adults who have
Both convergence and divergence fusional untreatable deep amblyopia, persistent
amplitudes can be increased by fusion training abnormal retinal correspondence and absence
using: of power of fusion. Patients having sensory
• Prisms or squint secondary to organic disorders (e.g. optic
• Major amblyoscope atrophy, central chorioretinitis) are also
(for details, see pages 213 and 214) corrected cosmetically only.
 Principles of Non-Surgical and Surgical Management of Strabismus 407

3. To relieve marked asthenopic symptoms as early as possible. In many cases with

squint surgery may be indicated, in some cases. intermittent squint the need for operation may
These include patients with phorias and be dispensed altogether with glasses and/or
intermittent tropias which could not be treated orthoptic treatment. However, before
by an active orthoptic treatment. abandoning the idea of surgery, it must be
4. To correct abnormal head posture which may ascertained that squint has completely
be assumed by some patients to relieve diplopia disappeared and not that there has occurred
(e.g. in superior oblique or lateral rectus mere reduction in the angle of deviation
weakness) or to improve vision (e.g. with (which may be satisfactory from the cosmetic
nystagmus and an eccentric null point). point of view but of no functional value).
5. To relieve mechanical restriction or to ii. In children old enough for orthoptic
improve appearance surgery may be required treatment (i.e. those above 4–5 years), following
sometimes in patients with hypertrophied considerations should be made:
conjunctiva or Tenon's capsule from prior • Before the surgery is performed for a constant
muscle surgery. squint, best possible efforts with optical and
orthoptic treatment should be made to treat
OPTIMAL TIME FOR SQUINT SURGERY the associated (if any) sensory adaptation
Optimal time for squint surgery varies (such as suppression, amblyopia and abnor-
depending upon the type of squint, age of the mal retinal correspondence). The efforts
patient and presence of various sensory should be aimed at developing good binocular
adaptations as below. vision with fusion amplitudes.
• In the presence of an abnormal retinal corres-
1. CONCOMITANT SQUINT
pondence, now most surgeons recommend
Although it is difficult to be dogmatic as to early surgery; since both cosmetic and
precisely when to operate upon a case of functional results may be obtained in many
concomitant squint, especially in a child, the cases (i.e. ARC disappears spontaneously after
following suggestions have been made by surgery).
experienced strabismologists:
• In the absence of a true fusion, the case may
i. Children too young for orthoptic treatment, have to be assessed purely for the cosmetic
i.e. those below 4–5 years should be considered effect. However, it has been reported that
for operation as follows: parallelism of the visual axes produced by an
• If a constant squint is present after wearing of early surgery, may sometimes result in
glasses (where indicated) for a month and the development of binocular fusion.
squint is an alternating one or that an early
alternation has been established by appropriate iii. In older children (above 12 years of age) and
occlusion, i.e. vision is almost equal in the two adult patients who have deep intractable
eyes; the child should be operated as early as amblyopia (functional or organic) the time of
possible. Such a recommendation has been surgery may be decided according to personal
made on the basis of the observation that in requirements for cosmetic purposes only, since
young children, if the visual axes are put there is hardly any scope for a functional cure.
within a few degrees of parallelism by means
of operative treatment, and good binocular 2. PARALYTIC SQUINT
vision may develop per se, provided that the The most important principle is to establish over
child already posseses some rudimentary a significant period of time that the condition is
development of his normal binocular reflexes. stable and that no spontaneous improvement is
• In intermittent squint with or without glasses, likely to occur. Therefore, a hastily surgical
the surgery should be delayed and child treatment may result in an over-correction of the
should be observed and refracted every six deviation in some cases. On the other hand in
months. Orthoptic treatment should be started some cases the condition may continue to
 408 Theory and Practice of Squint and Orthoptics

deteriorate so that ill-time surgical interference 3. Myectomy also weakens the muscle by
will prove to be inadequate. However, in most reducing the contractile fibres and is seldom
cases if the condition remains static for a period performed nowadays except by some surgeons
of 3–6 months, the surgical treatment may be especially for inferior oblique muscle.
considered. 4. Free tenotomy or disinsertion of the rectus
muscles may be performed in desparate cases.
Expectations from the treating ophthal- Oblique muscle tenotomy is practised by many
mologists regarding optimal time to operate surgeons to weaken this muscle.
It is expected that the operating ophthal- 5. Posterior fixation suture also known as
mologist should be ready with the following Faden operation or retropexy of an extraocular
spade work and home task when he decides that muscle is a weakening procedure that does not
it is the optimal time to operate: affect the deviation in primary position but
• He should have accomplished all the weakens the muscle action in patients who are
preoperative measures which are necessary already orthotropic. However, it reduces the
and helpful to achieve the basic three goals deviation in esotropic patients, when performed
of squint management. on medial rectus. Faden operation is performed
under following circumstances:
• He should have a plan as to what surgery will
be performed and why. – To correct the dissociated vertical deviation.
• By this time, he must educate the parents and/ – Patients having incomitant strabismus with
or patients as to the goals, his plan of attack, orthotropia in primary position.
the risks involved in surgery, the risks – To treat upshoot and downshoot of the
involved in not operating, and the possibility adducted eye in patients with Duane's
that more than one operation may be retraction syndrome type I.
necessary. – Esotropia with a variable angle.
• He should have plans for what to do post- – Persistent esotropia after maximal recession
operatively: and resection surgery.
– To dampen the nystagmus.
– If the patient is fusing,
– If the patient is overcorrected, and It has been reported by von Noorden that the
Faden operation is most effective, when
– If the patient is undercorrected.
performed on the medial rectus, less effective
on vertical rectus muscles and least effective on
TYPES OF SURGICAL TECHNIQUES FOR the lateral rectus muscle.
SQUINT CORRECTION 6. Recession of conjunctiva and Tenon's capsule
may also help in augmenting the weakening
A. MUSCLE WEAKENING PROCEDURES
effect of a rectus muscle especially in patients
1. Recession of an extraocular muscle is the most with large deviations of long standing where the
commonly performed weakening procedure. elasticity of conjunctiva and Tenon's capsule is
This procedure weakens the muscle action by impaired and where scars have been formed
changing its arc of contact with the globe. from previous surgery.
2. Marginal myotomy is infrequently indicated. 7. Muscle lengthening by insertion of a silicone
This procedure weakens the muscle by reducing expander or non-absorbable suture material has
the number of contractile fibres and not by been recommended as a more controlled
changing the arc of contact. Therefore, it is weakening procedure for superior oblique muscle.
effective in further weakening on already
maximally recessed muscle. This procedure is B. MUSCLE STRENGTHENING PROCEDURES
also indicated where recession cannot be 1. Resection is the most commonly performed
performed as in patients with very thin sclera muscle strengthening procedure. This procedure
and in those having buckle implants. strengthens the muscle by shortening its length.
 Principles of Non-Surgical and Surgical Management of Strabismus 409

One should avoid excessive resection of a age at the time of onset of squint, duration of
muscle, since this may restrict the eye squint, age at the time of operation, visual status,
movements in the opposite direction. convergence and accommodation status.
2. Advancement of the muscle insertion towards Therefore, degree of squint correction versus
limbus is usually not preferred as the primary amount of extraocular muscle manipulation
procedure alone. However, it may be combined required cannot be mathematically determined.
with the resection procedure or may be used as A discussion on this aspect of squint manage-
secondary procedure in already resected muscle ment can be considered under following heads:
to further strengthen it or in cases with • General considerations for planning squint
overcorrection due to recession of a muscle. surgery
3. Tucking of an extraocular muscle also • Guidelines for planning squint surgery
enhances its action. This procedure is not being • Rough estimates for amount of squint surgery
preferred for rectus muscles. However, a GENERAL CONSIDERATIONS FOR
superior oblique tucking is performed PLANNING SQUINT SURGERY
frequently to strengthen this muscle. This
As we know, it is not possible to provide a
procedure, when performed on the superior
readymade menu for correcting each patient
oblique muscle, is quite effective in improving
with strabismus, rather the plan of surgery has
the depression of the adducted eye and in
to be tailor made for the individual patient. The
counteracting the excyclotropia.
clinical factors other than the measurement of
C. PROCEDURES THAT CHANGE DIRECTION OF deviation in primary position which need to be
MUSCLE ACTION considered in the planning are as follows:
1. Amblyopia. As discussed under the 'optimal
1. Vertical transpositioning of the horizontal
time for surgery', the surgery should be delayed
recti is recommended in patients with A- or V-
till the vision has been made equal or nearly so
pattern without associated oblique muscle
by appropriate means such as glasses and
dysfunction.
amblyopia therapy, when needed. However, if
2. Horizontal transpositioning of the vertical the visual acuity cannot be made equal, the
recti has also been recommended by some surgery should preferably be performed on the
surgeons for correction of A-V pattern. eye with poor vision.
3. Slanting of the rectus muscle insertion has 2. Vertical incomitancy. Presence of A- or V-
also been recommended by some surgeons for pattern should be taken into consideration while
correction of A-V-patterns. However, at present, planning surgery for the horizontal strabismus.
it is not a preferred technique. The surgical treatment may include an
4. Transplantation of muscles in paralytic additional surgery on the oblique muscle or
squint (see page 439). vertical transpositioning of the horizontal recti.
3. Horizontal incomitancy. In the presence of a
D. PERIOSTEAL FIXATION OF THE GLOBE MEDIAL mechanical restriction or paretic limitation of the
1. Medial periosteal fixation of globe is eye movements, the deviation in left gaze and
recomendation in patients with third nerve right gaze may differ significantly from the
palsy deviation in primary gaze. Surgeon should aim
2. Lateral periosteal fixation of lateral rectus at making the alignment more nearly comitant
after disinsertion is also recomended in patients after surgery.
with third nerve palsy. For example, in a patient with 30° exotropia
in primary position, 20° in right gaze and 40° in
CHOICE OF OPERATION AND left gaze, the surgery may be modified as below:
AMOUNT OF SURGERY • If bilateral lateral rectus recession is planned,
Choice of operation and amount of extraocular a more recession on left than the right side may
muscle surgery to be performed depend upon be performed to have greater reduction of the
multiple factors, i.e. type and angle of squint, exotropia in left gaze.
 410 Theory and Practice of Squint and Orthoptics

• If a resect-recess procedure is planned to get 5. Previous surgery. The details of the previous
a greater reduction in exotropia in left gaze, a surgery performed (wherever possible), its
greater recession of the left lateral rectus and results and effects (any mechanical restriction,
less resection of the left medial rectus than the etc.) should be duly taken into consideration
standard amounts of surgery should be while planning a repeat surgery as follows:
performed. • Though planning for an under- or over-
In other words, testing of versions is very corrected squint should be made as for a fresh
important in deciding the appropriate surgical case of squint, it is preferable to operate on
technique as follows: muscles that have not had prior surgery.
• In esotropia associated with excessive adduction • In the presence of a mechanical restriction
and normal abduction, a maximal recession of from excessive resection/scarring or weakness
the medial rectus and a nominal resection of from excessive recession, reoperation on the
the lateral rectus should be performed. involved muscle may provide better results.
• In esotropia associated with normal adduction and • In multiple surgeries, one must ensure that at
a deficient abduction, a maximal resection of the least one rectus muscle remains unoperated
lateral rectus and a nominal recession of the in each eye.
medial rectus should be performed. 6. Distance and near measurements and AC/A
• In exotropia associated with excessive abduction ratio should also be taken into consideration
and normal adduction a maximal recession of while planning surgery for horizontal devia-
the lateral rectus and a nominal resection of tions. Duane classified horizontal deviations on
the medial rectus should be performed. the basis of distance/near measurements as
• In exotropia associated with normal abduction and follows:
deficient adduction, a maximal resection of the
Esodeviations
medial rectus and a nominal recession of the
lateral rectus should be preferred. • Basic esotropia—distance deviation equals near
deviation. Some surgeons prefer a monocular
• When the strabismus is associated with a normal
recession of the MR and resection of LR in such
abduction and adduction, one should prefer the
cases. While others prefer bilateral symmetrical
strengthening (resection) rather than the
recession of medial recti.
weakening (recession) procedure.
• Convergence excess type esotropia—near
• When the strabismus is associated with an
deviation greater than distance. Bilateral
excessive movement in one direction and deficient
medial rectus recession is preferred by some
on the other, one should prefer to do maximal
surgeons over monocular recess-resect
weakening of the muscle in the excessive
procedure.
movement and maximal strengthening of the
muscle with deficient movement. • Divergence insufficiency type esotropia—
All the above recommendations have been distance deviation greater than near. Bilateral
made by the workers with an aim to normalize lateral rectus resection is preferred by some
the excursions of the eyes along with correction surgeons over uniocular recess-resect
of deviation. procedure.
4. Lateral incomitancy. One must consider the Exodeviations
measurements in lateral gaze while planning • Basic exotropia—distance and near deviation is
surgery in a patient with intermittent exotropia. It equal. Some surgeons prefer a monocular
has been observed that standard amounts of recession of the LR and resection of MR in such
surgery may result in overcorrection in patients cases. While others prefer bilateral symmetrical
having lateral incomitancy (e.g. in a patient with recession of lateral recti.
exotropia of 30° in primary positioin and of 20° in • Convergence insufficiency type exotropia—near
right as well as left gaze). It has been recommended devition is greater than distance. This condition
that amount of recession of each lateral rectus rarely requires surgery. Some success has been
should be reduced by 1 mm in such patients. reported with bilateral MR resections.
 Principles of Non-Surgical and Surgical Management of Strabismus 411

• Divergence excess type exotropia—distance 3. Age of the patient and duration of squint. A
deviation greater than near deviation, normal more extensive surgery may be required in older
AC/A ratio, no increase in near deviation on children and adults having squint of long
occlusion. Bilateral lateral rectus recession is duration as compared to small children for the
preferred by many surgeons over uniocular same amount of deviation, since in the former,
recess-resect procedure. secondary anatomical changes take place in the
• Pseudo or simulated divergence exces type of muscles and fascia.
exotropia. In this condition, distance deviation 4. Recession versus resection. In general,
measures greater than near deviation on weakening of a muscle by recession produces
routine examination. But after occlusion test, more correction per millimetre of surgery vis-
near deviation increases to equal the distance a-vis strengthening of a muscle by resection.
deviation. Uniocular recess-resect procedure Therefore, relatively larger amounts of resection
is preferred by most surgeons. are required to produce an effect comparable to
that achieved by recession of the antagonist.
7. Special considerations for cyclovertical
5. Intractable amblyopia. In the presence of an
strabismus. While considering surgery for the
intractable amblyopia, it is not possible to predict
vertical strabismus, one must make the note of
the results of surgery. This point should be made
deviation in right gaze and left gaze and also in
amply clear to the patient and/or parents.
upgaze and downgaze of the same eye. And in
6. Medial versus lateral rectus surgery. In
general, surgery should be performed on those
general, a recession of the medial rectus muscle
muscles whose field of action is in the same field
is more effective than the same amount of
as the greatest vertical deviation.
recession performed on a lateral rectus muscle.
8. Forced duction test (FDT) should always be 7. Horizontal versus vertical rectus muscles.
performed before planning the surgery. In small Recession of the vertical rectus muscles is much
children, FDT should be performed under more effective than the recession performed on
general anaesthesia just before surgery, and if a the horizontal rectus muscles.
mechanical restriction is detected, the original 8. Combined recession-resection operation
surgical plan may have to be changed provides more correction than the added results
accordingly. However, it should be kept in mind of each procedure, when performed alone.
that when succinylcholine has been used, a Further, this procedure is more effective in
sustained contraction of the extraocular muscles stabilizing the surgical results vis-a-vis single
may occur for a period of about 20 minutes. procedure, since the resection procedure
Therefore, it is better to use a non-depolarizing reduces the amount of contracture that normally
muscle relaxant in squint surgery, since it will occurs in recessed antagonist.
not alter the FDT.
ROUGH ESTIMATES OF AMOUNT OF SQUINT SURGERY
GUIDELINES FOR PLANNING SQUINT SURGERY As mentioned earlier, it is not possible to
General guidelines based on the experience of provide a surgical dose-response curve or tables
various squint surgeons which can help in for correcting strabismus. Nevertheless, the
planning the squint surgery are as follows: conclusions drawn by experienced surgeons
may serve as rough estimates for the beginners.
1. Surgeon factor. Every surgeon gets a different However, once again it is stressed that surgeons
amount of correction vis-a-vis another surgeon should standardize their own approach by
for the same amount of surgery. Therefore, it is retrospectively and continuously reviewing
advisable that each surgeon must standardise their own results and adjusting the amount of
one's approximate effectiveness of a particular surgery for attaining the best possible results.
procedure based on review of his/her experience. For standardizing their surgery, it is mandatory
2. Degree of squint. The same amount of muscle that surgeons should use the ocular motility
surgery will give greater correction for larger measurements and judgement made by
deviations vis-a-vis smaller deviations. themselves. The rough estimates of surgical-
412 Theory and Practice of Squint and Orthoptics

Table 15.1 Rough estimate of amount of extraocular muscle surgery for esotropia
Deviation in Monocular surgery in mm Binocular surgery in mm
prism dioptres
Recession of + Resection of Bilateral or Bilateral or
MR LR MR recession LR resection
15 3.0 4 3.0 4.0
20 3.5 5 3.5 5.0
25 4.0 5 4.0 5.5
30 4.5 6 4.5 6.0
35 5.0 7 5.0 6.5
40 5.5 7 5.5 7.0
50 6.0 8 6.0 8.0
60 6.5 9 6.5 9.0
70 7.0 10 7.0 10.0

Table 15.2 Rough estimate of amount of extraocular muscle surgery for exotropia
Deviation in Monocular surgery in mm Binocular surgery in mm
prism dioptres Recession of + Resection of Bilateral or Bilateral
MR LR MR recession LR resection
15 4.0 3.0 4.0 3.0
20 5.0 4.0 5.0 3.5
25 6.0 4.5 5.5 4.5
30 7.0 5.0 6.0 5.5
35 7.5 5.0 6.5 6.0
40 8.0 6.0 7.0 6.5
50 9.0 7.0 8.0 7.5
60 10.0 8.0 9.5 8.0
70 10.0 10.0 8 mm bilateral LR recession +
8 mm MR resection of one eye
80 8 mm bilateral LR recession +
8 mm bilateral MR resection

dosage have been described along with the adequate analgesia without affecting the motor
different types of strabismus. However, for a supply of extraocular muscles and thus allowing
ready reference, they are again summarized in the readjustment of muscle position during
Tables 15.1 and 15.2. surgery to affect cosmetic or functional results.
It is specially useful where unpredicted results
ANAESTHESIA FOR SQUINT SURGERY are anticipated.
Three types of anaesthesia commonly used in
Technique. Topical anaesthesia can be achieved
strabismus surgery are:
by instillation of either 4% cocaine, 0.5%
• Topical anaesthesia proparacaine or 0.5% tetracaine drops, four
• Local anaesthesia times every 4 minutes, before the conjunctival
• General anaesthesia incision is made. It is important to note that after
the conjunctiva is opened, further anaesthetic
TOPICAL ANAESTHESIA drops should not be instilled, since paralysis of
Use and indications. It has been recommended extraocular muscles will occur and thus the main
that use of topical anaesthesia produces advantage of topical anaesthesia will be lost.
 Principles of Non-Surgical and Surgical Management of Strabismus 413

Prerequisites. Topical anaesthesia can be used localised akinesia of the orbicularis oculi muscle
in co-operative adults only. In addition, a very without associated facial paralysis.
fine handling is required during surgery under In this technique, 2.5 ml of anaesthetic solution
topical anaesthesia. Excessive pulling and is injected in deeper tissues just above the
manipulation produces pain; so topical eyebrow and just below the inferior orbital
anaesthesia is effective for simple recession margin, through a point about 2 cm behind the
procedures, and not for resection procedures or lateral orbital margin, level with outer canthus
for recession procedures involving restricted (Fig. 15.1).
muscles where exposure is difficult. Further, it
is recommended that no barbiturates or high
doses of analgesics be given preoperatively, since
these will affect the angle of deviation during
surgery and thus the mere purpose of topical
anaesthesia will be defeated. It should be ensured
that a suitable target and cover device should be
available in the operation theatre to check the
alignment during the procedure.
Disadvantages. Topical anaesthesia is not
effective in controlling the pain produced by
pulling on or against a muscle and thus not
suitable in all cases. Further, it can be used only
in very co-operative adults and is thus not much
popular. Fig. 15.1 Technique of van Lint's facial block.

LOCAL ANAESTHESIA
2. Facial nerve trunk block at the neck of mandible
Local anaesthesia is commonly used for squint
(O'Brien's block). In it, facial nerve is blocked
surgery in older co-operative children and
near the condyloid process. The condyle is
adults. It allows a very comfortable and smooth
located 1 cm anterior to the tragus. It is easily
surgery by producing lid and ocular anaesthesia
palpated, if the patient is asked to open and close
and akinesia.
the mouth with the operator's index finger
Techniques. Local anaesthesia can be achieved
located across the neck of the mandible. At this
either by—(1) a combination of surface
point, the needle is inserted until contact is made
anaesthesia, facial nerve block and retrobulbar
with the periosteum and then 4–6 ml of local
block or (2) a combination of surface anaesthesia
anaesthetic is injected while the needle is
and peribulbar block.
withdrawn (Fig. 15.2).
Surface and topical anaesthesia is achieved as
This technique is associated with pain at the
described above. injection site and unwanted facial paralysis.
Facial block. For intraocular surgery, it is
3. Nadbath block: In this technique, the facial
necessary to block the facial nerve which
nerve is blocked as it leaves the skull through
supplies the orbicularis oculi muscle, so that
the stylomastoid foramen. This block is also
patient cannot squeeze the eyelids.
painful.
Orbicularis akinesia can be achieved by
blocking the facial nerve at its terminal branches 4. Atkinson's block: In it, superior branches of the
(Van Lint block), superior branches (Atkinson facial nerve are blocked by injecting anaesthetic
solution at the inferior margin of the zygomatic
block) or proximal trunk (O'Brien or Nadbath
bone.
block).
1. Blocking the peripheral branches of facial nerve Retrobulbar block. It was introduced by
(Van Lint's block): This technique blocks the Herman Knapp in 1884. It is administered by
terminal branches of the facial nerve, producing injecting 2 ml of anaesthetic solution (2%
 414 Theory and Practice of Squint and Orthoptics

Fig. 15.4 Position of needle for peribulbar block in the

Fig. 15.2 Distribution of facial nerve and technique of
peripheral orbital space (A) and for retrobulbar block in the
O'Brien's block. muscle cone (B).

Complications encountered with it include

xylocaine with added hyaluronidase 5 IU/ml
retrobulbar haemorrhage, globe perforation,
and with or without adrenaline one in one lac)
optic nerve injury, and extraocular muscle
into the muscle cone behind the eyeball. It is
palsies.
usual to give the injection through the inferior
fornix or the skin of outer part of lower lid, with Peribulbar block. This technique described in
the eye in primary gaze (Fig. 15.3B). The needle 1986 by Davis and Mandel has almost replaced
is first directed straight backwards then slightly the time tested combination of retrobulbar and
upwards and inwards towards the apex of the facial blocks, because of its fewer complications
orbit, up to a depth of 2.5 to 3 cm (Fig. 15.4B). and by obviating the need for a separate facial
block.
Retrobular block anaesthetises the ciliary
Primarily, the technique involves the injection
nerves, ciliary ganglion and third and sixth
of 6–7 ml of local anaesthetic solution in the
cranial nerves thus producing globe akinesia,
peripheral space of the orbit (Fig. 15.4A), from
anaesthesia and analgesia. The superior oblique
where it diffuses into the muscle cone and lids,
muscle is not usually paralysed as the fourth
leading to globe and orbicularis akinesia and
cranial nerve is outside the muscle cone. anaesthesia. Classically, the peribulbar block is
administered by two injections; first through the
upper lid (at the junction of medial one-third
and lateral two-thirds) and second through the
lower lid (at the junction of lateral one-third and
medial two-thirds) (Fig. 15.3A). After injection
orbital compression for 10–15 minutes is applied
A
with digital massage, superpinky or any other
method.
The anaesthetic solution used for peribulbar
anaesthesia consists of a mixture of 2%
B lignocaine, and 0.5–0.75% bupivacaine (in a ratio
of 2:1) with hyaluronidase 5 IU/ml and
adrenaline one in one lac.

GENERAL ANAESTHESIA
Fig. 15.3 Position of needle on the lower eyelid skin for Indications. General anaesthesia is indicated for
peribulbar block (A) and retrobulbar block (B). squint surgery in infants, small children and also
 Principles of Non-Surgical and Surgical Management of Strabismus 415

in anxious, uncooperative and mentally retarded inexperienced surgeon, who may perform less
adults and those patients willing for surgery surgery, resulting in under-correction; and thus
only under general anaesthesia. another surgery may be required.
Special consideration during general anaes-
thesia for squint surgery COMMON OPERATIVE STEPS IN SQUINT
• It is recommended that if the child with SURGERY
esotropia is on miotic therapy (phospholine Fixation of the globe
iodide or other cholinestrase agents), it should For fixation and rotation of the globe in different
be discontinued at least 6 weeks prior to directions, 6-0 or 5-0 Mersilene or silk suture on
surgery so that the blood cholinestrase levels spatulated needle is passed through the
can be returned to normal before surgery. If conjunctiva and episcleral tissue near the limbus
this has not been possible, the anaesthesio- at following positions:
logist should be warned that the use of
• For horizontal rectus muscle surgery—at 12
succinylcholine is contraindicated.
and 6 o' clock positions (Fig. 15.5A).
• Since succinylcholine causes sustained
• For vertical rectus muscle surgery—at 9 and
contraction of extraocular muscles for about
3 o'clock positions.
20 minutes, so it will alter the forced duction
test (FDT). Therefore, it is better to use a non- • For inferior oblique muscle surgery—at about
depolarising muscle relaxant in squint 4½ o'clock in left eye and at about 7½ o'clock
surgery, since it will not alter the FDT. in right eye (i.e. near the limbus in infero-
temporal quadrant).
Advantages of general anaesthesia. The main
After applying the traction sutures the eyeball
advantage of general anaesthesia is that it
is rotated away from the muscle on which
produces complete analgesia and akinesia and
surgery is being performed, e.g. laterally for
does not require patient's cooperation during
surgery on the medial rectus (Fig. 15.5A).
the surgical procedure. Needless to say that
while performing surgery under general
anaesthesia, the ophthalmologist is most Conjunctival incision and exposure of the muscle
comfortable and is relieved of serious The conjunctival approaches recommended for
responsibilities. exposure of the rectus muscles are: (1) Limbal
Disadvantage of general anaesthesia in squint incision approach, (2) transconjunctival or Swan
surgery. (1) In general there is increased risk, i.e. approach, and (3) cul-de-sac or fornix approach.
not present in topical and local anaesthesia. The technique, advantages and disadvant-
(2) General anaesthesia is more costly and ages of each approach are described here in
requires services of an experienced anaesthesio- brief.
logist. (3) Incidence of oculocardiac reflex Limbal incision or von Noorden's approach
(bradycardia), oculodepressor reflex (hypo- Technique
tension) and oculorespiratory reflex (apnoea) is
higher under general anaesthesia. Therefore, it 1. At the limbus, conjunctiva and Tenon's
is recommended that a retrobulbar anaesthesia capsule are fused together. This conjoined tissue
should be given (to block the afferent pathway is grasped close to the limbus with forceps and
of these reflexes), even when the squint surgery a small radial incision perpendicular to the
is being done under general anaesthesia. limbus is made with the help of scissors
(4) Under general anaesthesia, it is not possible (Fig. 15.5B).
to relate eye position with the preoperative 2. The dissection is then carried concentric with
deviation and postoperative results. This the limbus by spreading the blades of a blunt-
preposition is to be particularly kept in mind in tipped Westcott spring-action scissors beneath
esotropia, since the eye under general the conjoined layer; which is then severed.
anaesthesia may look less esotropic or even 3. Generally, perpendicular incision of about
exotropic. This situation may intimidate an 4–5 mm long are made at both ends of the limbal
416 Theory and Practice of Squint and Orthoptics

Fig. 15.5 Surgical technique of medial rectus recession (for explanation, see text).
 Principles of Non-Surgical and Surgical Management of Strabismus 417

incision to facilitate exposure (Fig. 15.5C). Disadvantages

However, some surgeons prefer only one The disadvantages are a reasonably frequent
perpendicular incision. occurrence of dellen and retraction of the
4. The limbal incision provides a direct access conjunctival flap postoperatively but these are
to the Tenon's space. To separate the anterior minimal.
Tenon's capsule and intermuscular septum from
the sclera, the blades of a curved Steven's Over the muscle incision (Swan approach)
tenotomy scissors are inserted into the upper Technique
and lower quadrants and the blades are spread In this approach, an incision in both, the
gently only once (Fig. 15.5E). conjunctiva and Tenon's capsule is made over the
5. A muscle hook is then inserted below the muscle. For the medial rectus, the incision is
muscle near its insertion and the muscle is placed 1–2 mm anterior to the plica semilunaris
engaged by rotating the hook 180° (Fig. 15.5E). and for the lateral rectus, the incision is made
6. The muscle is made prominent by applying midway between the limbus and the lateral
traction with the hook and lifting away the fornix. The sub-Tenon's space is freed by
conjunctival flap (Fig. 15.5H). spreading the scissors points. The muscle is
7. For a good exposure, the borders of the muscle engaged by the muscle hook and is freed from
are then freed by sharp dissection of the facial facial connections by sharp dissection.
connections and the conjunctival surface of the Advantages
muscle in the anterior part is freed after severing
The only advantage of this approach is that
the check ligaments.
limbal area is not disturbed and so there are no
8. The muscle surgery is then performed as per chances of formation of cosmetically unsatis-
requirement (Fig. 15.5F to I) and after completion factory ridge at the limbus and thus no problem
of the procedure, the wound is closed by of dellen formation.
applying sutures through the edges of the
conjunctival flap and the limbal tissue near the Disadvantages
incision. Additional sutures are applied to close This incision has been reported to produce
the radial incision as per requirement (Fig. 15.5J). fibrosis over the muscle resulting sometimes in
a cosmetically unacceptable conjunctival scar
Advantages of limbal incision
and also making the resurgery (when required)
• It provides a direct access to the Tenon's space more difficult. So, most of the squint surgeons
and thus a very little dissection of Tenon's are not preferring this approach.
capsule is required to reach the muscle
insertion; this cuts down the bleeding and Cul-de-sac (fornix) incision (Park's approach)
scarring. Technique
• With this incision, the normal anatomic
1. In this approach, a combined conjunctival and
relations of Tenon's capsule and conjunctiva are
Tenon's capsule incision is made in the bulbar
least disturbed and that it allows a fine closure.
conjunctiva just near the cul-de-sac (1–2 mm to
• This technique is easy and quick and allows the limbal side of cul-de-sac). This incision is
optimal cosmetic and functional results. parallel to the fornix, approximately 8 mm in
• It is essential for a bare sclera closure (i.e. for length and depending upon the muscle to be
conjunctival recession). operated at following sites:
• It is the most comfortable approach for • For surgery on medial rectus, assistant is asked
adjustable muscle surgery. to grasp the limbus in the inferior nasal
Note. Because of the above advantages, the limbal quadrant and rotate the eyeball upward and
incision has become technique of choice for outward; and an incision is made in the infero-
conjuctival recession, adjustable suture surgery, nasal quadrant which terminates just temporal
repeat surgery and muscle transpositions. to the semilunar fold.
 418 Theory and Practice of Squint and Orthoptics

• For surgery on the lateral rectus, the assistant Closure of the conjunctival incision
should hold the limbus in the inferotemporal Following the completion of extraocular muscle
quadrant and rotate the eyeball up and in, and surgery, the conjunctival wound should be
the incision made in the inferotemporal closed by any of the following techniques
quadrant should extend temporally from an depending upon the type of incision given:
imaginary line dropped into the cul-de-sac 1. Closure of limbal incision is accomplished by
that divides the lateral and middle third of the interrupted sutures (Fig. 15.5J).
cornea. 2. Closure of over the muscle conjunctival incision
• For inferior rectus surgery, incision is made in can be carried out either with running sutures
the inferior cul-de-sac with its centre at or with the interrupted sutures.
6 o'clock position. 3. Swan closure technique comprises suturing of
Tenon's capsule and conjunctiva independently
• For superior rectus surgery, incision is made in
in two layers. Usually Tenon's capsule is closed
the superior cul-de-sac with its centre at
with vertical placed 6-0 Vicryl sutures and
12 o'clock position.
conjunctiva is closed with horizontally placed
• For surgery on inferior oblique muscle, incision 6-0 silk sutures.
is made in the inferotemporal quadrant 4. Horizontal closure following a vertical incision
(inferior cul-de-sac). by interrupted or running suture is preferred
• For surgery on the superior oblique muscle, an by a few surgeons.
incision is made in superior cul-de-sac, 5. Bare sclera closure technique is indicated in cases
situated just temporal to the superior rectus in which previous surgery or injury has led to
muscle. severe fibrosis of the conjunctiva and episcleral
2. The Tenon's capsule and intermuscular septa tissues, producing a mechanical limitation of
are cut down to bare sclera. motion. Making of the limbal incision is
mandatory, when bare sclera closure technique
3. The muscle concerned is hooked from the bare
is planned. In this technique, the conjunctiva is
sclera exposed, freed from the various fascial
attached directly to the sclera by interrupted
connections and the muscle surgery is
sutures, leaving a bare scleral area between the
performed as per requirement.
conjunctival margin and the limbus. The area
4. In the end of surgery, the conjunctiva is heals by the gradual proliferation of conjunctival
reposited back into the fonix by a gentle epithelium.
massage. Usually, no suture is required to close
Note. It is important to emphasize that the
unless an exposed, gaping wound is visible
conjunctiva and its closure play an important
between the palpebral fissure. role in the surgical results. It is mandatory that,
following the closure of the conjunctiva, forced
Advantages ductions be carried out to make sure that the
1. Often no sutures are required for closure. conjunctival closure does not provide any
2. There are no visible conjunctival or Tenon's mechanical limitation.
scars.
3. This approach is useful not only for RECESSION OF RECTUS MUSCLES
horizontal rectus surgery but also for RECESSION OF MEDIAL RECTUS
procedures on the vertical recti and obliques.
After the muscle has been exposed by any of
the above described techniques (most surgeons
Disadvantages are preferring von Noorden's limbal approach),
1. Technique is bit difficult as compared to the the salient points of the technique of medial
limbal approach. rectus recession are as follows:
2. The exposure is depended upon the technique 1. Exposure of the muscle (Fig. 15.5A to E). It is
of assistance. especially important on the medial rectus to free
 Principles of Non-Surgical and Surgical Management of Strabismus 419

the check ligament, since failure to do so may the medial rectus muscle should not be
result in retraction of the caruncle post- recessed more than 5.5 mm. In the presence
operatively. It is also important to ensure of a paralytic lateral rectus, the medial rectus
whether the muscle is completely engaged or can be recessed up to 6.5 mm. However, recent
not. It is done by passing a second muscle hook reports suggest that MR can be recessed up
repeatedly under the insertion from above or to 7–8 mm without producing significant
from below. limitation of adduction postoperatively.
2. Passing of sutures through muscle (Fig. 15.5F). • Minimal limits. It has been recommended that
After the muscle has been meticulously minimal limit for MR recession is 3 mm,
separated, the position of the insertion is noted because recession of MR less than 3 mm has
and two single armed sutures (6-0 Vicryl with hardly any effect.
spatulated needle) are placed and locked (by
whip suture technique) at both margins of the RECESSION OF LATERAL RECTUS
muscle close to the insertion. After the locking • The technique of recession of lateral rectus is
stiches, the suture ends are tagged with bulldog essentially the same as that of medial rectus
clamps for identification. recession.
3. Cutting the muscle (Fig. 15.5G). Traction to • It is important to remember that owing to close
the muscle hook and sutures is applied and the proximity of the inferior oblique insertion to the
muscle is cut free of the insertion from the sclera inferior border of lateral rectus muscle, the
using curved Stevens tenotomy-scissors. former is commonly hooked and sometimes
Bleeding from the cut insertion site is controlled even along with the latter dissected and inserted.
by applying pressure or using wet field cautery. Therefore, an extra care is required to prevent
4. Securing of muscle at the new insertion site this complication. It is recommended that
on the sclera (Fig. 15.5H). Eyeball is stabilized by preferably lateral rectus should be engaged/
grasping the stump of the insertion remaining on hooked from the superior border side.
the sclera with a forceps and the new insertion Limits of lateral rectus recession
(depending upon the predetermined amount of • Maximal limits. Under normal circumstances,
recession) is measured and marked with the help lateral rectus should not be recessed more
of a caliper. The needles are then passed through than 8–10 mm. However, in patients with a
the sclera parallel to the limbus at the previously large degree exotropia in blind eye, the
marked distance. It is important to note that amount of LR recession may be more, but in
needle should be visible at all times while passing such cases usually there is slight limitation of
through the scleral lamellae, so as to avoid abduction postoperatively.
perforation. The muscle is then secured to sclera • Minimal limits. Minimal amount of lateral rectus
by tying the sutures (Fig. 15.5I). It is important recession recommended is 5 mm, since LR
that the knots be tied securely and to the sclera recession less than this has little effect per se.
directly, rather than on top of the muscle.
Preferably the muscle should be spread out to its RECESSION OF SUPERIOR RECTUS
normal width. Following the completion of the • The technique of recession of superior rectus
reattachment of the muscle, the amount of is essentially the same as that described for
recession actually carried out should again be medial rectus.
measured with the help of caliper.
• It is important to remember that accidently
5. Closure of the conjunctival incision should superior oblique tendon may be hooked while
be done depending upon the type of incision engaging the superior rectus muscle. Care
used (see page 416, Fig. 15.5J). should be taken to avoid it.
Limits of medial rectus recession
• Maximal limits. Most of the previous reports RECESSION OF INFERIOR RECTUS
and conservative surgeons recommend that • Essentially, the technique is similar to
with a normally acting lateral rectus muscle, recession of any other rectus muscle.
 420 Theory and Practice of Squint and Orthoptics

However, one should dissect carefully the

intermuscular septum and all fascial
connections between the inferior rectus
muscle and Lockwood's ligament as far
posteriorly as possible.
• While dissecting the fascial connections of
inferior rectus muscle, one should take care
to avoid injury to the nerve to inferior oblique
muscle, which enters this muscle just as it
passes the lateral border of the inferior rectus
muscle, 12 mm posterior to the inferior rectus
insertion.

HANG-BACK TECHNIQUE OF RECTUS MUSCLE

RECESSION
Hang-back (HB) technique of rectus muscle
recession is a type of non-adjustable suspension
recession (SR) technique. It is reported to be
equally effective and comparatively safer than
the conventional recession technique. It is
performed for up to 7 mm of recessions.

Surgical steps
1. Initial steps up to isolation of the muscle are
similar to conventional recession (see page 351
and Fig. 15.5A to E).
2. Passing of suture through the muscle. A double
armed 6-0 Vicryl suture is passed through the
muscle 1.0 to 1.5 mm from the insertion. Locked
bites are taken at each end of the muscle (Fig. 15.6A).
3. Disinsertion of the muscle is then carried out
with the help of tenotomy scissors.
4. Placing of sutures on the sclera for hang-back
recession as described by Potter and Nelson are
as follows:
• The suture needles are passed through angled,
5 mm scleral tunnels that are 6 mm apart from
each other and begin where the posterior side
of the insertion merges with flat scleral fibres
and emerge anterior to the insertion as close
to each other as possible (Fig. 15.6.B).
• The sutures are then pulled forward until the
cut end of the muscle rests firmly against the
posterior aspect of the insertion (Fig. 15.6C).
• The calipers are opened equal to the amount
of recession and aligned perpendicular to the
suture arms. In this position, then the sutures
are clamped with a locking needle holder Fig. 15.6 Hang-back technique of rectus muscle recession
placed just inside the proximal caliper tip (for explanation, see text).
 Principles of Non-Surgical and Surgical Management of Strabismus 421

(Fig. 15.6C). The surgeon must observe metal- 5 mm). As in a conventional recession, the
to-metal (caliper tip to needle holder tip) calipers are used to mark the entrance points
contact during this step to pervent asymmetric for the scleral tunnels posterior to the
suture lengths and unequal recession of the insertion. The entry points are marked 6 mm
ends of the muscle. Four overhand knots are apart (Fig. 15.7A).
tied and trimmed while the needle holder • The sutures needles are then passed from the
clamps the sutures firmly. entry point through angled, 3 mm scleral
• The needle holder is then removed and the tunnels that emerge next to one another
muscle retracts until the knot rests against the (Fig. 15.7A).
insertion (Fig. 15.6D). The eye is rotated in the
• Further steps to produce another 5 mm of
opposite direction with the toothed forceps to
suspension recession are similar to hang-back
facilitate the posterior movement of the muscle.
technique (Fig. 15.7B).
• Finally, the caliper is placed from the insertion
to the anterior end edge of the muscle to verify • In the end, the calipers are reset for total
its position (Fig. 15.6D). amount of recession (10 mm or above) and the
total recession from the original insertion is
5. Conjunctival closure is carried out with 6-0
Vicryl sutures as usual (Fig. 15.5J). varified (Fig. 15.7C).
3. Conjunctival closure is performed as usual.
HEMIHANG-BACK TECHNIQUE OF RECTUS
MUSCLE RECESSION Advantages of hang-back and hemihang-back
Hemihang-back (HHB) technique of rectus techniques
muscle recession is a type of non-adjustable 1. The risk of scleral perforation is reduced in
suspension recession (SR) in which the muscle both procedures because the surgeon works
is reattached and suspended from the sclera from a comparatively more anterior site than
posterior to the original insertion. It is performed would be selected in a conventional recession.
for more than 7 mm of recession.
2. Advantage of HHB is that it minimizes
Surgical technique awkward needle placement in the sclera.
1. Initial steps up to disinsertion of the muscle are 3. These techniques avoid excessive manipulation
similar to hang-back technique. of the eye which may lead to decreased
2. Placing of sutures on the sclera in hemihang-back postoperative inflammation and more effective
technique (Potter and Nelson). muscle weakening.
• The calipers are first set on one-half of the total 4. Post-equatorial exposure which risks injury
proposed amount of recession (e.g. for 10 mm to the vortex veins is not required in these
recession, initial caliper setting should be techniques.

Fig. 15.7 Hemihang-back technique of rectus muscle recession (for explanation, see text).
 422 Theory and Practice of Squint and Orthoptics

5. Chances of postoperative, induced cyclo- (Fig. 15.8F). After pulling the cut muscle end up
vertical deviations, are less with suspension to the old insertion, each suture is tied with a
recession techniques. tripple knot (Fig. 15.8G).
Alternative technique of securing the muscle
RESECTION OF RECTUS MUSCLES
(technique-II). Some surgeons, after marking the
RESECTION OF MEDIAL RECTUS resection site, apply the muscle clamp just
anterior to it (Fig. 15.9A) and then disinsert the
Steps of the resection of the medial rectus muscle
muscle (Fig. 15.9B and C) followed by placing
are as follows:
2-double-armed sutures through the insertion
1. Conjunctival incision is similar to recession site (Fig. 15.9D). The sutures are then carried
technique (see page 415). through the muscle which is lifted by clamp
2. Exposure of muscle. Basic technique is same (Fig. 15.9D). The assistant then pulls the muscle
as described for recession procedure (see page 350). with the clamp towards the old insertion site
However, while exposing the muscle for and the surgeon ties each suture with a tripple
resection, the muscle should be freed only up knot (Fig. 15.9E). The muscle is then crushed just
to requirement for resection, keeping the fascial anterior to the sutures with the help of a
attachments intact as much as possible (c.f. haemostat (Fig. 15.9F) and then cut using
recession). tenotomy scissors (Fig. 15.9G).
3. Passing of sutures through the muscle. After 6. Spring-back balance test of Jampolsky should
the muscle has been meticulously separated, it preferably be carried out after the completion
is slightly stretched using two muscle hooks– of resection to prevent overcorrection. To
one just under the insertion and another about perform this test, eyeball is grasped at the limbus
10 mm away from it. The amount of muscle to with two fixation forceps (similar to forced
be resected is measured and marked with the duction test) and is rocked back and forth several
caliper (Fig. 15.8A). Two double armed 6-0 times in the desired plane and then quickly
Vicryl sutures are passed at the marked site and released noting the velocity of spring back and
are locked using the whip suture technique final position of the eyeball. Following
(Fig. 15.8B). A Jamson resection clamp or any observations may be made:
fine hemostat is applied just anterior to the • If, for example, medial rectus has been over
suture (Fig. 15.8C). corrected, the eye ball will come to rest in a
position of adduction. Under such circum-
4. Cutting of the muscle. The muscle hook near
stances, the surgeon should recess the just
the insertion is stretched and the muscle is
resected muscle to avoid overcorrection.
disinserted using Westcott or Stevens tenotomy
scissors (Fig. 15.8D). Then the muscle tissue • If the position of the eyeball after the spring-
anterior to the clamp/hemostat is excised with back balance test suggests the need for
the help of tenotomy scissors (Fig. 15.8E) (some additional strengthening, then the resected
surgeons do not apply any clamp and cut the muscle should be advanced 2 mm towards the
muscle tissue 1 mm anterior to the sutures after limbus.
crushing with a hemostat). 7. Closure of the conjunctiva is performed as
5. Securing of muscle to the insertion site usual (Fig. 15.5J).
(technique-I). After the requisite amount of
muscle has been resected, the four needles of RESECTION OF OTHER RECTUS MUSCLES
the two double-armed sutures are passed • The technique of resecting other rectus
through the superficial scleral lamellae just muscles is essentially the same as described
below the original insertion stump in a vertical for the medial rectus.
fashion; one needle of each suture being • Special points to be taken care for during each
placed close to the centre of the insertion site rectus muscle are same as described for the
and the other through the corresponding end recession technique.
 Principles of Non-Surgical and Surgical Management of Strabismus 423

Fig. 15.8 Surgical technique I of medial rectus resection (for explanation, see text).
424 Theory and Practice of Squint and Orthoptics

Fig. 15.9 Surgical technique II of medial rectus resection (for explanation, see text).
 Principles of Non-Surgical and Surgical Management of Strabismus 425

Limits of rectus muscle resection in mm anaesthesia' or 'awake strabismus surgery', post-

Maximal Minimal operative adjustable (two stage) surgery and
Medial rectus 8–10 4
'operation/reoperation technique'. Out of these,
Lateral rectus 12–14 4– 5 'postoperative adjustable (two-stage) suture
Superior rectus 5–6 2–3 technique' is most popular. Therefore, only this
Inferior Rectus 5–6 2–3 technique will be discussed here.

ADJUSTABLE SUTURES IN Steps of postoperative adjustable suture

technique with recession
RECTUS MUSCLE SURGERY
Keeping in view the unpredictable results of 1. Initial surgical steps up to meticulous exposure
strabismus surgery, the adjustable suture of the muscle are same as in usual recession
technique which permits alteration in the results procedure (see page 415).
in the immediate postoperative period, is 2. Passing of suture in the muscle. A double-armed
becoming popular. 6-0 Vicryl suture is passed and tied through the
centre of tendon and then passed and locked
Indications through its upper and lower edges, near the
Should adjustable suture surgery be performed insertion (Fig. 15.10A).
in every case of strabismus is controversial; no 3. Cutting of the muscle. The muscle is severed
convincing data are available to show that long- near its insertion with tenotomy scissors.
term results with this technique are superior to 4. Placing of sutures in the sclera. In this technique,
conventional methods. However, adjustable instead of placing sutures in the sclera at a
surgery is useful in conditions where the results measured distance away from the original
of strabismus surgery are comparatively less insertion (as done in usual recession), the sutures
predictable, as below: are passed directly through the insertional
• Patients with long-standing deviation. stump. To facilitate postoperative suture
• Acquired strabismus with fusional potential. adjustment, the scleral tunnel created by the
• Patients with paralytic squint requiring large suture is widened by moving the sutures back
recession and resection. and forth several times. The muscle is allowed
• Patients with restrictive strabismus in which to recede a desired amount of recession,
accurate assessment of muscle function is measured by caliper (Fig. 15.10B), and the
difficult. sutures are tied first with a single knot, and then
• Re-operation. with a bow knot (Fig.15.10C). The end of the
• Strabismus due to long-standing aphakia with suture that will open the knot is cut long for later
good visual acuity, bothered by diplopia. identification.
• All cases of strabismus where diplopia is the 5. Closure of conjunctiva. The knot is kept exposed
prime problem and thus needs precise by suturing the conjunctival flap on either side
postoperative results. of the insertion stump using bare sclera
technique of closure (Fig. 15.10C).
Prerequisites 6. Placing of traction suture. Lastly, a traction
Adjustment suture surgery needs an alert and suture (5-0 Mersilk) may be inserted through the
attentive patient who will co-operate for superficial sclera between the limbus and the
measurement of the deviation in required old tendon insertion. This will act as a 'globe-
position. In general, adjustable suture surgery control handle' and facilitate exposure by
can be undertaken in adults and children above rotating the globe during suture adjustment. It
11–12 years of age who will be able to co-operate is important to note that at the end of the
for the procedure. surgery, pad and bandage is done without using
ophthalmic ointment, since it can make muscle
Technique suture manipulation more difficult.
Various adjustable strabismus surgery techniques 7. Postoperative adjustment. One has to wait for
include, 'pull over (stay) sutures', 'topical the adjustment of suture till the anaesthetic effect
426 Theory and Practice of Squint and Orthoptics

Fig. 15.10 Surgical steps of recession using two-stage postoperative adjustable sutures technique.
 Principles of Non-Surgical and Surgical Management of Strabismus 427

has worn off. This is variable in the individual placed 8 mm from the insertion in a manner
cases. Most surgeons prefer to do it on the similar to that for recession.
second day. The procedure is done while the 3. Resection of muscle. 8 mm of the muscle
patient sits on a chair with back rest as follows: segment is then resected as done for usual
• Assessment of deviation. On second day, after resection.
removal of the patch, the amount of deviation 4. Placing of sutures in the sclera. The two
is assessed by prism cover test. In cases of needles of the double-armed suture are then
refractive error, this is done with optical passed under the original insertion stump
correction. In case of diplopia, Maddox rod similar to adjustable suture recession. The bow
is used appropriately. Two or three knot is then tied after allowing the muscle finally
measurements should be taken with 20–30 to rest 3 mm from the original insertion, thus
minutes interval before adjustments. effectively resulting in a 5 mm resection.
• Topical anaesthesia and separation of lids. Once 5. Rest all other steps up to final adjustment
the deviation is stabilized, topical anaesthetic are similar to the adjustable suture surgery for
drops are instilled twice or thrice into the recession.
conjunctival sac. The lids are separated by
using a speculum. The bow knot is loosened, Disadvantages of adjustable suture surgery
by using a needle holder and tying forceps and 1. Increases the total operation time during
is adjusted accordingly (Fig. 15.10D). primary surgery.
• Suture adjustment. In case of overcorrection, 2. Requires an additional procedure.
the muscle is pulled forward by the sutures 3. A vaso vagal attack can occur while pulling
while the globe is fixed with help of a forceps on the muscle.
applied at the insertion stump (Fig. 15.10E). 4. During adjustment, suture can break causing
In case of undercorrection, the muscle is a slipped muscle.
pushed backward. To do this, globe is pulled
with the help of traction suture or a forceps Contraindications of adjustable suture surgery
applied at the insertion stump in the opposite 1. Children and uncooperative adult patients.
direction, e.g. temporally in case of medial 2. Variable angle of deviation like in inter-
rectus recession (Fig. 15.10F) and the patient mittent exotropia, where the patient tries to
is asked to look in the direction of field of control the deviation.
action of the recessed muscle. This manoeuvre
3. Surgery involving angulation or displace-
allows the muscle to slide further posteriorly.
ment of the muscle.
Practically, it is easy to pull the muscle
forward rather than pushing it backward. For 4. Surgery on oblique muscles.
this reason, most of the surgeons prefer to do
1 or 2 mm more recession than the required MARGINAL MYOTOMY
amount. After the adjustment is completed the Marginal myotomy refers to weakening of a
sutures are permanently tied with a tripple muscle by giving an incision partly through the
knot (Fig. 15.10G). muscle margin.

Steps for postoperative suture adjustment with Indications

resection The operation is most often used, when further
1. Initial steps up to exposure of the muscle are weakening of a maximally recessed muscle is
same as for usual surgery. desired. For example, a marginal myotomy of
2. Placing of sutures in the muscle. While using one of the recessed muscle can be done, if a
adjustable sutures for resection, the sutures are patient has been undercorrected following
placed approximately 3 mm posterior to the bilateral lateral rectus recession for exotropia
desired amount of resection. For example, if the or bilateral medial rectus recession for
muscle to be resected is 5 mm, the sutures are esotropia.
 428 Theory and Practice of Squint and Orthoptics

Technique
1. Initial steps up to exposure of the muscle are
similar to usual recession.
2. Marginal myotomy. After the muscle has been
exposed meticulously, it is held under tension
with two large hooks. Haemostasis is obtained
by briefly crushing the tissue to be cut with
mosquito haemostats. The inferior haemostat is
applied 3–4 mm behind the insertion and the
superior haemostat is applied 3–4 mm posterior
to this (Fig. 15.11A). Marginal myotomy is then
performed with the help of tenotomy scissors,
cutting about 70% of the width of the muscle in
the crushed area (Fig. 15.11B). It is important to
note that posterior incision should always be
made before the anterior one to prevent
distortion of the muscle. Otherwise the
distortion of the muscle will make it difficult to
gauge the length of second incision. Figure
15.11C depicts the muscle lengthening after
completion of myotomy.
3. Closure of the conjunctiva is done as usual
for muscle surgery (Fig. 15.5J).

FADEN OPERATION
Faden operation also known as posterior fixation
suture or retroequatorial myopexy is a
weakening procedure for the rectus muscles that
weakens the muscle only in its field of action by
decreasing the mechanical advantage of the
muscle acting on the globe.

Indications
This operation was first described by Cuppers
in Germany. The term Faden in German means
a suture or a sling. Indications of Faden Fig. 15.11 Surgical technique of marginal myotomy.
operation are as follows:
1. Dissociated vertical deviation. Suture is 4. Vertical strabismus present only in down
placed in the superior rectus with or without gaze. Sutures are placed on the inferior rectus
recession to treat vertical deviations. of the hypotropic eye.
2. Nystagmus blockage syndrome. Suture is 5. Duane's retraction syndrome type I. The pro-
placed on the medial rectus with its recession to cedure is used to treat upshoot and downshoot
treat the esotropia present in primary position. of the adducted eye.
3. Incomitant strabismus. In patients who are 6. Laterally incomitant exotropias may also be
orthotropic in primary position but have managed by the Faden operation.
diplopia in peripheral position of gaze sutures 7. Non-accommodative type of convergences
are placed on the appropriate muscle for the excess esotropias are also reported to be
desired weakening effect. corrected by this procedure.
 Principles of Non-Surgical and Surgical Management of Strabismus 429

8. Near esotropias with high AC/A ratio which and, (5) a mechanical restriction from reverse
is poorly controlled with hyperopic correction, leash effect.
bifocals and miotics may be successfully treated After this operation, the weakened (sutured)
with bimedial recession combined with Faden muscle will require more innervation for a
operation. particular movement. According to Hering's law
of equal innervation, the increased innervation
Surgical technique will also go to its yoke muscle. Thus indirectly
1. Initial steps up to exposure of the muscle are this operation also strengthens the yoke muscle
similar to those described for a usual recession in the field of action (Fig. 15.12F and G).
procedure. For an appropriate effect von Noorden has
2. Rotation of the globe. Application of recommended the distance of posterior fixation
posterior fixation suture requires an excessive for different muscles as below:
rotation of the globe in opposite direction. This Muscle Distance of Idieal
can be accomplished by applying additional posterior insertion distances
traction suture at the insertion site (Fig. 15.12A). Medial rectus 12–15 mm 14 mm
3. Placing of posterior sutures. Faden operation Lateral rectus 13–16 mm 16 mm
is performed with or without recession of the Superior rectus 11–16 mm 14 mm
muscle as per indication. When combined with Inferior rectus 11–12 mm 12 mm
recession, first the muscle is disinserted from the
original insertion. The posterior fixation sutures Advantages of Faden operation
(5–0 Mersiline non-absorbable) are placed 1. There is decreased likelihood of over-
through the superficial scleral lamellae 12–15 adduction, especially in cases of non-accommo-
mm behind the original insertion (Fig. 15.12A). dative convergence excess.
Then the recession procedure is completed as 2. The upshoots and downshoots associated
usual and the posterior fixation sutures are with extreme adduction seen in Duane's
passed through the muscle and tied (Fig. 15.12B). retraction syndrome are lessened.
4. Conjunctival closure is done in the usual 3. Postoperative forced duction following this
fashion (Fig. 15.5J). procedure is free.
4. Last but not the least, it also saves the ciliary
Mechanics of the operation blood vessels from damage associated with
recession/resection.
When a posterior fixation suture is placed, the
effective insertion of the muscle is shifted Problems associated with Faden operation
posteriorly. This decreases the muscle power in
1. The procedure needs a vigorous traction of
its field of action without affecting the position
the eyeball to facilitate suture application.
of the eyes in primary position. The weakening
Even after this, it is difficult to apply sutures.
of the muscle power in this procedure can be
2. Vortex vein may be injured.
understood as an artificial paresis resulting
3. Chances of globe perforation are more.
from: (1) reduction of arc of contact (Fig. 15.12C),
(2) shortening of the active length of the muscle 4. Results are variable. A weakening effect of
(Fig. 15.12C), (3) the power of the rectus muscle 0 to 10 prism dioptre in the field of action of
to rotate the eyeball depends upon the leverage muscle is reported. Thus, either under
existing between the centre of rotation, C and correction or over correction may occur.
the line of pull of the muscle at tangential point
INFERIOR OBLIQUE WEAKENING
T (Fig. 15.12D), after Faden suture the moment
PROCEDURES
arm M of the lever system is decreased and more
muscle force (3+) is now required to rotate the Indications
globe by the same amount (Fig. 15.12E), 1. Primary inferior oblique overaction. Bilateral
(4) probably some muscular damage and inferior oblique weakening is indicated for
decrease effectiveness of the muscle contraction bilateral overaction, even if asymmetrical.
 430 Theory and Practice of Squint and Orthoptics

Fig. 15.12 Surgical technique of Faden operation with recession of a rectus muscle. (A and B) and mechanics of this
procedure (C to G). (C) Normal arc of contact (R) and reduced arc of contact (R') after Faden operation. (D) Normal length
of moment arm (M) of the rectus muscle lever system; (E) Reduced length of moment arm (M') after Faden operation; (F) The
innervation required (1+) to adduct the fixating nonparetic right eye is insufficient to abduct the left eye of a patient with left
lateral rectus palsy; (G) A posterior fixation of the right medial rectus increases the innervational requirment (3+) to adduct
this eye. According to Hering's law of equal innervation, this increase innervation will also flow to the yoke muscle in the fellow
eye and thus will improve the abduction of the left paretic eye. [C: Centre of rotation; A: Normal anatomical insertion;
A: Anatomical insertion after Faden operation; T: Tangential point (physiological insertion); R: Normal arc of contact; R': Reduced arc of contact after Faden
operation; M: Normal length of moment arm; M': Reduced length of moment arm after Faden operation].
 Principles of Non-Surgical and Surgical Management of Strabismus 431

2. Secondary overaction of inferior oblique Advantages

following superior oblique palsy. Unilateral 1. Procedure is very simple and easy.
inferior oblique weakening is indicated. 2. There is no danger of placing sutures over
3. Double elevator palsy. Inferior oblique muscle the macular area (of recession).
weakening is indicated in the contralateral eye. 3. Bleeding is minimum (of myectomy).
4. Upshoots in Duane's retraction syndrome can
Disadvantages
be controlled by the inferior oblique muscle
weakening. Incidence of recurrence of overaction is very
high.
Weakening procedures
INFERIOR OBLIQUE RECESSION
1. Disinsertion, i.e. cutting the muscle from the
Surgical technique
globe near its insertion.
2. Myectomy involves the excision of a segment 1. Rotation of globe. Lids are separated with
of muscle belly. universal eye speculum. A 5-0 Mersiline suture
is placed through the fused conjunctiva and
3. Extirpation refers to almost complete removal
Tenon's capsule near the limbus in the infero-
of the muscle.
temporal quadrant and globe is elevated and
4. Recession involves disinsertion followed by adducted by applying traction on this suture
reattachment at the planned site. (Fig. 15.13A).
5. Recession with anterior transposition 2. Conjunctival incision. Either cul-de-sac
involves disinsertion and reattachment near incision (Fig. 15.13A) or limbal incision is made
the lateral end of the inferior rectus insertion. in the inferotemporal quadrant.
The most commonly used procedures are 3. Exposure of muscle. With meticulous
myectomy and recession. Some surgeons prefer dissection of the fascial connections, lateral
disinsertion and others recession with anterior rectus and inferior rectus muscles are identified
transposition. Each technique has its own and hooked away by the assistant. Anterior
inherent advantages and disadvantages. And border of the fleshly inferior oblique can then
each surgeon has his own reasons to prefer a be directly visualized (Fig. 15.13B). A muscle
particular technique. hook is then passed between the sclera and
inferior oblique to engage the muscle. Inferior
DISINSERTION oblique is dissected free of the intermuscular
septum and the fascia which joins it with the
Surgical technique
lateral rectus (Fig. 15.13C).
Disinsertion alone is carried out through the
conjunctival incision placed at the inferior 4. Disinsertion of the muscle (Fig. 15.13D). After
border of the lateral rectus (Fig. 15.13A and B). the muscle insertion is exposed meticulously, it
After conjunctival incision, the lateral rectus is is disinserted by tenotomy scissors and the
hooked first, then a second hook is passed to detached muscle is held by assistant with
engage the inferior oblique muscle near its serrated forceps.
insertion (Fig. 15.13C). With traction between the 5. Reinsertion of the muscle. Two 6-0 Vicryl
two hooks, good exposure is obtained and the sutures are passed at its detached end and locked
muscle is severed from the globe with tenotomy by the whip suture technique (Fig. 15.13E). For
scissors (Fig. 15.13C). The hook is repassed so reinsertion of the muscle to various points on the
as to make sure that all fibres of the inferior sclera depending the degree of weakness
oblique have been successfully disinserted from required, following techniques are in vogue:
the sclera. It is also helpful to make sure that the i. Park's technique. Park recommended that for
inferior oblique retracts into the inferior orbital a 10 mm recession of the inferior oblique, the
area and that it does not reattach to the point medial needle should be passed through the
from which it was severed. sclera at a point 2 mm lateral and 3 mm
432 Theory and Practice of Squint and Orthoptics

Fig. 15.13 Surgical technique of inferior oblique disinsertion and recession (for expalnation, see text).
 Principles of Non-Surgical and Surgical Management of Strabismus 433

posterior to the lateral aspect of insertion of (Fig. 15.14A) and the muscle segment in between
the inferior rectus muscle. The lateral needle the haemostats is excised with tenotomy scissors
of the suture is placed more laterally (Fig. 15.14B and C). It is important to observe
according to width of inferior oblique and that all of the muscle fibres have been isolated
about 1 mm more inferiorly and the suture and cut. The stumps are cauterized to prevent:
are tied securely (Fig. 15.13F). – Postoperative haemorrhage,
It has been reported that 10 mm Park's – Reattachment of the muscle to the sclera, or
recession is more effective in weakening – Reattachment of the two stumps to each other.
vertical functions of the muscle than 10 mm
Fink's recession (described below). 3. Conjunctival closure is done as usual.
ii. Fink's technique. The classical Fink's technique Advantages
utilizes the lower end of lateral rectus muscle
• Technically easier to perform.
as the landmark. For an 8 mm recession, the
muscle is inserted at a point 6 mm posterior • Results are as good as recession.
and 6 mm down to the inferior edge of the Disadvantages
lateral rectus muscle. A point 2 mm up or
2 mm down the above point gives a recession • Effect cannot be graduated.
of 6 mm and 10 mm, respectively. • Myectomy is a destructive procedure and once
done cannot be controlled.
iii. Elliot and Nankin's technique. This technique
provides anterior positioning of the inferior • Results may be unpredictable depending on
oblique along with its recession. In this where the residual muscle stump reattaches.
technique, whole muscle is fixed just
temporal to the inferior rectus muscle SUPERIOR OBLIQUE WEAKENING PROCEDURES
insertion. Indications
6. Closure of the conjunctiva is done in the end. 1. Unilateral weakening of the superior oblique
is not commonly performed except as part of
Advantages the treatment of:
Presently, recession with or without anterior – Brown's syndrome, and
positioning of the inferior oblique muscle is – Isolated inferior oblique muscle weakness.
preferred over disinsertion and myectomy 2. Bilateral weakening of superior oblique is
because of the following advantages: often performed with or without horizontal
• Recession can be graduated to produce muscle surgery for A-pattern deviations. This
predictable results. surgery is expected to cause an eso-shift of up
• It can be always be revisited and the surgery to 30–40 prism dioptres in downgaze, little
can be undone, underdone and overdone. change in primary position and almost no effect
in upgaze.
Disadvantages
Recession of inferior oblique is technically Weakening Procedures
difficult than the disinsertion and myectomy. 1. Tenotomy, i.e. cutting of the tendon
2. Split lengthening of tendon
INFERIOR OBLIQUE MYECTOMY
3. Recession
Surgical technique 4. Silicon expander
1. Initial steps up to exposure of the muscle are 5. Translational recession of Prieto-Diaz
similar to those for the recession (Fig. 15.14A 6. Posterior tenotomy of superior oblique (PTSO)
to C).
2. Myectomy. After meticulous exposures the SUPERIOR OBLIQUE TENOTOMY
inferior oblique muscle is clamped with two Superior oblique tenotomy can be performed by
haemostats applied approximately 8 mm apart either nasal approach or a temporal approach.
 434 Theory and Practice of Squint and Orthoptics

Recently, most surgeons are preferring temporal

approach.

Surgical technique for temporal approach of

superior oblique tenotomy
1. Fixation of globe. Lids are separated with
universal eye speculum and a 5-0 Mersiline
suture is passed through the conjunctiva and
episcleral tissue near the limbus at approximately
11 o'clock position (right eye) or 1 o'clock
position (left eye). The eyeball is rotated
downward and inward by applying traction on
these sutures (Fig. 15.15A).
2. Conjunctival incision. An 8 mm cul-de-sac
incision is made in the superotemporal quadrant
starting just temporal to superior rectus insertion.
3. Exposure of the superior oblique tendon is carried
out taking following steps meticulously:
• After undermining the conjunctiva, the
intermuscular septum (between superior
rectus and lateral rectus) is buttonholed and
sclera is exposed.
• Superior rectus muscle is isolated, and engaged
in the muscle hook.
• Assistant further depresses and adducts the
eyeball by pulling on the superior rectus
muscle insertion with the help of muscle hook.
As this is done, the insertion of the superior
oblique is pulled down towards the incision.
• At this juncture, the conjunctival incision is
stretched temporally and also posteriorly with
the help of two separate muscle hooks or lid
retractors (Fig. 15.15B).
• Thin and filmy glistening fibres of superior
oblique tendon are identified below the superior
rectus muscle after retracting it medially with
Desmarre's retractor. Then the superior oblique
tendon is engaged by a sweeping action of
the muscle hook. After the superior oblique
tendon is engaged, it takes substance and
appears thick.
4. Tenotomy. After the superior oblique tendon
is isolated on the muscle hook, a complete
transection of it is done with the help of
tenotomy scissors (Fig. 15.15C). It is recom-
meded that in bilateral tenotomy, the transection
site on each tendon should be at same distance
Fig. 15.14 Surgical technique of inferior oblique myectomy. from these insertion point to get symmetric
 Principles of Non-Surgical and Surgical Management of Strabismus 435

Surgical technique for nasal approach of

superior oblique tenotomy
1. Conjunctival incision. The cul-de-sac incision
is made through the conjunctiva and Tenon's
capsule in the superonasal quadrant between the
medial and superior rectus muscles, about
4–5 mm behind the limbus. The wound is gently
spread with the help of tenotomy scissors
(Fig. 15.16A).
2. Exposure of the tendon. First of all the superior
rectus muscle is identified and engaged with a
muscle hook. The eyeball is rotated downward
and outward by applying traction on this muscle
hook. The upper edge of the wound is retracted
with a Desmarre's retractor and the glistening
white band of the superior oblique tendon is
identified (Fig. 15.16B).
3. Tenotomy. The superior oblique tendon is
engaged with a muscle hook and pulled forward
and is dissected meticulously from the
surrounding tissues. After the tendon is isolated
clearly, it is cut with the help of scissors
(Fig. 15.16C).
4. Conjunctiva is closed as usual.

Superior oblique tenotomy, temporal versus

nasal appraoch
1. Nasal approach for performing superior oblique
tenotomy without striping the intermuscular
septum is considered better for treating Brown's
syndrome. Since by this technique, the superior
oblique is weakened but some action is retained
because of undisturbed attachment of the
intermuscular septum to the tendon. It is
reported that this retained action of superior
oblique avoids the postoperative complication
of torsional diplopia.
2. Temporal approach is preferred in all other
indications, especially when bilateral tenotomy
is to be performed, because of the following
advantages:
• Exact measurement of the site of transaction
of the tendon can be obtained. This allows
Fig. 15.15 Surgical technique of temporal approach for
superior oblique tenotomy. bilateral symmetric results.
• Complications like blepharoptosis and
results. Such a measurement is possible with the superior rectus weakness known with nasal
temporal approach but not with the nasal approach are almost eliminated by temporal
approach. approach.
 436 Theory and Practice of Squint and Orthoptics

• Temporal approach is technically easier than

the nasal approach. However, injury to the
superior temporal vortex vein is obviously a
potential hazard of the temporal approach.

TECHNIQUES OF OTHER WEAKENING

PROCEDURES FOR SUPERIOR OBLIQUE
1. In general, temporal approach is preferred over
the nasal approach for superior oblique
weakening procedures.
2. Initial steps up to the exposure of the tendon
are similar to those described for superior
oblique tenotomy.
3. Final steps for different weakening procedures
are as follows:
i. In recession, the superior oblique tendon is
reinserted near the nasal border of superior
rectus muscle, about 4 mm posterior to its
insertion.
ii. In anteropositioning, the superior oblique
tendon is further anteriorized as compared
to recession.
iii. In translational recession, the anterior point of
superior oblique reinsertion is 4 mm nasal
from the superior rectus and 12 mm posterior
from the limbus. The advantage of the
translation recession is that it prevents the
limitation of depression in abduction which
occurs with recession or anteropositioning.
iv. In superior oblique expander technique some
surgeons insert 2 or 2.5 mm silicon tube
(or retinal band no. 240) in between the cut
ends of the superior oblique tendon 3 mm
nasal to the nasal border of superior rectus
muscle.
v. Posterior tenotomy of superior oblique (PTSO).
This operation is based on the observation
that posterior fibres of superior oblique
account for its vertical action. So tenotomy
of the posterior fibres of the superior oblique
is performed for a selective weakening of its
depressor action.

SUPERIOR OBLIQUE STRENGTHENING

PROCEDURES
Fig. 15.16 Surgical technique of nasal approach for
superior oblique tenotomy. SUPERIOR OBLIQUE TUCK
• Occurrence of transient Brown's syndrome has Indications
been reported following nasal but not with 1. In superior oblique paresis, the tucking
temporal approach. procedure helps in enhancing effect.
 Principles of Non-Surgical and Surgical Management of Strabismus 437

Fig. 15.17 Surgical technique of superior oblique tuck.

2. In DVD, superior oblique tucking with or of the original plane of the tendon by an
without inferior oblique weakening has been additional suture. However, an inadvertent
advocated. anterior pull on the tendon during this
manoeuver may decrease the vertical effect of
Surgical technique
the operation and cause pseudo-Brown's
1. Initial steps up to exposure of the superior
syndrome. Therefore, many surgeons prefer not
oblique tendon are similar to those described
to fasten the tucked portion to the sclera
for temporal approach for superior oblique
(Fig. 15.17D).
tenotomy (see page 483 and Fig. 15.15A and B).
3. Forced duction test is performed in the end to
2. Tucking procedure. After isolating , the tendon
determine the degree of restriction when
is transferred from the muscle hook to a Burch
elevating the adducted eye. The tuck is
tendon tucker or its von Noorden modification
considered adequate when mild elastic
(Fig. 15.17A). The tendon is then lifted upward
restriction is felt. If a severe restriction is
and pulled taut by turning the screw of the
encountered, the tucking should be undone
handle of the tendon tucker (Fig. 15.17B). After
followed by a lesser amount of tucking.
a sufficient (12–14 mm) tuck has been created
4. Conjunctival closure is done with one or two
on the tucker, two 5-0 Mersiline sutures are
7-0 Vicryl sutures.
placed near the base of tuck (Fig. 15.17C). The
sutures are secured and the tucker removed. Note. A transient postoperative limitation of
Previously most of the surgeons used to secure elevation of adducted eye (psuedo-Brown's
the tip of the tuck to the sclera in the direction syndrome) is a common phenomenon after
 438 Theory and Practice of Squint and Orthoptics

superior oblique tucking. Rarely a permanent

Brown's syndrome may also occur.

HARADA-ITO PROCEDURE
The Harada-Ito procedure is a selective
strengthening procedure for anterior fibres of
the superior oblique muscle. This procedure
essentially consists of anterior and lateral
displacement of the anterior fibres of the muscle
resulting in an advancement of its insertion
around the equator by several millimetres. This
operation is based on the idea that the anterior
fibres of the tendon are selectively concerned
with the torsional action of the superior oblique
muscle and that strengthening of these fibres
will enhance the incyclotropic effect thereby
correcting the excyclotropia.
Indications
This procedure has been reported to be useful
in patients with bilateral or unilateral superior
oblique palsy for correcting an excyclotropia of
10° or more but without significant primary
position vertical deviation.
Surgical technique
1. Initial steps up to exposure of the superior
oblique tendon are same as described for
superior oblique tenotomy (Fig. 15.15A
and B).
2. Anterior and lateral displacement of the anterior
fibres of superior oblique tendon is carried out, after
meticulous isolation of the tendon, by any of the
following techniques:
i. Conventional Harada-Ito technique. A 5-0
Mersiline (non-absorable) suture single-
armed is passed through the anterior half of
the tendon fibres and secured firmly with a
tripple knot (Fig. 15.18A). The needle is then
passed through the sclera at a point located
3 mm temporal and anterior to the insertion
(Fig. 15.18B). At this juncture, secure tying
of the suture over its scleral fixation will pull
the anterior fibres of the tendon anteriorly Fig. 15.18 Surgical technique of Harada-Ito procedure
(for explanation, see text).
and laterally.
ii. Fells modification over Harada-Ito technique. A anterior half of the insertion. The anterior half
visual estimate of half the width of superior of the tendon is then disinserted and
oblique tendon is made and its insertion is reattached to the sclera at a point where the
split parallel to its tendinous fibres. A single continuation of the lines of insertion of the
6-0 Vicryl suture is placed at the edge of the superior and lateral recti meet. A second
 Principles of Non-Surgical and Surgical Management of Strabismus 439

single-armed 6-0 Vicryl suture is used to • Correction of cyclotropia, and

secure the posterior point of the splitted and • Correction of compensatory head tilt to one
anteriorly transported anterior half of the shoulder in patients with a nystagmus having
tendon approximately 6 mm directly behind null zone in tertiary gaze position.
this point (Fig. 15.18C). 2. The direction in which the vertical and
iii. Harada-Ito procedure with adjustable suturing horizontal rectus muscles be transposed to cause
technique. Some surgeons have suggested the incycloduction or excycloduction of each eye is
use of adjustable sutures after splitting the as follows:
tendon as in Fells' modification. However, • To cause incyclodeviation, the superior rectus
not much advantages are associated with this should be shifted temporally and inferior
modification. rectus nasally (Fig. 15.19).
3. Conjunctival closure is performed with one or • To produce excyclodeviation, superior rectus
two of 7-0 Vicryl sutures. should be shifted nasally and inferior rectus
temporally.
MUSCLE TRANSPOSITION PROCEDURES • To produce incyclodeviation, the medial
Muscle transposition procedures essentially rectus should be shifted upward and lateral
involve moving the extraocular muscles out of rectus downward (Fig.15.20).
their original planes of action. Such procedures • To cause excyclodeviation the medial rectus
are generally reserved for treatment of paralytic should be shifted downward and lateral rectus
strabismus. The usual situations in which these upwards.
procedures are used include paralysis of cranial
3. Surgical technique of these procedures is
nerves III and VI as well as double elevator
similar to recession and resection procedure for
palsy. The muscle transposition procedures are these muscles, except that muscles are rein-
also useful in A- and V-patterns, cyclo- serted by shifting the insertion by one-half or
deviations and small vertical and horizontal one full muscle thickness, as per requirement.
deviations. A few commonly performed muscle Even the shifted reinsertion should be parallel
transposition procedures are described here. to limbus.
MUSCLE TRANSPOSITION PROCEDURES IN
TREATMENT OF A- AND V-PATTERNS
1. Vertical transposition of the horizontal rectus
muscles and horizontal transposition of the
vertical rectus muscles has been recommended
for correcting A- and V-patterns.
2. Surgical technique of these procedures is
similar to recession and resection procedures for
rectus muscles except that muscles are
reinserted by shifting the insertion between half
or one full muscle width as per requirement.
Important point to be noted is that even after
shifting, the muscle should be reinserted parallel
to the limbus.

MUSCLE TRANSPOSITION PROCEDURES FOR

TREATMENT OF CYCLODEVIATIONS
1. Horizontal transposition of vertical rectus Fig. 15.19 Temporal transposition of superior rectus tendon
muscles and vertical transposition of horizontal and nasal transposition of inferior rectus tendon to produce
rectus muscles has been recommended for: incyclodeviation.
 440 Theory and Practice of Squint and Orthoptics

Surgical technique
1. Conjunctival incision consists of a superior or
inferior limbal peritomy of approximately 210°
depending upon the indication.
2. Exposure of the muscles. The medial and lateral
recti and either superior or inferior rectus, are
exposed as described in recession for rectus
muscles.
3. Disinsertion of horizontal recti. After meti-
culous isolation, as in recession procedure, 6-0
Vicryl sutures are passed near the insertion of
medial and lateral recti and the muscles are
disinserted from the globe.
Fig. 15.20 Upward transposition of medial rectus and
downward transposition of lateral rectus to produce 4. Transposition of the horizontal recti. A
incyclodeviation. Desmarre's retractor is used to hold the
conjunctiva out of the surgical field and the
medial and lateral recti are reattached to the
MUSCLE TRANSPOSITION PROCEDURES FOR
PARALYTIC SQUINT globe at each corner of the insertion of either
superior rectus (in patients with double elevator
• It has been reported that the muscle trans- palsy (Fig. 15.21) or inferior rectus (in patients
position procedures are useful in paralytic with double depressor palsy).
squint by their mechanical effect and not by
any innervational adjustment. 5. Conjunctival closure is done in the end of
surgery.
• The muscle transpositions are useful in
paralytic squint, only if there is no restriction
to passive movements of the eyeball in the Jensen's procedure
paretic field of gaze. Therefore, if forced Indication
duction test reveals any restriction, it should Jensen's procedure is indicated in patients with
be removed first by a maximal recession of complete paralysis of lateral rectus muscle. It
the contractured antagonist of the paretic consists of transposition of half thickness
muscle with or without conjunctival recession muscle of superior and inferior recti to the lateral
depending upon the need. rectus at the level of equator.
• The commonly performed muscle trans-
position procedures for paralytic squint are
as follows:
– Knapp's procedure
– Hummelsheim procedure
– Jensen's procedure
– Transposition of superior oblique tendon
– Transposition of lateral rectus muscle

Knapp's procedure
Indication
Knapp procedure consists of transposition of the
insertion of medial and lateral recti to that of
superior rectus (in patients with double elevator
palsy) or inferior rectus (in patients with double
depressor paralysis). Fig. 15.21 Knapp's procedure for double elevator palsy.
 Principles of Non-Surgical and Surgical Management of Strabismus 441

Surgical technique medial rectus has been eliminated by a previous

1. Conjunctival incision consists of 180° peritomy or simultaneous medial rectus recession.
performed at the temporal half of the limbus. Surgical technique
2. Exposure of the muscles. The lateral, superior Hummelsheim procedure basically consists of
and inferior recti are meticulously exposed total transplant of the superior and inferior
beyond the equator as in recession procedure. rectus muscles to the insertion of the lateral
3. Transposition of the muscles. The tendons of rectus muscle (Fig. 15.23). The surgery is
superior, lateral and inferior rectus muscles are performed through a 180° temporal peritomy
isolated and split posteriorly for approximately and the technique is essentially similar to
8 mm with the help of a muscle hook. Then the Knapp's procedure.
temporal halves of the superior and inferior recti
are joined with the corresponding superior and Transposition of superior oblique tendon
inferior halves of the lateral rectus over the Indication
equator using 5-0 Mersiline suture (Fig. 15.22). Transposition of superior oblique tendon to the
It is important to note that at least one branch of insertion of medial rectus muscle is indicated in
the anterior ciliary vessel should remain in the patients with third cranial nerve palsy. This
nasal segment of each of the vertical recti that procedure is always combined with a maximal
are not incorporated in the muscle union. recession (10–12 mm) of the lateral rectus and
Further, one must ensure that sutures are tied resection (8–9 mm) of the medial rectus muscle.
loosely enough to prevent strangulation and
Surgical technique
necrosis of the muscle.
Superior oblique tendon transposition is
4. Recession of the medial rectus. It is imperative
performed by nasal approach. In this classical
that Jensen's procedure should always be
approach, superior oblique tendon is exposed
combined with a maximal recession of the
as described for nasal approach for tenotomy
ipsilateral medial rectus muscle with or without
(see page 435). A small closed mosquito
conjunctival recession to obtain successful
haemostat is then slid along the tendon until its
results. The recession is performed as usual. To
tip enters the pulley of the trochlea. The tendon
prevent anterior segment ischaemia, especially
is freed after fracturing the pulley by just
in older individuals, the recession of medial
opening the hemostat. The tendon is then
rectus and conjunctiva should be performed at
shortened by 10–12 mm and is sutured to the
least 4 to 5 months prior to the actual Jensen's
sclera near the superior border of the medial
procedure.
rectus muscle. Results of this procedure in
5. Conjunctival closure is done as usual.
improving the adduction are not always
Hummelsheim procedure encouraging.
Indication
This procedure is indicated in complete 6th
nerve palsy in which any contracture of the

Fig. 15.23 Surgical technique of Hummelsheim procedure

Fig. 15.22 Jensen's procedure for lateral rectus palsy. for lateral rectus muscle palsy.
 442 Theory and Practice of Squint and Orthoptics

Transposition of lateral rectus muscle 1. Cardiac arrest. Such a life-threatening

Some lateral rectus transposition procdures complication may occur during operation or
which have been recommended by some during recovery period. So a careful and
workers, for surgical management of third nerve meticulous monitoring is very important.
palsy include: Further, the ophthalmologist must be aware of
• Inferior nasal transposition of lateral rectus the updated means of managing such a
• Lateral rectus transposition to the nasal pole catastrophy and should be in a position to
of superior rectus muscle actively help the anaesthetist during the crisis
hours. A ready tray of emergency drugs filled
• Medial transposition of Y-split lateral rectus
in syringes is mandatory in the operation
muscle
theatre.
Periosteal fixation of extraocular muscles 2. Malignant hyperthermia. It is a comparatively
rare but again a life-threatening complication of
Medial rectus orbital wall (periosteal) fixation
anaesthesia.
Medial rectus orbital wall (periosteal) fixation
with the help of non-absorble 5-0 mersiline Prevention. In some families susceptibility to this
sutures through the retrocaruncular or skin condition is inherent and is apparently
approach (using DCR incision) has also been autosomal dominant. Therefore, careful history
described for surgical management of third and constant monitoring of the rectal temperature
nerve palsy. However, it shows limited especially in predisposed patients may help in
effectiveness. avoiding the crisis. Use of succinylcholine
should be better avoided, as chances of
Lateral rectus orbital (periosteal) fixation malignant hyperthermia are comparatively
Lateral rectus orbital (periosteal) fixation after more, when this drug is used.
disinserting the LR combined with 8–9 mm Management. The condition is diagnosed by the
resection of MR has been reported to show occurrence of tachycardia, arrhythmia, rapidly
reasonably good results in patients with third rising temperature, acidosis and shock. The first
nerve palsy. sign of malignant hyperthermia is muscular
rigidity and hyperthermia is a late sign. Once
COMPLICATIONS OF EXTRAOCULAR diagnosed, it should be treated energetically to
MUSCLE SURGERY prevent death by taking following measures:
Complications of extraocular muscle surgery are • Immediately stop anaesthesia
inevitable for any regularly operating stra- • Provide care and surface cooling
bismus surgeon. They vary in severity and • Correct metabolic acidosis
frequency. Complications of extraocular muscle • Intravenous use of the drug dantrolene
surgery, their prevention and management can sodium is quite effective incontrolling
be discussed under following headings: malignant hyperthermia.
• Complications of anaesthesia 3. Hepatic porphyria and suxamethonium
• Intraoperative complications sensitivity are other genetic disorders which can
• Postoperative complications cause serious complications during and after
general anaesthesia.
COMPLICATIONS OF ANAESTHESIA 4. Oculocardiorespiratory reflexes. The occur-
Undoubtedly, general anaesthesia has become rence of oculocardiorespiratory reflexes is
very safe in the recent days, however, it is never related to extraocular muscle manipulation and
entirely free from danger. Even deaths due to thus to be precise are operative complications.
anaesthesia to the tune of 2 per 10,000 have been However, since they occur more frequently with
reported during strabismic surgery. The general anaesthesia and are to be basically
potentially dangerous complications reported managed by the anaesthetist so they are
are as follows: discussed here. The oculocardiorespiratory
 Principles of Non-Surgical and Surgical Management of Strabismus 443

reflexes include oculocardiac reflex, oculo- perianaesthetic problems in general, family

respiratory reflex and oculodepressor reflex. history may sometimes be helpful in anticipating
it.
• Oculocardiac reflex (OCR) refers to slowing of
heart rate and/or disturbances in cardiac INTRAOPERATIVE COMPLICATIONS
rhythm resulting from manipulation of
1. Haemorrhage
extraocular muscles. It was first described by
Aschner in 1908 and since then an abundant • Mild surgical haemorrhage from subconjunctival
work has been done on various aspects of OCR. vessels is routine and is not preventable.
Its reported incidence varies from 32 to 90%. Rarely wet field cautery may be needed to
control bleeding from these vessels.
• Oculorespiratory reflex (ORR), also first
described by Aschner, refers to slowing of • A moderately excessive haemorrhage may occur,
respiration on extraocular muscle traction. if the muscle is cut inadvertently, which is
preventable if dissection is carried out
• Oculodepressor reflex (ODR) has recently been
meticulously. Compression for a while or wet
described by Khurana et al. to denote the
field cautery may be needed to control it.
significant fall in blood pressure observed
during extraocular muscle traction. One might • A profuse venous bleeding indicates vortex vein
expect the cardiac output and, therefore, arterial rupture. It is not an uncommon complication
blood pressure to fall in the presence of and is caused by careless dissection or
significant cardiac slowing due to oculocardiac anomalous location of vortex veins. The vortex
reflex (OCR). However, the significant vein, i.e. most easily ruptured is the inferior
hypotension observed by the authors even after temporal vortex veins. Rupture often occurs
abolition of OCR by vagotomy, atropine and during lateral rectus and inferior oblique
glycopyrronium have confirmed ODR to be surgery. The best way to handle this
distinct and independent of OCR. complication is that no attempt should be
made to try to clamp the vortex vein, since
• Prevention of OCR, ORR and ODR becomes clamping may result in further damage.
imperative during squint surgery since the Generally speaking, there are no postoperative
retrobulbar injection of 2% xylocaine blocks the complications from a tear in a vortex vein
afferent pathway of all three reflexes, its use has during surgery.
been recommended, even when the surgery is
performed under general anaesthesia. In 2. Lost muscle
addition, intravenous atropine or glycopyr- • Lost muscle is a serious complication of
ronium should also be used to block the efferent extraocular muscle surgery.
pathway of OCR. Further, controlled ventilation • Medial rectus is the most frequently lost
must be preferred to spontaneous breathing at muscle, because of its fewer attachments to
least before, after and during muscle traction. other muscles and ligaments. For instance,
inferior rectus is checked in its retraction into
5. Succinylcholine-induced apnoea. Succinyl-
the orbit by Lockwood's ligament, superior
choline-induced apnoea may occur in children
rectus by its attachment to levator and lateral
who are on miotics, echothiophate iodide. So it
rectus by its association with inferior oblique
is most important to not to schedule surgery
muscle.
until the miotics have been discontinued for a
• Lost muscle can occur intraoperatively or in
period of at least 2–3 weeks, thus avoiding this
immediate postoperative period.
problem. However, this problem can also occur
due to primary cholinesterase deficiency which • Lost muscle occurs because of following
is an autosomal recessive condition reported to reasons:
affect some individuals. These patients can have – Inadvertant transection of the muscle.
prolonged apnaea following succinylcholine – Slipping of the muscle from the suture.
administeration in the absence of prior treatment – Slipping of the muscle from the resection
with miotics. Making specific queries of unusual clamp.
 444 Theory and Practice of Squint and Orthoptics

• Prevention for lost muscle includes, a gentle, present before the operation, depending
meticulous and careful dissection, secure upon the muscle lost.
placement of sutures at preferred site and Management of postoperative slipped muscle
proper application of resection clamp. is similar to that of intraoperative lost muscle. It
• Intraoperative muscle loss should be managed is most important to note that exploration
as follows: should be carried out as early as possible, since
– If slippage occurs during a recession as time passes, finding the muscle becomes
procedure, flooding the area with irrigation progressively more difficult.
solution may usually reveal the cut end of a
3. Perforation of eyeball
tendon as a glistening white structure. If so,
Scleral perforation with or without perforation
the muscle can be easily retrieved and
of choroid or choroid and retina, during
resutured.
extraocular muscle surgery may occur in one of
– If the slippage occurs during resection
two ways:
procedure or when the above measures do
not help in finding the lost muscle in i. During cutting of the muscle insertion, sometimes
recession, one should gently examine the scleral laceration, with or without damage to
Tenon's capsule with forceps in both hands underlying tissue, may occur. This complication
used end over end. The tunnel in the Tenon's is most likely to occur, if the muscle is under
capsule in which the muscle course can be considerable traction with the hook and the
searched by this manoeuvre. Then one can sclera is very thin especially in patients with high
reach back through the tunnel and grasp the myopia and/or hereditary connective tissue
muscle with forceps and pull forward. The disease. It should be managed as below:
sutures are placed and the procedure is • Scleral perforation should be sutured with
carried out as planned. 10–0 nylon suture. If scleral hole is large, a
– If the muscle cannot be found, the best preserved scleral patch or a silicone patch may
choice is to perform a maximal recession on be applied to the area. If small and is located
the antagonist muscle along with a recession directly at the muscle insertion during
and Faden procedure on the yoke muscle of resection procedure; the resected muscle can
the lost muscle. If alignment in the primary be sutured over the defect.
position cannot be obtained by this, then • Pupil should be dilated and the retina should
one should perform a muscle transposition be examined with the indirect ophthalmoscope.
procedure. In this procedure, the adjacent • Prophylactic transcleral cryotherapy is
halves of the two closest rectus muscles are recommended around the site of injury. When
transposed to the scleral insertion site of the a retinal perforation is discovered, careful
lost muscle. postoperative follow-up examinations with
• Postoperative muscle lost occurs due to slippage indirect ophthalmoscopy should be performed.
of muscle not secured properly to the sclera. ii. During placement of needles for reinsertion of the
Possibility of a slipped muscle probably exists muscle, chances of scleral and chorioretinal
until the 5th or 6th postoperative day, when perforation are more than during cutting of the
granulation tissue has sufficiently attached the muscle. In fact, this complication may occur
muscle to sclera. It is diagnosed by following more frequently than is usually recognized or
observations: admitted. Undoubtedly, the rate of perforations
– Patient will be unable to move the eye into has decreased since the introduction of spatula
the field of action of lost muscle. needle. It is recommended that even on slightest
– Palpebral fissure will widen as the patient doubt of perforation with needle, the pupil
attempts to move the eye into the field of should be dilated and a careful indirect
action of lost muscle. ophthalmoscopy should be carried out. If a
– Patient will either have a marked over- retinal hole is detected, it should be managed
correction or a larger deviation than was as discussed above.
 Principles of Non-Surgical and Surgical Management of Strabismus 445

Though extremely rare, but serious compli- ii. Partial or complete severance of superior oblique
cations such as endophthalmitis, retinal tendon and sheath may occur while attempting
detachment and phthisis bulbi have been to hook the superior rectus muscle. Therefore,
reported following perforations. Therefore, a surgeon should be very careful while engaging
very careful watch is required for such cases. the superior rectus muscle. However, if this does
4. Operation on the wrong muscle happen, following measures should be taken:
Such a complication has also been reported • Inferior oblique should be recessed, so that a
occasionally under following circumstances: significant vertical deviation does not result.
• By mistake (or due to absentmindedness of • If the operation originally planned is resection
the surgeon), the muscle to be resected may of the superior rectus muscle the surgeon
be recessed and vice versa. should decrease the amount of surgery he
• In excessively rotated globe, the exact position planned to do.
of the muscle may be shifted. • If the operation is recession of the superior
• During reoperation, the previously operated rectus, surgeon should increase the amount
muscles may in an unusual location. of recession along with weakening of the
• Myectomy of the inferior rectus is a possible inferior oblique of the same side.
complication of myectomy of the inferior 6. Operation on the wrong eye
oblique at its origin through an inferior cul- Operation on the wrong eye is an embarassing
de-sac approach. complication, sometimes encountered by the
Prevention of such a complication includes: surgeon. The best way to avoid this compli-
• Exact marking of 3, 6, 9 and 12 o'clock cation is examination of the patient by the
meridian at limbus before the conjunctival surgeon himself in the morning before surgery.
incision is made. • Operation on the horizontal muscles of the
• To use the identifying check marks of the wrong eye may many a time correct the
various muscles such as close association of deviation in the affected eye; but undoubtedly
the: the patient and/or her/this guardians and
– Inferior oblique with lateral rectus, surgeon all are upset by this mistake.
– Superior oblique with superior rectus, and • Operation on the vertical muscle of the wrong
– Inferior oblique with inferior rectus. eye is always serious because the misalign-
ment will be exaggerated. If the mistake is
5. Inadvertent injury to the other muscles discovered immediately, it should be taken
Inadvertent injury to the following muscles has care of in the operation theatre there and then.
been reported during strabismus surgery: However, if the mistake is discovered post-
i. Partial or complete disinsertion of the inferior operatively, it should be managed like a new
oblique may occur during lateral rectus surgery. This fresh case of misalignment.
is because, it is extremely easy to hook part or
all of the inferior oblique tendon, when passing POSTOPERATIVE COMPLICATIONS
a hook under the lateral rectus muscle. To 1. Postoperative infections. Because of perfect
prevent this complication, it is recommended asepsis and better quality spatulated needles, the
that the lateral rectus be doubly hooked and that incidence of postoperative infection have
the area be carefully inspected to make sure that tremendously decreased. However, though
the inferior oblique is not incorporated and quite rare, following infections are reported:
disinserted during the freeing of the inferior
check ligaments and inferior intermuscular i. Endophthalmitis has been reported to occur
septum during a lateral rectus recession. If, following squint surgery because of following
however, the inferior oblique is inadvertently reasons:
partially or completely disinserted, a suture may • Introduction of an infectious organism
be placed in the muscle belly and sutured to the following sclerochorioretinal perforation.
globe at its original insertion. • Extension of cellulitis of extraocular muscles.
 446 Theory and Practice of Squint and Orthoptics

Early diagnosis and energetic management of blood supply to the anterior segment from
can save the useful vision. the anterior ciliary arteries.
ii. Orbital cellulitis, though rare but it has been In anterior segment ischaemia, cornea
reported in the literature postoperative becomes oedematous with stromal swelling and
complication of squint surgery. folds in Descemet's membrane. Anterior
iii. Localized suture abscess has also been reported, chamber shows heavy flare and cellular reaction.
possibly resulting from contaminated suture Lens may become cataractous.
material. Prevention of anterior segment ischaemia is
2. Suture reaction. Because of better quality most important since its treatment is
synthetic sutures, the reactions are now very unsatisfactory. Following measures have been
rare. However, suture reactions were common recommended for its prevention:
with the organic suture material. They used to • All four rectus muscles should never be
occur as acute allergic reactions within 24 hours disinserted simultaneously.
to 7 days of strabismus surgery and as delayed • Disinsertion of three muscles can be done
foreign body reaction after 6–8 weeks of surgery. safely in children, but should always be
Acute reaction is characterized by conjunctival avoided in adults.
hyperaemia, chemosis, itching and a dull red • In general, a waiting of at least 6 months in
smooth mass beneath the conjunctiva at the site adult patients after surgery on both horizontal
of muscle reattachment. It is treated by topical rectus muscles is recommended before
steroid eyedrops. operating on the vertical recti.
3. Conjunctival granuloma. Because of • Techniques for preservation of anterior ciliary
improved suture material and microsurgical vessels during extraocular muscle surgery
techniques, the incidence of conjunctival which have been recommended are as follows:
granuloma has decreased. Conjunctival – Microdissection of the anterior ciliary
granuloma occurs as non-allergic foreign body vessels from the muscle under operating
reaction to suture material, cotton fibres, glove microscope.
powder, an eyelash burried in the wound or
– Modified rectus tucking procedure as a
Tenon's capsule incarcerated into the wound.
strengthening procedure.
Conjunctival granuloma is characterized by a
– Muscle splitting procedures. The reliability
localized elevated hyperaemic conjunctival
of these procedures in preventing anterior
mass which may sometimes be even pedun-
segment ischaemia is yet to be ascertained.
culated. Treatment consists of topical steroid
eyedrops. Sometimes, even surgical excision Treatment of anterior segment ischaemia
may be required. consists of:
4. Conjunctival cyst. Conjunctival inclusion cyst • Topical steroids every 1–2 hour,
may occur following inadvertent closure of the
• Systemic steroids for about 0–15 days, and
conjunctival epithelium in the wound. It appears
as a subconjunctival translucent mass that • Topical atropine twice a day.
develops several days to weeks after strabismus Prognosis. The anterior chamber reaction is
surgery. Some conjunctival cysts will resorb usually controlled with the above treatment.
spontaneously. When indicated, surgical However, iris atrophy and cataract usually occur
treatment consists of complete excision of the as sequelae of the anterior segment ischaemia.
cyst. Sometimes the eyeball may go into phthisis
5. Dellen. Dellen refers to a localized area of bulbi.
corneal thinning due to dehydration. It is more 7. Necrotizing scleritis has been reported as an
common following limbal approach. infrequent complication following strabismus
6. Anterior segment ischaemia. It is a rare but surgery. Patients with systemic diseases like
potentially serious complication of extraocular autoimmune vasculitis are more prone to
muscle surgery which occurs due to disruption develop this complication.
 Principles of Non-Surgical and Surgical Management of Strabismus 447

8. Refractive error changes mostly astigmatic – A large recess-resect procedure on the

have been reported following strabismus horizontal rectus muscle, especially in a
surgery. Possibly, these changes occur because reoperation can cause slight narrowing of
of corneal curvature alterations caused by the palpebral tissue because of slight retraction
temporary imbalance of the muscle forces. of the globe.
9. Postoperative diplopia is a common occur- • Widening of the palpebral tissue may occur with
rence following strabismus surgery for comitant vertical rectus muscle recessions.
heterotropias. It may last from a few days, a few 16. Psychological complications. A multitude
weeks or even lifetime. Children are usually able of psychologically rooted behavioural changes
to suppress early and so diplopia is short lasting. have been reported following surgery in young
While in adults, it may sometimes be trouble- children and thus should be kept in mind. A few
some. Therefore, all adults opting for correction such disorders include enuresis, stuttering,
of strabismus, must be warned of this night terrors etc.
complication.
10. Postoperative retinal detachment. Retinal POSTOPERATIVE CARE AFTER STRABISMUS
detachment has been reported as very SURGERY
infrequent complication following strabismus 1. Immediate general care includes:
surgery. A missed scleroretinal perforation is • Vital signs should be observed for at least
considered the probable cause. 24 hours after the surgery, especially when it
11. Postoperative scarring. Scarring of the has been performed under general anaesthesia.
conjunctiva and extraocular muscles can occur • Antiemetics may be given, if required and diet
postoperatively and result in restriction. The is limited.
affected muscles and conjunctiva may need • Analgesics are rarely required following
recession. Postoperative scarring of Tenon's strabismus surgery, when required, usually
capsule may produce an L-deformity of the aspirin or ibuprofen will produce comfort.
inferior oblique and a J-deformity of a rectus 2. Dressing. It is useful to apply pad and bandage
muscle or cicatricial advancement of a rectus on the operated eye for at least 24 hours.
muscle (see page 352).
3. Topical antibiotics and steroids. Eyedrops
12. Adhesive syndrome has been reported to should be instilled 3 to 4 times a day and eye
occur following surgery on inferior oblique ointment at night for 2 to 3 weeks following
muscle (see page 352). surgery.
13. Under- or overcorrections are the most 4. Oral antibiotics are usually given for a period
common postoperative complications of of 5–7 days. Though their role in preventing rare
strabismus surgery. Their management has been complications like orbital cellulitis and
discussed along with the surgical treatment of endophthalmitis is debatable but it definitely
different types of heterotropia. saves the surgeons from legal complications in
14. Gaze incomitance. Gaze incomitance may case of any eventuality.
occur following in large recession large angle 5. Oral anti-inflammatory drug may also be
strabismus. Gaze palsy is also a known compli- given for a period of 3 to 4 days.
cation of Kestenbaum procedure especially the 6. Warm compresses to reduce swelling have
augmented procedure employed in patients also been recommended.
with congenital nystagmus. 7. Restrictions for the patient are to avoid
15. Alteration in palpebral fissure swimming, playing in a sand box or other
• Narrowing of palpebral tissue may occur with possible sources of postoperative infection.
following procedures: 8. Discharge from the hospital. If every thing
– Vertical rectus muscle resections, because of goes well, patient may be discharged from the
fascial attachments from the exterior surface hospital a day after surgery. Following tests
of the muscle and adjacent lid. should be done before discharging the patient:
 448 Theory and Practice of Squint and Orthoptics

• Conjunctival incision site should be examined • Prism and cover test

to make sure that it is closed properly and the • Maddox rod test
sutures have not broken. • Maddox wing test
• Approximate alignment of the eyes should be 5. Assessment of ocular movements.
determined by cover test and Hirschberg's test.
6. Measurement of amplitude of fusional con-
• Version test should be performed to rule out vergence.
the possibility of slipped muscle.
7. Measurement of accommodation.
• Presence of diplopia should be checked and if
found, the patient and or parents should be
Line of treatment
told that this is a good sign since it may be the
Exophoria
first step towards fusion.
1. Correct refractive error.
9. Postoperative follow-up examinations. At the
2. Equalize visual acuity, if necessary.
time of discharge from hospital, patient and/or
parents are instructed to dress the eyes with 3. Improve positive fusional convergence.
sterile water and to use the prescribed eyedrops 4. Surgery, when indicated.
and ointment.
Most patients are seen within one week Esophoria
postoperatively and then at weekly intervals for 1. Correct refractive error.
the next 2 weeks. And after that at 2 weeks intervals 2. Equalize visual acuity, if necessary.
for the next 2 months. It must be emphasized that 3. Improve negative fusional convergence.
patients should be followed closely in the 4. Miotics, when exercises not sufficient and
immediate postoperative period since this is a AC/A ratio is large.
critical time, if one is attempting to achieve fusional 5. Surgery, when indicated.
results. It is during this period that situation is most
plastic and that new characteristics of the motor Hyperphoria
and sensory systems are taking place.
1. Correct refractive error
During each follow-up visit, following tests
2. Equalize visual acuity, if necessary.
should be performed:
3. Prescribe prisms.
• Visual acuity testing 4. Surgery, when indicated.
• Tests for alignment of the eyes
• Ocular movements Cyclophoria
• Diplopia, if present, is identified as to types 1. Correct refractive error.
10. Postoperative orthoptic treatment. It will 2. Equalize visual acuity.
vary depending upon the preoperative 3. Surgery—when torsion due to oblique muscle
parameters, operative results, type of squint, age defect is present.
of the patient and has been discussed with
individual clinical entity. HETEROTROPIAS
Examination
OUTLINES OF STRABISMUS 1. Test visual acuity
MANAGEMENT 2. Refraction and fundus examination
3. Cover test
HETEROPHORIA
4. Measurement of deviation
Examination
– Synoptophore method
1. Measurement of visual acuity. – Prism bar cover test (PBCT)
2. Refraction and fundus examination. 5. Assessment of ocular movements
3. Cover-uncover test. 6. Assessment of accommodation, convergence
4. Measurement of deviation. and AC/A ratio
 Principles of Non-Surgical and Surgical Management of Strabismus 449

7. Assessment of fusional ability and amplitudes b. Tests

of fusion • Fusion with head position—recent onset
8. Assessment of binocular co-operation and • Measurements—ET > right and/or left gaze
sensory anomalies. – ET D > N slight A tendency
• Measure with OD, OS fix—primary/secondary
Esotropia deviation
Differential diagnosis • Versions/ductions, saccades—decreased LR
1. Infantile esotropia function
a. Additional history • Diplopia testing—horizontal, incomitant
• Onset before the age of six months young child may suppress
• Fixates with either eye • Vision—possible amblyopia
• Forced ductions—negative
• Constant ET
b. Tests 4. Duane's retraction syndrome type I
• Abduction—reluctant but intact a. Additional history
• Measurements—large ET, V-pattern • No trauma
• Cross-fixates—equal vision • Lid fissure changes with gaze
• Cycloplegic refraction—small refractive error • Abnormal head position
• Versions—possible IO overaction • Present since birth
• Cover-uncover—possible DVD b. Tests
• Versions, ductions—no abduction, lid fissure
2. Accommodative esotropia narrows during adduction
a. Additional history • Fusion with head position—fusion often
• E (T), N > D • Vision—may have amblyopia
• Eye preference 5. Consecutive esotropia
• Worse than when tired, concentrating
a. Additional history
• Typical age of onset—18 months to 3 years • Previous surgery for XT
• Range of onset—7 months to 10 years b. Tests
b. Tests • Fusion with ET corrected—fusion usually if
• P + C, CC + SC—accommodative component X or X (T) preopative.
• Cycloplegic refraction
6. Nystagmus compensation syndrome
– <4 D hyperopia, high AC/A
a. Additional history
– >4 D hyperopia, normal AC/A • Any nystagmus seen during abduction
• Fusion, CC—excellent usually • Large ET present since birth
• Vision—amblyopia frequently • Cross-fixates turning head to see
b. Tests
3. LR palsy (CN VI) • Krimsky—over-converges with "correcting
a. Additional history prism".
• Severe head/neck trauma • Versions/ductions—jerk nystagmus in
• ET greater to right gaze, left gaze, or both sides abduction, o.u.
• Head position to right or left • Cross-fixation—does not move fixing eye out
• Horizontal, diplopia, variable worse at distance of adducted position to mid-line.
• Sudden onset of ET/diplopia
7. Cyclic esotropia
• Present since birth, if congenital
• Associated with facial nerve palsy (Moebius a. Additional history
or brainstem tumour) • 24, 48, or 96 hours schedule of alternation
• Associated with ear pain (Gradenigo) between ET one cycle and straight next cycle
• Worsens with fatigue (myasthenia gravis) • Fairly sudden onset during childhood.
 450 Theory and Practice of Squint and Orthoptics

b. Tests b. Tests
• ET day • Hirschberg/cover uncover—no deviation
• Fusion—may have diplopia initially, then • External—epicanthal folds frequently
suppression/ARC • Angle kappa—negative
• PB + CT—large ET, comitant usually • Fusion—excellent, no suppression
• Straight day
• Versions/ductions—appears to have increasing
• Fusion—excellent
• ET to right and left gaze
• PB + CT—ortho or small exophoria
• Vision—may have amblyopia
Line of treatment
8. Strabismus fixus Refractive accommodative esotropia
a. Additional history 1. Correct refractive error
Long-standing ET 2. Treat amblyopia, if neccessary
b.Tests 3. Overcome suppression
• Forced ductions—positive MR tightening 4. Improve negative fusional convergence
• Measurements—large ET, greater in R and L 5. Surgery should be avoided
gaze Non-refractive accommodative esotropia
• Versions/ductions—decreased abduction, 1. Treat amblyopia, if necessary
fixed adduction
2. Bifocals with a +3 add over cycloplegic
• Vision—must cross-fixate and turn head to see
refraction
9. Divergence paralysis 3. Miotics, only if bifocals not accepted
a. Additional history 4. Orthoptic treatment to over come suppression
and improve negative fusional convergence
• General health—N raised intracranial pressure
• Infections, parasites, and travel abroad Mixed accommodative esotropia
• Trauma possible 1. Correct refractive error, bifocals, if AC/A is
b. Tests high
• Divergence amplitudes—nearly non-existent 2. Treat amblyopia, if necessary
• Versions/ductions—full, abduction okay 3. Orthoptic treatment to overcome suppression
• Diplopia testing—uncrossed, worse at distance and improve the negative fusional conver-
• Measurements—may fuse at near gence.
• Vision—equal 4. Surgery only for the remaining nonaccom-
modative part of squint.
10. Accommodative effort syndrome
Essential infantile esotropia
a. Additional history 1. Correct refractive error
• Near asthenopia, blurring, or diplopia 2. Amblyopia treatment, if necessary
b. Tests 3. Surgery by the age of 2 years
• NPA—normal
Essential late onset esotropia
• PB + PC—E', possibly E (T)'
1. Correct refractive error
• Divergence amplitudes—poor
2. Treat amblyopia, if necessary
• Plus lenses for near—help relieve symptoms
3. Perform surgical correction
11. Pseudoesotropia – Basic esotropia—recess/resect
a. Additional history – Convergence excess—bimedial recession
• Onset—usually since birth – Divergence insufficiency type—bilateral
• Incomitance lateral rectus resection.
 Principles of Non-Surgical and Surgical Management of Strabismus 451

Sensory esotropia 4. Duane’s retraction syndrome type II

1. Correct refractive error a. Additional history
2. Cosmetic surgery, if necessary • No trauma
• Lid fissure changes with gaze
Exotropia • Abnormal head position
Differential diagnosis • Present since birth
1. Primary exotropia b. Tests
a. Additional history • Versions/ductions—decreased adduction
• Intermittency with lid narrowing, abduction okay
• Worse at distance or near • Head position—done to achieve fusion
• Worse with fatigue, illness • Fusion with head—often present position
• Vision—may be amblyopic
b. Tests
5. Sensory exotropia (due to blind eye)
• Fusion—usually excellent
a. Additional history
Measurements—exodeviation, usually comitant • Age of blindness in one eye (if over 7, likely
Basic, divergence excess type, convergence to go exo)
insufficiency type Constant non-alternating XT
• Vision—equal b. Tests
• Convergence amplitudes—poor fusional • Vision—blind eye
convergence amps • Krimsky measurements—fairly comitant XT
2. Infantile exotropia 6. Consecutive exotropia
a. Additional history a. Additional history
• Present since birth • Previous surgery for ET
• Eye preference b. Tests
• Measurements—XT
b. Tests Fusion—diplopia or suppression/ARC
• Vision—decreased in non-preferred eye
7. Craniofacial anomalies
• Health of eyes—no specific disease of eye
a. Additional history
• Measurements—usually large; comitant XT
• Alpert syndrome
3. MR palsy/CN III palsy • Crouzon syndrome
a. Additional history b. Tests
• Present since birth, if congenital • Measurements —large XT, bilateral SO palsies
• Severe trauma often
• General health—possible headaches, • Versions/ductions—IO, overaction, SO
myasthenia gravis under-action
• Other signs—ipsilateral ptosis, mydriasis, • External—bilateral exophthalmos (Crouzon)
cycloplegia, hypodeviation, contralateral • Refraction—astigmatism (Alpert), progressive
body paralysis (Benedikt’s syndrome) hyperopia (Crouzon)
b. Tests 8. Convergence paralysis
• Versions/ductions—decreased adduction a. Additional history:
• Measurements—incomitant exodeviation, • Trauma
greater in adduction • General health—recent neurological condition
• Measure with OD, OS fix—primary and possible
secondary deviation b. Tests
• Vision—possible amblyopia, cycloplegia • Convergence amplitudes—nearly non-
• Head position—present, if to attain fusion existent
 452 Theory and Practice of Squint and Orthoptics

• Measurements—comitant exodeviation N>D Primary constant exotropia

• Versions/ductions—full 1. Surgical correction should be done as early
• Diplopia—crossed, worse at near as possible.
• Fusion—at distance only 2. Orthoptic treatment both before and after
• Vision—equal surgery should be continued, if fusion ability can
be demonstrated.
9. Internuclear ophthalmoplegia (INO)
Sensory exotropia
a. Additional history 1. Correct any refractive error
• Bilateral—systemic multiple sclerosis
2. Amblyopia therapy may be tried in children
• Unilateral—vascular accident, inflammation, with anisometropia or aphakia
infection, or tumour in brainstem, possibly 3. Cosmetic surgery is the usual treatment.
myasthenia gravis
Consecutive exotropia
b. Tests
1. Correct any refractive error
• Versions/ductions—decreased adduction
2. Assess state of binocular sensory cooperation
with jerk nystagmus in abducted eye
a. If normal regional correspondence and
• Convergence amplitudes—intact convergence bifoveal fusion can be demonstrated:
10. Pseudoexotropia i. Overcome suppressions
a. Additional history: ii. Improve fusional convergence
• Constant iii. Perform surgery
b. Tests b. If bifoveal fusion cannot be demonstrated;
• Krimsky/cover-uncover test. No eye assess cosmetic appearance.
deviation i. If good, leave alone
• Fusion—excellent, no suppression ii. If poor, perform cosmetic surgery.
• Angle kappa—positive
• Funduscopy—may have retinopathy of pre- Vertical and cyclodeviations
maturity with temporally dragged fovea Differential diagnosis
I. Isolated CN palsy (SO most common)
Line of treatment
a. Additional history
Primary intermittent exotropia
• Trauma, may be mild
1. Correct refractive error; myopia should be
• Head tilt
fully corrected hypermetropia up to 2.0 DS need
not be corrected. • Combined horizontal and vertical diplopia
2. Orthoptic treatment: • Diplopia/asthenopia worse to right and left
• Antisuppression exercises • Myasthenia gravis
• Exercises for positive relative convergence b. Tests
• Occlusion therapy • Fusion with head position—usually fuses
3. Prismotherapy in children where surgery is • PB CT, 9 positions—incomitant HT
to be postponed for some period. • B3ST—isolates EOM palsy
4. Surgery is indicated, when: • Versions/ductions
• Exotropia occurs more than 50% working – SO: May show underactive SO, overactive IO,
hours inhibitional palsy of contralateral (IPC) SR
• Deviation exceeds 20D – IO: May show underactive IO, overactive
• There develops secondary convergence SO, IPC IR
insufficiency with asthenopic symptoms. – SR: May show underactive SR; overactive
• Suppression develops IR, IPC SO
• There occurs gradual deterioration of stereopsis. – IR: May show underactive IR, overactive
• Age for surgery: Before 4 years SR, IPC IO
 Principles of Non-Surgical and Surgical Management of Strabismus 453

• Vertical amplitudes—may exist, if congenital/ – Hypotropia worsens towards upgaze

long-standing. No vertical amplitude, if recent – Restrictions of ductions—upgaze, may be
onset down, right, or left gaze
• Subjective torsion • Forced ductions—restriction of globe, upgaze
– May exist, if recent onset usually
– No torsion, if congenital/long-standing • X-ray/CT scan—orbital fracture, floor
palsy frequently, or nasal wall
– SO, SR—extorsion • Diplopia field—often has region of single
– IO, IR—intorsion binocular vision
• Look at old photographs—old head tilt, if • Exophthalmometry—often affected eye
congenital/long-standing enophthalmic
2. DVD
5. Double elevator palsy (DEP)
a. Additional history
a. Additional history
• Associated with congenital/infantile ET
• Present since birth
• One or both eyes seen to go up, neither eye
ever goes hypodeviation • Ptosis on affected side
• Chin up head position
• Intermittent HT, either eye
• No trauma
b. Tests
b. Tests
• Cover-uncover—either eye elevates under
cover without associated hypodeviation of the • Fusion with chin up—possible fusion
fellow eye • Versions/ductions—constant hypotropia of
affected eye
• PBCT—difficult to measure, variable
• Forced ductions—no restrictions of elevation
• Versions/ductions—rule out IO overaction as
unless very long-standing
“cause”
• External—pseudoptosis on affected side
• Red glass test
• Bielschowsky's phenomenon—positive 6. Graves' ophthalmopathy
a. Additional history
3. Brown’s syndrome
a. Additional history Thyroid dysfunction in past or present
• Trauma to trochlear region of globe b. Tests
• Sinus, orbital surgery • Ductions/versions—restriction of upgaze
• Juvenile rheumatoid arthritis (IR), lateral gaze (MR), or any EOM
• Present since birth, if congenital • Forced ductions—positive for restriction
• Fusion with head position—usually has fusion
b. Tests
• Diplopia—variable, vertical > horizontal
• Versions/ductions—no elevation of globe in
adduction. Eye elevates easily in abduction 7. Pseudohypertropia
• Forced ductions—restriction of globe up and in a. Additional history
• Fusion—often fuses in downgaze b. Tests
• Vision—amblyopia may or may not be present • Cover-uncover—no deviation
• Krimsky—hypotropia of affected eye, when • Lift lid—appearance of HT disappears
up and in • Assess pupils—possible asymmetry
4. Blow-out fracture • Document with photos—no deviation by light
reflex
a. Additional history
• Blunt trauma—orbital fracture Line of treatment
• Diplopia/discomfort worse in upgaze often Comitant vertical deviatons
b.Tests 1. Treat amblyopia and suppression
• Ductions/versions 2. Prescribe prism for deviations up to 10D:
 454 Theory and Practice of Squint and Orthoptics

– Minimum prismatic power that provides deviation occurs in the field of paretic
comfortable DSV should be prescribed. superior oblique without overaction of inferior
– Distribute prism power equally in two eyes oblique.
with base-down in front of the hypertropic 3. Bilateral tucking of superior oblique is
and base-up infront of hypotropic eye. required in patients with bilateral superior
oblique palsy producing V-pattern esotropia
3. Surgery is indicated for deviations larger than
and excyclodeviation.
10D.
• Vertical transplantation of horizontal rectus Excyclodeviations with no vertical deviation
muscle insertion is sufficient for 10–14 D 1. Harada-Ito procedure (anterolateral
vertical deviations associated with horizontal advancement of superior oblique tendon)
tropia. 2. Nasal transposition of inferior rectus muscle
• Recession of appropriate vertical muscle (3 to in patients with congenital absence of superior
4 mm) is required for larger deviations of 15D oblique tendon or in those where it has already
to 25D. been tenotomized.
3. Temporal transpositioning of superior rectus
Dissociated vertical deviations muscle.
For significant cosmetic problem due to DVD, Incyclodeviation with no vertical deviation
treatment is mainly surgical following Temporal transposition of inferior rectus along
operations may be performed: with nasal transposition of the superior rectus
1. Faden operation with superior rectus muscle.
recession.
Incomitant strabismus
2. Large recession of the superior rectus muscle.
3. Resection of the inferior rectus muscle. A- and V-pattern horizontal tropias
4. Recess-resect procedure on superior and 1. Treatment of V-pattern esotropia
inferior rectus muscles. i. For correction of horizontal deviation in primary
5. Recession of the inferior oblique with position:
anteriorization of its insertion. – Bilateral medial rectus recession or
– Medial rectus recession and lateral rectus
Inferior oblique overaction
resection
In significant cases, inferior oblique weakening
ii. For correction of V-pattern:
should be done by any of the following
– Inferior oblique muscle weakening–when
procedures:
overacting, otherwise
1. Disinsertion
– Infraplacement of medial recti or infra-
2. Myectomy placement of MR and supraplacement of LR.
3. Extirpation
2. Treatment of A-pattern esotropia
4. Recession i. When superior oblique overaction is
5. Recession with anterior transposition associated—bilateral tenotomy of superior
Superior oblique overaction oblique muscles should be performed and
1. Superior oblique tenotomy either bilateral medial rectus recession or
2. Superior oblique lengthening recess-resect procedure should be performed
for correction of horizontal deviation in the
Cyclodeviation with vertical deviation primary gaze after making adjustment for
1. Weakening of the offending inferior oblique 10–15 prism dioptres which is corrected by
muscle should be done, when excyclodeviation bilateral superior oblique tenotomy. Alter-
with hyperdeviation is associated with natively, horizontal rectus muscle surgery
secondary overaction of inferior oblique. may be deferred for a later date.
2. Tucking of superior oblique muscle should ii. When superior oblique overaction is not
be done, when excyclodeviation with hyper- present, the treatment of choice is either
 Principles of Non-Surgical and Surgical Management of Strabismus 455

bilateral medial rectus recession with 6. Treatment of  -pattern exotropia

supraplacement of the insertions, or Despite fusion in primary position, patients with
combined supraplacement of the medial -pattern exotropia may have considerable
rectus and infraplacement of the lateral difficulty with near work (i.e. in downgaze). No
rectus muscle with a recess-resect procedure. horizontal surgery is indicated, but following
procedures may be performed:
3. Treatment of V-pattern exotropia
i. If the superior obliques are overacting,
i. If inferior oblique muscle overaction is perform bilateral superior oblique teno-
present, these muscles should be recessed tomies.
and either a bilateral lateral rectus muscle
ii. If the superior obliques are not overacting,
recession or recess-resect procedure should
supraplace the medial recti without recessing
be performed for the horizontal deviation in
or infraplace the lateral recti without
primary position.
recessing.
ii. When inferior oblique overaction is not
present, the treatment of choice is either Paralytic strabismus
bilateral lateral rectus muscle recession with
• Investigate and treat the cause of paresis if
supraplacement of the insertion or combined
found.
supraplacement of LR and infraplacement of
• Conservative managemnet for 6–8 months.
MR with a recess-resect procedure.
• Surgery, if indicated after 6–8 months
4. Treatment of A-pattern exotropia depending upon the muscle involved.
i. If superior oblique overaction is present, its
Special restrictive ocular motility defects
bilateral tenotomies should be performed.
And keeping in mind the effect of superior Duane's retraction syndrome
oblique tenotomies on the horizontal Surgery is performed only if there is significant
deviation in primary position, either bilateral abnormal head posture and/or significant
lateral rectus recession or a recess-resect deviation in the primary position. In general
procedure should be planned. surgical results are not very encouraging.
ii. When superior oblique overaction is not Brown's syndrome
present, the treatment of choice is either Surgery to weaken the superior oblque should
bilateral lateral rectus muscle recession with be performed only in the presence of a
infraplacement of the insertion or combine significant head tilt and/or a large vertical
infraplacement of the LR and supra- deviation.
placement of MR with a recess-resect
procedure.
BIBLIOGRAPHY
5. Treatment of Y-pattern exotropia
1. Abraham, SV: The use of an echothiophate
i. No treatment is required, when the patient phenylephrine formulation (echophenyline-B3)
fuses in primary position and downward in the treatment of convergent strabismus and
gaze, has a normal head posture, and does amblyopia with special emphasis on iris cysts.
not have double vision in upward gaze. J Pediatr. Ophthalmol. 1:68, 1964.
ii. When chin elevation is present and there is 2. Abraham, SV: The use of miotics in the treatment
associated overaction of the inferior oblique, of convergent strabismus and anisometropia:a
preliminary report. Am. J. Ophthalmol. 32:233,
surgical weakening of both inferior oblique
1949.
muscles will close the Y-pattern.
3. Aust, W: The use of prisms in pre- and post-
iii. If the inferior obliques are not overacting, operative treatment. In Fells. P., editor: The
supraplace the lateral recti without recessing First Congress of the International Strabismo-
or infraplace the medial recti without logical Association. St. Louis. 1971, Mosby-Year
recessing. Book. Inc., p. 255.
456 Theory and Practice of Squint and Orthoptics

4. Basmadjian, G, LaSelle, P, and Dumas, J: Retinal following strabismus surgery. Ophthalmic Surg.
detachment after strabismus surgery. Am J 24:839, 1993.
Ophthalmol. 79:305, 1975. 22. Guibor, G.P: Some uses of ophthalmic prisms.
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hyperthermia. Arch. Ophthalmol. 93:232, 1975. symposium II. St. Louis, 1958, Mosby - Year
6. Breinin, GM, Chin, NB, and Ripps, H: A rationale Book, Inc, p.244.
for therapy of accommodative strabismus. Am 23. Harada, M, and Ito, Y:Srgical correction of
J Ophthalmol. 61:1030, 1966. cyclotropia. Jpn.J. Ophthalmol. 8:88, 1964.
7. Brown, HW: Complications in surgery of the 24. Helveston, EM: Muscle transposition
extraocular muscles. In Fasanella, RM, editor: procedures, Surv. Ophthalmol. 16:92, 1971.
Management of complications in eye surgery, 25. Ing. MR: Infection following strabismus surgery.
Philadelphia, 1957, WB Saunders Co. Ophthalmic Surg, 22:41, 1991.
8. Buckley, EG, and Flynn, JT: Superior oblique 26. Knapp, P: The surgical treatment of double-
recession versus tenotomy: a comparison of elevator paralysis, Trans. Am. Ophthalmol. Soc.
surgical results. J Pediatr. Ophthalmol. 67:304, 1969.
Strabismus 20:112, 1983. 27. Knapp, P: The surgical treatment of persistent
9. Burian, HM: Use of bifocal spectaclesin the squint. Doc. Ophthalmol. 34:221, 1973.
treatment of accommodative esotropia. Br 28. Knapp, P: The use of membrane prisms. Trans.
Orthopt. J 13:3, 1956. Am. Acad. Ophthalmol. Otolaryngol. 79: 718.,
10. Caldeira, JA: Graduated recession of the 1975.
superior oblique muscle. Br J Ophthalmol. 29. Kramer, ME: Clinical orthoptics: diagnosis and
59:553, 1975. treatment, St.Louis, 1949, Mosby - Year Book,
11. Cibis, GW: Incidence of inadvertent perforation Inc., p. 349.
in strabismus surgery. Ophthalmic Surg. 23:360, 30. Lyle, KT, and Bridgeman, GJO: Worth and
1992. Chavasse squint, ed, 9, London, 1959, Tindall
and Cox, p. 51.
12. Cole, JG, and cole HG: Recession of the
conjunctiva in complicated eye muscle 31. Milot, J, Jacob, JL, Blanc, VF, and Hardy, JF: The
operations. Am J Ophthalmol. 53:618, 1962. oculocardial reflex in strabismus surgery. Can.
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13. Costenbader. FD, and Kertesz, E: Relaxing
32. Noorden, GK von: Indications of the posterior
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14. Dottan, SA Hoffman, P, and Oliver, MD:
33. Noorden, GK von: Modification of the limbal
Astigmatism after strabismus surgery.
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80:94, 1968.
16. Fells, P: Management of paralytic strabismus. 35. Nooden, GK von, and Milam, J: Penalization in
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17. Fink, WH: Surgery of the oblique muscles of the experiences. Am J Ophthalmol. 88:511, 1979.
eye. St. Louis, 1951, Mosby- year Book, Inc,., p 36. Noorden, GK von, Morris, J, and Edelman, P:
296. Efficacy of bifocals in the treatment of
18. Gifford, SR: Some notes on the treatment of accommodative esotropia. Am J Ophthalmol.
strabismus. Br J Ophthalmol. 19:148, 1935. 85:829. 1978.
19. Gldstein. JH: The role of miotics in strabismus. 37. Parks MM: Fornix incision forhorizontal rectus
Surv. Ophthalmol. 13:31. 1968. muscle surgery. Am J Ophthalmol. 65:907, 1968.
20. Gonzales, C, and Klein, B: Myectomy and 38. Parks, MM: The weakening surgical procedures
anterior transposition of the inferior oblique for eliminating overaction of the inferior oblique
muscle: a new surgical procedure and its results muscle. Am J Ophthalmol. 73:107, 1972.
in 49 operations, Binoc. Vision 8:249, 1993. 39. Parks, MM, and Helveston, EM: Direct
21. Gross, SA Noorden, GK von, and Jones, DB: visualization of the superior oblique tendon.
Necrotizing scleritis and transient myopia Arch. Ophthalmol. 84:491, 1970.
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40. Plager, D.A., and Parks, MM: Recognition and 45. Scott, AB, Rosenbaum, A, and Collins, CC:
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and pharmacological penalization. Ophthal- 46. Sharma, P, Arya, AV, and Prakash, P: Scleral
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Ophthalmol. Soc, 79:734, 1981. 17:372, 1980.
 Index

A- and V-patterns 51, 279–285, 454 motor 206

definition 279 psychological 207, 273
etiology 280 sensory 168
treatment 283 Adduction 6
A-esotropia 279 Adherance syndrome 351
treatment 285 Adjustable sutures 425
A-exotropia 279 surgical technique 429
treatment 285 After image test of anomalous correspondence 133
Abducent nerve 16–19 Agonist muscles 30
anatomy 16 Alternating dissociated vertical deviations 265
paresis 309–315 Alternating esotropia 218
clinical features 312 Alternating exotropia 250
etiology 309 Amblyogenic factors 179
treatment 313, 441 Amblyopia 82, 177–202
Abduction 6, 26, 33 anisometropic 178
Abnormal (anomalous) retinal correspondence CAM treatment of 193
83, 202–205 classification and terminology of 178
angle of anomaly in 202 clinical features of 183
binocular triplopia in 205 congenital 178
clinical phenomenon associated 205 critical flicker frequency in 185
development of 203 crowding phenomenon 184
harmonious 202 definition 177–178
management of 205 of disuse (deprivation) 178
monocular diplopia in 205 electroretinography in 186
objective angle in 202 evaluation 187
paradoxic diplopia in 205 experimental in animal studies 181
suppression 203 fixation behaviour 186
synoptophore test 132 fixation pattern in 186
tests of 129–135 isoametropic 179
unharmonious 202 meridional 179
AC/A ratio 119, 224–227 neurophysiological basis of 180
in accommodative esotropia 224–225 occlusion 201
definition 119 occlusion treatment of 190
methds of determination 119 pathogenesis and pathophysiology of 179–183
Accommodation 163–165 pleoptics in treatment 193
age-related changes 164 prevention of 189
assessment of 118 prognosis of 201
definition 163 secondary of nystagmus 178
insufficiency of 164 strabismic 178
near point of 119, 163 suppression 168
paralysis of 164 treatment of 190
spasm of 165 CAM stimulator 193
Accommodative convergence 153 occlusion therapy 190–192
Accommodative esotropia 224–233 penalization 192
nonrefractive 228 perceptual learning 194
partially accommodative 232 pharmacologic 194
refractive 224 problems in 195
Accommodative insufficiency 164 visual acuity in 183
Active pulley hypothesis 29 crowding phenomenon in 184
Acquired Brown's syndrome 344 effect of neutral density filter 184, 188
Acquired motor fusion deficiency 162 visual deprivation 179
Acquired nonaccommodative esotropia 233 Amblyoscope major 136
Acquired nystagmus 382 Ametropic amblyopia 179
Active force generation test 302 (see isoametropic amblyopia)
Acuity of vision 37–51 Amplitude of accommodation 119
stereoscopic 70 Amplitude of convergence 117
Acute comitant esotropia 234 Amplitude of divergence 116
Adaptations in strabismus 167–177 Anatomy of extraocular muscles 1–12
459
 460 Theory and Practice of Squint and Orthoptics

Anatomy of third, fourth and sixth cranial nerves 12 Botulinum toxins, role in 404
Anaesthesia for strabismus surgery 412–415 nystagmus 394
complications of 442–447 paralytic strabismus 314
general 414 Boyce-Smith deviometer 107
local 413 Bruckner's test 105, 189
topical 412 Brown's syndrome 344–346, 455
Angle acquired 344
of anomaly in ARC 202 clinical features 344
of deviation—measurement 104–113 congenital 344
kappa 188 etiology 344
measurement of 138, 188 management 345
Anisometropic amblyopia 178 Brun's nystagmus 388
Annulus of Zinn 1
Anomalous head posture 288 CAM treatment of amblyopia 193
in AV patterns 206, 282 Cardinal positions of gaze 20
in nystagmus 206, 394 Carlson and Jampolsky transposition procedure 315
in paralytic squint 289 Centre of rotation of eyeball 20
Antagonist 30 Check ligaments 10
contralateral 31 Cheiroscope 144
inhibitional palsy of 288 Circle of Vieth-Muller 62
Anterior ciliary arteries 6 Cogwheeling 374
Anterior segment ischaemia 446 Complications of strabismus surgery 442–447
Antisuppression orthoptic exercises 174 from anaesthesia 442
Arc of contact 24 intraoperative 443
Asthenopia 211 postoperative 445
Atropine 404 Computer-based orthoptic programs 145
in penalization treatment 193 computer vergence system 146
in refraction 102 computer vision training 146
Axes diagnostic 145
oblique 22 neurovision therapy 148
optical and visual 188 perceptual visual tracking program 146
of rotation 22 therapeutic 146
x 22 vision therapy 146
y 22 Concomitant strabismus 217–253
z 22 esotropia 217
etiology of 97
Bagolini, striated glasses test of 129, 275
exotropia 239
Bagolini lenses 129
vertical deviations 255–271
Bare sclera closure 418
Congenital amblyopia 380
Basic esotropia 233
Basic exotropia 240 Congential cranial dysinnervation disorders 334–343
Benedikt's syndrome 327 Congenital esotropia (infantile esotropia) 218
Bielschowsky after image test 132 Congenital fibrosis of extraocular muscles (CFEOM) 342
Bielschowsky, head tilting test of 128, 293–296 Congenital nystagmus 380
Bielschowsky phenomenon 128 Conjugate gaze paralysis,
Bifocals in optical treatment of strabismus 401, 230 horizontal 370
Binocular field of fixation 127, 297 vertical 373
Binocular triplopia 205 Conjunctival incision 415
Binocular vision 57–88 closure of 418
corresponding retinal points in 60 techniques of 415–417
development of 74–76 Consecutive esotropia 239
disturbances in 81 Consecutive exotropia 452
fusion 58, 65 Constant exotropia, primary 249
grades of 57 Convergence 35, 152–161
horopter 61, 62 accommodative 153
maturation of 76 anomalies of 155–161
stereopsis and 58 fusional 153
tests of 83–88 insufficiency of 155
visual direction 59 insufficiency treatment trial 158
Blind spot mechanism 207 measurement of 117
Blind spot syndrome 206 near point of 154
Blood supply of extraocular muscles 5 proximal 154
Blow-out-fracture 348 tonic 152
 Index 461

units of measurement 154 Eccentric fixation 129, 140, 141, 185, 188
voluntary 152 Eccentric viewing 185
Convergence and divergence centre 360 Electro-oculography 186, 390
Convergence insufficiency 155 Electronic vision testing programs 145
clinical features 156 Electronystagmography 390
etiology 155 Emperical horopter 61, 62
treatment 157 Epicanthal fold 92
Convergence insufficiency treatment trial 156 Esodeviations 95, 217–239, 449
Convergence retraction nystagmus 388 classification of 95, 217
Convergence paralysis 159 Esophoria 209
Convergence spasm 160 Esotropia 95, 217–239, 449
Corneal reflex test 105 accommodative 224
Cover tests 103 hypoaccomodative 231
Cover-uncover test 103 infantile 233
Crowding phenomenon 184 nonrefractive 228
CSM method 187 partial accommodative 232
Cupper's binocular visuscope test 134 refractive 224
Cyclic estropia 237 acquired non-accommodative 233
Cyclic heterotropia 211 acute comitant 234
Cyclodeviations 271–277 basic 233
Cyclovergence 36 convergence excess type 233
cyclic esotropia 237
Deorsumductions 96, 265 esotropia divergence insufficiency type 234
Depth of perception 71–74 late onset esotropia 233
monocular clues 71 in myopia 233
non-stereoscopic clues 71 stress induced 233
stereoscopic 71 consecutive 239
Deviations (see strabismus) divergence paresis 237
Diagnostic positions of gaze 20, 107 essential infantile 218
Digital Hess screen 123 infantile (congential) esotropia 218
Digital synoptophor 139 intermittent 217
Diplopia 81, 286 sensory 238
in antisuppression training (diplopia exercises) 174 Essential infantile esotropia 218
binocular 286 Etiology of heterotropia 97–98
charting 121 Euthyscope 141
crossed 175
Exaggerated traction test 301
monocular in ARC 205
paradoxic in ARC 205 Examination of a patient with
physiologic 62 strabismus 100–135
uncrossed 175 Excycloduction 33
Diplopia exercises 174–176 Excyclotropia 271
Diplopia fields, charting 121 Exercises, orthoptic 213–216, 405–406
Diplopia test of ARC 131 Exodeviations
Diploscope 141 classification of 240
Disjugate movements 35 Exophoria 210
Disparity of fixation 64 Exotropia 239–253, 451
Dissociated horizontal deviations 271 congenital (infantile) 239
Dissociated vertical deviations 264–271, 453 constant 249
Divergence 35, 161–163 consecutive 252
anomalies of 161 intermittent 241
acquired motor fusion deficiency 162 primary 240
fusional 161 sensory 251
Divergence excess, simulated 240, 241 External ophthalmoplegia 333
Divergence insufficiency 161 Extraocular muscles 1–8
Divergence paralysis 162 actions of 6
Doll's head phenomenon 35, 219 agonists 30
Donder's law of ocular motility 31 anatomy of 1–6
Double depressor paralysis 325–326 antagonists 30
Double elevator paralysis 323–325, 453 blood supply of 5
Double prism test 113 fascial sheaths of 9
Duane's retraction syndrome 334–341, 455 field of action 29
Duction movements 33 mechanics of actions 22
Duction test 113 oblique, inferior 4
 462 Theory and Practice of Squint and Orthoptics

oblique, superior 3 Harada-Ito procedure 309, 438

paralysis of (paralysis) 285–333 Harmonious ARC 202
rectus muscles 1–3 Head posture
inferior 2 anomalous 288
lateral 2 in A- and V-patterns 206, 282
medial 2 in nystagmus 206, 395
superior 2 in paralytic strabismus 288
synergists 30 Head tilt test of Bielschowsky 128, 293–296
yoke muscle 30 Heredity, role in strabismus 98
Eye movements (see ocular movements) Hering's law of equal innervation 31, 288, 429
ductions 33 Hess screen test 122
persuits 34, 368 Heterophoria 209–216, 448
recording of 429 cyclophoria 211
saccades 34, 366 esophoria 209
vergences 35 exophoria 210
versions 33 hyperphoria 210
Faden operation 428 Heterophoria method of determining AC/A ratio 119
surgical technique of 429 Heterotropia 93, 448
Far point of accommodation 163 comitant 93
Fascial sheaths of extraocular muscles 9 incomitant 93
Fascia bulbi 8 Hirschberg test 105
Fick's axes 22 Horizontal conjugate gaze paralysis 370
Fixation pattern 129 Horizontal gaze palsy with progressive scoliosis 342
in amblyopia 184 Horizontal rectus muscles
eccentric 184 actions of 6
paradoxic 185 recession of 418
Fixation disparity 64 resection of 422
Forced duction test 300–302 Horopter 61, 62
Four D (4D) base-out prism test 171 Hummelscheim procedure 315, 441
Foveo-foveal test of Cupper 134 Hyperacuity 38
Foville's syndrome 311 Hypoaccommodative esotropia 231
Fracture orbital floor blowout 348 Hypotropic DVD 267
Fresnel prisms 401
Fusion, binocular 58, 63 Incomitant strabismus 278–352
motor 63 A-V patterns 279
sensory 63 classification 278
stereopsis 67 paralytic strabismus 285
tests of 83 restrictive ocular motility defects 333–352
Fusion deficiency acquired motor 162 Incycloduction 33
Fusion maldevelopment nystagmus syndrome 381 Incyclotropia 271
Fusional convergence 153 Infantile accommodative esotropia 233
Fusional divergence 161 Infantile esotropia 218
Infantile nystagmus syndrome (INS) 380
Gaze evoke nystagmus 387 Inferior oblique muscle
Gaze palsies actions of 6
horizontal 370 myectomy of 433
vertical 373 overaction 257–261
Gaze positions 20 paralysis 321–323
General anaesthesia for strabismus recession of 431
surgery 414 weakening procedure 431
Generated muscle force 302 Inferior rectus muscle
determination of 302 actions of 6
Global stereopsis 69 paralysis 316–318
Gradenigo syndrome 311 recession 419
Gradient method of determining AC/A ratio 120 Inhibitional palsy of contralateral antagonist 288
Graves ophthalmopathy 346, 453 Intermittent deviations
(see thyroid ophthalmopathy) esotropia 217
Haidinger's brushes 189 exotropia 240
Half-angle-rule 28 Intermuscular membrane 10
Haploscopic tests 113, 121–127 Internuclear ophthalmoplegia 333, 372, 452
Hess screen test 122 Intorsion (see incycloduction)
Lancaster red-green test 121 Inverse Duane's syndrome 338
Lees screen test 125 Isoametropia amblyopia 179
 Index 463

J-deformity 352 Medial longitudinal fasciculus 364

Jensen procedure in abducens palsy 315, 440 Medial rectus muscle
actions of 6
Kappa angle 188 anatomy of 2
(see angle kappa) palsy of 315–316
Kestenbaum-Anderson operation 395 recession of 418
Kestenbaum limbus test 114 resection of 422
Knapp procedure 326, 440 Meridional amblyopia 179
Krimsky's test 107 Meter angle 154
Kushner's classification of intermittent exotropila 241 Microtropia 235
Millard-Gubler syndrome 311
L-deformity 352
Miotics 194, 227, 403
Lambda pattern deviation 280
in accommodative strabismus 227
Lancaster red-green test 121
in amblyopia 194, 403
Landolt C test 46
Mobius syndrome 343
Lang test 86
Monocular diplopia 205
Late onset esotropia 233
Monocular elevation deficiency 323
Latent squint 209–216, 448
Monocular nystagmus of childhood 383
Lateral gaze incomitance 243
Monofixation syndrome 235
Lateral rectus muscle
Motor adaptation to strabismus 206
actions of 6
Motor fusion 63
anatomy of 2
Movements of eyeball 33–35
palsy of 309–315
Muscles extraocular (see extraocular muscles)
management of 313
Muscle sheaths 10
recession of 419
Muscle pulleys
resection of 422
anatomical arpects 10
Laws governing ocular motility
physiological aspects 28
Donder's law 31
kinemetics of 27
Hering's law 31
Myectomy of inferior oblique 429, 433
Listing's law 31
Myotomy, marginal 427
Sherrington's law 32
Leash restriction 300 Near point
Lees screen 125 of accommodation 119, 155
Lens gradient method 120 of convergence 118
Levocycloversion 34 Near vision 50
Levodopa, in amblyopia 194 Nerve fourth,
Levoversion 34 anatomy of 12
Limbal approach to conjunctiva 415 paralysis of 303
Limbus test of mobility 114 Nerve third
Line acuity in amblyopia 184 anatomy of 12
Listing's law 31 paralysis of 326
Listing's plane 22 Nerve sixth
Livingston binocular guage 139
anatomy of 12
Local anaesthesia for strabismus surgery 413
paralysis of 309
Local stereopsis 69
Nerve supply of extraocular muscles 5
Lockwood's ligament 10
Neurophysiological basis of amblyopia 180
Macular massage 176 Neurovision therapy 148
Maddox double prism test 274 Neutral density filter test 184, 188
Maddox double rod test 273 Newcastle scoring system 244
Maddox rod 109 Nonaccommodative convergence excess 233
Maddox rod test 109 Nonaccommodative (acquired) esotropia 233–239
Maddox tangent scale 109 Nonrefractive accommodative esotropia 228
Maddox wing 111 Normal retinal correspondance 60
Maddox wing test 111 Nystagmus 376–399
Major amblyoscope 136–139 acquired nystagmus 382
Malignant hyperthermia 442 classification of 379
Marginal myotomy 427 clinical evaluation 389
Maxwell's spot, eccentric fixation congenital 380
determination by 186 electrophysiological recording 390
Mayo scale for control of intermittent exotropia 244 features of 376
Measurement of deviation 104–113 fusion maldevelopment nystagmus syndrome 381
Mechanical-restrictive strabismus (restrictive infantile nystagmus sundrome (INS) 380
strabismus) 333–352 manifest latent 381
 464 Theory and Practice of Squint and Orthoptics

neuroimaging 392 occlusion in 190

optokinetic 380 penalization in 192
pathophysiology of 379 pleoptics in 193
physiological 380 antisuppression exercises 174
surgical treatment of 394 for convergence insufficiency 157
treatment of 393–398 for esophoria 213
Nystagmus blockage syndrome 236 for exophoria 214
surgical treatment of 237 in primary exotropia 245–246
Nystagums dampening syndrome 236
surgical treatment of 237 Panum's area 64
Nystagmus like ocular motor oscillation 398 Paradoxical diplopia in ARC 205
Paradoxic fixation 185
Objective angle of squint 111, 202 Paralysis (of extraocular muscles)
Oblique axis 22 of convergence 159
Oblique muscles of divergence 162
inferior oblique 4 of double depressor 325–326
anatomy of 4 of double elevator 323–324, 453
surgery of 431, 433 of lateral rectus 309
superior oblique of superior oblique 303
anatomy of 3 of third nerve 326
sheath syndrome of 344–346 Paralytic strabismus 285–333
surgery of 433–439 congenital versus acquired 298
Oblique school of A and V pattern etiology 280 clinical features 286
Occulocephalic (doll's eye) reflex test 370 diagnostic tests in 292–302
Occlusion 190–192 etiology 285
Occlusion amblyopia 201 individual muscles in 303–326
Occlusion test 243 paralytic versus restrictive 298
for primary exodeviations 243 past pointing in 287
Occlusion therapy of amblyopia 190–192 primary and secondary deviations in 286, 287
Ocular dysmetria 374 supranuclear gaze paralysis in 369
Ocular flutter 374 treatment of (see treatment of lateral rectus palsy)
Ocular motility (movements) Paramedian pontine reticular formation (PPRF) 360
assessment of 113–118 Partial accommodative esotropia 232
ductions 33 Past pointing in paralytic strabismus 287
physiology of 20–36 Penalization in treatment of amblyopia 192
basic kinematics of 20 Pharmacologic treatment of strabismus 403
laws of 31 Physiologic diplopia 62
pursuits 34 Physiology of ocular moticity 20–36
saccades 34, 366 Pleoptics in treatment of amblyopia 193
vergences 35, 368 Position of gaze 20
versions 33 cardinal 20
Ocular torticollis 291 primary 20
Oculocardiac reflex 443 secondary 20
Oculodepressor reflex 443 Posterior commissure 360
Oculorespiratory reflex 443 Posterior fixation suture surgery for
Oculomotor apraxia 370 strabismus (Faden operation) 428
Oculomotor nerve 12 Posterior tenectomy of superior oblique 263
One-and-half syndrome 373 Postoperative care after strabismus surgery 447
Ophthalmoplegia Preferential looking (viewing test) 43
external 333 Primary constant exotropia 249
internuclear 333, 372 Primary deviation in paralytic strabismus 286
total 333 Primary exotropias 240
Opsoclonus 374 Primary overaction of inferior oblique muscle 257
Optical treatment of strabismus 400 Prism adaptation test 401
Optokinetic nystagmus test 41, 380 Prism bar and cover test (PBCT) 105
Optokinetic system 369 Prism bar and red filter test 131
Orbital fascia 8 Prism reflex test 107
Orthophoria 91, 209 Prism test
Orthoposition 91 4D base-out 171
Orthoptic instrument 135–145 25D base-in 187
Orthoptics in treatment of strabismus 405 Prisms
for amblyopia 190 for concomitant hyperdeviations 255
CAM treatment in 193 for esophoria 216
 Index 465

for nystagmus 393 Spread of comitance 287

in primary exodeviations 245 Spring-back balance test 302, 422
in treatment of strabismus 401 Squint (see strabismus)
Prolonged occlusion test 243 Stereogram random dot 85
Proximal convergence 154 Stereopsis 67–71
Pseudoesotropia 92 Frisby test 86
Pseudoexotropia 92 and fusion 70
Pseudohypertropia 92 global 69
Psychologic adaptation in strabismus 207 Lang test in 86
Pursuit movements 34, 368 local 69
random dot stereogram test 85
Random dot stereogram 85 test of 83
Raymond's syndrome 311 Titmus stereo test in 84
Reading bars 143 TNO test in 86
Recession of two pencil test in 88
inferior oblique 431 Stimulus deprivation amblyopia 178
inferior rectus 419 Strabismic amblyopia 178
lateral rectus 419 Strabismus
medial rectus 418 A and V patterns of 279–285
superior oblique 433 acute strabismus 234
superior rectus 419 classification of 92
Rectus muscles comitant
actions of 6, 22–29 esotropias 217–239
anatomy of 1–5 exotropias 239–253
recession of 418 vertical deviations 255, 256
resection of 422 cover test 103
Red glass test (see diplopia test) deorsoadductorius 96, 256
Red-green test, Lancaster 121 etiology of 97–98
Refractive accommodative esotropia 224 evaluation of a case 100–135
Remy separator 143 fixus 350
Resection of rectus muscles 422 incomitant 278–354
Restrictive ocular motility defect 333–352 paralytic 285
Brown's syndrome 344 restrictive 333
congenital cranial dysinnervation disorders 334–343 vertically incomitant 279
Duane's retraction syndrome 334–341 non-surgical management 400
Retinal correspondence sursoadductorius 96, 257
normal (NRC) 60 Strengthening operations of
anomalous (ARC) 202–205 rectus muscles 418, 419
tests for 129–131 superior oblique 436
Retinal massage Striated glasses test of Bagolini's 129
(macular massage) 176 Subjective angle in ARC 112, 202
Retraction syndrome (Duane's) 334–341, 455 Superior colliculus 361
Retraction syndrome (pseudo Duane's) 340 Superior oblique click syndrome 345
Rostral interstitial nucleus of MLF 360 Superior oblique muscle
anatomy of 3, 4
Saccadic eye movements 34, 366 overaction of 261–264
Secondary deviation in paralytic squint 287 paralysis of 303
Secondary overaction of inferior oblique muscle 257 recession of 436
Secondary positions of gaze 20 strengthening of 436
Sensory adaptations in strabismus 168 tenotomy 434
Sensory esotropia 238 weakening procedure 433
Sensory exotropia 251 Superior oblique tendon sheath syndrome (Brown)
Sensory fusion 63 344–346
Sheridan-Gardiner test 46 Superior rectus contracture syndrome 267
Sherrington's law of reciprocal innervation 32 Superior rectus muscle
Simulated divergence excess exotropia 240 anatomy of 2–3
Single binocular vision 57 paralysis of 318–321
Skew deviation 374 recession of 419
Snellen's test type 48 Suppression 81, 168–177
Spasm of accommodation 165 and ARC 83, 203
Spasm of convergence 160 facultative 170
Spasmus Nutans syndrome 382 measurement of depth of 173
Spiral of Tillaux 3 mechanism and seat of 170
 466 Theory and Practice of Squint and Orthoptics

obligatory 170 Upbeat nystagmus 385

tests for 171–173 Unharmonious ARC 202
treatment of 173–177
Suppression amblyopia 178 V-esotropia 280
V-exotropia 280
Supranuclear control of eye movements 355–369
Vergences 34
Supranuclear disorders of ocular motility 369–374
measurement of 116–118
Supranuclear gaze paralysis 370
Versions 33
Surgical management of strabismus 406–448 assessment of 115
Surgical techniques 406–448 Vertical and cyclofusional movements 272
for adjustable sutures 425 Vertical conjugate gaze paralysis 373
for conjunctival incision and muscle exposure 415 Vertical deviations 255–271, 452
for fixation of globe 415 Vertical rectus muscles 1, 2
for Harada-Ito procedure 309, 438 Vertical school of A and V pattern etiology 280
for marginal myotomy 427
Vertical strabismus 255–271, 452
for muscle transposition procedures 439, 440
comitant 255
for myectomy of inferior oblique muscle 433
incomitant 256
for posterior fixation suture (Faden operation) 428
Vertical vergence 35
for recession of rectus muscles 418
Vestibular apparatus 362
hang-back techniques 420
hemihang-back techniques 421 Vestibulo-ocular reflex test 370
for resection of rectus muscle 422 Videonystagmography 391
for slanting of rectus muscle insertions 277 Vieth-Muller circle 62
for strengthening action of superior oblique muscle 436 Vision therapy 146
for weakening action of superior Visual acuity 37–54
oblique muscle 433 components of 38
Sursumadductorius 257 measurement of 38–51
Sursumduction alternating, (see DVD) in infants 40
Suspensory ligament of Lockwood 10 in preschool children 45
Synergistic muscles 30 in school age children 48
Synoptophore 136–139 projec-o-chart test 173
measurement of angle of deviation 111, 112 Visual angle 38
test of ARC 132 Visual axes 188
Visual deprivation amblyopia 178
Tangent scale 109 Visually evoked potential 42
Tenon's capsule 8
Visually evoked responses 42
Tenon's space 8
Visuscope 140
Third nerve palsy (total) 326–332
Voluntary convergence 152
acquired 327
congenital 327 Weakening procedure for
Thyroid ophthalmopathy 346, 453 extraocular muscles 408
Tibbs binocular trainer 144 inferior oblique 429
Tillaux, spiral of 3 superior oblique 433
TNO test 86 Weber's syndrome 327
Tonic convergence 152 Worth four dot test 132, 171
Torsional kestenbaum 397
Torticollis, ocular 291 X axis 22
Total ophthalmoplegia 333 X-pattern deviation 280
Transposition of extraocular muscles 439 Y axis 22
Triplopias, binocular 205 Y cells 181
Trochlear nerve 13 Y-pattern deviation 280
Tucking of superior oblique muscle 436 Yoke muscles 30
Tumbling E-pad test 46
Two pencil test 88 Z axis 22
Type IV Duane's syndrome 337 Zinn, annulus of 1