A Systematic Approach to Strabismus, 2nd Edition
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Library of Congress Cataloging-in-Publication Data
Karlsson, Virginia.
A systematic approach to strabismus / Virginia Karlsson. -- 2nd ed.
p. ; cm.
Includes bibliographical references and index.
ISBN-13: 9781556427947 (alk. paper)
1. Strabismus. 2. Eye--Examination. 3. Ophthalmic assistants. I. Title.
[DNLM: 1. Strabismus. 2. Strabismus--diagnosis. WW 415 K18s 2008]
RE771.H36 2008
617.7'62--dc22
2008029541
ISBN: 9781556427947 (pbk)
ISBN: 9781003526681 (ebk)
DOI: 10.1201/9781003526681
Dedication
In memory of
Steven William Salevouris
1956 – 2006
Orthoptist, technologist, student, true gentleman, and friend.
Contents
Dedication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . v
Acknowledgments. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . viii
About the Author . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ix
Foreword by Jonathan M. Holmes, MD . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . x
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xi
Chapter 1. The Systematic Approach to Strabismus . . . . . . . . . . . . . . . . . . . . . . 1
Chapter 2. The Four-Part Exam. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
Chapter 3. Approaching Children and Infants. . . . . . . . . . . . . . . . . . . . . . . . . . 27
Chapter 4. Approaching Adults . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35
Chapter 5. Extraocular Muscles—Anatomy and Function . . . . . . . . . . . . . . . . 45
Chapter 6. Binocularity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59
Chapter 7. Differential Diagnosis—Ins, Outs, Ups, and Downs. . . . . . . . . . . . 63
Chapter 8. Syndromes With Ocular Manifestations . . . . . . . . . . . . . . . . . . . . . 85
Chapter 9. Nonsurgical Treatment of Strabismus . . . . . . . . . . . . . . . . . . . . . . . 93
Appendix . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107
Index. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109
Acknowledgments
Another 10 or so years have gone by since A Systematic Approach to Strabismus was first
written for SLACK in response to their success with the original 12-volume series for ophthalmic
personnel. Now, we have this second edition: my third book, but probably not the last.
Again, I’ve learned a lot in the past 10 years and am grateful to the pediatric ophthalmologists
at the Mayo Clinic with whom I’ve had the pleasure to work. They have encouraged me to have
the best of both worlds: seeing real patients within a team approach and teaching and academics.
Drs. Jonathan Holmes, Michael Brodsky, Brian Mohney, and George Hohberger all have shared
a common joy in the care of our patients and a wonderful variety in styles of doing so!
I blinked, and it happened: my children have grown up considerably. Thank you Dory for
proving that the impossible can happen. Thank you Lars for taking on an impossible role. Thank
you Carlson for fitting in impossible places. Thank you Greta for knowing that everything is pos-
sible, and thank you Colby for reminding me daily that today might just possibly be the best.
About the Author
Virginia Carlson’s orthoptic career began at the age of 2 when she climbed over into the front
seat of her parents’ 1956 Chevy Bel Air and gave her mother a corneal abrasion with her finger-
nail. Unfortunately, this abrasion was to her mother’s NON-amblyopic eye and required 3 days of
pressure patching to heal. From that point onward, however, her mother always claimed that her
amblyopic eye could see just a little bit better after all that albeit unintentional, occlusive therapy
that had been overlooked in her childhood.
Virginia Karlsson, now spelling her last name the way her paternal grandfather had before
immigrating to the United States, celebrated her 30th year as a certified orthoptist in 2008.
Colby-Sawyer College in New London, New Hampshire, provided her introduction to orthop-
tics as a career, providing an excellent undergraduate experience. The University of Florida
orthoptic training program in Gainesville provided something unique 32 years ago—simultane-
ous training as a tech and as an orthoptist. After graduating, Carlson now Hansen returned to
New England where she took two part-time jobs: one as a tech to support her second part-time
job as an orthoptist in western Massachusetts. Two years later, she became the director of the
orthoptic training program at Tufts-New England Medical Center in Boston. After the birth of
her first child, she filled in (twice each) for other orthoptists out on maternity leave themselves
at the University of Massachusetts in Worcester; the Lions Orthoptic Clinic in Springfield, Mas-
sachusetts; and the Newington Children’s Hospital in Newington, Connecticut.
She returned to work full-time in 1990 when her family moved to Minneapolis; she worked
for a large health system and then a private practice. In 2005, Karlsson took the orthoptic posi-
tion at the Mayo Clinic and has thoroughly enjoyed the extraordinarily challenging patients, the
completely ordinary patients, the academic environment, and the peaceful commute from Min-
neapolis to Rochester, Minnesota.
Five children with varied interests don’t leave much time for Mom, but most recently she
has remembered how to ski, speak Swedish, play kubb (poorly), and definitely laugh more.
“Don’t blink!” is the advice she tells new parents about their baby’s first year of life. Maybe she
shouldn’t have blinked so much over the past 30 years of being an orthoptist!
Foreword
Many of us can remember the sense of inadequacy we first felt when we tried to approach a
young child to glean any information from his or her eyes. Pediatric ophthalmology and strabis-
mus are “lore unto themselves”; it seems that nothing we learn from examining adults applies to
these hypermobile, hyperdistractable bundles of joy. In this book, Ginny Karlsson shares with us
many superbly practical tips for “making it happen”; acquiring that information from the child;
allowing us to discover, treat, and help; while still having fun. Fun for them and fun for us.
For beginning orthoptists, ophthalmic technicians, and ophthalmology residents, this book is
crammed full of helpful pearls and insights based on the many years of Ginny’s experience. It has
been a joy to work with Ginny taking care of children. May you also find a sense of joy as you
embark on the adventure of taking care of children who have eye problems.
Jonathan M. Holmes, MD
Professor and Chair of Ophthalmology
Mayo Clinic
Rochester, Minnesota
Introduction
As an orthoptic student I remember staring at my big, blank exam sheet and wondering what
the patient in the exam chair had. Worse was staring at my big filled-in exam sheet and wondering
what the patient in the exam chair had. Now I’m looking at a computerized chart note with drop-
down boxes of cookie-cutter choices and realize that no matter how much gets filled in, we still
have to think about how to put our whole exam together and be able to make sense of the infor-
mation. Over time (up to the present and beyond), the examination of the patient with strabismus
has become clearer to me, and it is that exam that I have tried to put into this book.
Many other texts cover pediatric ophthalmology and strabismus in immense detail. My only
intention here is to get the beginner started turning that big, blank exam sheet; computer monitor;
or some yet-to-be-invented tool into a skillfully diagnosed patient with a treatment plan that the
patient and parents will work with.
Virginia C. Karlsson, CO, COMT
Certified Orthoptist
Mayo Clinic
Rochester, Minnesota
Chapter 1
The Systematic
Approach to
Strabismus
K E Y P O I N T S
• Visual maturity commonly occurs at 9 years of age.
• Exam pollution is avoided by conducting the exam in a system-
atic order—history, fusion, alignment, and (finally) vision.
• Practice tests on cooperative adults before attempting to do
them on children.
DOI: 10.1201/9781003526681-1
2 Chapter 1
Congratulations! You have bravely entered the world of strabismus and ocular motility. This is
the same world that has terrified residents and technicians, brought experienced ophthalmologists
to their knees, and placed a smile on the orthoptist’s face. This book is designed to get you past
the common core of knowledge that you possess and move you boldly into the exam room face-
to-face with a 3-year-old child. You know how to test vision and you have read how to measure
stereopsis. The technique for doing a prism and cover test really did not sound that difficult. So
why the terror when your next chart reads, “3 year old with RET”?
Two Eyes
Strabismus and its related subjects bring the added dimension of a second eye to the eye
exam. Although you are always testing two eyes when testing vision, refracting, or testing pres-
sure, each eye is treated as a separate entity. Start thinking of the two eyes as a pair, a combination
to be reckoned with as a single unit. Despite familiarity with the basic tests required to complete
a motility exam, performing these tests gracefully, accurately, and in an orderly and timely fash-
ion on that 3-year-old child with the right esotropia may be a sticking point in your repertoire of
techniques.
Four Hints for the Child’s Eye Exam
1. Visual maturity can be your friend or your enemy. Visual maturity refers to that magical
age, unique to every individual, at which time the visual system has matured to its full
capacity and is no longer malleable. A normal infant’s vision develops at a rapid rate after
birth, reaching 20/20 adult-like vision by 6 months of age. An infant’s control of his or her
eye movements should be normal and therefore completely straight by 4 months of age.
Both vision and motility, however, continue to change for years to come. While each indi-
vidual is different, age 9 is commonly cited as the age at which visual maturity frequently
occurs. This means that a child’s vision may no longer improve after that age if therapy is
attempted, and conversely, may no longer be lost if therapy is discontinued at his or her own
personal age of visual maturity.
2. Your examination technique must change when examining children or babies. The new set
of rules requires speed. The quicker the exam on a child can be accomplished, the better.
Measurements are more accurate on a happy, relaxed, and attentive child (and parent). The
exam will go considerably smoother on a child who is dazzled by the flurry of activity you
perform in front of him or her. When something is happening to the child all of the time
and the child is distracted, there is less chance that the child will be uncooperative. In order
to do this, you, the examiner, must plan and practice. First, practice the skills you need on
cooperative adults; do not waste your time trying to learn on a squirming child. Anticipate
the tests you will need to accomplish on this particular individual before you have involved
the child in the exam. Plan so as to limit the time the child will actually sit in the chair (my
personal preference is always on the parent’s lap if under age 5 or 6). To work within this
limited amount of time, take your history with the parent/child sitting in regular chairs in
the exam room rather than in the exam chair. Once you are completely ready to stop asking
the boring questions and get to the fun part of the exam, move the child with parent to the
chair, inviting him or her to look at your cool stuff. Do not ever actually say that it is an eye
The Systematic Approach to Strabismus 3
exam, which puts many children on guard for a frightening experience. If you really want
to get the child’s attention, speak softly. That will get everyone’s attention.
3. Not every test needs to be done on every patient. Do only those that are necessary. Assum-
ing that your young patient’s cooperation will only last a finite period of time, plan on
doing that part of the exam that is most necessary while the child is still cooperative. For
instance, if a child is destined to be cyclopleged with a postcyclo refraction, do not waste
time trying to get a manifest refraction. The tables in Chapter 7 will help you put together
an exam strategy.
4. Try to limit the level of exam pollution. Exam pollution happens when performing one
test alters the outcome of a subsequent test. An example of this in general ophthalmology
would be to anesthetize the cornea for a pressure check before checking corneal sensitivity.
Testing order is critical. A strabismus exam has four key parts: history, vision, alignment
measurements, and fusion. But those tests are never done in that order!
Fusion is most easily disturbed by measuring alignment or vision, so fusion is usually mea-
sured first. Since the most sensitive fusion test should be done initially, stereopsis is measured
first. This would be followed by Worth four-dot testing if desired and then the single cover–
uncover test (which does not measure alignment but tests for motor fusion control; for more on
this, see Chapter 2).
Ideally, alignment measurement should be done before vision testing, since prolonged patch-
ing of the eye for vision testing could alter the everyday ocular alignment and will certainly
disrupt fusion. Alignment measurements require the proper fixation target, appropriate measuring
test, and your polished technique.
Vision testing on children requires your utmost patience. The ability of the patient must be
considered and the appropriate test selected long before the patch goes on the child’s eye. An
adhesive patch should always be used to prevent cheating and peeking, unintentional or other-
wise. Finally, while the patch is still on, a dry retinoscopy or manifest refraction (to be balanced
later) should be done, and vision rechecked for improvement, with that correction in place.
You can learn how to do all of this by keeping the guidelines in order and by mastering your
skills practicing on cooperative adults.
Chapter 2
The Four-Part
Exam
K E Y P O I N T S
• History and observation provide clues to the potential diagno-
sis.
• The exam is done in a particular order—history first, then
fusion, then alignment, and vision last.
• The potential diagnosis determines what additional tests are
necessary to complete the exam.
DOI: 10.1201/9781003526681-2
6 Chapter 2
The ocular motility exam generally has four parts, which are performed in the following order:
history clues (so you will hopefully know what you are looking for), fusion testing, alignment
assessment, and vision. This order of testing is essential to reduce pollution of the test results.
Part 1. History Clues—The Story Begins
Some patients/parents have no idea what is wrong with them/their child. It is our job to
attempt to narrow down the differential diagnosis by asking pertinent history questions. The per-
tinent questions are the ones your doctor would like you to ask. So ask your attending physician
just how involved he or she would like your history taking to be. If the doctor does not care about
a full-term baby’s birth weight, do not waste time asking.
Taking a history allows you to size up both the patient and parent. How accurately does the
parent observe the child? Has he or she noticed that 2-year-old Erin tilts her head to the side
(which you already noticed in the waiting room)? Or is that a big surprise to the parents? Always
ask the child some benign question to determine: A) if the parent will allow his or her child to
participate in the exam, and B) if the child will be participating in the exam. All you want is a
response, verbal or otherwise, so just ask a yes/no question: “Did you see the goldfish in our
tank?” “Did you drive all the way here? ...or did Mommy?” “Do you know these people?” as you
smile and gesture to his or her parents.
To assess the child’s developmental capabilities, probe further by asking a simple question
but one that requires a thoughtful answer. A 2 year old should be able to answer: “How old are
you?” or “What’s your baby’s name?” A 3 to 4 year old should be able to tell you: “Are you plain
3, or 3-and-a-half? How old is your brother?” A 5 to 6 year old should know if he or she is going
to school/kindergarten in the fall. A child in first grade and up should respond appropriately to
“What grade are you in? What’s your teacher’s name? Is she nice? Is he smart? Is your teacher
fair? What’s your favorite thing about second grade?” While all of this sounds like idle chit-chat,
you will have gained a wealth of information, particularly about the child’s ability to understand
your questions; his or her cognition, hearing, and speech/verbal skills; and his or her attitude
about school. That attitude in particular is important because a significant number of children are
brought in for eye exams by parents who are worried about the child’s school performance.
Only ask the questions you need to know, and be extremely specific. Do not ask, “Have you
ever had any eye problems?” if you do not care about the minor corneal abrasion the patient had
10 years ago. Do not ask, “Is there any family history of strabismus?” if you want to know “Are
there any immediate family members with an eye that turns in, or out, or who had to wear a patch
when they were younger?” If you want to know if there are any high myopes in the family, ask
specifically if any family members are known to wear strong glasses and at a young age. Then
you might try to determine if they were plus or minus.
Use common language. Instead of asking the patient if there is any family history of glau-
coma, ask if any family members had high pressure in the eye and had to take eye drops every
day. Instead of inquiring if the school was worried about amblyopia, ask if the school found a
difference in vision in the two eyes. Instead of asking if the child has any ptosis, ask if one lid is
droopy or different from the other.
Ask questions in such a way as to get accurate answers. “What brings you to our office
today?” discourages patients from telling about their last eye exam at the mall or the springtime
allergies they had 6 months ago. Your job is to determine whether the problem is visual, symp-
tomatic, cosmetic, or a combination of these. Use all of your history-taking skills to probe at any
The Four-Part Exam 7
problem: “What makes it better/worse? When did it start? Who notices it?” (Grandparents can
be exceedingly accurate regarding grandchildren who they see sporadically with a fresh, albeit
scrutinizing eye.)
A child who was able to articulate a visual complaint to his or her parents certainly should be
able to intelligently respond to questions put forth by a professional: “When you see the double
vision, is it side-to-side like this (demonstrate with your hands) or up and down like this? Is it
blurry when you read a book or when you sit at the back of the room and try to read the board or
overhead projector? Is it blurry to everyone else in class (because of glare, angle, poor penman-
ship) or just to you?”
It is often difficult for patients/parents to verbalize medical problems. A parent may be
reluctant to blurt out the details of the child’s problem(s) in front of the child. A patient is often
kept unaware of the diagnosis until it has been confirmed. If you get nowhere when you ask the
patient if there are any ongoing health problems, ask what other doctors he or she has seen. The
patient may have no idea that the reason he or she is suddenly seeing double, has lost weight,
has been sent to see an endocrinologist who did blood work, and now needs a motility/eye exam
is because someone thinks that he or she probably has Graves’ disease or a thyroid problem. We
often encounter patients who deny that they have high blood pressure because they are on medi-
cation that controls it. Ask what medications the patient is on, but find out if it is important to
know how much medication and what time of day it is taken. You need to know when the patient
last took his or her glaucoma drops, but does it really matter when his or her last antihistamine
was taken? Knowing which doctors are currently following the patient and what medications the
patient is on will help complete the patient’s history.
Record the patient’s allergies to medication, food, and the environment. Patients often do not
realize that an eye drop can actually affect their heart or that an allergy to peanuts could be fatal
in a child given the eye drop phospholine iodide.
As far as the parents are concerned, visual history can be divided into two categories: the
child needs glasses in order to see properly, or more seriously, the child might have uncorrectable
vision and be blind. Amblyopia, which frequently requires occlusion therapy along with glasses,
falls halfway between those two categories since although it is only one eye, it results in per-
manent loss of vision if left untreated. Ask the parents, “How do you think Gus sees?” followed
by, “Who wonders if he sees okay?” This will usually get the answers necessary to determine
if blindness is a true concern or if the visit is merely due to a failed vision screening in a 4 year
old who otherwise appears normal. These questions will usually elicit the parents’ real concerns,
which range from their genuine fear that their baby is blind to disappointment that their child
will be wearing spectacles.
Symptomatic history also has two categories: behavior that is observed (by the parents, teach-
ers, or mother-in-law) and symptoms that the child complains about (cannot see, does not want
to go to school, headaches, does not want to go to school, does not want glasses, does not want
to go to school, sees double, does not want to go to school). The child’s complaints may be real
or fictitious. Table 2-1 lists observed behavior and the potential diagnoses. Table 2-2 lists typical
childhood complaints.
Cosmetic history immediately implies a surgical correction in the minds of parents and chil-
dren. An honest response to “How do you think the eyes look?” may be tainted by their fear of
surgery. When strabismus is obviously present, ask the child if his or her friends ever say anything
about his or her eye alignment. Ask if people who are not the child’s friends ever comment on
it and if that bothers him or her. Ask the parents who else notices the child’s misalignment. The
