Acquired Nonaccommodative Esotropia in

Childhood
Brian G. Mohney, MD

Purpose: Acquired nonaccommodative esotropia (ANAET) in childhood is reported to occur infrequently and is
often associated with an underlying neurologic or neoplastic disorder. The primary objective of this study was to
ascertain the prevalence and clinical characteristics of this form of childhood esotropia. Methods: A cohort of all
children younger than 11 years with esotropia from a predominantly rural Appalachian region was prospectively
identified from August 1, 1995, through July 31, 1998. The age at onset, family history of strabismus, perinatal and
medical history, ophthalmologic findings, and surgical results were reviewed for all patients with ANAET. Results:
Twenty-three (10.4%) of 221 consecutive children with esotropia were diagnosed with ANAET compared with 12
(5.4%) diagnosed with congenital esotropia. The median age at esotropia onset for the 23 children with ANAET was
31.4 months (range, 8-63 months) with a mean initial angle of esotropia of 24 PD. Although at least 2 children pre-
sented with diplopia, none of the 23 patients were known to have harbored intracranial tumors or other lesions of
the central nervous system during the follow-up period. Fourteen of the 19 patients who underwent surgery attend-
ed follow-up visits for at least 6 months after their last surgical procedure: 13 were within 8 PD or less of orthotropia,
whereas the final patient had persistent esotropia. Twelve of the 13 patients within 8 PD of orthotropia demonstrat-
ed some level of stereopsis, including 2 children with bifoveal fixation. Two (10.5 %) of the 19 operated patients later
required a low hyperopic spectacle correction to control their deviation. Conclusions: ANAET was more prevalent
than congenital esotropia in this cohort of children with esotropia. This clinically distinct form of strabismus typi-
cally begins between 1 and 5 years of age and appears to be infrequently associated with underlying disease. The
angle of deviation is relatively small and early surgical correction is more likely to achieve bifoveal fixation for these
patients than for those with congenital esotropia. (J AAPOS 2001;5:85-9)

plastic disease.9-26 The primary objective of this study was

E
sotropia is a disorder of ocular alignment that
affects 3% to 4% of the pediatric population.1,2 to ascertain the frequency and clinical characteristics of
Although there exists a great body of literature ANAET among a cohort of 221 children with esotropia
concerning the congenital and accommodative forms, less prospectively studied during a 3-year period.
is known about other types of childhood esodeviations.
Acquired nonaccommodative esotropia (ANAET) is a SUBJECTS AND METHODS
prime example. This form of strabismus is considered A cohort of 221 consecutive children younger than 11
uncommon3,4 and may be confused with an acute form of years who were diagnosed with esotropia in the Division of
esotropia that presents in older children and adults.5-9 Ophthalmology at East Tennessee State University
Moreover, recent reports have emphasized that some chil- College of Medicine, Johnson City, Tennessee, from
dren presenting with an acquired nonaccommodative August 1, 1995, through July 31, 1998, was prospectively
esotropia may harbor an underlying neurologic or neo- studied. The geographic area of the study is located in a
predominantly rural region of Southern Appalachia.
From the Division of Ophthalmology, Department of Surgery and the Department of Johnson City, the largest metropolis within the area of
Pediatrics, James H. Quillen College of Medicine, East Tennessee State University, Johnson investigation, had a 1995 population of 52,545. The study
City, Tennessee. patients were referred primarily from pediatricians and
Presented in part at the 26th Annual Meeting of the American Association for Pediatric
Ophthalmology and Strabismus, San Diego, California, April 12-16, 2000. family practitioners or presented at the volition of their
Submitted April 15, 2000. caretakers.
Revisions accepted November 28, 2000. Each child underwent a comprehensive medical history
Reprint requests: Brian G. Mohney, MD, East Tennessee State University College of
Medicine, Department of Surgery, Box 70575, Johnson City, TN 37614 (e-mail: and ocular examination. The age at onset, family history of
mohney@[Link]). strabismus, perinatal and developmental history, and ocu-
Copyright © 2001 by the American Association for Pediatric Ophthalmology and lar history were recorded on all patients. The age at onset
Strabismus.
1091-8531/2001/$35.00 + 0 75/1/113313 was determined by the examining ophthalmologist and
doi:10.1067/mpa.2001.113313 often required the review of family photographs for con-

Journal of AAPOS April 2001 85

 Journal of AAPOS
86 Mohney Volume 5 Number 2 April 2001

TABLE 1. Historical characteristics and initial ophthalmologic findings of 10 PD or more with the use of spectacle correction)
among 23 children with ANAET form of deviation or children with a previously diagnosed
No. of males/females 15/8 congenital or developmental central nervous system disor-
No. born premature* (%) 3 (13) der were excluded from the current study.
No. with family history of strabismus (%) 9 (39.1) Surgical correction was recommended for all patients
Mean age at onset in months (range) 31.4 (8 - 63) who displayed a constant esotropia of 12 PD or more at
Mean age at diagnosis in months (range) 39 (14 - 87) both 6 m and 33 cm. Except for those children with dense
No. with amblyopia† (%) 5 (21.7)
unilateral amblyopia, bimedial rectus recession was per-
Mean angle of deviation, PD (range) 24 (6 - 40)
No. with IO overaction (%) 3 (13)
formed as the initial surgery for all patients who required
No. with DVD (%) 1 (4.3) intervention.
Mean cycloplegic refraction (range) +1.71 (+.50 - +3.00) RESULTS
*Less than 37 weeks’ gestation.
†Amblyopia was defined as a difference of 2 or more Snellen lines between the 2 The 23 children with acquired nonaccommodative esotropia
eyes or fixation that did not last more than 2 seconds beyond cover removal. comprised 10.4% of the 221 study patients compared with
DVD, Dissociated vertical deviation; IO, inferior oblique. 12 children (5.4%) with congenital esotropia. Pertinent
historical characteristics and initial ophthalmologic find-
ings for the 23 patients with ANAET are shown in Table
firmation. A family history of strabismus was considered 1. The mean age at onset of esotropia was 31.4 months.
positive if any blood relative had any form of strabismus. Approximately two thirds of the patients were boys, and 3
Patients with a history of prior strabismus surgery were of the children were born before 37 weeks’ gestation. The
eliminated from the study because a precise diagnosis mean initial angle of esotropia was 24 PD, with at least 11
could not be assured. These children tended to present at (47.8%) of the 23 patients exhibiting a progressive increase
an older age with a poor or unobtainable history or med- in their deviation with time. Three of the study patients
ical record. The ophthalmologic examination included had inferior oblique overaction and 1 child, who at the ini-
visual acuity or fixation assessment, ocular deviation in tial examination at 13 months of age demonstrated no
prism diopters, ocular rotations, sensory testing (whenev- measurable strabismus, later developed esotropia and the
er possible), cycloplegic refraction, and evaluation of the only case of dissociated vertical deviation. The mean
anterior and posterior segments. Only those children who refractive error for all 23 patients was +1.71 (+0.50 to
developed an intermittent or constant esotropia of 10 PD +3.00). None of the 7 patients with a hypermetropia of
or more during the study period were enrolled. The angle +2.00 D or more demonstrated a reduction in their devia-
of deviation was determined by the Krimsky prism tech- tion with the correction of their cycloplegic refraction.
nique in infants and young children until prism and alter- Two of the 23 study children presented with diplopia as
nate cover measurements were feasible. Most neurologi- their primary complaint. Magnetic resonance imaging of
cally intact children older than 7 months were evaluated by the head was performed in both patients and the results
the latter technique in the primary position at both dis- were negative. None of the 23 patients were found to have
tance and near. The cycloplegic refraction was performed nystagmus, paralytic strabismus, divergence paralysis, or
at least 30 minutes after the patient received 1 drop to each any systemic manifestations of neurologic disease.
eye of 2% cyclopentolate or 2 drops of 1% cyclopentolate Surgery was ultimately recommended for all 23 patients.
in children younger than 1 year or with Down Syndrome. Nineteen patients underwent surgery whereas the remain-
All cycloplegic eye drops were preceded by 1 drop of a top- ing 4 were lost to follow-up (Table 2). Four (21%) of the
ical anesthetic and were administered by the author. 19 operated patients required more than 1 procedure: 3
The precise forms of childhood esotropia from this children had 2 surgeries, and the fourth patient underwent
cohort have already been reported.27 The medical records 3 surgeries. The mean follow-up for the 19 children who
of those patients who displayed ANAET, defined as a underwent surgery was 16.9 months (range, 3 days to 42
nonaccommodative esodeviation that developed after 6 months) after their last surgical procedure. Fourteen of the
months of age, were reviewed and made up the principal 19 operated patients attended follow-up for at least 6
findings of this current study. These patients were pre- months: 13 were within 8 PD or less of orthotropia,
sumed to have a normal neurologic status although imag- whereas the final patient had persistent esotropia. Twelve
ing of the central nervous system was pursued in any child of the 13 patients with a deviation of 8 PD or less demon-
whose history or ophthalmologic examination suggested strated some level of stereopsis, including 2 children with
an unrecognized underlying neurologic or neoplastic dis- bifoveal fixation.
order. Patients found to have a cycloplegic refraction with Two (10.5 %) of the 19 operated patients, whose preop-
a spherical equivalent of +2.00 D or more were first treat- erative cycloplegic refractive error was less than +2.00 D,
ed with a 4-week period of full-time use of their cyclo- subsequently required a low hyperopic spectacle correc-
plegic correction. Esotropic children with a paralytic, sen- tion to control intermittent esotropia. Both children, who
sory, or accommodative (reducing the esotropic deviation attended follow-up for a mean of 29 months after their last
Journal of AAPOS
Volume 5 Number 2 April 2001 Mohney 87

surgical procedure, were aligned within 4 PD of orthotropia TABLE 2. Surgical results and follow-up among 19 children with ANAET
and demonstrated at least 400 seconds of arc on stereo- Mean age at surgery in months (range) 55 (27-110)
acuity testing. Mean angle of deviation at surgery, 30.7 (15-60)
PD (range)
DISCUSSION Initial surgery BMR
In this cohort of 221 consecutive children with esotropia, No. requiring second surgery (%) 4 (21)
10.4% were found to have an acquired nonaccommodative Reason for second surgery ET in 3*; Consecutive XT in 1†
deviation. Although there are no known incidence studies of No. requiring third surgery (%) 1 (5.3)
this type of esotropia, classic teaching suggests that this is an Reason for third surgery Consecutive XT‡
Mean follow-up in months after 16.9 (3 d-42 mo)
infrequent form of childhood strabismus.3,4 In his textbook,
last surgery (range)
Wright4 devotes a single paragraph to the entity and charac- No. developing postoperative 2 (10.5)
terizes its occurrence as uncommon. Two recent reviews on accommodative component (%)
acquired esotropia failed to include this form.24,28 However, Mean final deviation, PD (range)§ 3.8 ET (orthotropia to 18 ET)
these views are in contrast to the indirect findings of other Mean final stereoacuity in 12 705 (40-3000 secs of arc)
reports. Fletcher and Silverman29 and Graham1 reported patients ≤ 3000 secs of arc (range)§
that nonaccommodative esotropia (including both acquired *Two patients underwent resection of both lateral rectus muscles and 1 patient
and congenital esotropia) made up approximately 30% of received re-recession of both medial rectus muscles.
childhood esotropia. Dankner et al30 have described 54 clin- †Managed with advancement of both medial rectus muscles.
‡Managed with bilateral lateral rectus recession.
ically similar patients younger than 6 years who had under- §Findings among all (14) patients followed-up for 6 months or more after their last
gone strabismus surgery during a 21-year study period, sug- surgical procedure.
gesting a relatively frequent occurrence. BMR, Bimedial rectus recession; ET, esotropia; XT, exotropia.
The frequency of ANAET found in this cohort of eso-
tropic children was nearly twice as prevalent as congenital
esotropia. Although congenital esotropia is often considered The primary features that differentiate type II AACE
the most common form of childhood strabismus,31,32 recent from ANAET are the age at presentation and angle of
population-based data have supported a more infrequent deviation. Type II AACE has not been described in chil-
occurrence.33 Because the ophthalmologic characteristics of dren younger than 5 years. In this study, 20 of the 23 chil-
ANAET are similar to those of congenital esotropia, prior dren were younger than 5 years when diagnosed, which
studies may have classified some children with an acquired may explain why only 2 children complained of diplopia.
deviation as having congenital esotropia, particularly if the Secondly, the angle of deviation in those with type II
patients were first examined after their first birthday. AACE typically ranges from 25 to 70 PD, whereas the
ANAET presenting in children must be distinguished patients in this study presented with a deviation ranging
other forms of acquired esotropia. Separating ANAET from 6 to 40 PD, although the strabismic angle tended to
from accommodative esotropia is generally a simple process increase over time for many of the 23 study patients.
because of the relatively high hypermetropia of the latter Children with ANAET must also be distinguished from
condition. None of the children in this study displayed an those whose deviation is a harbinger of serious neurologic
accommodative component to their deviation at their ini- disease. The occurrence of acquired comitant esotropia
tial evaluation. Children with esotropia that had a paralyt- has been associated with neurologic defects9,11,17,20,26 and
ic or sensory etiology, or with a previously diagnosed con- brain tumors.10,12-16,18,19,21-25 Although some authors
genital or developmental abnormality of the central ner- have suggested that increased intracranial pressure is the
vous system, were specifically excluded from this study. most likely cause for the acute deviation,22 Hoyt and
Patients with ANAET must also be distinguished from Good34 have argued that neither hydrocephalus nor
those presenting with an acute form of deviation, otherwise abducens palsy can alone account for all children who pre-
known as acute acquired comitant esotropia (AACE). Burian sent with acute esotropia and an underlying intracranial
and Miller6 reviewed the 3 basic forms of this esodeviation process. These authors have emphasized the clues that
and included 8 of their own patients between the ages of 6 warrant further evaluation: the finding of other neurolog-
and 72 years. Type II AACE, also termed the Franceschetti ic or systemic signs and symptoms, nystagmus, or the lack
type, is the most likely of the 3 forms to be confused with of fusional ability with prisms or the synoptophore.34 The
ANAET. This esodeviation is characterized by an acute 23 children with ANAET in this study were younger than
onset of a relatively large angle of comitant esotropia and most children who were reported to have an underlying
minimal hypermetropia. More recent articles7-9 on AACE abnormality of the central nervous system. Additionally,
have emphasized the benign nature of this disorder when the none of the patients in this study displayed nystagmus,
ophthalmologic and neurologic examination are otherwise divergence paralysis, incomitance, or other indicators of
unremarkable, although Legmann Simon and Borchert9 neurologic disease.
included several children with refractive esotropia and 1 with An important finding in this series of patients was that
an Arnold-Chiari type I malformation. 2 (10.5%) of the 19 operated children achieved 40 seconds
 Journal of AAPOS
88 Mohney Volume 5 Number 2 April 2001

of arc. Theoretically, patients who maintain normal motor thalmologists and primary care physicians to identify chil-
and sensory alignment during the first 6 months of life dren with esotropia early to maximize their fine motor and
should achieve bifoveal fixation if their strabismic angle is sensory potential.
corrected relatively soon after appearing.3,4 There may be The conclusions of this study suggest that ANAET
other postoperative patients in this study who attained occurs frequently and may be more prevalent than con-
bifoveal fixation but were too young to fully cooperate genital esotropia. This acquired form of childhood eso-
with sensory testing. The 2 children who read 40 seconds tropia is also far more common than AACE, as well as
of arc did so at 84 and 98 months of age. Patients with con- those conditions with a hidden neurologic or neoplastic
genital esotropia, on the other hand, have only rarely disorder, which tend to occur in older children and adults.
achieved bifixation.35-37 Parks36 and Wright et al37 have ANAET is a clinically distinct form of strabismus that typ-
presented the most convincing evidence that bifoveal fixa- ically begins between 1 and 5 years of age and appears to
tion is attainable in children with congenital esotropia, be infrequently associated with underlying disease. The
although these isolated reports emphasize its rarity. Ten of angle of deviation is relatively small but progressive and
the remaining 11 children who were within 8 PD of early surgical correction is more likely to achieve bifoveal
orthotropia (and attended follow-up for a minimum of 6 fixation than for those children with congenital esotropia.
months after their last surgical procedure) demonstrated a
range of stereopsis from 3000 to 80 seconds of arc. References
The findings of this study should be viewed in light of
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3. von Noorden GK. Binocular vision and ocular motility. St Louis:
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Mosby; 1985.
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12. Anderson WD, Lubow M. Astrocytoma of the corpus callosum pre-
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Journal of AAPOS
Volume 5 Number 2 April 2001 Mohney 89

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An Eye on the Arts – The Arts on the Eye

The eye is drawn, when it is desired to represent the individual in his total-
ity. The eye is the person himself, and possession of the eye gives control over
the whole energy of the owner and power over his life. A legend of the Tlingit
Indians relates how a man gouged out an eye of everyone he met and affixed it
to the rim of his boat. He did not need to fear any of those people any more,
but rather exercised full power over them.
—A. R. Pope (from The Eros Aspect of the Eye p 6)