Evaluation and surgical outcome of acquired

nonaccommodative esotropia among older children

Bo Li, MD, Sapna Sharan, FRCSC
ABSTRACT ●
Objective: To describe the presentation, clinical evaluation, work-up, surgical management, and surgical outcomes in children older
than 8 years with spontaneous, comitant, acquired nonaccommodative esotropia (ANAET).
Design: Retrospective chart review.
Participants: Children who underwent bilateral medial rectus recession surgery for ANAET with initial esotropia onset later than
8 years of age.
Methods: The medical records of children older than 8 years presenting with ANAET from 2009 to 2015 were retrospectively
reviewed. The clinical presentation, work-up, surgical intervention, preoperative and postoperative deviations, and surgical
outcomes were recorded.
Results: A total of 7 healthy patients were identified. The average age of onset was 11.9 years. All patients presented with
symptoms of diplopia with large-angle esotropia. Most patients had no preceding illness and presented with minimal refractive
error. All 7 patients had unremarkable neurological and general pediatric evaluations without findings of acute intracranial
pathology on neuroimaging. Bilateral medial recession surgery was performed for all 7 patients with resolution of diplopia and
excellent stereopsis postoperatively.
Conclusions: Diplopia is the most common presenting symptom among older children presenting with ANAET. Bilateral medial
recession surgery achieved excellent postoperative results with resolution of diplopia and excellent stereopsis.

Acquired nonaccommodative estropia (ANAET) is a type
of strabismus characterized by constant and comitant eso-
deviation that presents after 6 months of age without a
significant refractive component.1–3 ANAET is considered
either the second or third most common type of eso-
deviation, followed by accommodative or infantile esotro-
pia.2,4 However, the prevalence of spontaneous ANAET
among older children (after 5 years of age) remains
unknown and is considered rare.5,6
To date, most published reports on ANAET have
focused on younger children. Mohney described 23
children with ANAET at a median age of 31.4 months
and concluded that ANAET typically presents between
1 and 5 years of age.7 In 2011, Jacob et al. reported the
largest series of 174 children with ANAET and a mean age
of onset at 3 years.8 Kothari reported 15 patients with
ANAET who presented at an average age of 7.2 years.5
However, most of their patients initially presented at a
much younger age; therefore, limited insights can be
drawn for older children presenting with ANAET.
We reviewed the presentation, clinical characteristics, work-
up, surgical management, and outcomes in a case series with
children older than 8 years presenting with spontaneous
ANAET. To our knowledge, this is the first report to examine
ANAET exclusively among older children.
SUBJECTS AND METHODS
The medical records of children older than 8 years
presenting with spontaneous, comitant ANAET to a single
pediatric ophthalmologist (S.S.) at the Ivey Eye Institute,
University of Western Ontario (London, Ont.) from 2009
to 2015 were reviewed retrospectively. Research ethics
approval was obtained from University of Western
Ontario Health Science Research Ethics Board (HSREB
No. 106985). It was believed that older children with
ANAET were presenting in the clinic more frequently
than had been noted in the past years in the same clinic.
Patients with history or findings of other types of
esotropia, such as accommodative, refractive, partially
accommodative, or infantile esotropia, or who first pre-
sented with ANAET before the age of 8 years were
excluded. The following information was collected from
patient charts: age of onset, sex, duration of symptoms,
patient symptoms, medical history, ocular history, family
history, general pediatric and neurological assessment,
presence of preceding illness, cycloplegic refraction, inves-
tigations performed, preoperative and postoperative devia-
tion at near and distance, surgery performed, and
preoperative and postoperative stereopsis. Intracranial
anomalies and thyroid eye disease were ruled out.
RESULTS
A total of 7 cases (5 males and 2 females) of sponta-
neous ANAET in children older than 8 years were
identified. Detailed patient information is listed in
Table 1. The average age of symptom onset was 11.9
years. All patients had symptoms of sudden onset of

& 2017 Canadian Ophthalmological Society.

Published by Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.jcjo.2017.07.021
ISSN 0008-4182/17

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ANAET among older children—Li et al.

Table 1—Clinical findings and surgical outcome of older children presenting with ANAET

Age of Preop Postop Postop

Onset Duration of Deviation Deviation Fusion
Pt (y)/ Symptoms Presenting Cycloplegic MRI (Prism Surgery and Clinical Status at 1/ Postop
No. Sex (mo) Symptoms MHx/Preceding Illness Refraction Findings Diopter) Performed Outcome 3m/6m Stereopsis
1 11/M 1 Diplopia Healthy/URTI þ1.0 OU Normal LET 45 BMR E3, no BSV/BSV 40 secs of
6.5 mm diplopia arc
2 9/F 6 Diplopia Healthy/none þ1.0 OU Normal RET 20 BMR E2, no BSV/? 40 secs of
5.0 mm diplopia diplopic arc
3 17/M 1 Diplopia Depression, ADHD/none OD: −8.25 Normal RET 35 BMR RET14, no Diplopia/ None
to 0.25 × 6.0 mm diplopia right
080 OS: suppression
−7.25 to
0.75 × 105
4 12/M Revealed only Diplopia Central auditory þ0.5 OU Normal, ET 18 BMR E(T)8, no BSV/BSV 40 secs of
in clinic that processing disorder, possible 3.5 mm diplopia arc
diplopia chronic left ptosis, ADHD, previous
experienced chromosomal copy left
for several number variation at thalamus
months 1q21.2 locus/none infarct
5 13/F 10 Diplopia Healthy/none -0.5 OU Normal ET 35 BMR E8, no BSV/BSV 40 secs of
5.5 mm diplopia arc
6 10.5/M 6 Headache Healthy/none þ1.0 OU Normal E(T) 20 BMR Orthophoria, BSV/BSV 40 secs of
and 4.0 mm no headache arc
diplopia or diplopia
7 11/M 6 Headache Healthy/none OD: plano Normal E(T) 18 BMR E(T)4, no Diplopia/ 40 secs of
and −1.0 × 90 3.5 mm headache or BSV arc
diplopia OS: plano diplopia
−1.0 × 90

Pt, patient; MHx, medical history; URTI, upper respiratory tract infection; ADHD, attention-deficit hyperactivity disorder; ET, esotropia; E(T), intermittent esotropia; BMR, bilateral medial rectus
recession; E, esophoria; BSV, binocular single vision.

diplopia at disease onset. Two patients experienced
intermittent headaches in addition to symptoms of dip-
lopia. The duration of symptoms ranged from 1 to 10
months. One patient was not able to identify the duration
of symptoms because he did not voluntarily disclose his
symptoms of diplopia to his parents or pediatrician; in
fact, his parents realized only in the clinic that he was
experiencing diplopia. The majority (5 out of 6 patients)
of the other patients presented within 6 months of
symptom onset.
Five of the 7 patients were previously healthy without any
identifiable medical or ocular history. Patient 3 had depres-
sion and attention-deficit hyperactivity disorder. Patient 4 had
chromosomal copy number variation at the 1q21.2 locus of
unknown significance, as well as central auditory processing
disorder, chronic left ptosis, and attention-deficit hyperactivity
disorder. Before the onset of ANAET, only 1 patient had
experienced preceding illness in the form of a mild upper
respiratory tract infection. Six patients had no identifiable
family history of strabismus or other ocular pathologies.
Patient 3 had an older sibling who had required strabismus
surgery at a young age.
All 7 patients had unremarkable anterior and posterior
segment examinations. Six patients had minimal refractive
error with a spherical equivalent of no more than 1.0 diopter
of either hypermetropia or myopia, and all 6 patients had
uncorrected or corrected vision of 20/20 OU or better.
Patient 3 showed high myopia with best-corrected vision of
20/40 OD and 20/25 OS. Preoperative strabismus evaluation
showed that all 7 patients showed comitant deviation with
identical measurements at distance and near. Five patients
showed manifest esotropia, and 2 patients demonstrated
intermittent poorly controlled esotropia. The average meas-
ured esotropia was 26 prism diopters, and the range of
measured deviation was 18–45 prism diopters. No inferior
oblique muscle or superior oblique muscle overaction,
nystagmus, or dissociated vertical deviation was found in
any of these patients.
Magnetic resonance imaging (MRI) of the brain was
performed in all patients to rule out any intracranial
abnormalities. Six of the 7 patients had completely unre-
markable MRI studies, whereas patient 4 demonstrated
chronic changes over the left thalamus, suggesting possible
previous ischemic infarct. Blood work, including thyroid
function tests, was carried out at the discretion of the general
pediatrician. All 7 patients had unremarkable blood work
results.
All 7 patients underwent strabismus surgery to correct
ANAET. Bilateral medial recessions ranged from 3.5 to
6.5 mm and were performed by a single pediatric
ophthalmologist (S.S.). The amount of bilateral medial
recessions was guided based on the amount of presenting
deviation. Postoperatively, all 7 patients had complete
resolution of symptoms of diplopia. The 2 patients who
presented with intermittent headaches also had resolution
of headaches. Postoperative measurement performed
2 months after surgery demonstrated that all 7 patients
had significant reduction in their measured deviation.
Four patients had residual deviation less than 5 prism
diopters. Patient 3 had the largest residual esotropia at 14
prism diopters. There was no measured consecutive exo-
deviation. Six of the 7 patients regained excellent stereopsis

2 CAN J OPHTHALMOL — VOL. ], NO. ], ] 2017


at 40 seconds of arc. Patient 3 failed to demonstrate
stereopsis both preoperatively and postoperatively.
DISCUSSION
Among different types of eso-deviations, ANAET is
considered less common than accommodative and refrac-
tive forms of eso-deviation.2–4 Furthermore, ANAET is
considered even rarer among older children. As a result,
there is a lack of published reports on ANAET in this age
group. Here we report a series of 7 children with
spontaneous onset of ANAET at more than 8 years of age.
What is unique about ANAET among older children is
that all patients in our series reported subjective symptoms of
diplopia with or without headaches; subjective reports are
more difficult to elicit from younger children, given the rapid
onset of suppression, the patient’s inability to articulate the
symptoms, and/or recall bias. For example, the parents of
patient 2, patient 4, and patient 6 in our study were surprised
when they heard about symptoms of diplopia from their
children for the first time in clinic. The parents had simply
brought their children to their primary care physicians for the
“sudden eye turn.” Therefore, careful and skilful questioning
for the symptoms of diplopia and headache is essential while
obtaining history from older children and their parents with
spontaneous presentation of strabismus.
The other important point for the management of
ANAET among older children is the favourable surgical
outcome.8 Given the nature of late-onset ANAET, most
children presenting at an older age have already established
binocular single vision and stereopsis before the onset of
ANAET. As a result, they are excellent surgical candidates
with good surgical prognosis for binocular vision and
stereopsis. As seen in our series, although all patients lost
stereopsis with the onset of ANAET, all but 1 patient had
complete recovery of stereopsis to 40 seconds of arc
postoperatively; the only patient who failed to demonstrate
stereopsis postoperatively probably had prior eye misalign-
ment with limited or no stereopsis before the onset of
ANAET. Excellent surgical outcomes were achieved in our
series with bilateral medial recession surgery, with 4 out of
7 patients achieving manifest or latent deviation of less
than 5 prism diopters. Only 1 patient remained with a
residual deviation of more than 10 prism diopters. The
symptoms of diplopia with or without headaches resolved
in all patients. The excellent postoperative alignment and
stereopsis measurement and 100% resolution rate of
patients’ subjective symptoms highlight the importance
and favourable prognosis of surgical intervention in this
patient population.
In the literature, ANAET has been categorized into
5 different subtypes: type 1 (Swan)—esotropia caused by
disruption of fusion; type 2 (Burian-Francesschetti)—
esotropia associated with physical or psychological stress
and minimal hypermetropia; type 3 (Bielchowsky)—
esotropia associated with high myopia in adults; type 4
ANAET among older children—Li et al.
—esotropia associated with high hypermetropia that can
be controlled with refractive correction; type 5—esotropia
associated with intracranial pathology.5,9–13 Based on this
categorization, 5 patients fit into type 2 ANAET, patient
3 can be considered type 3 ANAET given his high myopia
and older age, and patient 4 would be considered
indeterminate given the possible thalamus lesion shown
on MRI imaging. Patient 3 had the largest residual eso-
deviation despite a bilateral medial recession surgery of
6.0 mm. This poses the question whether type 3 ANAET
has a different response to strabismus surgery compared
with type 2 ANAET and whether the amount and/or type
of surgical correction needs to be modified or augmented
accordingly.
The association between ANAET and intracranial pathol-
ogy, especially posterior fossa tumour, has been well docu-
mented.13–18 In our report, none of the patients had any
acute neuroimaging findings, and 1 patient had chronic
findings suggestive of a previous ischemic lesion. Despite the
apparent low yield, we would still advocate for careful clinical
examination for red flags of intracranial lesions, such as sixth
nerve palsy and papilledema, and routine neuroimaging with
MRI as part of the preoperative work-up and assessment.
In conclusion, spontaneous onset of ANAET among
older children is a rare form of eso-deviation that has
excellent surgical and functional prognosis. Careful history
taking for symptoms of diplopia and headaches and
clinical and neuroimaging work-up for other potential
causes of the deviation are essential before surgical
management.
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Footnotes and Disclosure:
The authors have no proprietary or commercial interest in any
materials discussed in this article.
From the Ivey Eye Institute, Department of Ophthalmology,
Western University, London, Ont.
Originally received May. 10, 2017. Final revision Jul. 7, 2017.
Accepted Jul. 20, 2017.
Correspondence to Bo Li, MD, Ivey Eye Institute, St Joseph’s
Health Care, 268 Grosvenor Street, London, Ont. N6A 4V2;
bolihere@gmail.com

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