Incidence and Types of Childhood Esotropia

A Population-Based Study
Amy E. Greenberg, BS,1 Brian G. Mohney, MD,2 Nancy N. Diehl, BS,3 James P. Burke, PhD4

Objective: To describe the incidence and types of childhood esotropia in a defined population.
Design: Retrospective population-based cohort.
Participants: All pediatric (⬍19 years of age) residents of Olmsted County, Minnesota, diagnosed with an
esodeviation (ⱖ10 prism diopters) from January 1, 1985, through December 31, 1994.
Methods: The medical records of all potential patients identified by the resources of the Rochester Epide-
miology Project were reviewed.
Main Outcome Measures: Incidence and types of childhood esotropia.
Results: Three hundred eighty-five cases of childhood esotropia were identified during the 10-year period,
yielding an annual age- and gender-adjusted incidence of 111.0 (95% confidence interval, 99.9 –122.1) per
100 000 patients younger than 19 years of age. This rate corresponds to a cumulative prevalence of approxi-
mately 2.0% of all children younger than 6 years, with a significant decrease in older ages (P⬍0.0001). Of the 385
study children, the specific forms and percentages of esotropia diagnosed were as follows: fully accommodative,
140 (36.4%); acquired nonaccommodative, 64 (16.6%); esotropia associated with an abnormal central nervous
system, 44 (11.4%); partially accommodative, 39 (10.1%); congenital, 31 (8.1%); sensory, 25 (6.5%); paralytic, 25
(6.5%); undetermined, 13 (3.4%); and other, 4 (1.0%).
Conclusions: The incidence of childhood esotropia from this self-referred population-based study is com-
parable with prevalence rates reported among Western populations. Esotropia is most common during the first
decade of life, with the accommodative and acquired nonaccommodative forms occurring most frequently. The
congenital, sensory, and paralytic forms of childhood esotropia were less common in this population.
Ophthalmology 2007;114:170 –174 © 2007 by the American Academy of Ophthalmology.

Esotropia is a disorder of ocular alignment characterized by
an inward deviation of the eyes. Although recently reported
to occur less frequently than exotropia in Asians,1,2 esode-
viations are much more common than exodeviations among
Western populations.3,4 However, existing epidemiologic
reports of childhood esotropia are almost exclusively stud-
ies of prevalence, often by nonophthalmic specialists, and
provide little information on the true population-based in-
cidence of this disorder.2–12 The primary objective of this
study was to determine the incidence and types of esotropia
among patients younger than 19 years diagnosed during a
Originally received: March 14, 2006.
Accepted: May 31, 2006. Manuscript no. 2006-328.
1
Mayo Clinic College of Medicine, Mayo Clinic and Mayo Foundation,
Rochester, Minnesota.
2
Department of Ophthalmology, Mayo Clinic and Mayo Foundation,
Rochester, Minnesota.
3
Division of Biostatistics, Mayo Clinic and Mayo Foundation, Rochester,
Minnesota.
4
Division of Epidemiology, Mayo Clinic and Mayo Foundation, Roches-
ter, Minnesota.
Supported in part by an unrestricted grant from Research to Prevent
Blindness, Inc., New York, New York.
Correspondence to Brian G. Mohney, MD, Department of Ophthalmology,
Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905. E-mail:
mohney@mayo.edu.
10-year period using a population-based medical record
retrieval system.
Patients and Methods
The medical records of all patients younger than 19 years of age
who were residing in Olmsted County, Minnesota, and were di-
agnosed with esotropia from January 1, 1985, through December
31, 1994, were reviewed retrospectively. Potential cases of esotro-
pia were identified by using the resources of the Rochester Epi-
demiology Project, a medical records linkage system designed to
capture data on any patient–physician encounter in Olmsted
County, Minnesota.13,14 The racial distribution of Olmsted County
residents in 1990 was 95.7% white, 3.0% Asian American, 0.7%
African American, and 0.3% each for Native American and other.
The population of this county (106 470 in 1990) is relatively
isolated from other urban areas, and virtually all medical care is
provided to residents by the Mayo Clinic or Olmsted Medical
Group and their affiliated hospitals. Unaffiliated area optometrists
rarely were the sole provider of eye care to children with strabis-
mus during the study years of this investigation.15
Institutional review board approval was obtained for this study.
We identified the medical records of all patients 0 through 18 years
of age who were diagnosed by an ophthalmologist as having any
form of esotropia or unspecified strabismus from 1984 through
1999. We also obtained the records of pediatric patients who were,
during the same interval, diagnosed with cranial nerve palsies,
retinal disorders, cataract, corneal scarring, optic nerve disorders,

170 © 2007 by the American Academy of Ophthalmology ISSN 0161-6420/07/$–see front matter
Published by Elsevier Inc. doi:10.1016/j.ophtha.2006.05.072
 Greenberg et al 䡠 Incidence and Types of Childhood Esotropia
Figure 1. Incidence rates of childhood esotropia by age in Olmsted
County, Minnesota, from 1985 through 1994 (3-year running average).
and developmental delay to avoid missing any potential cases of
secondary esotropia. All diagnoses were entered into the Rochester
Epidemiology Project database by trained personnel who reviewed
the entire medical record. Trained residency checkers verified the
patients’ residency status at the time of birth and at diagnosis using
information from city and county directories.
Ten (0.7%) of the 1530 potential patients declined research
authorization; the records of the remaining 1520 patients were
reviewed. Children not residing in Olmsted County at the time of
their diagnosis were excluded. All remaining patients diagnosed
with an intermittent or constant esotropia of ⱖ10 prism diopters
(PD; at either distance or near in the primary position) during the
study period were included. The angle of deviation was determined
by the prism-alternate cover test at both distance and near for most
patients, although the Krimsky test was necessary in infants, in
some young children, and in those with neurologic deficits. A
cycloplegic refraction was performed in all patients, the vast
majority receiving 1 to 2 drops of 1% cyclopentolate 30 minutes or
more before retinoscopy. The entire medical record of those diag-
nosed with esotropia was reviewed carefully for any developmen-
tal, neurologic, or ocular disorder that might have caused or been
associated with the esotropic deviation.
The following criteria were used to define the various types of
childhood esotropia.16 The congenital form, also known as infan-
tile or essential infantile esotropia, included neurologically intact
children with a constant nonaccommodative esotropia that devel-
Table 1. Incidence of Childhood Esotropia in Olmsted County, Minnesota, 1985 through 1994
Female Incidence Rates
(per 100 000 Female
Age Group (yrs) Patients <19 yrs)
0–1 342.3
2–3 284.1
4–5 220.9
6–7 78.0
8–9 43.3
10–11 0 23.2
12–13 27.4
14–15 14.1
16–17 6.8
18 30.5
Total 116.6
Age- and gender-adjusted total
oped by 6 months of age. Accommodative esotropia was diag-
nosed in those patients with an acquired constant or intermittent
deviation that was corrected or reduced 10 PD or more after
wearing hyperopic spectacles full time for at least 3 weeks. Fully
accommodative esotropia included patients whose deviation re-
duced to ⱕ8 PD, whereas partially accommodative esotropia des-
ignated those with a residual deviation of 10 PD or more. Acquired
nonaccommodative esotropia included children whose deviation
developed after 6 months of age and was not associated with
accommodative effort. The finding of a nonaccommodative esotro-
pia in an older child with an imprecise date of onset was classified
as an unknown form of esotropia. Esotropic children with a de-
velopmental or neurologic disorder were grouped under CNS
defects regardless of the age at onset or form of esotropia. Because
it was impossible to correlate CNS disease severity with the
development of strabismus, any form of neurologic impairment
except isolated speech delay was included in this category. Sen-
sory esotropia included patients with a unilateral or bilateral ocular
condition that prevented normal fusion. Other forms of esotropia
included any esotropia not classified by the above groups. Post-
operative consecutive esotropia was not included in this series
because it is not a naturally occurring form of strabismus. No
patient was placed in more than 1 diagnostic group.
Annual age- and gender-specific incidence rates were constructed
using the age- and gender-specific population figures for Olmsted
County, Minnesota, from the United States census. Age- and
gender-specific denominators for individual years were generated
from linear interpolation of the 1970, 1980, 1990, and 2000 census
figures. The 95% confidence intervals were calculated with as-
sumptions based on the Poisson distribution. Trends over time, by
age, and between genders were investigated using Poisson regres-
sion models.
Results
Three hundred eighty-five new cases of childhood esotropia were
identified in Olmsted County, Minnesota, during the 10-year study
period. There were 176 female (45.7%) and 209 male (54.3%)
incident cases. The age- and gender-adjusted annual incidence rate
for esotropia was 111.0 per 100 000 patients younger than 19 years
of age (Table 1), with a significant decrease in older ages
(P⬍0.0001), as shown in Figure 1. Although boys (118.4 per
100 000) were more likely than girls (103.2 per 100 000) to be
diagnosed with esotropia, the difference was not statistically sig-
nificant (P ⫽ 0.198), nor were there significant gender differences
among the various forms of esotropia (P ⫽ 0.369). The number of
Male Incidence Rates Total Incidence Rates
(per 100 000 Male (per 100 000 Patients
Patients <19 yrs) <19 yrs)
350.8 346.7
351.5 317.8
251.2 236.5
107.4 93.2
17.0 29.5
12.0
46.5 37.1
13.0 13.5
7.0 6.9
29.3 29.9
132.2 124.6
111.0
171
 Ophthalmology Volume 114, Number 1, January 2007

Table 2. Cumulative Incidence of Childhood Esotropia by Table 4. Disorders Contributing to Documented Central
Age at Diagnosis in Olmsted County, Minnesota, 1985 Nervous System Impairment among 44 Children with Esotropia
through 1994
Central Nervous System Disorder Number
Age Group (yrs) Cumulative Incidence (%) Cumulative (n)
Cerebral palsy 9
0–⬍1 0.38 63 Developmental delay 8
0–1 0.74 122 Down syndrome 8
0–2 1.09 179 Seizure disorder 6
0–3 1.42 234 Encephalopathy 2
0–4 1.70 279 Microcephaly 2
0–5 1.94 318 Agenesis of corpus callosum 1
0–6 2.07 340 Ataxia-telangectasia 1
0–7 2.13 350 Fibrillary astrocytoma, grade III 1
0–8 2.17 356 Hanhart’s syndrome 1
0–9 2.19 360 Mosaic trisomy 16 1
0–10 2.20 362 Motor-tic disorder 1
0–11 2.22 364 Pseudotrisomy 18 1
0–12 2.25 369 Supernumary pseudoisodicentric chromosome 15 1
0–13 2.28 375 Williams syndrome 1
0–14 2.31 379
0–15 2.31 379
0–16 2.31 380
0–17 2.32 381 with Duane’s retraction syndrome or a sixth cranial nerve palsy
0–18 2.34 385 (Table 6). Congenital esotropia was diagnosed in 31 children,
corresponding to an incidence of 8.5 per 100 000. The 4 remaining
cases of other esotropia included 2 patients with late sudden-onset
children diagnosed with esotropia was similar throughout the esotropia, and 1 patient each with cyclic esotropia and post-scleral
10-year period. buckle esotropia.
Table 2 shows the cumulative incidence rates by age calculated
from our incidence data. Although the cumulative incidence
steadily increases through age 18 years, the vast majority of Discussion
patients were diagnosed by age 5 years, corresponding to a prev-
alence of approximately 2.0%. The additive nature of Table 2
assumes no spontaneous resolution of the esotropias diagnosed in This study provides population-based data on the incidence
this study. of childhood esotropia from an upper Midwest population
Table 3 shows the relative frequencies and incidence rates for of the United States. We found an annual age- and gender-
the various types of childhood esotropia. Accommodative (both adjusted incidence of 111.0 (95% confidence interval, 99.9 –
fully and partially) esotropia was the most frequently diagnosed 122.1) per 100 000 patients younger than 19 years. The
form of esotropia, comprising 46.5% of all study patients with an incidence rate was highest in the first 5 years of life, with a
incidence rate of 54.3 per 100 000 patients younger than 19 years. prevalence or cumulative incidence of 1.94%. New cases of
Fourteen (10%) of the 140 patients with fully accommodative esotropia significantly decreased with an increase in age.
esotropia later required horizontal strabismus surgery for a decom- There was no significant difference in the incidence be-
pensating angle at a mean of 4.9 years after their initial diagnosis.
Acquired nonaccommodative esotropia was found in 64 children
tween the sexes, nor was there a significant change in the
(16.6%), corresponding to an incidence of 18.6 per 100 000. The incidence rate over the 10-year study period.
specific CNS defects associated with 44 of the esotropic children Published studies on the prevalence of strabismus have
are shown in Table 4, whereas the causes of sensory esotropia in included preverbal children,5–7 children 6 to 7 years of
the 25 children with this condition are shown in Table 5. Paralytic age,4,8 –11 preadolescents,2,12 and a more comprehensive re-
esotropia, diagnosed in 25 patients, primarily included children view of children between birth and 19 years.3 However, these

Table 3. Observed Types of Esotropia among 385 Consecutive Patients Younger than 19 Years in
Olmsted County, Minnesota, 1985 through 1994

Incidence per 100 000 Patients <19

Esotropia Type Number % years (Confidence Interval)*
Fully accommodative 140 36.4 40.4 (33.7–47.1)
Acquired nonaccommodative 64 16.6 18.6 (14.0–23.2)
Abnormal central nervous system 44 11.4 12.5 (8.8–16.2)
Partially accommodative 39 10.1 10.9 (7.5–14.4)
Congenital 31 8.1 8.5 (5.5–11.6)
Paralytic 25 6.5 7.2 (4.4–10.2)
Sensory 25 6.5 7.2 (4.4–10.0)
Unknown 13 3.4 4.1 (1.8–6.3)
Other 4 1.0 1.4 (0–2.8)

*Age- and gender-adjusted 95% confidence interval.

172
 Greenberg et al 䡠 Incidence and Types of Childhood Esotropia

Table 5. Causes of Sensory Esotropia among 25 Children described a prevalence rate of 2.28% in a population-based
study of 1582 children aged 8 to 9 years in Northern Ireland.
Ocular Defect Number We found a similar cumulative incidence rate of 2.19% in
Anisometropic amblyopia 13 children younger than 10 years. In patients up to the age of
Cataract, bilateral (n ⫽ 3) or unilateral (n ⫽ 1) 4 12 years, our study reports a cumulative esotropia incidence
Combined hamartoma of the retina and retinal pigment 1 of 2.25%. A recent study of Japanese children of a similar
epithelium, unilateral
Corneal scar secondary to trauma, unilateral 1 age, however, reported a much lower prevalence of 0.28%.2
Ocular albinism 1 This discrepancy may be explained by a number of factors.
Optic nerve anomaly (congenital), unilateral 1 An incomplete return rate of the study questionnaire elim-
Optic nerve coloboma, bilateral 1 inated one fourth of the eligible population. Additionally, a
Persistent fetal vasculature, unilateral 1
Retinopathy of prematurity (stage V), unilateral 1
distinction between esotropia and exotropia was not possi-
Vitreous hemorrhage from proliferative diabetic 1 ble in 22% of the children with known strabismus. Finally,
retinopathy, bilateral recent reports have demonstrated that esotropia is far less
common than exotropia among Asian communities, the
reverse of which has been reported for Western populations.
reports are almost exclusively studies of prevalence, often by Frandsen’s3 comprehensive report on childhood strabis-
nonophthalmic specialists, and provide little information on the mus, based on data from 16 046 children in nurseries,
true population-based incidence of this disorder. Despite these schools, and mental institutions in Copenhagen during the
limitations, the prevalence rates from these reports neverthe- 1950s, analyzed children between birth and 19 years of age.
less may be compared with the cumulative incidence values Frandsen reported an esotropia incidence of 4.1%, with an
calculated from this study (Table 2) by adjusting for the age at incidence of 3.5% for normal children and 9.0% for children
which the prior studies were conducted. with mental retardation. Our study reports a cumulative
Three reports, all from Western populations, have de- incidence of esotropia of 2.34% for children from birth to
scribed the prevalence of esotropia among preverbal chil- age 19 years. Although our incidence of childhood esotropia
dren.5–7 Kornder et al5 reported an esotropia prevalence of was approximately half the rate described by Frandsen, we
2.0% among 1074 Canadian children from 6 to 30 months found a nearly identical incidence of exotropia in the same
of age. A lower prevalence of 1.0% has been reported in population.15
larger cohorts of similarly aged patients from both Israel6 The findings from this study suggest that esotropia may
and Britain.7 This study found a cumulative incidence of be somewhat less prevalent in the Midwestern United States
1.09% in children younger than 3 years of age, comparable compared with Western Europe, in contrast to the preva-
with the findings of both the Israeli and British studies. The lence of exotropia, which seems to be nearly identical in the
higher rate described by Kornder et al is likely the result of 2 regions. However, esotropia prevalence among Asian
the preferential inclusion of children with a positive family populations is significantly lower than in either the United
history of strabismus. States or Europe. These differences in part may be the result
Most prevalence studies of strabismus focus on children of, as was recently suggested, refractive error differences
in the first year of primary school, in which esotropia is among populations in which esotropia is associated with
reported to occur in 1.50% to 3.62% of that popula- hyperopia and exotropia is associated with myopia.1,17
tion.4,8 –11 Kvarnström et al8 describe the lowest rate This study also reports the relative incidence of various
(1.50%) based on their analysis of 3126 Swedish children types of childhood esotropia. Congenital or infantile esotro-
younger than 8 years who were identified by a national pia, often considered the most common form of childhood
screening program. Our cumulative incidence of 2.13% esotropia, was relatively uncommon in this population. Al-
(Table 2) for patients between birth and age 7 years falls though congenital esotropia remains preeminent, this and
between the Swedish study and Kornder et al’s prevalence other recent epidemiologic reports of childhood esotro-
rate of 2.7%.9 However, as noted above, the Canadian pia1,12,16,18 from Western and Asian populations confirms
findings may be artificially elevated because the investiga- the relatively infrequent occurrence of congenital esotropia
tors focused their screening on those with a family history and the predominance of the accommodative form. Accom-
of strabismus. Chew et al10 reported an esotropia prevalence modative esotropia, including both the partially and fully
of 3.0% among 39 227 children born and evaluated through accommodative forms, comprises approximately one half
age 7 years in 1 of 12 United States university medical
centers. The study, however, is flawed by referral bias and the
exclusive reliance on the pediatrician or neurologist to make Table 6. Types of Paralytic Esotropia among 25 Children
the diagnosis of strabismus. Nevertheless, even higher preva- Ocular Defect Number
lence rates of approximately 3.50% have been reported from
Wales4 as well as in an earlier study from Sweden.11 Although Unilateral (n ⫽ 12) or bilateral (n ⫽ 1) Duane’s retraction 13
syndrome type I
the report from Wales described a prevalence of exotropia Unilateral (n ⫽ 5) or bilateral (n ⫽ 1) sixth cranial nerve 6
nearly identical to our findings for the same-aged children in palsy
Olmsted County,15 the prevalence of esotropia in Wales was Unilateral (n ⫽ 3) or bilateral (n ⫽ 1) Duane’s retraction 4
nearly twice the rate of our population. syndrome type III
The prevalence of esotropia among preadolescent children Aberrant regeneration of cranial nerve III 1
Myasthenia gravis 1
has been reported in 2 studies.2,12 Donnelly et al12 recently

173
 Ophthalmology Volume 114, Number 1, January 2007
of all pediatric esotropia in the United States. Acquired
nonaccommodative esotropia and esotropia in the setting of
an abnormal CNS each make up approximately 10% to 20%
of esotropic children, whereas the congenital, sensory, and
paralytic forms generally comprise less than 10% each.16
As with other reports on strabismus in this population,
there are several limitations to this study, the bulk of which
may result in an underestimation of the true incidence of
esotropia. Patients with an intermittent or small angle of
deviation may have gone unnoticed by the caretaker,
thereby avoiding an evaluation by the study ophthalmolo-
gists. A second potential weakness was the inability to
review the medical records of optometrists practicing within
the study region. However, a computer review of these
charts confirmed that these practices rarely evaluated pedi-
atric patients with strabismus. Additionally, some residents
of this population may have sought care outside Olmsted
County, although the relative isolation of this population
from other urban areas makes this occurrence rare.
This study is further limited by the region in which it was
performed. Because the ethnic composition of Olmsted
County was 95% white during the years of the study, our
results are best extrapolated to the semiurban white popu-
lations of the United States. Esotropia has been shown to be
less common among black and Asian populations compared
with white populations.1,2,10 Recognizing these weaknesses
and assuming a population of 67 224 000 citizens younger
than 19 years of age (1990 US Census), we estimate 74 619
new cases of childhood esotropia each year in the United
States. Nearly one half of these children presumably will
develop some form of accommodative esotropia.
The incidence of childhood esotropia derived from this
population-based study is similar to that of prior studies of
prevalence among Western populations. We found a cumula-
tive incidence of 2.34% for all children younger than 19 years
of age. Accommodative esotropia, acquired nonaccommoda-
tive esotropia, and esotropia in the setting of an abnormal CNS
were the most commonly diagnosed forms in this population.
References
1. Yu CB, Fan DS, Wong VW, et al. Changing patterns of
strabismus: a decade of experience in Hong Kong. Br J Oph-
thalmol 2002;86:854 – 6.
174
2. Matsuo T, Matsuo C. The prevalence of strabismus and am-
blyopia in Japanese elementary school children. Ophthalmic
Epidemiol 2005;12:31– 6.
3. Frandsen AD. Occurrence of squint: a clinical-statistical
study on the prevalence of squint and associated signs in
different groups and ages of the Danish population [disser-
tation]. Copenhagen: Munksgaard; 1960. Acta Ophthalmol
Suppl vol 62.
4. Graham PA. Epidemiology of strabismus. Br J Ophthalmol
1974;58:224 –31.
5. Kornder LD, Nursey JN, Pratt-Johnson JA, Beattie A. Detec-
tion of manifest strabismus in young children. 1. A prospec-
tive study. Am J Ophthalmol 1974;77:207–10.
6. Friedman Z, Neumann E, Hyams SW, Peleg B. Ophthalmic
screening of 38,000 children, age 1 to 2 1/2 years, in child
welfare clinics. J Pediatr Ophthalmol Strabismus 1980;17:
261–7.
7. Stayte M, Johnson A, Wortham C. Ocular and visual defects
in a geographically defined population of 2-year-old children.
Br J Ophthalmol 1990;74:465– 8.
8. Kvarnström G, Jakobsson P, Lennerstrand G. Visual screening
of Swedish children: an ophthalmological evaluation. Acta
Ophthalmol Scand 2001;79:240 – 4.
9. Kornder LD, Nursey JN, Pratt-Johnson JA, Beattie A. Detec-
tion of manifest strabismus in young children. 2. A retrospec-
tive study. Am J Ophthalmol 1974;77:211– 4.
10. Chew E, Remaley NA, Tamboli A, et al. Risk factors for esotro-
pia and exotropia. Arch Ophthalmol 1994;112:1349 –55.
11. Nordlow W. Squint—the frequency of onset at different ages,
and the incidence of some associated defects in a Swedish
population. Acta Ophthalmol (Copenh) 1964;42:1015–37.
12. Donnelly UM, Stewart NM, Hollinger M. Prevalence and
outcomes of childhood visual disorders. Ophthalmic Epide-
miol 2005;12:243–50.
13. Kurland LT, Molgaard CA. The patient record in epidemiol-
ogy. Sci Am 1981;245:54 – 63.
14. Melton LJ III. History of the Rochester Epidemiology Project.
Mayo Clin Proc 1996;71:266 –74.
15. Govindan M, Mohney BG, Diehl NN, Burke JP. Incidence and
types of childhood exotropia. A population-based study. Oph-
thalmology 2005;112:104 – 8.
16. Mohney BG. Common forms of childhood esotropia. Oph-
thalmology 2001;108:805–9.
17. Lambert SR. Are there more exotropes than esotropes in Hong
Kong? Br J Ophthalmol 2002;86:835– 6.
18. Mohney BG, Erie JC, Hodge DO, Jacobsen SJ. Congenital
esotropia in Olmsted County, Minnesota. Ophthalmology 1998;
105:846 –50.