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Amblyopia in Childhood Eyelid Ptosis
Amblyopia in Childhood Eyelid Ptosis
PURPOSE: To report the prevalence and causes of over a 40-year period while residing in Olmsted County,
amblyopia among children with ptosis diagnosed in Minnesota.
a well-defined population over a 40-year period.
DESIGN: Retrospective, population-based cohort study.
METHODS: We retrospectively reviewed the charts of
107 patients younger than 19 years for the prevalence METHODS
and causes of amblyopia who were diagnosed with child-
THE MEDICAL RECORDS OF 107 PATIENTS YOUNGER THAN
hood ptosis and were residents of Olmsted County,
19 years with childhood ptosis who were residents of
Minnesota, from January 1, 1965, through December
Olmsted County, Minnesota, and were diagnosed bet-
31, 2004.
ween January 1, 1965, and December 31, 2004, were
RESULTS: Amblyopia was diagnosed in 16 (14.9%) of
reviewed retrospectively. The inclusion criteria for study,
the 107 patients with childhood ptosis. Fourteen
incidence, and demographic data of these 107 patients
(14.6%) of 96 patients diagnosed with a congenital form
have been reported previously.12 Institutional review
of ptosis demonstrated amblyopia. Twelve (14.8%) of
board approval was obtained for this study. The cases
the 81 patients diagnosed with simple congenital ptosis
were identified using the resources of the Rochester
had amblyopia, 7 (8.6%) cases of which solely were the
Epidemiology Project, a medical record linkage system
result of eyelid occlusion of the visual axis. The causes
designed to capture data on any patient–physician
of amblyopia in the remaining 5 patients were significant
encounter in Olmsted County, Minnesota.13 The popula-
refractive error in 3 patients and strabismus in 2 patients.
tion of this county is relatively isolated from other urban
CONCLUSIONS: Amblyopia occurred in 1 in 7 children
areas, and virtually all medical care is provided to its resi-
diagnosed with ptosis in this population-based cohort. In
dents by Mayo Clinic, Olmsted Medical Group, and their
approximately half of those with amblyopia, or less than
affiliated hospitals. Patients not residing in Olmsted
10% of all patients, the disease solely was the result of
County at the time of their diagnosis with childhood
eyelid occlusion of the visual axis. (Am J Ophthalmol
ptosis were excluded from the study.
2013;155:1125–1128. Ó 2013 by Elsevier Inc. All
Amblyopia was defined as 2 lines or more difference
rights reserved.)
between the 2 eyes while wearing the proper prescription
or lack of central, steady, and maintained fixation in the
ptotic eye with presence of normal central, steady, and
A
MBLYOPIA IS THE MOST COMMON CAUSE OF
monocular vision loss in children.1,2 Among maintained fixation in the nonptotic eye. Each patient
patients with childhood ptosis, however, the diagnosed with amblyopia was managed by an ophthalmol-
incidence of amblyopia has been reported to be higher ogist (pediatric, oculoplastic, or comprehensive ophthal-
than that in the general population.3–11 Although this mologist). The initial and subsequent refractions were
disparity is thought to be the result of an increased determined in most patients after the topical administra-
prevalence of strabismus, significant refractive error, and tion of 1% cyclopentolate in younger patients and by
eyelid occlusion of the visual axis, none of the prior a manifest refraction for older patients. All refractions
reports are population based. The purpose of this study were converted into their spherical equivalent. Significant
was to report the prevalence and causes of amblyopia refractive error was defined as the presence of anisome-
among a cohort of 107 children diagnosed with ptosis tropia of at least a 1-diopter difference between the spher-
ical equivalents of each eye, hyperopia of 3 diopters or
more, or astigmatism of 1 diopter or more. Amblyopia as
a result of strabismus was diagnosed in those with an inter-
Accepted for publication Dec 19, 2012.
From the Department of Ophthalmology, Mayo Clinic and Mayo mittent or constant horizontal deviation of at least 10 prism
Foundation, Rochester, Minnesota (G.J.G., B.G.M.); and the Division diopters or a vertical deviation of at least 2 prism diopters
of Biostatistics, Mayo Clinic and Mayo Foundation, Jacksonville, Florida and in whom no other cause for decreased vision was
(N.D.).
Dr Griepentrog is currently at the Section of Oculofacial and Orbital apparent. If the examination results met the above criteria
Surgery, Department of Ophthalmology, Medical College of Wisconsin, for both significant refractive error and strabismus, the
Milwaukee, Wisconsin. cause of amblyopia was listed as ‘‘combination.’’ Cases of
Inquiries to Brian G. Mohney, Department of Ophthalmology, Mayo
Clinic, 200 First Street Southwest, Rochester, MN 55905; e-mail: amblyopia secondary to eyelid occlusion of the visual axis
mohney@mayo.edu alone, without frontalis muscle recruitment or chin-up
Ptosis Cause No. (%) Occlusion (Ptosis) Strabismus Refractive Error Total
Congenital
Simple congenital ptosis 81 (75.7) 7 (8.6)a 2 (2.5) 3 (3.7) 12 (14.8)
Blepharophimosis 3 (2.8) 0 0 0 0
Congenital CN III palsy 3 (2.8) 0 2 (66.6) 0 2 (66.6)
Marcus-Gunn jaw wink 3 (3.8) 0 0 0 0
Congenital Horner syndrome 2 (1.9) 0 0 0 0
Central core myopathy 1 (0.93) 0 0 0 0
CFEOM 1 (0.93) 0 0 0 0
Myotonic dystrophy 1 (0.93) 0 0 0 0
Noonan syndrome 1 (0.93) 0 0 0 0
Acquired
Aponeurotic dehiscence 4 (3.7) 0 0 1 (25) 1 (25)
Acquired CN III palsy 2 (1.9) 0 0 0 0
Acquired Horner syndrome 2 (1.9) 0 0 0 0
Traumatic structural ptosis 2 (1.9) 0 0 0 0
Childhood myasthenia gravis 1 (1.9) 0 1 (100) 0 1 (100)
Total 107 7 (6.5) 5 (4.6) 4 (3.7) 16 (14.9)
were diagnosed with amblyopia, 16 (12.3%) cases of with blepharophimosis-ptosis-epicanthus inversus syn-
which were the result of strabismus and 9 (6.9%) cases of drome at birth with mild unilateral ptosis had no signs of
which were the result of occlusion.7 More recently, in amblyopia by age 14 years.
a study of 92 patients with congenital ptosis, 22 (23.9%) There are several limitations to the findings in this
of patients were diagnosed with amblyopia, with almost study. Although relatively isolated, some residents of
every case occurring in the context of coexisting anisome- Olmsted County with ptosis may have sought care
tropia or strabismus.11 The same authors note that congen- outside the region, potentially underestimating the inci-
ital ptosis patients are at risk of developing anisometropic dence of amblyopia in this population. Further, the pres-
and strabismic amblyopia even if not originally detected, ence of a compensatory head tilt has been correlated
and routine monitoring that includes regular cycloplegic with a high incidence of amblyopia in patients with
refractions is recommended.11 congenital ptosis in the absence of significant anisome-
All cases of amblyopia occurred in patients with unilat- tropia and strabismus.7,24 In the current study, there
eral ptosis, with the exception of 1 patient with childhood were recording inconsistencies regarding the presence
myasthenia gravis. The child had bilateral ptosis with or absence of this pertinent finding during the 40-year
symmetric palpebral fissure heights (4 mm) and 30 prism period. Because of the young age of patients in the study
diopters of exotropia. Because of the significant degree of (median age at diagnosis for simple congenital ptosis, 1.3
exotropia, strabismus was recorded as the cause of ambly- years; range, 32 days to 16.7 years),12 we were unable to
opia, although it has been speculated elsewhere that ptosis assess stereoacuity to identify amblyopia indirectly, thus
may be the initial cause of amblyopia that leads to stra- potentially underestimating the overall prevalence of
bismus.7 amblyopia. Finally, the ability to generalize the findings
Three of the 96 patients with congenital ptosis in the of the current report is limited by the demographics of
current study were diagnosed with blepharophimosis- Olmsted County, a relatively homogeneous semiurban
ptosis-epicanthus inversus syndrome.12 Although ambly- white population.
opia was not noted in this small group, the rate of amblyopia The findings of this study provide population-based prev-
in patients with blepharophimosis-ptosis-epicanthus inver- alence rates for amblyopia in childhood ptosis diagnosed
sus syndrome has been reported previously to be as high as over a 40-year period. Amblyopia affected approximately
56.4%, and early surgery is recommended.23 Two of the 3 1 in 7 patients diagnosed with childhood ptosis, of which
patients underwent surgery at a mean age of 54.4 months nearly half solely were the result of eyelid occlusion of
(range, 44.4 to 64.4 months), whereas 1 patient diagnosed the visual axis.