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Amer. Orthoptic Jrnl. 59(1):76-83 (2009); doi:10.3368/aoj.59.1.76
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Refractive and Ocular Motility Findings in Children with Epidermolysis Bullosa

Kate A. V. Smith, B.Sc. (Hons) Orthoptics, Sophie M. Jones, M.R.C.Ophth. and Ken K. Nischal, F.R.C.Ophth.

From the Clinical and Academic Department of Ophthalmology, Great Ormond Street Hospital for Children, London, United Kingdom.

Correspondence: Requests for reprints should be addressed to: Kate A.V. Smith, Department of Ophthalmology, Great Ormond Street Hospital for Children, London WC1N 3JH, U.K.


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Background and Purpose: Epidermolysis bullosa (EB) is a genetic disorder resulting in blistering of the skin and mucous membranes. While ocular surface complications in EB patients are well documented, there are no reports* on the incidence of refractive errors and orthoptic problems in these patients.

Patients and Methods: Fifty-five consecutive EB patients underwent examination including visual acuity, cover test, ocular motility and assessment of binocular single vision, cycloplegic refraction, and fundus examination.

Results: Twenty-one of the 55 patients (38%) had reduced visual acuity (0.3 logMAR or less) in at least one eye, and 13 / 55 (24%) had bilaterally reduced visual acuity.

Twenty-nine percent of patients had refractive errors; 20% hypermetropic, 5% myopic, 16% astigmatism, and 11% anisometropic. Fifteen percent of patients had a constant or intermittent tropia; 9% exotropia and 4% esotropia. Thirteen percent of patients had a significant phoria (> 10{Delta}); all were exophoric. Sixteen percent had convergence insufficiency. This cohort of EB patients demonstrated a high prevalence of reduced visual acuity, strabismus and refractive errors compared with normal pediatric data from the literature.

Conclusions: A large proportion of patients with EB across the subgroups have clinically significant refractive errors and strabismus. In view of the improved survival of EB patients, visual morbidity is of increasing importance. This study highlights the need for careful orthoptic and ophthalmic surveillance of children with EB.

Key words: epidermolysis bullosa, ocular motility, refractive errors


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This Article
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