Bilateral Ischemic Retinopathy Associated With Upshaw-Schulman Syndrome: A Case Report

PurposeThis is the first report to our knowledge of ischemic retinopathy in a pediatric patient with Upshaw-Schulman syndrome (USS). MethodsA 6-year-old girl previously diagnosed with USS was referred to our clinic with exodeviation of the left eye and a 2-month-long decrease in vision of both eyes....

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Bibliographic Details
Published in:Journal of vitreoretinal diseases 2022, Vol.6 (2), p.163-166
Main Authors: Cernichiaro-Espinosa, Mariam, Meizner-Grezemkovsky, Daniela, López-Santiago, Norma, Borbolla-Pertierra, Ana M, Morales-Cantón, Virgilio, Cernichiaro-Espinosa, Linda A
Format: Report
Language:English
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Summary:PurposeThis is the first report to our knowledge of ischemic retinopathy in a pediatric patient with Upshaw-Schulman syndrome (USS). MethodsA 6-year-old girl previously diagnosed with USS was referred to our clinic with exodeviation of the left eye and a 2-month-long decrease in vision of both eyes. A dilated fundus examination showed a total vitreous hemorrhage in both eyes. The first course of action was conservative treatment, with the patient experiencing visual-acuity improvement in her right eye. ResultsAn ischemic retina and optic nerve atrophy was found once the left eye was cleared of the hemorrhage. ConclusionsWe present a case of a vitreous hemorrhage, possibly secondary to an episode of severe thrombocytopenia. Following USS diagnosis, providers should perform dilated ophthalmologic examinations as part of initial and follow-up general evaluations. This case exemplifies that, in understudied and underdescribed pediatric retinal diseases, extreme therapeutic decisions-such as surgery-should not be rushed.
ISSN:2474-1272
DOI:10.1177/24741264211049694