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Abstract

Purpose:

This is the first report to our knowledge of ischemic retinopathy in a pediatric patient with Upshaw-Schulman syndrome (USS).

Methods:

A 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.

Results:

An ischemic retina and optic nerve atrophy was found once the left eye was cleared of the hemorrhage.

Conclusions:

We 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.

Keywords

congenital thrombotic thrombocytopenic purpura ischemic retina pediatric ischemic retina Upshaw-Schulman syndrome vitreous hemorrhage

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