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Bilateral Ischemic Retinopathy Associated With Upshaw-Schulman Syndrome: A Case Report
Mariam Cernichiaro-Espinosa1, Daniela Meizner-Grezemkovsky1, Norma López-Santiago2
1Pediatric Retina Unit, Retina and Vitreous Department, Asociación para Evitar la Ceguera en México, I.A.P. Ciudad de México, Mexico.
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.

