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

• Published in: Journal of vitreoretinal diseases (Print) 2022-03, 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: Article
• Language: English
• Subjects: Case Reports
• ISSN: 2474-1264; 2474-1272
• DOI: 10.1177/24741264211049694

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.