Retinal vasoproliferative tumors in 6 patients with neurofibromatosis type 1

JAMA Ophthalmology
J A ShieldsC L Shields

Abstract

Retinal vasoproliferative tumors (RVPTs) are an important ocular finding in patients with neurofibromatosis type 1 (NF1), and early detection of this association and prompt initiation of treatment may prevent vision loss and blindness in affected patients. To describe the clinical findings of RVPTs in patients with NF1 and to underscore the risk of severe vision loss in such patients. DESIGN, SETTING, AND PATIENTS We performed a retrospective medical record review of 6 patients with RVPTs and NF1 treated at the Ocular Oncology Service, Wills Eye Hospital. The demographics, clinical features, clinical course, and outcomes of the 6 patients with RVPTs were recorded. RESULTS Of 275 patients with RVPTs, 6 (2.2%) had NF1. At the time of diagnosis of RVPT, the median patient age was 12 years (range, 9-36 years). Visual acuity was variable, ranging from 6/7.5 to light perception on initial presentation to the oncology service. The RVPT was located between the equator and ora serrata in all patients. The mean basal tumor diameter was 11 mm, and the mean tumor thickness was 4 mm. Associated features included subretinal fluid (n = 6), subretinal exudation (n = 6), epiretinal membrane (n = 3), retinal hemorrhage (n = 2), vitreous hemorrha...Continue Reading

Citations

Dec 26, 2015·Survey of Ophthalmology·Frederick A JakobiecAlan D Proia
Feb 24, 2016·Clinics in Dermatology·Karen A Chernoff, Julie V Schaffer
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