PAAO-YO Research: Victor Villegas MD
25 June 2021
Victor Villegas MD, is a specialist in Ocular Oncology, Retina & Vitreous and Pediatric Ophthalmology at the University of Puerto Rico, Department of Ophthalmology, in San Juan, Puerto Rico.
He and his colleagues recently published this article:
Multimodal Imaging in the Diagnosis of Exophytic Juxtapapillary Retinal Capillary Hemangioblastoma
Retinal capillary hemangioblastomas (RCHS) are uncommon tumors of the retina that can occur sporadically or as part of the von Hippel–Lindau (VHL) syndrome. RCH can grow in an exophytic pattern, in the direction of the subretinal space, in which case they often have a more subtle appearance that can be challenging to diagnose. The purpose of this study is to determine how modern multimodal imaging can assist in the diagnosis of exophytic juxtapapillary RCH (JRCH). Medical records and multimodal imaging studies were reviewed on all patients diagnosed with RCH at Bascom Palmer Eye Institute, Miami, Florida, between January 2013 and December 2019. Patients with exophytic lesions within 2 mm of the disc were included. One patient from the Baylor College of Medicine, Houston, Texas was included. Fundus photography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, optical coherence tomography (OCT), OCT angiography, and B-scan images were reviewed. Twelve patients were identified with exophytic JRCH. The mean age was 54 years (range 38-73 years). Five patients had von Hippel–Lindau syndrome.
The most common referral diagnoses were choroidal neovascularization and neuroretinitis.
Imaging features included nodular outer retinal thickening with shadowing and intra-/subretinal fluid on OCT, hypoautofluorescence on fundus autofluorescence, middle to outer retinal hypervascularity on OCT angiography, early hyperfluorescence with late leakage on fluorescein angiography, and lack of choroidal vascular lesion on indocyanine green angiography. Early lens extraction might be considered in cases with very shallow AC or primary angle-closure suspect/primary angle-closure.
A key to the accurate diagnosis of exophytic JRCH is its intraretinal location, typically involving the outer retinal layers, which results in a clinical appearance that is distinct from the more common and easily recognizable endophytic RCH.
Multimodal imaging can aid in ruling out choroidal neovascularization and disc edema by demonstrating an absence of involvement of those structures..
Victor M. Villegas, MD
Areas of specialization are Ocular Oncology, Retina & Vitreous, and Pediatric Ophthalmology at the Department of Ophthalmology, University of Puerto Rico.
