Optical Coherence Tomography Angiography of Early Stage 1a Retinal Hemangioblastoma in Von-Hippel-Lindau

Abstract

Von-Hippel-Lindau (VHL) syndrome is characterized by focal vasoproliferative tumors of retinal capillaries called retinal capillary hemangioblastomas (RCH). These tumors are initially small and can be easily missed if not looked for carefully. As they grow, these tumors are more demanding to treat and hence the importance of detecting them early and treating them. Herein, we describe and review the optical coherence tomography angiography (OCTA) of the early- stage lesion, which suggested the involvement of superficial and a deeper retinal capillary plexus. In addition, to helping us detect these lesions earlier, OCTA may also help to understand the in vivo changes occurring at an earlier phase.

Keywords: OCTA; VHL; hemangioblastoma; retinal capillary hemangioblastoma.

Figure 1

Fundus images shows lasered areas, multiple RCH, and localized retinal detachment. One of these lesions is a barely visible early stage 1a lesion (A- white arrow). B-scan OCT image (B) through this lesion showed the angioma is entirely intraretinal, well defined, and vertically oval. At the lower end, there is basal hypo-reflectivity with absence of an ellipsoid zone and an external limiting membrane. At the upper end, there is a focal hump but with maintained architecture of overlying inner retinal layers. En face structural appearance of the lesion on OCTA (C). An enface image of the superficial retinal layer (D), a focal increase in density and irregularity of the superficial capillary network with no prominent feeder vessels is seen. Adjacent capillary network is normal. An en face image of the deep retinal layers; a relatively more compact, well defined hyperreflective lesion is seen (E). VHL: Von-Hippel-Lindau; RCH: retinal capillary hemangioblastoma; OCTA: optical coherence tomography angiography.