SEGMENTAL DIFFUSE VASCULAR LEAKAGE: A FLUORESCEIN ANGIOGRAPHIC FINDING IN PATIENTS WITH VON HIPPEL-LINDAU DISEASE

To describe a novel fluorescein angiographic finding in patients with von Hippel-Lindau disease.

Retrospective case series of patients diagnosed with retinal capillary hemangioblastoma (RCH) in the setting of von Hippel-Lindau.

We identified six eyes of three patients with von Hippel-Lindau and leaky retinal vessels. All eyes showed segmental diffuse vascular leakage (SDVL) that was seen in the late phase of the angiogram and that originated from third order and more peripheral retinal veins and adjacent capillaries. These vessels did not drain from the RCHs. Segmental diffuse vascular leakage was mainly seen in the mid and far periphery. In some cases, it was located near the RCHs, while in other cases, it was remote. Segmental diffuse vascular leakage was also seen in one eye without RCHs. On follow-up, the extent and intensity of segmental diffuse vascular leakage did not change after the RCHs were treated with laser or cryotherapy.

Diffuse vascular leakage from retinal venules around and away from RCHs in patients with von Hippel-Lindau disease is seen, but the clinical and prognostic importance of this finding is uncertain.