Von Hippel–Lindau disease (VHL) is a neoplastic disorder that can lead to the multisystem formation of tumors and cysts in the central nervous system, adrenal glands, kidneys, pancreas, reproductive system, and retina [1]. The earliest ocular findings of VHL are retinal capillary hemangiomas, and loss of vision can result from tumor growth, extravasation of fluid and lipid exudates from the lesions, and retinal detachment. The evaluation of retinal manifestations of VHL includes the clinical exam and fundus fluorescein angiography (FFA), but FFA is invasive and findings of small lesions can be equivocal, making the decision for treatment challenging. Optical coherence tomography with angiography (OCTA) is a novel, noninvasive imaging technique that can provide in vivo images of the retinal vasculature with higher resolution than conventional techniques. However, the ability of OCTA to confirm both the presence and absence of potential retinal lesions seen with FFA in VHL has not yet been described. We have observed two cases of retinal hemangiomas with a systemic history of VHL.

As demonstrated in the cases described, OCTA has promising potential as an adjunctive tool to enhance the evaluation and management of patients with VHL. In Case 1, OCTA was able to confirm the presence of a vascular lesion that had not been observed on the dilated fundus exam and an equivocal appearance on FFA. In Case 2, using OCTA in the evaluation of a small area of hyperfluorescence observed with FFA demonstrated that there was no hemangioma. In both cases, the use of OCTA aided in the clinical management of the patients. The frequency of retinal lesions ranges from 49 to 85% in individuals with VHL [2, 3] and the potential damage to the retinal tissue and subsequent vision loss underscore the importance of high-quality clinical imaging in the ophthalmic management of this patient population. The size, location, and progression of the ocular lesions in VHL guide the decision for appropriate therapy, which includes laser photocoagulation, cryotherapy, diathermy, external beam radiation, plaque brachytherapy, intravitreal anti-VEGF administration, and pars plana vitrectomy. OCTA has been used to identify feeding and draining vessels and to characterize lesions after laser treatment of retinal angiomas that were previously diagnosed by exam and/or FFA [4]. Limitations of OCTA include its inability to image the peripheral retina. Although OCTA is a relatively new technology, its increased utilization and future advances in its imaging capabilities may improve the medical management of patients with suspected vascular lesions of their retina.

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