Background: von Hippel-Lindau (VHL) syndrome is a multisystem neoplastic disorder involving eyes, central nervous system, kidneys, spine, and other tissues. A retinal capillary hemangioma (RCH) is the earliest manifestation of the VHL disease in most cases.Areas covered:This paper aims to provide an up-to-date review of the current literature about von Hippel-Lindau syndrome. Molecular background, systemic and ocular features of the diseases as well as the utility of newer imaging modalities in diagnosis and monitoring of ocular VHL disease have been described. Besides, we have discussed newer treatment modalities and therapeutic targets.Conclusion: Modern imaging technologies like optical coherence tomography and optical coherence tomography angiography are tools of the trade, in making an appropriate diagnosis and monitoring disease activity and response to treatment. Peripheral RCH may be treated using laser photocoagulation in tumors up to 3000 µm. Vascular endothelial growth factor suppression can help in reducing tumor activity and stabilize the tumor size; however, it does not regress the RCH.
Keywords: OCT angio of retinal capillary hemangioma; classification; retinal capillary hemangioma; retinal tumors; von Hippel–Lindau disease.