Purpose: Cavernous hemangioma of the retina (CHR) is a rare retinal vascular hamartoma. The objective of this study was to summarize the current knowledge of this rare condition based on a detailed review of 96 documented cases.
Methods: The incidence, clinical and histological appearance, differential diagnosis, treatment, and prognosis of CHR were analyzed by evaluating clinical data obtained from 96 cases in the literature since the first case report in 1934.
Results: More than 90% of cases of CHR have been reported in white individuals with even gender distribution. A majority of patients presented a unilateral unifocal lesion without positive family history. Our analysis showed bilateral cases were strongly correlated with family history, which suggests the importance of screenings for family members in bilateral CHR cases. Intracranial involvement was reported in 14% of CHR cases. Most patients were asymptomatic, but in patients with history of headaches, transient visual disturbance, or seizures 80% had intracranial involvement.
Conclusion: It is important to screen family members in bilateral CHR cases, and it is critical to exclude cerebral vascular abnormalities in view of relative high incidence of intracranial involvement and high morbidity.