von Hippel angioma in South Indian subjects--a clinical study

To assess the management strategies for retinal capillary hemangioma in von Hippel-Lindau disease.

A retrospective, nonconsecutive case series of 12 patients.

We reviewed the records of patients diagnosed with retinal capillary hemangioma from January 1996 to June 2002. Age at diagnosis, visual acuity, tumor pattern and location, associated clinical features, type of treatment, and visual and anatomical outcomes were analyzed.

None of the patients had evidence of extraocular hemangiomas. The patients were observed for 2 to 24 months. Ten of 12 hemangiomas were endophytic. Subretinal exudation was present in all patients. Cryotherapy was the most common treatment option with successful anatomical (7 of 8 patients) and visual (5 of 8 patients) outcomes. Photocoagulation was successful in 1 of 2 patients. Two patients in the cryotherapy group and two in the photocoagulation group had complications, including macular scar, macular hole, epimacular membrane, and combined traction and rhegmatogenous retinal detachment. Two patients were lost to follow-up after cryotherapy. Seven of 10 patients with a minimum follow-up of 2 months had improved visual acuity.

Endophytic peripheral retinal capillary hemangioma with subretinal exudation was the commonest presentation in this series. Although cryotherapy and photocoagulation regress the tumor, these treatment modalities are associated with complications that may require surgical intervention. Retinal detachments in this group of patients are amenable to surgical intervention.