SOLITARY RETINAL CAPILLARY HEMANGIOMA IN A PATIENT WITH BILATERAL CHORIORETINAL COLOBOMA

Abstract

Purpose: To report a case showing a very rare association of chorioretinal coloboma and retinal capillary hemangioma in a previously healthy patient.

Methods: Observational case report.

Results: A 21-year-old woman presented at our clinic for vision screening. She had a history of bilateral chorioretinal coloboma with amblyopia in her left eye. Her best-corrected visual acuity was 20/25 in her right eye and 20/200 in her left eye. On slit-lamp examination, iris coloboma was observed in the left eye. Fundus evaluation showed inferior chorioretinal coloboma in both eyes, and an elevated, round, and orange-red retinal lesion with feeder vessels localized in the midperipheral region of the right eye. The patient was diagnosed as having solitary retinal capillary hemangioma and underwent argon laser therapy for treating the tumoral lesion. Diagnostic studies were negative for von Hippel-Lindau disease. This is the first reported case of solitary retinal capillary hemangioma associated with bilateral chorioretinal coloboma.

Conclusion: Chorioretinal coloboma is a congenital defect of the eye caused by improper closure of the embryonic fissure. Retinal capillary hemangioma is a vascular retinal tumor that may occur sporadically or as part of the von Hippel-Lindau syndrome. We report a rare association of chorioretinal coloboma and retinal capillary hemangioma in a previously healthy patient.

Fig. 1.

Color fundus photography. A. Dilated fundus examination revealed a raised, rounded, and orange-red retinal lesion, with two-disk diameters, localized in the nasal midperipheral region distant from the chorioretinal coloboma in her right eye. Note the large vessels feeding the tumor. B. Chorioretinal coloboma is also observed in her left eye.

Fig. 2.

Fluorescein angiogram. Initial consult (A) early hypofluorescence over the area of the tumoral lesion. The mid (B) to late (C) sequences showed leakage around the lesion. Feeder vessels are observed in all pictures.

Fig. 3.

Sequence of the laser treatment on the tumoral lesion. First visit (A) 2 months after starting argon laser therapy on and around the lesion until collapse and cessation of blood flow in the collector vessels (B).

Fig. 4.

After 6 months of remission, a new engorged vessel appeared to feed the tumor again. Note the presence of new collector vessels feeding the tumor. Reactivation of the tumoral activity was observed with fluid collection around the retinal hemangioma.

Fig. 5.

Color fundus photography. Two years of follow-up. Tumoral lesion exudation was controlled using with argon laser therapy.