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December 1979

Cavernous Hemangioma of the RetinaA Four-Generation Pedigree With Neurocutaneous Manifestations and an Example of Bilateral Retinal Involvement

Author Affiliations

From the Vascular Unit (Drs Goldberg and Pheasant) and the Oncology Unit (Dr Shields), Retina Service, Wills Eye Hospital, Philadelphia, and the Retina Service, Department of Surgery, Holy Redeemer Hospital, Meadowbrook, Pa (Dr Goldberg). Dr Pheasant is now with M. S. Hershey Medical Center Division of Ophthalmology, Hershey, Pa.

Arch Ophthalmol. 1979;97(12):2321-2324. doi:10.1001/archopht.1979.01020020537005

• Cavernous hemangioma of the retina is an unusual vascular hamartoma whose coexistence with vascular anomalies of the skin and central nervous system has been recognized recently. A 39-year-old woman, who had an acute palsy of the right third cranial nerve, had a history of seizures, cutaneous vascular anomalies, and a cavernous hemangioma of the retina of the right eye. One of her daughters demonstrated bilateral retinal cavernous hemangiomas, and another daughter, who developed seizures when febrile, displayed cutaneous vascular anomalies. A four-generation pedigree showed a number of cutaneous vascular anomalies, seizures, and stroke-related deaths. The pedigree suggests further support for considering this disorder an authentic oculoneurocutaneous triad.

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