Concomitant multiple cavernous hemangiomas and venous angioma of the orbit.

PURPOSE: The coexistence of multiple cavernous hemangiomas and venous malformation is an extremely rare clinical condition. In this report, we describe a patient showing initial multiple cavernous hemangiomas followed years later by a recurrence of multiple cavernous hemangiomas along with the appearance of venous angioma coexisting in the same orbit.

METHODS: A 52-year-old woman was referred with a gradually progressive proptosis and upward displacement of her left eye that were present for 10 years. The clinical features, computed tomography and ultrasonography examination, surgery, and histopathologic findings from this patient with multiple cavernous hemangiomas coexisting with venous angioma in the same orbit are presented.

RESULTS: Color Doppler ultrasonography and computed tomography scan displayed more than 10 well-defined homogenous masses in the left orbit measuring 0.5 × 0.5 to 1.0 × 0.8 cm with no blood flow. Anterior orbitotomy of the left eye was performed. Fifteen accessible, distinct, red-purple, round masses were excised with tumors measuring 0.5 × 0.5 × 0.6 to 1.0 × 1.0 × 1.0 cm. Histopathologic analysis confirmed the diagnosis of multiple cavernous hemangiomas. Eight years later, she again developed left proptosis. Color Doppler ultrasonography and computed tomography disclosed multiple smoothly outlined homogeneous masses in the left orbit ranging in size from 5 × 4 to 28 × 16 mm, along with some scattered, high-density, vein-stone shadows within the mass. An anterior orbitotomy was again performed. At surgery, 4 violaceous, well-defined, and cone-shaped masses were removed with minimal bleeding. Histopathologic examination confirmed the diagnosis of multiple cavernous hemangiomas with coexisting venous angioma. There was no recurrence of orbital hemangioma at 2 years after the second surgery.

CONCLUSIONS: Orbital cavernous hemangioma and venous angioma may share a yet-to-be-described common link.