Two cases of orbital lymphangioma associated with vascular abnormalities of the retina and iris

Author(s): Vavvas D, Fay A, Watkins L


Purpose: To report 2 patients with combined intraocular and orbital vascular abnormalities.

Design: Two interventional case reports.

Main outcome measures: Clinical and pathologic findings. INTERVENTION/TESTING: Orbitotomy, fluorescein angiography, magnetic resonance imaging, and radiation therapy.

Results: Two patients presented with vision loss, intermittent proptosis, and cosmetic deformity. These patients had orbital lymphangioma, ipsilateral retinal and iris vascular malformations, and smaller corneal diameter on the affected side.

Conclusions: The coexistence of these diverse vascular anomalies supports the established hypothesis of pluripotential orbital vascular anlagen. Disruptive influences of various types during embryogenesis or development may produce a variety of congenital orbital and intraocular vascular malformations.

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