Ipsilateral facial and uveal arteriovenous and capillary angioma, microphthalmos, heterochromia of the iris, and hypotony: an oculocutaneous syndrome simulating Sturge-Weber syndrome.
Sturge-Weber syndrome is a disorder characterized by ipsilateral cavernous hemangioma of the face, uvea, and brain in patients who may present with an enlarged eye, exudative retinal detachment, glaucoma, and seizures. This report presents the clinicopathologic findings of an otherwise healthy infant with ipsilateral arteriovenous and capillary hemangiomas of the face and uveal tract, microphthalmos, iris heterochromia, hypotony, and absence of central nervous system involvement. The association of an arteriovenous-capillary angioma of the ocular adnexa and ipsilateral uveal tract is a syndrome that is distinct from Sturge-Weber syndrome.
Gass
J D
JD
Vanderbilt University Department of Ophthalmology, Nashville, Tennessee, USA.
D001165:Arteriovenous Malformations / Q000175:diagnosis*; D003937:Diagnosis, Differential; D015353:Eye Enucleation; D005153:Facial Neoplasms / Q000175:diagnosis*; D005260:Female; D018324:Hemangioma, Capillary / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D007499:Iris Diseases / Q000175:diagnosis*; D008850:Microphthalmos / Q000175:diagnosis*; D015814:Ocular Hypotension / Q000175:diagnosis*; D012171:Retinal Vessels / Q000002:abnormalities*; D013341:Sturge-Weber Syndrome / Q000175:diagnosis*; D014602:Uvea / Q000098:blood supply; D014604:Uveal Neoplasms / Q000175:diagnosis*