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Cutis marmorata telangiectatica congenita associated with bilateral congenital retinal detachment.

Shields J A, Shields C L, Koller H P, Federman J L, Koblenzer P, Barbera L S


AI Summary

CMTC, a rare skin disorder, was found linked to bilateral congenital retinal detachment and secondary neovascular glaucoma, expanding its known ophthalmic associations beyond unilateral glaucoma.

Abstract

Cutis marmorata telangiectatica congenita is a rare congenital vascular disorder of the skin, characterized by persistent telangiectasia of the cutaneous blood vessels often associated with cutaneous ulcers. The only previously recognized ophthalmic association with this condition has been rare instances of unilateral congenital open angle glaucoma. The authors report their observations in a child in whom this cutaneous disorder was associated with congenital bilateral total retinal detachments and secondary neovascular glaucoma. The retinal detachments produced bilateral leukocoria simulating retinoblastoma. The cutaneous disorder and the ocular findings were confirmed histopathologically.


MeSH Terms

Corneal DiseasesEye EnucleationEye HemorrhageFemaleGlaucoma, NeovascularHumansInfant, NewbornRetinal DetachmentSkinTelangiectasisUltrasonography

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