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Klippel–Trenauney–Weber syndrome is a neurocutaneous vascular disease characterised by deep venous dysplasia affecting the head and limbs.1 Affected patients are born with bilateral facial port-wine stains, and develop progressive limb hypertrophy secondary to high tissue pressure from chronic venous and lymphatic outflow obstruction.1 Reported ophthalmological signs include conjunctival and retinal varicosities, choroidal thickening, orbital varices and iris heterochromia.2 This article describes treatment of a patient with Klippel–Trenauney–Weber syndrome and congenital glaucoma who developed circumpapillary choroidal varices in the right eye over a 15-year period of observation.
Case report
A 1-year-old girl had bilateral facial port-wine stains, lumbosacral and lower limb port-wine stains with associated limb hypertrophy, and congenital glaucoma in both eyes (fig 1A). After surgery, intraocular pressure (IOP) was controlled by …
Footnotes
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Funding: This study was supported in part by an unrestricted grant from the Research to Prevent Blindness (New York) and the Pat & Willard Walker Eye Research Center, Jones Eye Institute, University of Arkansas for Medical Sciences (Little Rock, Arkansas).
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Competing interests: None declared.
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Informed consent was obtained for publication of fig 1.