Article Text

Circumpapillary choroidal “haemorrhoid” in Klippel–Trenauney–Weber syndrome
  1. M C Brodsky
  1. Departments of Ophthalmology and Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA
  1. Correspondence to: Dr M C Brodsky Arkansas Children’s Hospital, 800 Marshall, Little Rock, AR 72202, USA; brodskymichaelc{at}

Statistics from

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 …

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