Abstract

P 213

Unilateral congenital glaucoma, anisometropia and hypertrophy of the limbs in Klippel-Trenaunay-Weber syndrome

Susanne Butros, Gerhard K. Lang, Stephan Eckert
Universitäts-Augenklinik Ulm

Objective
The Klippel-Trenaunay-Weber syndrome (Angioosteohypertrophic syndrome) is a congenital rare phacomatosis of uncertain aetiology, characterized by vascular abnormalities. The cardinal signs are cutaneous angiomas, varicosities, bone- and tissue hypertrophy and arteriovenous fistulas. Mostly one region of the body or a limb is affected, rarely several limbs or even the hole body.
Methods
A 17-year-old male patient with Klippel-Trenaunay-Weber syndrome and unilateral congential glaucoma on his left eye presented with a not regulated intraocular pressure and a progression of visual field loss.
Results
Both arms and legs of the patient were erythematous. All four limbs showed a hypertrophy of the soft tissue on the left side more than on the right side, with associated finger and toe deformity. Face exhibited multiple teleangiectasias and also a hypertrophy on the left. In 1991 an iridectomy and in 1998 a filtration surgery on his left glaucoma eye was performed.  Visual acuity was 1.0 on his right eye with emmetropia. Anisometropia caused an amblyopia on his left eye with myopia of -7.0 dpts and visual acuity of 1/40. The right eye was morphological normal. The left eye showed a scarred filtering bleb and chamber angle was almost completely closed by distinctive anterior synechias. Retinal vessels appeared normal. Optical disc had a glaucomatous atrophy. Goldmann visual field generated a central residual visual field. Because of an intraocular pressure extended to 42 mmHg despite of maximum antiglaucomatous therapy, a cyclophotocoagulation was carried out.
Conclusions
Klippel-Trenaunay-Weber syndrome can be associated with different ocular symptomes. In addition to arteriovenous malformations appearing in anterior and posterior globe, there is especially a disposition to congenital glaucoma. Therefore early follow-ups in childhood should be performed to prevent blindness.

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