Juveniles Offenwinkelglaukom mit Mikrocornea bei okulo-dento-digitaler Dysplasie (Meyer-Schwickerath-Weyers)

Summary

History An 8-year-old boy underwent bilateral repeat trabeculectomies with large iridectomies at age 4 and 5 and cyclocryocoagulation of the right eye at age seven for glaucoma.

Findings The first examination in our department revealed a visual acuity of OD light perception, OS 0.2. The right eye was exotropic and hypotropie. The intraocular pressure was 30 mm Hg in both eyes. The corneal diameters were OD 9.0×7.0 and OS 8.5×6.5 mm, while the axial length was OD 25.3 and OS 23.6 mm. Both eyes had slightly prominent filter blebs with uveal incarceration. The chamber angles were open in both eyes and partly covered with uveal tissue, the E/P ratio was OD 1.0, OS 0.9. Extraocular changes were: dysplastic teeth, dental enamel hypoplasia, thickened jaw, small nose with pronounced nose bridge. Both hands showed shortening of the metacarpals and of the IVth digits as compared to the Und and Illrd digits. Syndactyly digits IV and V had been surgically separated earlier.

Diagnosis Our diagnosis was juvenile open-angle glaucoma in oculo-dento-digital dysplasia.

Therapy After trabeculotomy and cyclocryocoagulation IOP remained around 10 mm Hg.

Conclusions Despite small corneal diameters, congenital glaucoma needs to be ruled out early in patients with oculo-dento-digital dysplasia. These patients should be treated in close cooperation with pediatric neurologists, orthopedic surgeons, dentists and otorhinolaryngologists.

Schlüsselwörter

Juveniles Offenwinkeiglaukom - Trabekulotomie - Mikrocornea - okulo-dento-digitale Dysplasie - Meyer-Schwickerath-Weyers

Key words

Juvenile open angle glaucoma - trabeculotomy - microcornea - oculo-dento-digital Dysplasia - Meyer-Schwickerath-Weyers