[Juvenile open angle glaucoma with microcornea in oculo-dento-digital dysplasia (Meyer-Schwickerath-Weyers syndrome)]

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

The first examination in our department revealed a visual acuity of OD light perception, OS 0.2. The right eye was exotropic and hypotropic. The intraocular pressure was 30 mm Hg in both eyes. The corneal diameters were OD 9.0 x 7.0 and OS 8.5 x 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 IInd and IIIrd digits. Syndactylic digits IV and V had been surgically separated earlier.

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

After trabeculotomy and cyclocryocoagulation IOP remained around 10 mm Hg.

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.