Braun M, Seitz B, Naumann G O
Augenklinik mit Poliklinik, Friedrich-Alexander-Universität Erlangen-Nürnberg.
Klin Monbl Augenheilkd. 1996 Apr;208(4):262-3. doi: 10.1055/s-2008-1035208.
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.
一名8岁男孩在4岁和5岁时接受了双侧重复小梁切除术及虹膜切除术，7岁时因青光眼对右眼进行了睫状体冷凝术。
在我们科室的首次检查显示，右眼视力为光感，左眼视力为0.2。右眼为外斜视和下斜视。双眼眼压均为30 mmHg。角膜直径右眼为9.0×7.0，左眼为8.5×6.5 mm，眼轴长度右眼为25.3，左眼为23.6 mm。双眼均有轻微突出的滤过泡，伴有葡萄膜嵌顿。双眼房角开放，部分被葡萄膜组织覆盖，E/P比值右眼为1.0，左眼为0.9。眼外改变包括：牙齿发育异常、牙釉质发育不全、下颌增厚、鼻梁突出的小鼻子。与第二和第三指相比，双手的掌骨和第四指均缩短。第四和第五指并指畸形此前已通过手术分离。
我们的诊断是眼-牙-指发育不良合并青少年开角型青光眼。
小梁切开术和睫状体冷凝术后眼压维持在10 mmHg左右。
尽管角膜直径较小，但对于眼-牙-指发育不良的患者，仍需早期排除先天性青光眼。这些患者应与儿科神经科医生、骨科医生、牙医和耳鼻喉科医生密切合作进行治疗。
