Bodanowitz S, Hesse L, Pöstgens H, Kroll P
Medizinisches Zentrum für Augenheilkunde, Marburg.
Ophthalmologe. 1997 Sep;94(9):634-7. doi: 10.1007/s003470050173.
Ehlers-Danlos syndrome (EDS) is an hereditary connective tissue disorder caused by defective collagen synthesis, the main features being hyperelasticity and vulnerability of the skin, recurrent bleeding from fragile blood vessels, and secondary deformities of the joints. Ocular involvement is a rare occurrence, e.g., corneal and scleral rupture from minor blunt injury, lens displacement, rhegmatogenous retinal detachment. To date, few reports exist concerning the treatment of retinal detachment in Ehlers-Danlos syndrome, all of them dealing exclusively with conventional scleral buckling surgery.
We report on a 47-year-old male patient suffering from EDS type VI (so-called ocular type, lysine-hydroxylase deficiency). He presented with rhegmatogenous retinal detachment in his only eye. A scleral buckling procedure was not feasible because of marked scleral atrophy. A three-port vitrectomy was therefore carried out.
During the operation, pronounced choroidal detachment and bleeding developed, subsiding within weeks postoperatively. Closure of the sclerotomies was difficult due to scleral thinning. Two revitrectomies were necessary because anterior PVR with traction retinal detachment occurred. The last revitrectomy was performed 18 months ago, and the retina has been completely reattached under 5000 cs silicone oil since then. Visual acuity is 0.1.
Primary vitrectomy permits successful treatment of retinal detachment in EDS patients if a buckling procedure cannot be performed because of scleral atrophy. However, serious complications may occur. Surgical procedures other than primary vitrectomy should therefore always be carefully considered, e.g., pneumatic retinopexy, temporary balloon, dura patch with episcleral pocket.
埃勒斯-当洛综合征（EDS）是一种由胶原蛋白合成缺陷引起的遗传性结缔组织疾病，主要特征为皮肤弹性过度和易损性、脆弱血管反复出血以及关节继发性畸形。眼部受累较为罕见，例如轻微钝性损伤导致角膜和巩膜破裂、晶状体移位、孔源性视网膜脱离。迄今为止，关于埃勒斯-当洛综合征视网膜脱离治疗的报道很少，所有报道均仅涉及传统的巩膜扣带术。
我们报告一例47岁男性患者，患有VI型EDS（所谓的眼型，赖氨酸羟化酶缺乏症）。他唯一的眼睛出现了孔源性视网膜脱离。由于明显的巩膜萎缩，巩膜扣带手术不可行。因此进行了三通道玻璃体切除术。
手术过程中出现了明显的脉络膜脱离和出血，术后数周消退。由于巩膜变薄，巩膜切口闭合困难。由于发生了前部增殖性玻璃体视网膜病变伴牵引性视网膜脱离，需要进行两次再次玻璃体切除术。最后一次再次玻璃体切除术于18个月前进行，从那时起视网膜在5000厘沲硅油下完全复位。视力为0.1。
如果由于巩膜萎缩无法进行扣带手术，原发性玻璃体切除术可成功治疗EDS患者的视网膜脱离。然而，可能会发生严重并发症。因此，应始终仔细考虑除原发性玻璃体切除术之外的其他手术方法，例如气体视网膜固定术、临时球囊、带巩膜下间隙的硬脑膜补片。
