Uemura A, Uto M
Department of Ophthalmology, Kagoshima University Faculty of Medicine, Japan.
Jpn J Ophthalmol. 1992;36(1):97-102.
A 39-year-old man developed bilateral, insidiously progressing detachment of the retina and nonpigmented epithelium of the ciliary body because of a large break in the pars plicata. The anterior segments had multiple malformations including ectopia of the lens associated with coloboma of the lens and hypoplastic ciliary processes. The pars plicata breaks were present in areas corresponding to the anomalies of the lens-zonule-ciliary body complex and caused progressive, shallow rhegmatogenous retinal detachments with anterior chamber cells and elevation of intraocular pressure. Surgeries with scleral buckling procedure combined with vitrectomy and lensectomy brought about reattachment of the retina. This case indicates that subtle anomalies in the lens-zonule-ciliary body complex might be an indication of a break in the nonpigmented epithelium of the ciliary body which could lead to rhegmatogenous retinal detachment. The mode of inheritance of the clinical syndrome remained undefined because of negative family history.
一名39岁男性因睫状突皱襞处的大裂孔，出现双侧隐匿性进展的视网膜脱离和睫状体无色素上皮脱离。眼前节有多种畸形，包括晶状体异位伴晶状体缺损和睫状突发育不全。睫状突皱襞处的裂孔出现在与晶状体-悬韧带-睫状体复合体异常相对应的区域，导致进行性、浅孔源性视网膜脱离，并伴有前房细胞和眼压升高。巩膜扣带术联合玻璃体切除术和晶状体切除术使视网膜重新附着。该病例表明，晶状体-悬韧带-睫状体复合体的细微异常可能提示睫状体无色素上皮破裂，进而导致孔源性视网膜脱离。由于家族史阴性，该临床综合征的遗传方式尚不确定。
