Omulecki W, Nawrocki J, Palenga-Pydyn D, Sempinska-Szewczyk J
Department of Ophthalmology, Medical University of Lodz, Poland.
Ophthalmic Surg Lasers. 1998 May;29(5):375-9.
Congenital lens subluxation may be a difficult therapeutic problem. Surgical treatment options include iris manipulation or lens decision, aspiration, intracapsular or extracapsular extraction, and lensectomy through the pars plana. It is not established which kind of aphakic correction is the most appropriate in these cases.
A father and his two sons with Marfan's syndrome were operatively treated for lens dislocation in both eyes. Pars plana vitrectomy was done in all eyes. Dislocated lenses were removed by lensectomy in three eyes and with an intracapsular method in three eyes. The outside-in scleral fixation technique was used for primary posterior chamber intraocular lens (PC IOL) implantation in all cases.
All eyes achieved good visual acuity (20/20-20/25). Time of observation ranged between 8 and 20 months. There were no intraoperative or post-operative complications.
Pars plana vitrectomy and primary scleral-fixated IOL implantation is a safe procedure and gives good visual rehabilitation in adult patients with Marfan's syndrome.
先天性晶状体半脱位可能是一个棘手的治疗难题。手术治疗方案包括虹膜操作或晶状体摘除、抽吸、囊内或囊外摘除，以及经睫状体扁平部进行晶状体切除术。在这些病例中，哪种无晶状体矫正方法最为合适尚无定论。
一名父亲及其两个患有马凡综合征的儿子接受了双眼晶状体脱位的手术治疗。所有眼睛均进行了经睫状体扁平部玻璃体切除术。三只眼睛通过晶状体切除术摘除脱位晶状体，三只眼睛采用囊内法摘除。所有病例均采用由外向内巩膜固定技术进行一期后房型人工晶状体（PC IOL）植入。
所有眼睛均获得了良好视力（20/20 - 20/25）。观察时间为8至20个月。未发生术中或术后并发症。
经睫状体扁平部玻璃体切除术及一期巩膜固定人工晶状体植入术是一种安全的手术方法，可为成年马凡综合征患者提供良好的视力恢复。
