Jules Gonin University Eye Hospital, Lausanne, Switzerland.
Klin Monbl Augenheilkd. 2022 Apr;239(4):490-493. doi: 10.1055/a-1766-6381. Epub 2022 Apr 26.
Marfan Syndrome is an autosomal dominant disease with multiple ocular abnormalities including ectopia lentis and a high incidence of rhegmatogenous retinal detachment (RRD). The management of RRD may be challenging in cases of aphakic patients with Marfan.
To report on the management of four cases of simultaneous RRD and aphakia with vitrectomy, silicone oil tamponade, and retropupillary iris-claw intraocular lens (IOL) implantation in patients with Marfan that have been operated at the Jules-Gonin Eye Hospital between 2019 and 2020.
Ages at presentation were 20, 30, 32, and 31 years, respectively. All patients had a history of extraction of a dislocated lens. None of the patients had a previous posterior vitrectomy. Two patients had records of previous measurements for IOL calculation by optical biometry (IOL Master, Carl Zeiss Meditec AG, Jena, Germany) about 1 year prior to the RRD development. In two cases, measurements for IOL calculation by optical biometry were based on the contralateral eye.
All patients underwent 23 G vitrectomy, peripheral iridotomy, and retropupillary iris-claw IOL. No intraoperative complications were encountered. All patients had silicone oil tamponade, one of which required heavy silicone oil. Silicone oil was removed 3 months following primary surgery. Minimum follow-up was 1 year. The single surgery anatomic success rate was 100%. All patients had visual acuity of at least 0.8 at the last follow-up (1.25, 1.0, 0.8, and 0.8 respectively). The targeted refractive results were accurately achieved in all four cases postoperatively. One patient presented ocular hypertension 2 weeks after surgery due to presumed steroid response and was managed conservatively. None of the patients had silicone oil migration into the anterior chamber.
Retropupillary iris-claw IOL implantation in cases of RRD and aphakia creates a barrier to tamponades from the posterior segment, effectively preventing them from entering the anterior segment of the eye. Therefore, the management of aphakia and retinal detachment with simultaneous vitrectomy and a retropupillary iris-claw IOL may be a successful strategy in reducing postoperative complications in patients with Marfan syndrome.
马凡综合征是一种常染色体显性遗传病，眼部异常包括晶状体异位和孔源性视网膜脱离（RRD）的高发病率。马凡综合征患者无晶状体眼的 RRD 处理可能具有挑战性。
报告 2019 年至 2020 年在 Jules-Gonin 眼科医院对 4 例马凡综合征伴 RRD 和无晶状体患者进行玻璃体切除、硅油填充和后房型虹膜扣带式人工晶状体（IOL）植入的治疗情况。
患者年龄分别为 20、30、32 和 31 岁，均有晶状体脱位摘除史，均无玻璃体切除手术史。2 例患者在 RRD 发生前约 1 年有 IOL 计算的光学生物测量（德国卡尔蔡司公司的 IOL Master）记录。2 例患者的 IOL 计算通过对侧眼进行光学生物测量。
所有患者均接受 23G 玻璃体切除术、周边虹膜切开术和后房型虹膜扣带式 IOL。无术中并发症。所有患者均行硅油填充，其中 1 例需重硅油填充。初次手术后 3 个月取出硅油。随访时间最短 1 年。单次手术解剖成功率为 100%。末次随访时，所有患者视力均至少为 0.8（分别为 1.25、1.0、0.8 和 0.8）。术后 4 例均达到了预期的屈光效果。1 例患者术后 2 周因推测的类固醇反应出现眼压升高，给予保守治疗。无硅油向眼前房迁移。
RRD 和无晶状体患者后房型虹膜扣带式 IOL 植入术在后部节段形成了一个阻止填充物进入眼球前段的屏障，有效地防止了填充物进入眼前段。因此，玻璃体切除联合后房型虹膜扣带式 IOL 治疗无晶状体眼和视网膜脱离可能是减少马凡综合征患者术后并发症的一种成功策略。
