Savastano Alfonso, Carlà Matteo Mario, Gambini Gloria, Caporossi Tomaso, Giannuzzi Federico, Boselli Francesco, Rizzo Stanislao
Ophthalmology Unit, "Fondazione Policlinico Universitario A. Gemelli, IRCCS", Rome, Italy.
Catholic University "Sacro Cuore", Rome, Italy.
Am J Ophthalmol Case Rep. 2023 Apr 1;30:101840. doi: 10.1016/j.ajoc.2023.101840. eCollection 2023 Jun.
We present a case of a patient with Marfan Syndrome who underwent a personalized approach to manage a spontaneous ciliary body detachment and degeneration of the ciliary processes, causing refractive ocular hypotony.
A 20-year-old MFS man, with a history of bilateral juvenile cataract surgery with failed IOL positioning due to subluxation and consequent explantation, was referred to our clinic after the discovery of persistent ocular hypotonia in his left eye for the past two months, non-responder to corticosteroids. Slit-lamp examination showed shallow anterior chamber and aphakia, with chorioretinal folds, optic disc swelling and mild peripheral retinal lifting. Intraocular pressure (IOP) was 4 mmHg. Ultrasound biomicroscopy (UBM) revealed the presence of a flat, annular ciliochoroidal detachment, posterior pole congestion and total ciliary body separation. One-month systemic therapy with corticosteroids revealed ineffective, and a newly performed UBM showed a significant reduction in the number and thickness of the ciliary processes. At this point, he underwent 23-gauge pars plana vitrectomy with silicone oil endotamponade, combined with a focal -scleral cryopexy of the ciliary body, 2 mm behind the limbus and one spot per quadrant, in order to favor ciliary body reattachment. Post-operative IOP was 28 mmHg and the choroidal detachment was resolved, with UBM highlighting ciliary body reattachment. Six months after, with good IOP control using topical therapy, silicone oil was removed. One year after, visual acuity had inccreased to 6/10 and a good IOP control was still maintained with eye drops.
A rare case of spontaneous ciliary body detachment in a long standing aphakic MFS patient was successfully managed with focal -scleral cryopexy of the ciliary body combined with pars plana vitrectomy and silicone oil endotamponade.
我们报告一例马凡综合征患者，该患者采用个性化方法治疗自发性睫状体脱离和睫状突变性，导致屈光性低眼压。
一名20岁的马凡综合征男性患者，有双侧青少年白内障手术史，因晶状体半脱位导致人工晶状体植入失败并随后取出。在过去两个月发现左眼持续低眼压且对皮质类固醇无反应后，转诊至我院。裂隙灯检查显示前房浅、无晶状体，伴有脉络膜视网膜皱褶、视盘肿胀和轻度周边视网膜隆起。眼压（IOP）为4 mmHg。超声生物显微镜检查（UBM）显示存在扁平的环形睫状体脉络膜脱离、后极充血和睫状体完全分离。为期一个月的皮质类固醇全身治疗无效，新进行的UBM显示睫状突数量和厚度显著减少。此时，他接受了23G经平坦部玻璃体切除术联合硅油眼内填充，并在角膜缘后2 mm处对睫状体进行象限性局部巩膜冷冻治疗，以促进睫状体重新附着。术后眼压为28 mmHg，脉络膜脱离得到解决，UBM显示睫状体重新附着。六个月后，通过局部治疗眼压控制良好，取出硅油。一年后，视力提高到6/10，使用眼药水仍能维持良好的眼压控制。
一名长期无晶状体的马凡综合征患者发生自发性睫状体脱离的罕见病例，通过睫状体局部巩膜冷冻治疗联合经平坦部玻璃体切除术和硅油眼内填充成功得到治疗。
