Kannan Naresh Babu, Sen Sagnik, Damodaran Sourav, Debbarma Meri, Rajan Renu P, Kumar Karthik, Ramasamy Kim
Department of Vitreo-Retina, Aravind Eye Hospital, Madurai, TN, India.
J Vitreoretin Dis. 2020 Jul 27;4(6):479-483. doi: 10.1177/2474126420936187. eCollection 2020 Nov-Dec.
Spherophakia is a rare, debilitating congenital ocular disorder.
This institution-based, retrospective, interventional study evaluated medical records of 16 patients with spherophakia with a median age of 19.5 years. Twenty-six eyes underwent scleral-fixated intraocular lens implantation. The technique involved 2 parallel-to-limbus scleral tunnels fashioned using a microincision vitrectomy blade, followed by lensectomy, core vitrectomy, externalization of haptics through 2 separate diametrically opposite sclerotomies, and tucking in of the haptics into the tunnels. Anterior and posterior segment examination, visual acuity, and intraocular pressure (IOP) were noted at baseline and final follow-up.
Baseline best-corrected visual acuity (BCVA) of all eyes was 0.49 ± 0.51 logarithm of the minimum angle of resolution (logMAR) (Snellen equivalent, 6/18). BCVA of more than 6/60 was present in 28 of 32 (87.5%) eyes, and 13 of 26 (50%) eyes that were operated on had a baseline BCVA of more than 6/18. Postoperative BCVA in 26 eyes improved from 0.43 ± 0.32 logMAR to 0.19 ± 0.21 logMAR (Snellen equivalent, 6/9) ( = .002). Postoperative BCVA was more than 6/18 in 20 of 26 (76.9%) operated-on eyes ( = .046), and all eyes had a final BCVA of more than 6/60. In 20 of 26 (76.9%) eyes, BCVA improved from preoperative status. Postoperatively, spherical equivalent improved from -9.55 ± 5.17 diopters to -0.29 ± 1.45 diopters ( < .001). Apart from 2 patients with Marfan syndrome, 1 with Weill-Marchesani syndrome, and 1 with homocystinuria, the remaining patients had isolated spherophakia. Six eyes presented with an IOP greater than 21 mm Hg, and 50% of these eyes had a final IOP that decreased to less than 15 mm Hg after lensectomy.
Lensectomy with pars plana vitrectomy and scleral-fixated intraocular lens is an effective method of refractive rehabilitation for patients with spherophakia presenting with visual disability.
球形晶状体是一种罕见的、使人衰弱的先天性眼部疾病。
本基于机构的回顾性干预研究评估了16例球形晶状体患者的病历，这些患者的中位年龄为19.5岁。26只眼睛接受了巩膜固定人工晶状体植入术。该技术包括使用微切口玻璃体切割刀制作2个平行于角膜缘的巩膜隧道，随后进行晶状体切除术、核心玻璃体切除术，通过2个单独的直径相对的巩膜切口将襻引出，并将襻塞入隧道。在基线和最终随访时记录眼前节和后节检查、视力及眼压（IOP）情况。
所有眼睛的基线最佳矫正视力（BCVA）为0.49±0.51最小分辨角对数（logMAR）（相当于Snellen视力表的6/18）。32只眼中的28只（87.5%）BCVA大于6/60，接受手术的26只眼中有13只（50%）基线BCVA大于6/18。26只眼术后BCVA从0.43±0.32 logMAR提高到0.19±0.21 logMAR（相当于Snellen视力表的6/9）（P = .002）。接受手术的26只眼中有20只（76.9%）术后BCVA大于6/18（P = .046），所有眼睛最终BCVA均大于6/60。26只眼中有20只（76.9%）视力较术前有所改善。术后等效球镜度从-9.55±5.17屈光度改善至-0.29±1.45屈光度（P < .001）。除2例马凡综合征患者、1例韦尔-马切桑尼综合征患者和1例同型胱氨酸尿症患者外，其余患者为单纯性球形晶状体。6只眼眼压高于21 mmHg，其中50%的眼睛在晶状体切除术后最终眼压降至15 mmHg以下。
晶状体切除联合扁平部玻璃体切除术及巩膜固定人工晶状体植入术是治疗有视力障碍的球形晶状体患者屈光康复的有效方法。
