不同年龄Marfan综合征晶状体半脱位两种术式的疗效观察

目的 探讨两种晶状体摘除联合一期人工晶状体（IOL）悬吊术治疗不同年龄Marfan综合征晶状体半脱位的效果。设计 回顾性病例系列。研究对象 Marfan综合征先天性晶状体半脱位患者10例（18眼）。方法 ＞12岁患者3例（5眼）采用晶状体超声乳化术摘除晶状体同时行一期IOL悬吊术（乳化组）；≤12岁患者7例（13眼）采用晶状体切除联合一期IOL悬吊术，同时行前段玻璃体切除术（晶切组）。平均随访（1.40±1.02）年。主要指标 最佳矫正视力、眼压、并发症。 结果 18眼术前平均最佳矫正视力（0.29±0.20），术后（0.51±0.24）（P=0.008）。 乳化组术前平均最佳矫正视力（0.17±0.04），术后（0.41±0.16）（P=0.026）；晶切组术前平均最佳矫正视力（0.34±0.22），术后（0.55±0.25）（P=0.000）。两组间比较，平均矫正视力改善无统计学差异（P=0.062）。所有患者术前等效球镜为（-2.76±14.1）D，术后为（-0.79±1.95）D。无一例出现严重并发症。结论 晶状体超声乳化与晶状体切除及前部玻璃体切除联合一期IOL悬吊术两种手术方式均能有效改善不同年龄Marfan综合征晶状体半脱位患者视力，手术方式选择与视力改善无明显关系。（眼科，2016, 25： 86-89）

Lens extraction combined with scleral-fixated intraocular lens implantation for ectopia lentis in Marfan syndrome

Objective To evaluate the clinical effects of phacoemulsificatio or limbal-approach lensectomy and vitrectomy combined with scleral-fixated intraocular lens(IOL) implantation for ectopia lentis in Marfan syndrome.  Design Retrospective case series Participants 10 patients (18 eyes) with ectopia lentis in Marfan syndrome were reviewed. Methods 3 patient (older than 12 years, 5 eyes) were treated with phacoemulsification combined with the scleral-fixated IOL implantation. The other 7 patients (younger than 12 years, 13 eyes) were treated with limbal-approach lensectomy and vitrectomy combined with scleral-fixated IOL implantation. The mean follow-up period was (1.40±1.02) years  (range 0.5 to 3 years). Main Outcome Measures The changes of visual activity, intraocular pressure and surgery complications. Results The mean best-corrected visual acuity (BCVA) of the 18 eyes improved significantly, from preoperative 0.29±0.20,  to 0.51±0.24 (P=0.008). The mean BCVA of phacoemulsification group was 0.17±0.04 before operation, which significantly improved to 0.41±0.16 after operation (P=0.026). The mean BCVA of lensectomy group was (0.34±0.22) before operation, and (0.55±0.25) after operation. The improvement was significant (P=0.000). The pre- and post-operative visual acuity change was no significant between the two groups (P=0.062). The mean pre-opertive spherical equivalent refractive error was -2.76±14.1 diopters (D), which improved to -0.79±1.95 D after operation. Conclusion The two surgical approaches to treat ectopia lentis are both safe and effective. There was no significant difference between the two approaches in terms of visual improvement and refractive error. (Ophthalmol CHN, 2016, 25: 86-89)