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马凡综合征晶状体异位症患儿前房虹膜夹型人工晶状体植入的长期疗效。

Long-term results of anterior chamber iris claw intraocular lens implantation in children with ectopia lentis in Marfan syndrome.

机构信息

Department of Ophthalmology, Leiden University Medical Center, Leiden, The Netherlands.

Department of Ophthalmology, Noordwest Ziekenhuisgroep Alkmaar, Alkmaar, The Netherlands.

出版信息

J AAPOS. 2024 Jun;28(3):103922. doi: 10.1016/j.jaapos.2024.103922. Epub 2024 Apr 27.

DOI:10.1016/j.jaapos.2024.103922
PMID:38679138
Abstract

PURPOSE

To report the long-term clinical and endothelial cell count (ECC) results of lensectomy with primary anterior chamber iris claw lens implantation in the eyes of patients ≤18-year-old with ectopia lentis due to Marfan syndrome.

METHODS

The medical records of Marfan patients operated on at a single institution from September 2007 to August 2020, with minimum follow-up of 2 years, were reviewed retrospectively. The following data were analyzed: sex, age at surgery, indication for surgery, the position of the lens in relation to the undilated and dilated pupil, corneal endothelial cell counts (ECC), peri- and postoperative complications, pre- and postoperative best-corrected visual acuity.

RESULTS

A total of forty-two eyes of 23 patients (12 girls and 11 boys) were included. At least two or more postoperative ECCs were collected from 33 eyes (17 patients). Median age at IOL implantation was 6.1 years (range, 1.8-18). Median overall follow-up time was 6.2 years (range, 2-13.5). Median ECC follow-up time was 6.2 years (range, 2-10). Mean best-corrected visual acuity was 0.71 ± 0.38 logMAR before surgery and 0.02 ± 0.25 logMAR at final follow-up. The mean annual ECC decline was 0.71% ± 2.24. Total cell loss from first to last postoperative measurement was 150 cells ± 394 cells/mm (4.81%). Pre- and first postoperative data were available for 17 eyes of 10 patients, with a mean cell loss before and directly after surgery of 269 ± 268 cells (7.94%). Surgery related complications were iris bombé due to blockage of peripheral iridectomy in 3 eyes and claw dislocation due to direct impact trauma in 3 eyes.

CONCLUSIONS

In our large, pediatric study cohort, anterior chamber iris claw IOL implantation resulted in an excellent visual outcome and normal endothelial cell loss compared with normative data. Safety measures are recommended to avoid traumatic dislocation of IOLs.

摘要

目的

报告 2007 年 9 月至 2020 年 8 月在一家单中心接受晶状体切除术联合原发性前房虹膜爪型人工晶状体植入术的马凡综合征患者(年龄≤18 岁)的长期临床和内皮细胞计数(ECC)结果。

方法

回顾性分析了 2007 年 9 月至 2020 年 8 月期间在一家单机构接受手术的马凡综合征患者的病历,这些患者的随访时间至少为 2 年。分析的数据包括:性别、手术年龄、手术适应证、晶状体在未散瞳和散瞳瞳孔中的位置、角膜内皮细胞计数(ECC)、围手术期并发症、术前和术后最佳矫正视力。

结果

共纳入 23 例患者(12 名女孩和 11 名男孩)的 42 只眼。33 只眼(17 例)至少有 2 次或更多次术后 ECC 采集。人工晶状体植入时的中位年龄为 6.1 岁(范围,1.8-18 岁)。中位总随访时间为 6.2 年(范围,2-13.5 年)。中位 ECC 随访时间为 6.2 年(范围,2-10 年)。术前最佳矫正视力平均为 0.71 ± 0.38 logMAR,末次随访时为 0.02 ± 0.25 logMAR。平均每年 ECC 下降 0.71%±2.24%。从第一次到最后一次术后测量,细胞总数损失 150 个±394 个/平方毫米(4.81%)。17 只眼(10 例患者)有术前和首次术后数据,术前和术后直接测量的细胞损失平均为 269±268 个(7.94%)。与手术相关的并发症有 3 只眼因周边虹膜切除术阻塞引起虹膜膨隆,3 只眼因直接撞击性外伤引起爪形脱位。

结论

在我们的大型儿科研究队列中,与正常数据相比,前房虹膜爪型人工晶状体植入术可获得良好的视力结果和正常的内皮细胞丢失。建议采取安全措施,避免人工晶状体的外伤性脱位。

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