先天性白内障合并永存胚胎血管的手术治疗和预后

Surgical Treatment and Prognosis of Congenital Cataract with Persistent Fetal Vasculature

目的：观察先天性白内障合并永存胚胎血管（PFV）患儿的临床特征及手术治疗效果。方法：回顾性系列病例研究。分析2011 年5 月至2017 年6 月在温州医科大学附属眼视光医院杭州院区接受手术治疗的先天性白内障合并PFV患儿24例（28眼）的临床资料，记录手术年龄、手术方式、手术前后眼部检查情况以及手术前后视力。结果：术前合并先天性瞳孔虹膜异常14 眼，晶状体脐状凹陷或悬韧带异常6 眼，后囊膜缺损9 眼，先天性小角膜及角膜缘干细胞异常2 眼，眼底病变2 眼，眼球震颤5 眼，斜视7 眼，全身合并腭裂1 例。大于3 岁患儿7 例（10 眼），行“晶状体切除＋前段玻璃体切割＋人工晶状体（IOL）植入术”，随访8.0（2.5～41.0）个月，末次随访视力均在0.1 以上，2 例（2 眼）在0.3 以上；小于1岁患儿17例（18眼），行“晶状体切除＋前段玻璃体切割术”，选择性注视卡Snellen视力均达0.1以上，注视追视及眼球震颤均明显改善；6例（6眼）患儿一期先行“晶状体切除＋前段玻璃体切割术”，年龄（5.5±2.6）个月，二期再行IOL植入术，年龄（23.5±8.1）个月，1例（1眼）达到0.6，接近正常同龄儿童，5 例（5 眼）达到标准对数视力表视力0.1 以上。手术后随访4 个月至6 年（中位数12.0 个月）， 无严重并发症。结论：先天性白内障合并PFV大部分为单眼发病。微创晶状体切除联合玻璃体切割术后并发症的发生率非常低，术后视力明显提高，少部分可以达到正常视力。

Objective： To observe the clinical characteristics and prognosis of surgical intervention in congenital cataract associated with persistent fetal vasculature （PFV）. Methods： In this retrospective case series study, 28 eyes of 24 congenital cataract children with PFV were enrolled from May 2011 to June 2017 at Eye Hospital, Wenzhou Medical University （Hangzhou）. Age at surgery, surgical approaches, clinical signs, and visual acuity （VA） were collected both before and after surgery. Results： We found congenital pupil anomalies in 14 eyes, umbilication of lens or zonular anomalies in 6 eyes, posterior capsule defect in 9 eyes, congenital microcornea or corneal limbal stem cell anomalies in 2 eyes, fundus lesion in 2 eyes, nystagmus in 5 eyes, strabismus in 7 eyes, and cleft palate in 1 children. Seven children over 3-years old （10 eyes）underwent lensectomy combined with anterior vitrectomy and intraocular lens （IOL） implantation. The median follow-up duration was 8 months （quartile range, 2.5-41.0 months）, and all patients had achieved VAs over 0.1 at the last follow up, and 2 patients had VAs over 0.3. Seventeen children （18 eyes） under 1 year of age underwent lensectomy combined with anterior vitrectomy. The Snellen VA measured by preferential looking cards was over 0.1 in all patients, with significant improvement of pursuit and nystagmus. Six children （6 eyes） underwent both primary lensectomy combined with anterior vitrectomy （mean ± standard deviation 5.5 ± 2.6 months） and secondary IOL implantation （mean age 23.5 ± 8.1 months）. One eye achieved a VA of 0.6, close to normal for children of the same age, and 5 eyes reached the standard logarithmic chart VA of more than 0.1. The follow-up duration ranged from 4 months to 6 years, and the median follow-up time was 12 months. No serious postoperative complications occurred.Conclusions： Most congenital cataracts with PFV are unilateral. The incidence of complications after microincision lensectomy combined with anterior vitrectomy is low, with significantly improved visual outcomes. Normal visual acuity can be expected in a few cases.