Loukovaara Sirpa
Unit of Vitreoretinal Surgery, Department of Ophthalmology, Helsinki University Hospital, Helsinki, Finland.
Individualized Drug Therapy Research Program, University of Helsinki, Helsinki, Finland.
Clin Ophthalmol. 2024 Aug 24;18:2387-2396. doi: 10.2147/OPTH.S472028. eCollection 2024.
To delineate the characteristics, surgical interventions, and visual outcomes among children diagnosed with congenital cataract and persistent fetal vasculature (PFV).
Retrospective observational single-center study was conducted between January 1, 2009, and December 31, 2019, at Helsinki University Hospital. The national cohort encompassed 82 children aged from birth to 15 years who underwent lensectomy, 3-port vitrectomy, or a combined procedure, with the objective of achieving visual rehabilitation. Among the surgical cohort, paediatric cases with International Classification of Disease (ICD-10) codes Q14.0 for PFV and Q12.0 for congenital cataract were identified and analyzed. Data were collected through a comprehensive review of medical records, encompassing clinical history (birth weight), gender distribution, ocular parameters (laterality, intraocular pressure [IOP], visual acuity [VA]), details of cataract and vitreoretinal surgical interventions, indications for surgery, postoperative ophthalmic complications, as well as evaluations of functional and anatomical outcomes.
The cohort consisted of 11 children, ranging in age from 6 months to 12 years. Surgical intervention resulted in the attainment of at least light perception vision in nine of the operated eyes, representing 81.8% of cases. Among these, two eyes (18.2%) achieved hand motion vision, while 5 eyes (55.6%) achieved vision of finger counting or better. Additionally, two eyes (18.2%) achieved visual acuity measurable on the Snellen chart. However, one eye (9.1%) experienced complete vision loss, while the contralateral eye developed sympathetic ophthalmia.
Congenital cataract with PHV without anterior segment pathology associated with best postoperative VA, while eyes with anterior segment pathology or a complex posterior segment pathology had poor visual prognosis. Case selection for surgery is considered of great importance, since sympathetic ophthalmia may occur postoperatively. Understanding more deeply pathogenesis of PFV is warranted.
描述被诊断患有先天性白内障和永存原始玻璃体增生症（PFV）的儿童的特征、手术干预措施及视觉预后。
于2009年1月1日至2019年12月31日在赫尔辛基大学医院进行了一项回顾性观察单中心研究。全国队列包括82名年龄从出生至15岁接受晶状体切除术、三通道玻璃体切除术或联合手术以实现视觉康复的儿童。在手术队列中，识别并分析了国际疾病分类（ICD - 10）编码为Q14.0的PFV和Q12.0的先天性白内障的儿科病例。通过全面查阅病历收集数据，包括临床病史（出生体重）、性别分布、眼部参数（眼别、眼压[IOP]、视力[VA]）、白内障和玻璃体视网膜手术干预细节、手术指征、术后眼科并发症以及功能和解剖学预后评估。
该队列由11名年龄在6个月至12岁之间的儿童组成。手术干预使9只手术眼至少达到光感视力，占病例的81.8%。其中，2只眼（18.2%）达到手动视力，5只眼（55.6%）达到数指视力或更好。此外,2只眼（18.2%）达到了Snellen视力表可测量的视力。然而，1只眼（9.1%）出现完全视力丧失，对侧眼发生交感性眼炎。
无前节病变的先天性白内障合并PHV术后视力最佳，而有前节病变或复杂后节病变的眼视觉预后较差。手术病例选择至关重要，因为术后可能发生交感性眼炎。有必要更深入地了解PFV的发病机制。
