129例永存原始玻璃体增生症的临床特征和术后疗效分析

Clinical characteristics and surgical outcomes of 129 cases of persistent hyperplastic primary vitreous

目的分析永存原始玻璃体增生症(PHPV)的临床特征及术后疗效。方法回顾性分析了129例PHPV患儿的相关临床资料,包括术前视力、眼压、眼轴长度以及PHPV分型,以单眼患儿的对侧眼作为正常对照组。并对适应证患儿进行手术治疗,手术方法以晶状体切除和玻璃体切除为主;随访时间1～40个月。结果 129例PHPV病例(138眼)中,男性82例(63.6%),女性47例(36.4%);初诊年龄6 d～13岁,平均(2.8±3.1)岁。PHPV分型:前部型占5.1%(7/138),后部型占15.2%(21/138),混合型占79.7%(110/138)。术前检查显示:33.3%(11/33)的患眼视力≥0.1,27.3%(9/33)的患眼视力低于手指数(包括无光感及光感和手动),28只正常对照眼视力均≥0.1;77.1%(37/48)的患眼眼压正常,95.2%(40/42)的正常对照眼眼压正常;术前不同年龄段儿童患眼(共45眼)的平均眼轴长度均小于正常对照眼(共36眼)。47例患儿(49眼)获得随访,其中手术治疗34例,术后患眼并发症基本得以解除,共有10只患眼测得视力,30%(3/10)的患眼视力≥0.1,10%(1/10)的患眼视力低于手指数。结论该组PHPV患儿群体平均就诊年龄偏大、年龄跨度大,以混合型及后部型病例为主。手术病例视力改善有限,但对有用视力保存、并发症防治有较好的效果。

Objective To investigate the clinical characteristics and surgical outcomes of persistent hyperplastic primary vitreous （PHPV）. Methods The clinical data of 129 children with PHPV were retrospectively analysed, including preoperative visual acuity, intraocular pressure, ocular axial length and PHPV types, and the contralateral eyes of the affected ones were served as normal control group. Surgical treatment was performed in children with indications, and the major surgical procedures were lensectomy and vitrectomy. Patients were followed up for 1 to 40 months. Results A total of 129 patients （138 eyes） were enrolled, among whom 82 were males （63.6%） and 47 were females （36.4%）. The age of initial diagnosis ranged from 6 d to 13 years, with the average of （2.8 ± 3.1） years. As to the PHPV types, anterior type accounted for 5.1% （7/138）, combined type 79.7% （110/138） and posterior type 15.2% （21/138）. Examinations before surgery indicated that the visual acuity of 33.3% （11/33） of the affected eyes was ≥ 0.1, that of 27.3% of the affected eyes was lower than finger counting, and that of the 28 normal control eyes was ≥0.1. The intraocular pressure was normal in 77.1% （37/48） of the affected eyes, and that was normal in 95.2% of the normal controls. The average ocular axial length of the 45 affected eyes was shorter than that of the 36 normal controls. Forty-seven children （49 eyes） were followed up, including 34 cases with surgery. After surgery, most complications of the affected eyes were eliminated, and visual acuity was detected in 10 eyes, including visual acuity ≥0.1 in 3 affected eyes and that lower than finger counting in 1 affected eye. Conclusion The age is big and the age span is large for initial diagnosis of children with PHPV, which are mainly presented as combined type and posterior type. Improvement in visual acuity is limited for surgical cases, but better results may be achieved in preservation of useful vision and prevention and treatment of complications.