目的：分析永存原始玻璃体增生症(PHPV)和先天性纤维血管瞳孔膜(CFPM)的临床特征异同。

方法：回顾性分析2006-03/2021-12在空军军医大学西京医院眼科接受手术治疗的PHPV(PHPV组)和CFPM患儿(CFPM组)的眼部生物测量参数、临床表现、病变的形态学特点。

结果：纳入PHPV患儿56例61眼，CFPM患儿24例25眼； PHPV和CFPM的发病年龄相似、无性别差异，均以单眼患病为主，其占比分别为91%和96%。PHPV合并白内障患眼可有多种并发症和眼发育异常，CFPM主要为不同程度的瞳孔区堵塞及形态异常。PHPV组和CFPM组单眼患病患儿患眼前房深度(ACD)均小于对侧眼，手术年龄≤24月龄患儿患眼眼轴长度(AL)均小于对侧眼(P<0.05)； PHPV组单眼患病患儿患眼角膜直径(CD)小于对侧眼、眼压高于对侧眼(均P<0.05)； CFPM组单眼患病患儿患眼与对侧眼CD、IOP比较均无显著差异(P>0.05)。PHPV组患儿患眼ACD小于CFPM组患眼(P<0.05)。术中发现PHPV纤维血管膜组织位于晶状体后、玻璃体腔内，而CFPM纤维血管膜位于虹膜与晶状体前囊膜之间，很少累及晶状体。

结论：PHPV和CFPM有非常相似的临床特点，提示PHPV和CFPM可能是永存胚胎血管(PFV)的不同表现形式，但PHPV病变范围更广、病情更复杂。

METHODS: Retrospectively analyze the ocular biometric parameters, clinical features and morphological changes in children with PHPV(PHPV group)and CFPM(CFPM group)who received surgery at the department of ophthalmology, Xijing Hospital from March 2006 to December 2021.

RESULTS: The study included 56 cases(61 eyes)of PHPV and 24 cases(25 eyes)of CFPM. There were no differences on the gender and age of onset between PHPV and CFPM, and both of them were mainly unilaterally affected, with the ratio of 91% and 96%. Children with PHPV and cataract combined with other complications and ocular developmental abnormalities. CFPM was mainly presented different degrees of blockage and morphological abnormalities of pupillary area. In unilateral cases of PHPV and CFPM, the anterior chamber depth(ACD)of affected eyes was smaller than that of the fellow eyes, and in subgroups with age of operation ≤24mo, the axial length(AL)of affected eyes was smaller than that of the fellow eyes(PSymbol|@@0.05). The corneal diameter(CD)of the affected eyes in PHPV group was smaller and the intraocular pressure(IOP)was higher than those of the fellow eyes(all PSymbol|@@0.05); while there were no significant differences on CD and IOP between affected eyes and the fellow eyes in CFPM group(P>0.05). The ACD of affected eyes in PHPV group was significantly smaller than that of CFPM group(PSymbol|@@0.05). The fibrovascular membrane in PHPV group was located in the posterior part of the lens and vitreous cavity; while the fibrovascular membrane in CFPM group was located between the iris and the anterior capsule of the lens, rarely involving the lens.

CONCLUSION: PHPV and CFPM had the similar clinical features, suggesting that they may belong to the different variants of persistent fetal vasculature(PFV). However, PHPV had a wider range of lesions and more complex conditions.