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Title: [Clinical characteristics and treatment of congenital fibrovascular pupillary membranes].
Author: Liang TW, Zhang CY, Bai DY, Peng CX, Bai XQ, Wu Q, Zhao JY, Li L.
Journal: Zhonghua Yan Ke Za Zhi; 2018 Nov 11; 54(11):849-854. PubMed ID: 30440157.
Abstract:
Objective: To investigate the clinical characteristics, surgical approaches and postoperative effects associated with congenital fibrovascular pupillary membranes. Methods: A retrospective study design was used. Thirteen children (13 eyes) with congenital fibrovascular pupillary membranes, treated in Beijing Children's Hospital from January 2014 to December 2017 were included. The morphology of the membrane and the anterior chamber was evaluated using a digital wide-area fundus imaging system. The ophthalmic signs, examination results, operation methods, intraocular pressure and ocular position were analyzed. Results: There were 13 children (13 eyes) were enrolled, including 9 males and 4 females. The age at surgery ranged from 2.0 months to 34.5 months, with an median of 5.1 months. According to the degree of obstruction of the pupil and the intraocular pressure, the eyes were divided into three groups. In the 5 eyes of group A, the pupil membrane did not completely cover the pupil, and the depth of the anterior chamber was normal. Among them, 4 eyes had normal intraocular pressure (9-12 mmHg) (1 mmHg=0.133kPa), and 1 eye had elevated intraocular pressure (18 mmHg). In the 5 eyes of group B, the pupillary membrane completely covered the pupil into a pinhole, the anterior chamber was normal or slightly shallow, and the intraocular pressure was normal (6-16 mmHg). In the 3 eyes of group C, the pupillary membrane completely covered the pupil, the anterior chamber was shallow or disappeared, and the intraocular pressure was high (24-45 mmHg). Membranectomy and pupilloplasty were performed in group A, and trabeculectomy was combined when there was glaucoma; postoperative intraocular pressure was normal (4-10 mmHg). Membranectomy, pupilloplasty and iridectomy were performed in group B; postoperative intraocular pressure was normal (7-13 mmHg). Membranectomy, pupilloplasty, iridectomy and goniosychialysis were performed in group C; after surgery, intraocular pressure was normal in 2 eyes (10 mmHg and 13 mmHg) and 25 mmHg in 1 eye. All eyes were orthophoric before and after operation in group A. In group B, 1 eye was esotropic, 2 eyes were exotropic (worse after surgery in 1 eye), and 2 eyes were orthophoric before surgery. In group C, one eye was esotropic, one eye was exotropic, and one eye was orthophoric before surgery, and all eyes were exotropic after operation. Conclusions: Congenital fibrovascular pupillary membranes are unilaterally a continuation of the iris covering the pupil at different degrees, with or without glaucoma. Surgical treatment should be performed promptly when there is obscuring of the visual axis or incorporating of glaucoma. The main surgical procedures are membranectomy and pupilloplasty and iridectomy. Postoperative intraocular pressure can be well controlled, and strabismus has no improvement. (Chin J Ophthalmol, 2018, 54:849-854). 目的： 总结先天性纤维血管性瞳孔膜的临床特点、手术方式和术后效果。 方法： 回顾性系列病例研究。连续纳入2014年1月至2017年12月北京儿童医院眼科先天性纤维血管瞳孔膜的患儿13例（13只眼）的临床资料。膜和房角的形态采用数字化广域眼底成像系统评估。对患儿眼部体征、检查结果、采用的手术方式及术后的眼压和眼位情况进行总结分析。 结果： 13例患儿中男性9例，女性4例，手术年龄2.0~34.5个月，中位数5.1个月。根据膜遮挡瞳孔程度和眼压将患儿分为3组：A组5例（5只眼），瞳孔膜未完全遮盖瞳孔，前房深度正常，其中4只眼眼压正常（9~12 mmHg）（1 mmHg=0.133kPa），1只眼眼压升高（18 mmHg）；B组5例（5只眼）：瞳孔膜完全遮盖瞳孔成针孔样，前房正常或者稍浅，眼压正常（6~16 mmHg）；C组3例（3只眼）：瞳孔膜完全遮盖瞳孔，前房浅或者消失，眼压高（24~45 mmHg）。A组均行膜切除瞳孔成形术，伴随青光眼的患眼联合小梁切开术，术后眼压均正常（4~10 mmHg）；B组均行膜切除瞳孔成形+虹膜周切术，术后眼压均正常（7~13 mmHg）；C组均行膜切除瞳孔成形+虹膜周切+房角分离术，术后1只眼眼压25 mmHg，2只眼眼压正常（10、13 mmHg）。A组术前、术后5只眼眼位均为正位；B组术前1只眼内斜、2只眼外斜、2只眼正位，术后1只眼外斜较术前加重，其余无明显变化；C组术前1只眼内斜、1只眼外斜、1只眼正位，术后3只眼均为外斜。 结论： 先天性纤维血管瞳孔膜是虹膜瞳孔领延续的白膜，不同程度遮盖瞳孔，伴有或不伴有青光眼，为单眼发病。当遮挡视轴或合并青光眼时要及时手术治疗，术式主要是膜切除瞳孔成形虹膜周切术，术后眼压控制良好，斜视改善不良。（中华眼科杂志，2018，54：849-854）.

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