Clinical and Ultrasound Biomicroscopic Characteristics of Congenital Fibrovascular Pupillary Membrane-Induced Secondary Glaucoma.

Yingting Zhu,Shufen Lin,Pingping Liu,Liming Chen,Julius Oatts,Yihua Su,Xiaodi Zhou,Yimin Zhong,Lei Fang,Xing Liu,Ying Han

doi:10.3389/fmed.2021.763137

Yingting Zhu, Shufen Lin + Show 9 more

Open Access

https://doi.org/10.3389/fmed.2021.763137

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Abstract

Purpose: The purpose of this study was to describe and summarize the clinical features of congenital fibrovascular pupillary membrane-induced secondary glaucoma (CFPMSG).Design: Cross-sectional case series.Methods: Eyes of 32 patients with CFPMSG were enrolled. Demographic data, including gender, laterality, age at presentation, and age at onset of glaucoma were collected. Patients underwent comprehensive ophthalmic examinations and ultrasound biomicroscopy (UBM). CFPMSG eyes were classified into three groups based on UBM findings and intergroup analysis was performed using ANOVA.Results: The average age at presentation was 2.4 ± 4.6 months (mean ± SD) and at glaucoma onset was 3.8 ± 4.5 months. Compared to normal fellow eyes, all affected eyes had increased intraocular pressure (IOP), axial length, corneal diameter, and central corneal thickness, and decreased anterior chamber depth (ACD) (all P ≤ 0.001). Twenty-two affected eyes (68.8%) had evidence of glaucomatous optic neuropathy. Based on iris configuration on UBM, eyes were classified as 53% type I (“U” shape), 34% type II (“Y” shape), and 13% type III (no anterior chamber). IOP in types II (33.8 ± 5.9 mmHg) and III (35.2 ± 5.9 mmHg) was significantly higher than in type I eyes (26.5 ± 5.1 mmHg). The ACD was shallower in type II compared to type I (P = 0.045).Conclusion: Congenital fibrovascular pupillary membrane-induced secondary glaucoma is characterized by ocular hypertension, corneal enlargement and edema, axial length elongation, and glaucomatous optic neuropathy. Glaucoma in this condition is secondary to pupillary block and angle-closure. UBM provides important information for the diagnosis and classification of CFPMSG. This novel classification system demonstrated varying levels of severity and may guide on management of this disease.

Highlights

Congenital fibrovascular pupillary membrane (CFPM) refers to a condition where a congenital white membrane covers part or all of the pupil and may be accompanied by anterior chamber angle dysplasia [1]
Seventeen patients were noted to have an abnormality by their parents at birth, and four (24%) presented with symptoms associated with glaucoma within 1 month of age
We summarize the clinical characteristics and propose a novel classification system of congenital fibrovascular pupillary membrane-induced secondary glaucoma (CFPMSG) base on ultrasound biomicroscopy (UBM) images of 32 subjects: the largest series of this rare condition published to date

Summary

Introduction

Congenital fibrovascular pupillary membrane (CFPM) refers to a condition where a congenital white membrane covers part or all of the pupil and may be accompanied by anterior chamber angle dysplasia [1]. Subsequent cases have been described [2,3,4,5,6,7], though without detailed imaging of the anterior chamber Another group described the clinical characteristics of 13 cases of CFPM, though reported unsatisfactory surgical results with a high recurrence rate and poor surgical success [8]. This emphasizes the importance of better understanding and classifying this condition to improve visual outcomes

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