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前部巨眼畸形的生物特征和眼部结构表现

Biometric and Structural Ocular Manifestations of Anterior Megalophthalmos.

作者信息

Chen Tian-Hui, Chen Ze-Xu, Zhang Min, Chen Jia-Hui, Lan Li-Na, Jiang Yongxiang

机构信息

Department of Ophthalmology and Vision Science, Eye & ENT Hospital of Fudan University, Shanghai, China.

Key Laboratory of Myopia of State Health Ministry, Shanghai Key Laboratory of Visual Impairment and Restoration, Shanghai, China.

出版信息

Front Med (Lausanne). 2022 May 19;9:732452. doi: 10.3389/fmed.2022.732452. eCollection 2022.

DOI:10.3389/fmed.2022.732452
PMID:35665341
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9160329/
Abstract

OBJECTIVE

The aim of this study was to examine the biometric ocular manifestations and structural ocular features of anterior megalophthalmos (AM).

METHODS

Fifteen patients with AM (30 eyes) from the Eye & ENT Hospital of Fudan University were included. The age-matched control group consisted of 30 participants (30 eyes) who underwent Pentacam HR and IOLMaster 700 measurements for one normal eye. Data on demographics, biometric manifestations, and genotypes were carefully compared.

RESULTS

A total of 15 patients with AM and 30 control patients were enrolled. There were no differences in age (37.27 ± 19.1 vs. 31.43 ± 19.69 years, = 0.249) between these two groups. AM eyes were characterized by premature cataracts (11/30, 36.67%) and zonular weakness with lens subluxation (22/30, 73.33%) compared with the control group. Notably, 20 of the 30 AM eyes (66.67%) had significant posterior iris bowing, and 16 of the 30 AM eyes (53.33%) showed an enlarged ciliary ring on ultrasound biomicroscopy (UBM). Mean corneal curvature was lower in the AM eyes (42.01 ± 2.06 D vs. 43.14 ± 1.38 D, = 0.023). There was no significant difference in corneal pachymetry and central endothelial cell count between the AM and control groups. Significant differences were found in terms of the anterior chamber and white-to-white (WTW) among the Pentacam HR and IOLMaster 700 in patients with AM ( < 0.05). The difference was 0.53 ± 0.48 mm and 0.36 ± 0.14 mm, respectively ( < 0.001).

CONCLUSION

The results of this cohort study conclude the biometric and structural ocular manifestations in Chinese cohorts. Posterior iris bowing (66.67%) and lens subluxation (73.33%) are the most characteristic findings in patients with AM with anatomical abnormalities of megalocornea and a deep anterior chamber, although corneal biometric manifestations of AM included flatter cornea and lower total corneal astigmatism. The knowledge of ocular manifestations of AM is important for diagnosis and preparation for the operation in advance to avoid intraoperative and postoperative complications. Significant differences were found in the anterior chamber and WTW values between the Pentacam HR and IOLMaster 700. Thus, we suggest that various examinations should be carefully considered before determining an AM diagnosis.

摘要

目的

本研究旨在探讨前部巨眼症(AM)的生物特征性眼部表现和眼部结构特征。

方法

纳入来自复旦大学附属眼耳鼻喉科医院的15例AM患者(30只眼)。年龄匹配的对照组由30名参与者(30只眼)组成,他们对一只正常眼睛进行了Pentacam HR和IOLMaster 700测量。仔细比较了人口统计学、生物特征表现和基因型数据。

结果

共纳入15例AM患者和30例对照患者。两组之间年龄无差异(37.27±19.1岁 vs. 31.43±19.69岁,P = 0.249)。与对照组相比,AM眼的特征为白内障早熟(11/30,36.67%)和晶状体悬韧带薄弱伴晶状体半脱位(22/30,73.33%)。值得注意的是,30只AM眼中有20只(66.67%)出现明显的虹膜后凸,30只AM眼中有16只(53.33%)在超声生物显微镜检查(UBM)中显示睫状环扩大。AM眼的平均角膜曲率较低(42.01±2.06 D vs. 43.14±1.38 D,P = 0.023)。AM组与对照组之间的角膜厚度和中央内皮细胞计数无显著差异。AM患者的Pentacam HR和IOLMaster 700在前房和白对白(WTW)方面存在显著差异(P<0.05)。差异分别为0.53±0.48 mm和0.36±0.14 mm(P<0.001)。

结论

这项队列研究的结果总结了中国队列中的生物特征性和结构性眼部表现。虹膜后凸(66.67%)和晶状体半脱位(73.33%)是AM患者最具特征性的表现,伴有大角膜和前房深的解剖异常,尽管AM的角膜生物特征表现包括角膜更平坦和总角膜散光更低。了解AM的眼部表现对于诊断和提前准备手术以避免术中及术后并发症很重要。Pentacam HR和IOLMaster 700在前房和WTW值方面存在显著差异。因此,我们建议在确定AM诊断之前应仔细考虑各种检查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ceb/9160329/c5e26a5b8cdf/fmed-09-732452-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ceb/9160329/d0c173484443/fmed-09-732452-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ceb/9160329/c5e26a5b8cdf/fmed-09-732452-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ceb/9160329/d0c173484443/fmed-09-732452-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ceb/9160329/c5e26a5b8cdf/fmed-09-732452-g002.jpg

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本文引用的文献

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CPAMD8, a New Gene for Anterior Segment Dysgenesis and Childhood Glaucoma.CPAMD8,一种导致眼前节发育异常和儿童青光眼的新基因。
Ophthalmology. 2020 Jun;127(6):767-768. doi: 10.1016/j.ophtha.2020.02.035.
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How many challenges we may encounter in anterior megalophthalmos with white cataract: a case report.大白内障合并前部巨眼症会遇到多少挑战:一例报告
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