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Disease-related and age-related changes of anterior chamber angle structures in patients with primary congenital glaucoma: An in vivo high-frequency ultrasound biomicroscopy-based study.

ABSTRACT: OBJECTIVES:To provide in vivo measurements of anterior chamber angle structures and their relationship with age as evaluated by high-frequency ultrasound biomicroscopy (UBM) in patients with primary congenital glaucoma (PCG). METHODS:High-frequency UBM was done for 51 PCG eyes from 40 patients (aged from 3 to 96 months) and 11 unaffected contralateral eyes. Parameters, including the proportion of observable abnormal tissue membrane and Schlemm's canal, the largest cross-sectional area (CSA) of Schlemm's canal (SC), SC meridional diameter, trabecular-iris angle (TIA), trabecular meshwork (TM) thickness, iris thickness, ciliary process length, and corneal limbus thickness were compared between the two groups and their relationship with age was explored in PCG eyes. RESULTS:Abnormal tissue membrane was detected in 27.5% of PCG eyes and none in unaffected eyes. SC was observed in 73.1% of PGC eyes compared to 100% in unaffected eyes (P<0.001). The largest CSA of SC, SC meridional diameter, iris thickness, and corneal limbus thickness were all significantly smaller in PCG eyes compared to unaffected eyes (all P<0.05). TIA and ciliary process length in unaffected eyes were smaller than PCG eyes (both P<0.05). The largest CSA of SC, TM thickness, iris thickness, and ciliary process length were all significantly correlated to age in PCG eyes (P<0.05). CONCLUSIONS:The anatomical information evaluated by high-frequency UBM may provide glaucoma specialists a useful tool to aid in understanding the dysgenesis and changes with age of anterior chamber angle in PCG.

SUBMITTER: Shi Y

PROVIDER: S-EPMC6986727 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Disease-related and age-related changes of anterior chamber angle structures in patients with primary congenital glaucoma: An in vivo high-frequency ultrasound biomicroscopy-based study.

Shi Yan Y Han Ying Y Xin Chen C Hu Man M Oatts Julius J Cao Kai K Wang Huaizhou H Wang Ningli N

PloS one 20200128 1

<h4>Objectives</h4>To provide in vivo measurements of anterior chamber angle structures and their relationship with age as evaluated by high-frequency ultrasound biomicroscopy (UBM) in patients with primary congenital glaucoma (PCG).<h4>Methods</h4>High-frequency UBM was done for 51 PCG eyes from 40 patients (aged from 3 to 96 months) and 11 unaffected contralateral eyes. Parameters, including the proportion of observable abnormal tissue membrane and Schlemm's canal, the largest cross-sectional ...[more]

PMID: 31990918

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Project description:BackgroundAxenfeld-Rieger syndrome (ARS), a developmental disorder, involves anterior segment abnormalities and can lead to glaucoma. However, limited research has addressed the ultrasound biomicroscopy (UBM) characteristics of ARS. This study aimed to assess the anterior chamber angle features using UBM in ARS and determine their correlation with glaucoma severity and mutant genes.MethodsUBM examination was conducted for 42 patients diagnosed with ARS and glaucoma. The morphology of the anterior chamber angle was classified into 6 types (type A, pure high iris insertion; type B, posterior embryotoxon; type C, iris process; type D, trabecular-iris synechia; type E, peripheral iridocorneal adhesion; type F, goniodysgenesis). Candidate genes were sequenced with next-generation sequencing. Correlations of clinical characteristics with angle dysgenesis types and mutant genes were analyzed.ResultsAmong the 42 patients recruited, 6 eyes were excluded for poor quality UBM images or lack of glaucoma development. The remaining 78 eyes were categorized into 6 groups according to their dominant type of anterior chamber angle (>2 quadrants). There were statistically significant differences in onset age of glaucoma (P<0.001), untreated intraocular pressure (IOP) (P=0.016), vertical cup to disc ratio (P=0.001), and age at surgery (P<0.001) among the groups. Eyes in the type C and D groups developed glaucoma and underwent surgery at an earlier age, while eyes in the type B, E, and F groups developed glaucoma at a relatively later age. Eyes in type A group developed glaucoma and underwent surgery at the latest age, and had the lowest untreated IOP compared to the other groups. Patients with FOXC1 defects were more likely to have angle type B, type C, and type D (accounting for 93.8% of the total), whereas patients with PITX2 defects were more likely to have angle type A, type E, and type F (accounting for 92.1% of the total).ConclusionsUBM is powerful for evaluating the anterior segment abnormalities in ARS. Combined with genetic testing results, the morphological classification helps to assess the severity of glaucoma.

Dataset Information

Disease-related and age-related changes of anterior chamber angle structures in patients with primary congenital glaucoma: An in vivo high-frequency ultrasound biomicroscopy-based study.

Publications

Disease-related and age-related changes of anterior chamber angle structures in patients with primary congenital glaucoma: An in vivo high-frequency ultrasound biomicroscopy-based study.

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