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Section II · Clinical Forms

Chapter 13: Congenital Glaucomas

Shields' Textbook of Glaucoma, 6th edition

Showing 1–20 of 617 articles

17.
Cohort Study

Incidence of Strabismus in Childhood Glaucoma.

Rickels Kaersti L, Chauhan Muhammad Z, Dagi Linda R et al.

Ophthalmol SciJul 2025

Childhood glaucoma significantly increases strabismus risk (7-14x higher than controls), with younger age, nystagmus, and amblyopia further elevating risk, highlighting the need for vigilance.

18.
Observational Study

Two-Year Results of Gonioscopy-Assisted Transluminal Trabeculotomy Versus Ab Externo Visco Circumferential Suture Trabeculotomy in Primary Congenital Glaucoma.

Elwehidy Ahmed Samy, Elhofi AbdElhamid Shaker, Abdelkader Amr Mohammed Elsayed et al.

J GlaucomaJul 2025

This study found two surgical techniques, GATT and AVCST, similarly and significantly lowered eye pressure in primary congenital glaucoma over two years, with comparable safety. Both are effective options for children.

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Optical coherence tomography (OCT) provides structural information that facilitates early diagnosis, informs assessment of disease severity, guides management decisions, and enables longitudinal follow-up across pediatric eye diseases, including prematurity-related pathology, congenital optic disc anomalies, foveal hypoplasia, optic neuropathies, glaucoma, retinal vascular and inflammatory disorders, inherited retinal dystrophies, ocular tumors, and neuro-ophthalmic conditions.

DiagnosisReviewn=Not applicable

Parafoveal degeneration of the outer retinal layers was observed in 59% of patients with genetically confirmed Mucopolysaccharidosis type III (n = 27, median age 6.5 years, range 1-16 years) in a retrospective monocenter case series.

DiagnosisCase seriesRetrospective monocenter case seriesn=27 patients with genetically confirmed MPS III

Foveal thickening of the external limiting membrane was noted in 67% of patients with genetically confirmed Mucopolysaccharidosis type III (n = 27, median age 6.5 years, range 1-16 years) in a retrospective monocenter case series.

DiagnosisCase seriesRetrospective monocenter case seriesn=27 patients with genetically confirmed MPS III

One patient with Mucopolysaccharidosis type IIIA (out of 16 MPS IIIA patients) showed severe foveal atrophy, and another presented with foveal intraretinal fluid accumulation, in a retrospective monocenter case series of 27 patients with genetically confirmed MPS III.

DiagnosisCase seriesRetrospective monocenter case seriesn=16 MPS IIIA patients within a cohort of 27 MPS III patients

Quantitative retinal thickness analysis in 27 patients with genetically confirmed Mucopolysaccharidosis type III (median age 6.5 years, range 1-16 years) showed median values at the lower end of the reference spectrum in the inner perifoveal ring and below the normal range in the outer perifoveal ring, with no distinct pattern distinguishing subtypes or phenotypes, in a retrospective monocenter case series.

DiagnosisCase seriesRetrospective monocenter case seriesn=27 patients with genetically confirmed MPS III

Retinal nerve fiber layer (RNFL) thickness was unremarkable in all 27 patients with genetically confirmed Mucopolysaccharidosis type III (median age 6.5 years, range 1-16 years) in a retrospective monocenter case series.

DiagnosisCase seriesRetrospective monocenter case seriesn=27 patients with genetically confirmed MPS III

In a posthoc cohort analysis from a multicenter randomized controlled trial in the United States (NCT00212134) involving 110 participants with unilateral congenital cataract who underwent cataract surgery between 2004 and 2007 and were followed for 10.5 years, subjects diagnosed with glaucoma had an anterior chamber depth (ACD) of 2.76 ± 0.48 mm compared to 3.08 ± 0.38 mm among subjects without glaucoma or glaucoma suspect (mean difference = 0.32 mm, P = .003).

PrognosisCohortPosthoc Cohort Analysisn=110 participants with unilateral congenital cataract

In a posthoc cohort analysis from a multicenter randomized controlled trial in the United States (NCT00212134) involving 110 participants with unilateral congenital cataract who underwent cataract surgery between 2004 and 2007 and were followed for 10.5 years, logistic regression models found shallower anterior chamber depth (ACD) was a strong predictor of glaucoma (OR 5.8 [1.8, 18.9], P = .004) at 10.5 years following congenital cataract surgery.

PrognosisCohortPosthoc Cohort Analysisn=110 participants with unilateral congenital cataract

In a posthoc cohort analysis from a multicenter randomized controlled trial in the United States (NCT00212134) involving 110 participants with unilateral congenital cataract who underwent cataract surgery between 2004 and 2007 and were followed for 10.5 years, other factors such as shorter axial length (OR 1.0 [0.7, 1.4], P = .9), thinner lens (OR 1.5 [0.7, 3.1], P = .3), decreased corneal diameter (OR 1.6 [0.95, 2.9], P = .08), and younger age (OR 1.01 [1.0, 1.02], P = .04) were not as strong predictors of glaucoma compared to shallower anterior chamber depth.

PrognosisCohortPosthoc Cohort Analysisn=110 participants with unilateral congenital cataract

A posthoc cohort analysis from a multicenter randomized controlled trial (NCT00212134) included 114 participants with unilateral congenital cataract who underwent cataract surgery between 2004 and 2007 and were followed for 10.5 years as part of the Infant Aphakia Treatment Study, with 110 participants analyzed and 88 having at least one biometry measurement available.

MethodologyCohortPosthoc Cohort Analysisn=110 participants with unilateral congenital cataract

In a posthoc cohort analysis from a multicenter randomized controlled trial in the United States (NCT00212134) involving 110 participants with unilateral congenital cataract who underwent cataract surgery between 2004 and 2007 and were followed for 10.5 years, overall, 41% of the cohort was diagnosed as glaucoma or glaucoma suspect by study endpoint.

EpidemiologyCohortPosthoc Cohort Analysisn=110 participants with unilateral congenital cataract

The prevalence of primary congenital glaucoma (PCG) was significantly higher in patients with fibrous ingrowth (FI-CSR group) (8/12 [66.7%]) compared to those without abnormal ingrowth (CSR group) (11/28 [39.3%] cases, P < 0.001) undergoing capsulectomy shunt revision (CSR) for failed Ahmed Glaucoma Valves (AGVs).

PrognosisCase seriesProspective interventional case seriesn=40 eyes of 40 patients