Long-term functional outcomes of different subtypes of primary congenital glaucoma.

AIM: To analyse long-term visual outcomes across different subtypes of primary congenital glaucoma (PCG). METHODS: Patients with PCG with a minimum of 5-year follow-up post surgery were included in the study. Snellen visual acuity recordings taken at their last follow-up were analysed. We evaluated the results using Kaplan-Meier curves to predict the probability of maintaining good vision (as defined by a visual acuity of 6/18 or better) in our patients after 30-year follow-up. The results were also analysed to determine whether there were any differences in the long-term visual acuities with time between the neonatal and infantile PCG. We also analysed the reasons for poor visual outcomes. RESULTS: We assessed a cohort of 140 patients with PCG (235 eyes) with an average follow-up of 127±62.8 months (range 60-400 months). Overall, the proportion of eyes with good visual acuity was 89 (37.9%), those with fair visual acuity between 6/60 and 6/18 was 41 (17.4%), and those with poor visual acuity (≤6/60) was 105 (44.7%). We found a significant difference (p=0.047) between neonatal and infantile patients with PCG whereby the neonatal cohort fared worse off in terms of visual morbidity. On Kaplan-Meier analysis, the cumulative probability of survival of a visual acuity of 6/18 or better was more among the infantile PCG in comparison to the neonatal PCG (p=0.039) eyes, and more among the bilateral than the unilateral affected eyes (p=0.029). Amblyopia was the most important cause for poor visual acuity as shown on a Cox proportional-hazards regression model. CONCLUSIONS: Long-term visual outcomes of infantile are better than neonatal PCG. Eyes with unilateral have worse visual outcomes compared with those with bilateral PCG because of the development of dense amblyopia.

Early Predictors of Long-term Outcomes in Childhood Glaucoma.

PURPOSE: A data-based staging system for childhood glaucoma is likely to improve the prediction of visual acuity and intraocular pressure (IOP) control outcomes. We investigated early clinical factors associated with poor long-term visual, and IOP control outcomes in early-onset glaucoma as the initial steps to constructing a severity staging system. DESIGN: Statistical modeling of retrospective case series data. PARTICIPANTS: Glaucoma patients younger than 3 years of age who presented to Bascom Palmer Eye Institute between 1990 and 2010 with at least 5 years of follow up. METHODS: Statistical modeling of retrospective case series data from first, second, third, 3-year, 5-year, and final visits. MAIN OUTCOME MEASURES: Association of early clinical characteristics to final IOP-control outcomes and visual acuities. RESULTS: A total of 26 eyes of 15 children were included. Nine of 15 (60%) of patients were male. Mean age at initial presentation: 9.98±10.55 months. Mean duration between initial and final visits: 11.13±3.55 years. By the third visit (mean 6.69 mo after presentation), presence of nystagmus, anterior segment dysgenesis (ASD) or having failed angle surgery increased the final LogMAR visual acuity by 0.76 (P=0.0516), 0.64 (P=0.0618), and 0.58 (P=0.0159), respectively. At year 3, failed amblyopia therapy, failed angle surgery, nystagmus, media opacity (MO), or ASD increased the final LogMAR by 1.30, 1.34, 1.21, 0.85, 0.64, respectively (all P<0.02). Failed angle surgery or MO increased the chance of uncontrolled IOP at the final visit (proportional odds ratio of 6.77 and 12.88, respectively). CONCLUSIONS: In this pilot study of a modest size cohort, the presence of nystagmus, ASD or failed angle surgery early in the course of infantile-onset glaucoma management predicted poor final visual outcome, whereas failed angle surgery and presence of MO predicted poor final IOP control. These predictors of poor outcomes will serve as the initial steps in constructing a severity staging system.

A retrospective survey of childhood glaucoma prevalence according to Childhood Glaucoma Research Network classification.

PURPOSE: To evaluate the Childhood Glaucoma Research Network (CGRN) classification system and describe the prevalence of each subtype according to this classification. MATERIALS AND METHODS: Retrospectively, the medical records of 205 consecutive childhood glaucoma and glaucoma suspect patients at an urban tertiary care center were reviewed. The initial diagnosis and new diagnosis according to CGRN classification were recorded. RESULTS: All patients fit one of the seven categories of the new classification. Seventy-one percent of diagnoses were changed upon reclassification. Twenty-three percent of patients had primary glaucoma (juvenile open-angle glaucoma and primary congenital glaucoma [PCG]); 36% had secondary glaucoma (glaucoma associated with nonacquired ocular anomalies; glaucoma associated with nonacquired systemic disease or syndrome; glaucoma associated with acquired condition; and glaucoma following cataract surgery); and 39% were glaucoma suspect. Of the patients diagnosed with glaucoma, PCG was the most common diagnosis, seen in 32% of patients. CONCLUSION: The CGRN classification provides a useful method of classifying childhood glaucoma.