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PA Quiram, DO Hodge, J Burke, DH Johnson, MS Good; Congenital Glaucoma: A Population-based Study and Analysis of Cases at the Mayo Clinic . Invest. Ophthalmol. Vis. Sci. 2002;43(13):3320.
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Purpose: To evaluate the incidence, clinical features at diagnosis and long-term outcome of congenital glaucoma (CG) in Olmsted County, Minnesota from 1976-1996. Methods: Study patients were identified using the Rochester Epidemiology Project (REP) database, a medical records linkage system established to study the occurrence and natural progression of diseases among residents in Olmsted County, Minnesota (population 100,000). We reviewed records of pediatric patients less than four years of age diagnosed with congenital glaucoma or glaucoma secondary to acquired or systemic illness during a 21year period (1976-1996). To evaluate long-term outcome and clinical features at diagnosis, we documented visual acuity, intraocular pressure (IOP), and optic disc appearance at the time of diagnosis and at 1, 3, 5, 10, 15, 20 years and last follow up visit. Results: Two infants born in Olmsted County during this interval were diagnosed with congenital glaucoma. There were 34,927 births during this 21year period, resulting in an incidence of 0.6 per 10,000 (95% CI: 0.1-2.1 per 10,000). In addition, 20 cases of CG in infants not born but treated in Olmsted Country were identified. Long term follow up studies show a significant correlation between time to diagnosis and a relative decrease in visual acuity by 15 years of age (r= 0.77, p=0.02). The mean IOP at diagnosis was 30 +/- 9 and the cup to disk ratio was 0.5 +/- 0.2. At diagnosis, corneal cloudiness occurred in 81% of patients, photophobia in 67%, and epiphora in 52%. There were an additional 32 cases of secondary glaucoma treated. Sturge-Weber was the most common (28%), followed by PHPV/ROP (16%), aphakic glaucoma (13%), and rubella (6%). Conclusion: In a well-defined population, the incidence of CG was 0.6 per 10,000. Long-term outcome data shows a significant correlation between time to diagnosis and decrease in visual acuity by 15 years of age.
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