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Primary congenital glaucoma is a rare disease that is not usually seen by the general ophthalmologist. Only one out of 10 000–18 000 newly born children in European populations presents with this disease. However, being a disease of usually recessive inheritance, the incidence may be 10 times higher in communities with high consanguinity, and in these cases the disease may manifest early and often in a more severe form. Cases presenting soon after birth are difficult to treat, especially in the presence of cloudy corneas. Most cases without cloudy corneas are diagnosed late, either by the family members or, less frequently, by an ophthalmologist or paediatrician at a routine neonatal examination. Even after the condition has been suspected, the mean interval between detection and first presentation at the ophthalmologist was still 2.2 months according to a recent study1 in Germany despite adequate access to ophthalmic care.
Despite its low incidence, the management of congenital glaucoma is lifelong and largely dependent on surgical intervention. Primary congenital glaucoma occurs once every 12 000 births, giving an incidence of 0.008% as compared with 0.8% in primary open angle glaucoma in adult caucasians.2 3 Hence, only one of 100 new glaucoma patients will be a patient with primary congenital glaucoma. However, the life expectancy of a newborn with primary congenital glaucoma is 5 times longer than the life expectancy of the elderly patient with POAG. Even with a similar frequency of follow-up visits, a patient with primary congenital glaucoma patient would comprise 5% of a general glaucoma practice. However, because of the frequency of follow-up, the proportion may be much …
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