A CASE REPORT ON CONGENITAL GLAUCOMA
Dr. Seema, Dr. Ramesh Kaundal* and Dr. Deepti Parmar
PCG is a rare eye disorder which accounts for 0.01–0.04% of total blindness. The disease is usually manifested at birth or early childhood (before 3 years of age). The incidence of PCG is different in different populations. The majority of patients (about 60%) are diagnosed by the age of 6 months, and 80% are diagnosed within the first year of life. A slight predominance of males is common (about 65%), and involvement is usually bilateral (about 70%).Most cases of PCG occur sporadically. Patients with a familial pattern usually show a recessive pattern with incomplete or variable penetrance and possibly multifactorial inheritance. An autosomal dominant and pseudo-dominant mode of inheritance has also been reported.[2,3] PCG refers to a specific form of developmental glaucoma characterized by an isolated maldevelopment of the trabecular meshwork (isolated trabeculodysgenesis) not associated with other developmental ocular anomalies or ocular disease that can raise the IOP. Also called primary infantile glaucoma, it is the most common form of developmental glaucoma. Most western textbooks describe a classic triad of symptoms comprising epiphora, photophobia and blepharospasm (attributable to IOP-induced corneal epithelial edema). However, one study conducted in a tertiary institution shows that large eyeball size and hazy eyes (from corneal edema) may be the more common presenting features in the Indian subcontinent. Medical therapy usually provides a supportive role to reduce the IOP temporarily, to clear the cornea, and to facilitate surgical intervention. Laser therapy has a limited role in developmental glaucomas. The most effective and definitive form of treatment of most developmental glaucomas is surgical. Primary surgical treatment is usually with goniotomy or trabeculotomy, although combined trabeculotomy with trabeculectomy may be useful in certain populations with a high risk of failure of goniotomy or trabeculotomy. Refractory pediatric glaucomas may be managed by trabeculectomy with anti-fibrosis drugs, glaucoma drainage implants and cyclodestructive procedures.
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