Glaucoma · Congenital glaucoma

Buphthalmos / Congenital glaucoma

Primary congenital glaucoma (PCG) is a rare form of childhood glaucoma resulting from abnormal development of the eye's drainage system, leading to elevated intraocular pressure (IOP). This increase in IOP damages the optic nerve, potentially causing irreversible vision loss if left untreated. Subtypes of PCG correlate with the age of onset of the disease. These subtypes are: True congenital glaucoma: Also known as newborn glaucoma, this subtype accounts for approximately 25% of cases of PCG. In true congenital glaucoma, the child is either born with ocular enlargement or enlargement of the eyes is noticed within the 1st month of life. The elevation IOP likely occurs during fetal life. Infantile glaucoma: This subtype of PCG includes patients who manifest symptoms between ages 1 and 36 months and accounts for approximately 65% of patients with PCG. Juvenile glaucoma: This subtype accounts for approximately 10% of cases of PCG and includes patients with signs of elevated IOP that manifest between 3 years and adulthood. The Hoskin classification is another classification system for PCG. This system is based on the area of dysgenesis and comprises 3 types. Hoskin type I is referred to as PCG; types II and III are considered secondary congenital glaucoma. Type I, Trabeculodysgenesis: The defect lies in the development of trabecular meshwork. Type II, Iridotrabeculodysgenesis: This includes hypoplasia or hyperplasia of the stroma, anomalous iris vessels, or structural defects in the form of coloboma or aniridia. Type III, Corneotrabeculodysgenesis : This includes complex cases of congenital glaucoma like Axenfield, Rieger, or Peters anomaly.

Contributed byDr. Mohammed Arif KaderiResident, JNMC, Wardha

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