The childhood glaucomas

Abstract

The childhood glaucomas are a diverse group of ocular disorders that must be recognized early and treated to prevent secondary loss of vision. Primary congenital glaucoma is the most common type and is typically recognized by 1 year of age and responds well to surgery. Childhood glaucoma secondary to other diseases and to ocular injury must also be managed to save vision. The filtration angle pathology in affected eyes with pediatric glaucoma is varied and includes both primary congenital anomalies and abnormalities secondary to increased intraocular pressure and secondary surgery. The occurrence of glaucoma in childhood is unusual with an incidence of approximately 1:10,000. The CYPB1 gene is the most frequent identified mutated gene abnormality. The clinical signs of these glaucomas are variable dependent on the age of the patient and the severity of the intraocular pressure abnormality. In infancy corneal opacification and enlargement is typical, and later in childhood diagnosis is made by recognizing secondary loss of vision. Some children are at increased risk for glaucoma including those with a facial nevus flammeus, chronic intraocular inflammation, history of infantile cataract surgery, and a familyhistory of early-onset familial glaucoma. Successful tonometry is an essential component of theexamination of affected children for both diagnosis and follow-up assessments after surgery. Gonioscopy provides essential information for accurately classifying childhood glaucoma and for judging the severity of the causal angle anomalies. Medical therapy is often the initial glaucoma treatment, but most young glaucoma patients soon require glaucoma surgery. Goniosurgery is the most frequent procedure and is very successful for many primary congenital glaucoma patients.For patients unresponsive to goniosurgery, glaucoma tube-shunt procedures and filtration procedures may be very successful. Refractive and strabismus amblyopia can develop and must be treated to prevent related loss of vision in patients with well-controlled pressures.