Thursday, December 28, 2006

Chapter 10: Pediatric Glaucoma

Section 10-A: Types of pediatric glaucoma

Pediatric glaucoma (also referred to as childhood glaucoma, infantile glaucoma or congenital glaucoma) is a relatively rare disease, as most patients with glaucoma are adults. However, pediatric glaucoma can lead to loss of vision and blindness in a young child and will profoundly affect the child’s life, if not diagnosed promptly and treated appropriately. Pediatric glaucoma can include a number of different diagnoses. Primary congenital glaucoma occurs in the first three years of life (usually within the first 6 months of life) without associated ocular or systemic abnormalities. Other pediatric glaucomas are associated with ocular and/or systemic abnormalities, or due (secondary) to another disease (Table 10-1). The remainder of this chapter describes the primary congenital glaucoma.

Primary pediatric glaucoma associated with ocular abnormalities

Primary pediatric glaucoma associated with systemic abnormalities

Secondary pediatric glaucoma


Axenfeld-Rieger syndrome


Axenfeld-Rieger syndrome

Congenital rubella

Intraocular tumors (e.g., retinoblastoma)

Congenital hereditary endothelial dystrophy (CHED)

Cutis marmorata telangiectasia congenita

Uveitis (ocular inflammation)

Congenital microcornea with myopia

Marfan syndrome


Congenital ocular melanosis (Nevus of Ota)

Neurofibromatosis 1

Aphakic (after cataract surgery without lens implant)

Peters anomaly

Oculocerebrorenal (Lowe) syndrome


Posterior polymorphous dystrophy (PPMD)

Stickler syndrome

Ocular infection


Sturge-Weber syndrome

Angle-closure (e.g., retinopathy of prematurity)

Table 10-1. Different types of pediatric glaucomas.

The primary congenital glaucoma is thought to be an autosomal recessive disease and can be associated with positive family history. However, in many cases of primary congenital glaucoma, there is no obvious family history. It occurs approximately one in 30,000 live births, and the risk for congenital glaucoma is increased if there is a pre-existing family history. Recently, molecular genetic studies have identified CYP1B1 gene as a cause for congenital glaucoma in patients with positive family history (see Chapter 11 for more).

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