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
Aniridia	Axenfeld-Rieger syndrome	Trauma
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	Lens-induced
Congenital ocular melanosis (Nevus of Ota)	Neurofibromatosis 1	Aphakic (after cataract surgery without lens implant)
Peters anomaly	Oculocerebrorenal (Lowe) syndrome	Steroid-induced
Posterior polymorphous dystrophy (PPMD)	Stickler syndrome	Ocular infection
Sclerocornea	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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