Secondary Childhood Glaucoma

All other types of glaucoma are considered secondary—they are caused by other congenital or acquired ocular anomalies or are associated with systemic disease. Some common secondary glaucomas are discussed in the following sections.

Glaucoma Associated With Nonacquired Ocular Anomalies

Anterior segment abnormalities

Aniridia Aniridia is a bilateral condition characterized by complete or nearly complete absence of the iris. Glaucoma develops frequently in this disorder. Aniridia is discussed in Chapter 20.

Anterior segment developmental anomalies Glaucoma occurs in more than 50% of patients with Axenfeld-Rieger syndrome. Peters anomaly, sclerocornea, microcornea, and congenital iris ectropion are also associated with childhood glaucoma. See also Chapter 20.

Posterior segment abnormalities

Glaucoma may be associated with persistent fetal vasculature (PFV), retinopathy of prematurity (ROP), familial exudative vitreoretinopathy (FEVR), or retinal, iris, or ciliary body tumors.

Glaucoma Associated With Nonacquired Systemic Disease or Syndrome

Sturge-Weber syndrome

Sturge-Weber syndrome (SWS) is a phakomatosis that includes a port-wine stain (nevus flammeus) of the face, intracranial calcifications, and glaucoma. See Chapter 28 for a discussion of glaucoma associated with SWS.

Neurofibromatosis

Glaucoma associated with neurofibromatosis 1 (NF1) can be bilateral or unilateral (see Chapter 28).

Lowe syndrome

Lowe syndrome (oculocerebrorenal syndrome) is an X-linked disorder that presents with glaucoma and bilateral disciform cataracts (see Chapter 28).

Lens-associated disorders

Secondary mechanical glaucoma occurs in Marfan syndrome, homocystinuria, Weill-Marchesani syndrome, and microspherophakia (see Chapter 23).

Secondary Glaucoma Associated With an Acquired Condition

In children, as in adults, glaucoma may also develop secondary to corticosteroid use, uveitis, infection, or ocular trauma. Topiramate causes acute, usually bilateral angle-closure glaucoma secondary to ciliary effusion. Forward displacement of the iris–lens diaphragm causes acute high myopia followed by elevated IOP. Peripheral iridectomy is not effective as treatment, but timely cessation of the medication is.

Glaucoma Following Cataract Surgery

Aphakic glaucoma is a common cause of secondary glaucoma in childhood. The incidence of openangle aphakic glaucoma after congenital cataract surgery varies from 15% to 50% or higher. Aphakic glaucoma often develops years after cataract surgery, but it can occur within weeks to months of surgery and remains a lifelong risk. Consequently, patients who have undergone cataract surgery in