Treatment and Prognosis

Treatment of PCG typically requires surgical intervention following a thorough examination with the patient under anesthesia (see the Surgical Management section later in this chapter). Medical therapy has limited long-term value but may be used to temporize or reduce corneal edema to improve visualization during surgery.

PCG generally has a better visual prognosis than do most secondary pediatric glaucomas. In US retrospective reviews, approximately two-thirds of patients with PCG have a visual acuity of 20/70 or better at final follow-up. However, in cases of newborn PCG, in cases of PCG diagnosed after age 1, or in patients with corneal diameters larger than 14 mm at diagnosis the prognoses may be very poor; severe dysgenesis can make IOP control difficult or there may be late-stage optic nerve damage at the time of diagnosis. More than 50% of patients with newborn PCG progress to legal blindness. The prognosis is best for patients whose glaucoma is diagnosed between the ages of 3 and 12 months, because most of these cases respond to angle surgery.

• Ko F, Papadopoulos M, Khaw PT. Primary congenital glaucoma. Prog Brain Res. 2015; 221:177–189.

Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.