Prognosis and Follow-Up
The development of effective surgical techniques has greatly improved the long-term prognosis for pediatric glaucoma patients, particularly PCG patients asymptomatic at birth who present with onset of symptoms between 3 and 12 months of age. These patients typically have a good prognosis; many achieve visual acuity of at least 20/70 with 5-year follow up, although multiple surgeries may be required, and vision may still decline with longer followup. When symptoms are present at birth or when the disease is diagnosed after 12 months of age, the prognosis is poor, and the risk of blindness is high. Children with secondary pediatric glaucomas tend to have the worst prognosis; up to 50% lose light perception despite treatment. Early referral to vision rehabilitation may be helpful to patients and family.
Pediatric patients whose IOP is controlled by surgery may still experience morbidities related to previous IOP elevation, including amblyopia, corneal scarring, strabismus, anisometropia, cataract, lens subluxation, susceptibility to trauma due to scleral fragility, and recurrent IOP elevation in the affected or unaffected eye. These morbidities can cause serious long-term visual compromise and thus should be addressed promptly.
Amblyopia is a common cause of visual compromise, particularly in patients with unilateral glaucoma, corneal opacification, and/or anisometropia. It is important to treat amblyopia aggressively, addressing conditions contributing to its development, such as refractive error, strabismus, cataract, and corneal pathology. Haab striae and corneal scarring may cause astigmatism. Elevated IOP can lead to buphthalmos in patients with PCG and to progressive myopia and anisometropia in patients with JOAG. Refractive errors should be corrected with spectacles or contact lenses, and use of protective eyewear should be encouraged.
Strabismus may result from glaucoma tube shunt surgery or amblyopia. When performing surgery to correct strabismus, the surgeon should try to minimize conjunctival scarring in anticipation of future glaucoma surgeries and should be cognizant of the sites of prior trabeculectomies and tube shunt implants.
All cases of pediatric glaucoma require lifelong follow-up to monitor IOP, potential complications from prior surgeries, and secondary vision-threatening complications. Because IOP elevation may recur even years later, glaucoma and pediatric ophthalmologists should coordinate care. A team approach to care will involve low vision rehabilitation specialists, pediatricians, genetic counselors, educators, and parents or caregivers. Educating parents or caregivers about the need for lifelong care of a child with glaucoma and involving these children in their own care enhances the long-term management of this challenging disease (for more on low vision rehabilitation in pediatric patients, see BCSC Section 6, Pediatric Ophthalmology and Strabismus).
de Silva DJ, Khaw PT, Brookes JL. Long-term outcome of primary congenital glaucoma. J AAPOS. 2011;15(2):148–152.
Khitri MR, Mills MD, Ying GS, Davidson SL, Quinn GE. Visual acuity outcomes in pediatric glaucomas. J AAPOS. 2012;16(4):376–381.
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.