Secondary Glaucomas due to Acquired Conditions
Many of the causes of secondary glaucoma in infants and children are similar to those in adults, including trauma, inflammation, steroid use, and topiramate-induced angle closure. The signs and symptoms at presentation will depend on the age of the child (whether the child is younger than or older than 3–4 years) and the extent of the IOP elevation and severity of vision loss. Lens-associated disorders that cause angle-closure glaucoma may occur in patients with Marfan syndrome, homocystinuria, Weill-Marchesani syndrome, and microspherophakia. Posterior segment disorders such as per sis tent fetal vasculature, retinopathy of prematurity, and familial exudative vitreoretinopathy, as well as tumors of the retina, iris, or ciliary body, can also result in glaucoma. The intraocular tumors known to lead to secondary glaucoma in infants and children include retinoblastoma, juvenile xanthogranuloma, and medulloepithelioma. Rubella and congenital cataract are also associated with secondary pediatric glaucoma.
Glaucoma Following Cataract Surgery
Glaucoma and suspicion of glaucoma develop in up to 50% or more of children who have undergone surgery for congenital cataract. The glaucoma is predominantly an open-angle type; however, angle closure may also occur as a late consequence of an enlarging Sommering ring that pushes the iris forward. The term “aphakic glaucoma” is commonly used to refer to this group of glaucomas; today, this term may be considered outdated because many of these young patients receive intraocular lens implants. Risk factors include cataract surgery in the first year of life (risk is greatest in individuals who have undergone surgery in first 6 weeks of life), postoperative complications, and small corneal diameter. The risk of developing glaucoma is the same whether patients are left aphakic or they receive an intraocular lens implant at the time of cataract extraction. Although most glaucoma following congenital cataract surgery develops in patients within 3 years of cataract surgery, these patients are always at risk for glaucoma and thus require lifelong follow-up. The underlying mechanism is unclear, but likely etiologies for open-angle cases include congenital anomalies of the outflow pathway, surgically induced inflammation, and altered intraocular anatomy postoperatively. Removing all residual cortex during cataract surgery may reduce the risk of IOP elevation.
Freedman SF, Lynn MJ, Beck AD, Bothun ED, Örge FH, Lambert SR; Infant Aphakia Treatment Study Group. Glaucoma-related adverse events in the first 5 years after unilateral cataract removal in the Infant Aphakia Treatment Study. JAMA Ophthalmol. 2015;133(8):907–914.
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.