The glaucomas of childhood and adolescence (herein called pediatric glaucomas) are a heterogeneous group of disorders associated with elevated intraocular pressure (IOP). These disorders can cause damage to the optic nerve, visual field, and, up to about age 4, the cornea and other structures. Various presentations and etiologies characterize these rare glaucomas. The Childhood Glaucoma Research Network has defined both glaucoma and glaucoma suspect for pediatric populations (Table 11-1). Although pediatric glaucomas share many characteristics with adult-onset glaucomas, there are numerous management issues that are unique to the pediatric and adolescent populations.
Pediatric glaucoma is typically classified as primary or secondary (Table 11-2). Isolated angle abnormalities are seen in the primary pediatric glaucomas. In primary congenital glaucoma (PCG), angle dysgenesis leads to outflow resistance and elevated IOP, which in turn leads to the classic features of PCG: enlarged and/or cloudy corneas, Haab striae, and an enlarged globe (buphthalmos). In juvenile open-angle glaucoma (JOAG), another high-pressure primary glaucoma, an isolated angle abnormality may be present; this glaucoma develops later in childhood (generally after age 4) or in early adulthood (Table 11-3).
Secondary pediatric glaucomas are associated with other ocular or systemic conditions. These glaucomas are further classified according to whether the condition is acquired after birth or is present at birth (nonacquired). Nonacquired pediatric glaucoma is categorized according to whether the signs are mainly ocular or systemic (Tables 11-4 through 11-7). Glaucoma following extraction of a congenital cataract comprises its own separate category, outside the nonacquired group. See BCSC Section 6, Pediatric Ophthalmology and Strabismus, for additional discussion of many of the topics covered in this chapter.
Table 11-1 Definitions of Pediatric Glaucoma and Glaucoma Suspect
Table 11-2 Classification of Pediatric Glaucoma
Table 11-3 Features of Primary Pediatric Glaucomas
Table 11-4 Features of Secondary Pediatric Glaucomas
Table 11-5 Features of Glaucoma Associated With Nonacquired Ocular Anomalies
Table 11-6 Features of Glaucoma Associated With Nonacquired Systemic Disease or Syndrome
Table 11-7 Features of Glaucoma Associated With Acquired Conditions
Thau A, Lloyd M, Freedman S, Beck A, Grajewski A, Levin AV. New classification system for pediatric glaucoma: implications for clinical care and research registry. Curr Opin Ophthalmol. 2018;29(5):385–394.
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.