Glaucoma is a group of diseases that damage the optic nerve, typically resulting from elevated pressure within the eye. While most commonly associated with older adults, it can affect individuals at any age, including infants and children. Glaucoma in young patients, often termed pediatric glaucoma, is rare, affecting approximately one in every 10,000 infants. Because children’s eyes are still developing, high internal pressure can lead to rapid and permanent damage that requires specialized and immediate intervention.
Distinct Categories of Glaucoma in Young Patients
The presentation of glaucoma in young patients is categorized based on the age of onset, which reflects different underlying causes and anatomical issues. The most frequent type is Primary Congenital Glaucoma (PCG), which is not caused by another medical condition or trauma. PCG is sub-classified based on when the symptoms first become apparent.
True congenital glaucoma presents either at birth or within the first month of life, while infantile glaucoma develops between one month and three years of age. These early-onset forms are typically caused by developmental abnormalities in the eye’s drainage system, known as the trabecular meshwork, which prevents the internal fluid from exiting properly. This anatomical defect leads to a buildup of intraocular pressure that stretches the still-elastic eye tissues.
Glaucoma with an onset after age three, but often before age 40, is known as Juvenile Open-Angle Glaucoma (JOAG). In JOAG, the eye’s drainage angle appears open, but a microscopic defect in the trabecular meshwork still obstructs fluid outflow, causing pressure elevation. This form often progresses more aggressively than adult-onset glaucoma and may be linked to specific gene mutations.
Secondary Glaucoma develops as a complication of another underlying condition, eye injury, or surgical procedure. This can occur in children with syndromes like Sturge-Weber or neurofibromatosis, or following cataract removal surgery. The specific cause dictates the most appropriate course of treatment and monitoring schedule.
Recognizing the Signs and Symptoms
Identifying glaucoma in infants and young children is challenging because they cannot verbally communicate symptoms, necessitating vigilance from parents and pediatricians. For infants with congenital or infantile glaucoma, a distinct set of signs often provides the first indication of a problem. These include the classic triad of excessive tearing (epiphora), sensitivity to light (photophobia), and involuntary eye closure or twitching (blepharospasm).
The elevated internal pressure in a developing eye causes the eyeball to stretch, leading to an abnormally large appearance known as buphthalmos. This stretching can result in a cloudy or hazy cornea, often prompting the initial referral to a specialist. Parents may also notice a bluish discoloration of the eye or that one eye is noticeably larger than the other.
Older children and adolescents with Juvenile Open-Angle Glaucoma often exhibit subtle symptoms that resemble the adult form of the disease, such as blurred vision, headaches, or eye pain. Because the eye is less elastic at this age, the physical signs seen in infants, such as buphthalmos and a cloudy cornea, are typically absent. Many young patients remain asymptomatic until the condition is advanced, with vision loss first manifesting as a reduction in peripheral vision.
Treatment Approaches for Developing Eyes
The management of glaucoma in young patients prioritizes surgical intervention, especially for Primary Congenital Glaucoma. Surgery is considered the first-line treatment because it addresses the underlying anatomical defect in the eye’s drainage system to achieve long-term control of the intraocular pressure.
Two common surgical procedures are goniotomy and trabeculotomy. A goniotomy involves making a precise incision into the abnormal tissue of the drainage angle from inside the eye to facilitate fluid outflow. If the cornea is too cloudy to allow for a clear view, a trabeculotomy is performed, which accesses the drainage system from an external approach.
Topical medications are typically used as secondary or adjunctive therapy, or as a temporary measure until surgery can be performed. Long-term medication use in growing children carries unique risks, including potential systemic absorption that can cause side effects. Alpha-adrenergic agonists are generally avoided in infants due to the risk of central nervous system side effects like somnolence or apnea.
Beyond controlling eye pressure, a primary component of treatment for pediatric glaucoma is the management of amblyopia, or “lazy eye,” and refractive errors. High pressure and corneal changes can severely impact visual development, and visual rehabilitation must be managed alongside pressure control. If left untreated, amblyopia and corneal scarring can lead to permanent vision loss, even if the internal pressure is successfully normalized.