Glaucoma is characterized by damage to the optic nerve, most often due to elevated pressure inside the eye. This pressure rises when the fluid inside the eye (aqueous humor) malfunctions and cannot drain properly, harming the delicate nerve fibers. While commonly associated with older adults, children and even infants can develop glaucoma. Childhood glaucoma requires specialized and prompt care to prevent permanent vision loss. Because a child’s eye tissues are more elastic, the disease can present with unique observable signs and may progress more aggressively than in adults.
Classifying Childhood Glaucoma
The classification of childhood glaucoma is based on the age of onset and whether the condition occurs in isolation (primary) or results from another disease (secondary). This categorization helps doctors determine the underlying cause and treatment plan.
Primary childhood glaucoma occurs without an underlying medical condition or injury. This category is broken down by age. Primary Congenital Glaucoma (PCG) is the most common form, typically identified in infants and toddlers from birth up to three years of age. These primary forms are generally linked to developmental abnormalities in the eye’s drainage system.
Juvenile Open-Angle Glaucoma (JOAG) is diagnosed in children older than three years, often extending into adolescence. Unlike PCG, JOAG usually lacks the dramatic physical signs seen in infants, making early detection more challenging.
Secondary glaucoma results from a known cause, such as an eye injury, prior eye surgery, or an underlying medical syndrome. This form can develop at any point during childhood. Common examples include glaucoma following cataract surgery or glaucoma associated with conditions like Sturge-Weber syndrome.
Identifying Symptoms in Infants and Children
The symptoms of childhood glaucoma differ significantly from those in adults and vary based on the child’s age. For infants and very young children (under three years old), the elasticity of their eye tissues leads to visible physical changes when pressure builds.
Infant symptoms include excessive tearing (epiphora) and extreme sensitivity to light (photophobia), which may cause them to squint or become irritable in bright rooms. The cornea, the clear front surface of the eye, may appear cloudy, hazy, or white, indicating fluid buildup. A dramatic sign is buphthalmos, where the eyeball enlarges due to increased pressure stretching the eye’s structure, sometimes making one eye appear larger than the other.
In older children, often those with Juvenile Open-Angle Glaucoma, the symptoms are more subtle and may mimic the adult form. Parents or teachers might notice a progressive decline in vision or the child frequently failing school vision tests. Older children may verbalize symptoms such as blurry vision, eye pain, or frequent headaches. Since the eye structure is less flexible after age three, physical signs like eye enlargement are usually absent, making routine eye exams important for early detection.
Underlying Causes and Associated Risks
The fundamental cause of childhood glaucoma is a malfunction in the eye’s system for draining aqueous humor. In primary forms, the cause is often developmental, stemming from an abnormality in the trabecular meshwork, the tissue responsible for fluid outflow. This structural issue prevents the fluid from exiting the eye efficiently, causing pressure to rise and damage the optic nerve.
Genetic factors play a significant role, especially in Primary Congenital Glaucoma, where approximately 10% of cases are inherited. Specific gene mutations linked to improper development of the drainage angle have been identified. A family history of childhood glaucoma significantly increases risk.
Secondary glaucoma arises from external factors or pre-existing medical issues. Causes and associated risks include:
- Severe trauma or injury to the eye, which can disrupt drainage structures.
- Surgery to remove a congenital cataract, particularly if performed in the first six months of life.
- Systemic diseases and syndromes, including Sturge-Weber syndrome, Axenfeld-Rieger syndrome, and neurofibromatosis.
- Extended use of certain medications, such as long-term steroid eye drops or oral steroids, which can increase intraocular pressure.
Diagnosis and Management Strategies
Diagnosing childhood glaucoma requires specialized expertise, as young children often cannot cooperate with standard adult testing methods. For infants and toddlers, an Examination Under Anesthesia (EUA) is frequently necessary to obtain accurate measurements. During an EUA, a pediatric ophthalmologist precisely measures the intraocular pressure (IOP) using a tonometer, since crying or struggling can artificially inflate readings.
The examination includes assessing the optic nerve for damage and measuring the corneal diameter and eye length (axial length). Enlargement of the cornea or a rapid increase in axial length indicates poorly controlled glaucoma. The doctor also looks for breaks in the cornea, known as Haab’s striae, which signal past pressure elevation.
Management strategies for primary childhood glaucoma prioritize surgical intervention to correct the underlying drainage defect. Procedures like goniotomy and trabeculotomy are considered primary treatments for Primary Congenital Glaucoma. Goniotomy involves making an incision into the trabecular meshwork under direct view. Trabeculotomy uses an external approach to access and open the drainage canal.
If the cornea is cloudy, obstructing the view for a goniotomy, trabeculotomy is often the preferred choice. Medications, such as eye drops, are used temporarily to lower IOP and clear the cornea before surgery, or as an adjunct treatment afterward. Long-term follow-up is necessary to monitor IOP and address resulting vision issues like amblyopia (lazy eye), which may require patching or glasses.