Primary infantile glaucoma is a rare developmental defect in the iridocorneal filtration angle of the anterior chamber that prevents aqueous humor from properly draining from the eye. This obstruction increases the intraocular pressure, which, if untreated, damages the optic nerve. Diagnosis is based on clinical findings and is confirmed by measurements of intraocular pressure and of the eyeball and cornea. Treatment is surgical. Infantile glaucoma can cause complete blindness if left untreated.
(See also Overview of Glaucoma in adults.)
Primary infantile glaucoma occurs in infants and young children and may be unilateral or, more commonly, bilateral. Intraocular pressure increases above the normal range (10 to 22 mm Hg).
Glaucoma associated with another disorder that affects the eyes, such as aniridia, Lowe syndrome, or Sturge-Weber syndrome, is called secondary glaucoma. Glaucoma can also occur in infants after trauma or intraocular surgery (eg, cataract extraction).
Aqueous Humor Production and Flow
Aqueous humor is produced in the ciliary body behind the iris (in the posterior chamber), passes into the front of the eye (anterior chamber), and then exits through the drainage canals or the uveoscleral pathway (black arrows). |
In primary infantile glaucoma or early childhood glaucoma, the affected eye or eyes become enlarged because the increased intraocular pressure causes the collagen of the sclera and cornea to stretch. This enlargement does not occur in adult glaucoma. The large-diameter (> 12 mm) cornea is thinned and sometimes cloudy. The infant may have tearing and photophobia.
Diagnosis of Primary Infantile Glaucoma
Clinical examination
Measurement of intraocular pressure and other measurements of the eyeball and cornea
Diagnosis is suspected when the symptom triad of blepharospasm (excessive blinking), photophobia (light sensitivity), and epiphora (tearing) is seen.
The ophthalmologist is often able to measure intraocular pressure in the office or clinic; however, an examination under anesthesia in an operating room is necessary to obtain measurements of corneal diameter, corneal thickness, axial length of the eyeball, anterior chamber angle, and refractive error. These measurements further help confirm the diagnosis.
Treatment of Primary Infantile Glaucoma
Surgical intervention
Early surgical intervention (eg, goniotomy, trabeculotomy, trabeculectomy) is the mainstay of treatment.
If untreated, corneal clouding progresses, the optic nerve is damaged (as evidenced clinically by optic nerve cupping), and blindness can occur.
