Section 10-B: Diagnosis of congenital glaucoma

Congenital glaucoma occurs in infants up to 3 years of age. Most commonly, patients are diagnosed between 3-6 months of age. In the US, boys are slightly more commonly affected than girls. Approximately 70% of congenital glaucoma patients have both eyes affected, while the remaining 30% have only one eye affected. There are 3 common symptoms associated with congenital glaucoma (Table 10-2). These include tearing (epiphora. Figure 10-1), light sensitivity (photophobia, Figure 10-2), and spasm and closure of the eyelids (blepharospasm, Figure 10-2) due to the patient’s sensitivity to light.

Figure 10-1. Light sensitivity and tearing in a teenage patient with a long history of congenital glaucoma. He also has misalignment of the eyes (strabismus).

Figure 10-2. Spasm and closure of the eyelids (blepharospasm) and light sensitivity (photophobia) of a patient with congenital glaucoma.

Common symptoms of congenital glaucoma	Common signs of congenital glaucoma
Tearing (epiphora)	Elevated intraocular pressure (IOP)
Light sensitivity (photophobia)	Enlarged cornea
Spasm and closure of eyelids (blepharospasm)	Cloudy, hazy cornea (corneal edema)
	Tears in Descemet’s membrane in cornea (Haab’s striae)
	Enlarged length of the eye (buphthalmos)
	Optic nerve damage (cupping)

Table 10-2. Common symptoms and signs of primary congenital glaucoma

On eye examination, there are certain features that are commonly associated with congenital glaucoma (Table 10-2). Patients often avoid bright lights because they are very sensitive to light. This is from the cloudy, hazy cornea that disperses the light and causes glare. Instead of a normally clear cornea, these patients develop a thick, cloudy, and hazy cornea (frosted glass appearance) from elevated intraocular pressure (IOP). In severe cases, the patient’s pupil may not even be visible due to the cloudy cornea. Over time, the elevated IOP can cause “stretch marks” in the cornea from excessive stretching (Haab’s striae, Figure 10-3), and also enlarge the size of the eye itself (buphthalmos, Figure 10-4, same as 4-10).

Figure 10-3. Stretch marks in the cornea (Haab’s striae) from the high intraocular pressure in a patient with congenital glaucoma (need cropping and arrows).

Figure 10-4. The left eye of this congenital glaucoma patient is noticeably larger than the right eye. The patient has buphthalmos of the left eye (same as Figure 4-10).

Abnormal enlargement of one or both eyes in an infant is an important sign of congenital glaucoma and should not be ignored. Eventually, as in adult glaucoma, the optic nerve will become damaged (cupping, see Chapter 1). However, unlike adult glaucoma, the optic nerve damage in congenital glaucoma is considered reversible in the early stages if the glaucoma is treated promptly and effectively.

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