In infants, PCG presents with the classic triad of epiphora, photophobia, and blepharospasm. Until about 4 years of age, elevated IOP causes the cornea to stretch, leading to increased corneal diameter and enlargement of the globe (buphthalmos; Fig 11-2A), along with stretching of the scleral canal housing the optic nerve. The corneal stretching produces Haab striae, or breaks in Descemet membrane, and may lead to corneal edema and corneal opacification (Fig 11-2B; also see Figs 22-2 and 22-3 in BCSC Section 6, Pediatric Ophthalmology and Strabismus). As the cornea swells, the child may become irritable and photophobic. After age 3–4 years, the cornea ceases to enlarge further. Scleral stretching also ceases around this age. However, persistently elevated IOP may result in continued optic nerve damage.
Figure 11-1 Anterior chamber angle in primary congenital glaucoma (PCG). A, Illustration of a gonioscopic view of the anterior chamber angle in primary congenital glaucoma reveals a deep angle with no angle recess; the iris appears as a scalloped line with less density of the iris fibers (rarefaction). B, Goniophotograph of the angle showing a similar view. Iris blood vessels are more prominently visible in eyes with PCG.
(Part A courtesy of Lee Allen and Wallace L.M. Alward, MD; part B courtesy of Robert Honkanen, MD.)
Figure 11-2 Primary congenital glaucoma. A, photograph of a child with unilateral buphthalmos resulting from uncontrolled elevated intraocular pressure (IOP) in the left eye prior to surgery. B, photograph of Haab striae, or breaks in Descemet membrane, which are visible after corneal edema clears. The striae are both horizontal and circumferential.
(Part A courtesy of JoAnn A. Giaconi, MD; part B courtesy of Deepak Edward, MD.)
Table 11-8 Differential Diagnosis for Symptoms and Signs of Primary Congenital Glaucoma
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.