(See Figure 8.11.1.)
Critical
Enlarged globe and corneal diameter (horizontal corneal diameter >12 mm before 1 year of age is suggestive), corneal edema, Haab striae (curvilinear tears in Descemet membrane of the cornea, with scalloped edges with or without associated stromal haze), increased cup/disc ratio, high intraocular pressure (IOP), axial myopia, commonly bilateral (80%). Classic findings are tearing, photophobia, blepharospasm, corneal clouding, and a large eye (buphthalmos).
Other
Corneal stromal scarring or opacification; high iris insertion on gonioscopy; other signs of iris dysgenesis, including heterochromia, may exist.
Common
Less Common
Rare
NOTE: |
IOP may be reduced by general anesthesia, particularly halothane (sevoflurane or desflurane less likely), and over ventilation (low end-tidal CO2); IOP may be elevated with ketamine hydrochloride, succinylcholine, endotracheal intubation (for 2 to 5 minutes), pressure from the anesthetic mask, speculum use, or inadequate ventilation with elevated end-tidal CO2. |
Definitive treatment is surgical, particularly in primary congenital glaucoma. Medical therapy is utilized as a temporizing measure before surgery and to help clear the cornea in preparation for possible goniotomy.
NOTE: |
Brimonidine is contraindicated in children under the age of 1 year because of the risk of apnea/hypotension/bradycardia/hypothermia from bloodbrain permeability. Caution should be used in children under 5 years old or <20 kg or intracranial pathology (such as SturgeWeber syndrome). |
NOTE: |
Amblyopia is the most common cause of visual loss in pediatric glaucoma and should be treated appropriately. See 8.7, AMBLYOPIA. |