Primary congenital glaucoma

Definition

Primary congenital glaucoma (PCG) is characterized by elevated intraocular pressure (IOP), enlargement of the globe (buphthalmos), edema, and opacification of the cornea with rupture of Descemet's membrane (Haab's striae), thinning of the anterior sclera and iris atrophy, anomalously deep anterior chamber, and structurally normal posterior segment except for progressive glaucomatous optic atrophy. Symptoms include photophobia, blepharospasm, and excessive tearing. Typically, the diagnosis is made in the first year of life. Depending on when treatment is instituted, visual acuity may be reduced and/or visual fields may be restricted. In untreated individuals, blindness invariably occurs.

Also known as primary congenital glaucoma, primary congenital glaucoma (disease) — per MONDO

Also identified as

• DOID 0050593 per MONDO
• NCIT C150251 per MONDO
• SCTID 415176004 per MONDO
• UMLS C1533041 per MONDO

Affected Organs