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. [ https://www.ncbi.nlm.nih.gov/books/NBK1135/ ]
Synonyms: primary congenital glaucoma (disease) primary congenital glaucoma
Term information
- UMLS:C1533041 (MONDO:equivalentTo)
- HP:0008007 (MONDO:otherHierarchy)
- SCTID:415176004 (MONDO:equivalentTo)
- NCIT:C150251 (MONDO:equivalentTo)
- DOID:0050593 (MONDO:equivalentTo)
rare, inferred_rare
http://linkedlifedata.com/resource/umls/id/C1533041
http://identifiers.org/snomedct/415176004
http://purl.obolibrary.org/obo/NCIT_C150251
http://purl.obolibrary.org/obo/DOID_0050593