Persistent hyperplastic primary vitreous

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A rare ophthalmic disorder characterized by mostly unilateral failure of the regression of a fetal ocular vessel component, the tunica vasculosa lentis and/or the hyaloid system, resulting in an anterior (presenting with microphthalmia, leukocoria, cataract, glaucoma, elongated ciliary processes, shallow anterior chamber, and retrolental fibrovascular membranes, among others) or posterior disease subtype (with microphthalmia, leukocoria, presence of a retinal fold or detachment, hypo- or dysplastic optic nerve, and vitreous membranes and stalk), respectively. Most patients present with a combination of the two subtypes.

Synonyms: Persistent fetal vasculature syndrome PFVS Non-syndromic congenital retinal non-attachment PHPV Congenital retinal detachment NCRNA disease

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Term information

database cross reference
hasDbXref

UMLS:C1857299

ICD-10:Q14.0

UMLS:C0266568

OMIM:221900

OMIM:611308

MeSH:D054514

notation

ORPHA:91495