vitalwiki

Exstrophy-epispadias complex

ORPHA:322· ICD-10 Q64.1

Definition

A rare non-syndromic uro-genital tract malformation characterized by a spectrum of manifestations ranging in severity: epispadias (E) is the mildest form, classic exstrophy of the bladder (CEB) is the intermediate form and cloacal exstrophy (CE) the most severe form. Exstrophy-epispadias complex (EEC) involves the bladder, the genitalia, the lower abdominal wall, the pelvis and pelvic floor, and depending on the phenotype, the spine and the anus.

Prevalence
Unknown
Inheritance
Multigenic/multifactorial
Age of onset
Antenatal, Neonatal