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