Cloacal exstrophy
ORPHA:93929· ICD-10 Q64.1
Definition
A rare congenital genitourinary malformation characterized by urogenital, gastrointestinal, skeletal and neurospinal anomalies. It is the most severe part of the spectrum of the exstrophy-epispadias complex (EEC) and is also referred as the OEIS complex (omphalocele, exstrophy, imperforate anus and spinal defects).
- Prevalence
- 1-9 / 1 000 000
- Inheritance
- Multigenic/multifactorial
- Age of onset
- Infancy, Neonatal