Monday 16 June 2008
The term ’exstrophy,’ derived from the Greek work ekstriphein, which literally means ’turn inside out,’ was first used by Chaussier in 1780.
A primary developmental field defect
Exstrophy of the cloaca (cloacal exstrophy) and exstrophy of the bladder (vesical exstrophy) are 2 different expressions of a primary developmental field defect. But Martinez-Frías et al. (2001) have pointed out that bladder exstrophy and cloacal exstrophy seem to be distinct clinical entities.
Cloacal exstrophy is a feature of the OEIS (omphalocele-exstrophy-imperforate anus-spinal defects) complex (MIM.258040). Exstrophy of the cloaca includes the persistence and exstrophy of a common cloaca that receives ureters, ileum, and a rudimentary hindgut and is associated with failure of fusion of the genital tubercles and pubic rami, incomplete development of the lumbosacral vertebrae with spinal dysraphism, imperforate anus, cryptorchidism and epispadias in males and anomalies of the mullerian duct derivatives in females, and a wide range of urinary tract anomalies. Omphalocele is common in OEIS, and most patients have a single umbilical artery.