Cloacal exstrophy

Cloacal exstrophy (CE) is an extensive congenital abdominal wall defect which encompasses:

The estimated prevalence is at around 1 in 50,000 to 200,000 live births. There is a recognised male predilection with a M:F ratio of ~2:1. 

It results from persistence and premature perforation of the cloacal membrane. Cloacal exstrophy is considered to be the most severe end of the spectrum of disease ranging from epispadias at one end to cloacal exstrophy at the other.

Genetics

Most cases are sporadic.

Associations

Recognised wider associations include:

Markers
Antenatal ultrasound

A large ventral wall defect may be seen. Some antenatal-postnatal ultrasound findings can overlap with that of bladder exstrophy and include:

  • failure to visualize a normal urinary bladder 
  • pubic diastasis: can difficult to identify in-utero
  • presence of a soft-tissue mass protruding from the lower anterior abdominal wall surface 

Additional features present with cloacal exstrophy includes:

  • presence of an omphalocoele
  • presence of lumbo-sacral anomalies

While not usually lethal, cloacal exstrophy often carries a poor prognosis where a series of operations are often required.

Share Article

Article Information

rID: 13232
Tag: cases
Synonyms or Alternate Spellings:
  • Exstrophy of the cloaca
  • Cloacal exstrophy (CE)

Support Radiopaedia and see fewer ads

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.