Bladder exstrophy

Dr Henry Knipe and Radswiki et al.

Bladder exstrophy (also known as ectopia vesicae) refers to a herniation of the urinary bladder through an anterior abdominal wall defect. The severity of these defects is widely variable.

Epidemiology

The estimated incidence of bladder exstrophy is 1:10,000-50,000 live births 4,6. There is a recognised male predilection with a male to female ratio of ~3:1 6. Most cases are sporadic.

Pathology

Bladder exstrophy is thought to be caused by a developmental defect of the cloacal membrane which results in a subsequent eversion of the bladder mucosa. This then protrudes out as a mass-like lesion.

Associations
General
In females
  • vaginal duplication
  • clitoral cleft
Serological markers

Radiographic features

Imaging findings include a soft-tissue mass extending from a large infra-umbilical anterior wall defect which may be close to the umbilical arterial exits. The absence of a normal urinary bladder and a low-lying umbilical cord insertion 5 may also indicate the diagnosis. 

Failure of the pubic bones to meet in the midline (widened pubic symphysis). This appearance on AP plain radiograph of the pelvis has been likened to a manta ray swimming towards you (manta ray sign8.   

Amniotic fluid volumes are often normal.

Treatment and prognosis

Treatment is with surgical intervention (primary closure/excision with urinary diversion) and prognosis is generally good.

Complications 

See also

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Article Information

rID: 12729
Section: Pathology
Tag: congenital
Synonyms or Alternate Spellings:
  • Urinary bladder exstrophy
  • Exstrophy of the urinary bladder
  • Exstrophy of the bladder
  • Ectopia vesicae
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