Bladder exstrophy

Last revised by Andrew Murphy on 26 Oct 2022

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.

The estimated incidence of bladder exstrophy is 1:10,000-50,000 live births 3,5. There is a recognized male predilection with a male to female ratio of ~3:1 6. Most cases are sporadic.

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.

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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 4 may also indicate the diagnosis. 

Failure of the pubic bones to meet in the midline (widened pubic symphysis). There will be shortening of the pubic rami and acetabular retroversion. This appearance on AP plain radiograph of the pelvis has been likened to a manta ray swimming towards you (manta ray sign7Hurley stick appearance of distal ureters has been described in excretory urogram 8.

Amniotic fluid volumes are often normal.

Treatment is with surgical intervention - staged multidisciplinary reconstruction and the prognosis is generally good.

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Cases and figures

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