Blasenekstrophie

Newborn with
a defect of the lower abdominal wallAXR AP shows diastasis of the symphysis pubis and multiple spinal segmentation defects while the AXR lateral shows a small amount of bowel herniated anterior to the abdomen and inferior to the umbilicus along with a large skin covered spinal dysraphism posteriorly and a radioopaque marker being held in place over where the anus should be.The diagnosis was cloacal exstrophy with bladder exstrophy, anorectal malformation and spinal segmentation anomalies.
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Prenatal
diagnosis of diphallia in association with bladder exstrophy: a case report. Ultrasound image at 32 weeks; large cystic structure disappeared. Large abdominal wall defect clearly seen
bmcpregnancychildbirth.biomedcentral.com • CC-by-4.0
Prenatal
diagnosis of diphallia in association with bladder exstrophy: a case report. Post-delivery examination revealed a large abdominal wall defect, exomphalos. Diphallia with empty scrotum
bmcpregnancychildbirth.biomedcentral.com • CC-by-4.0
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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 . There is a recognized male predilection with a male to female ratio of ~3:1 . 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
  • raised maternal alpha-fetoprotein levels

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 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 sign) . Hurley stick appearance of distal ureters has been described in excretory urogram .

Amniotic fluid volumes are often normal.

Treatment and prognosis

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

Complications 

See also

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Blasenekstrophie:
Kryptorchismus
 
pediatricimaging.org • CC-by-nc-sa 4.0
akute
Pyelonephritis
pediatricimaging.org • CC-by-nc-sa 4.0
Kloakenekstrophie
 
pediatricimaging.org • CC-by-nc-sa 4.0
Inguinalhernie
 
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