Kloakenekstrophie

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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Newborn with
anterior abdominal wall defect. AXR shows diastasis of the symphysis pubis, multiple loops of bowel outside the contour of the abdomen inferiorly, and multiple segmentation anomalies in the lower lumbar spine and sacrum.The diagnosis was cloacal exstrophy in a patient with Omphalocele-Exstrophy of cloaca-Imperforate anus–Spinal defects (OEIS) syndrome.
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Cloacal exstrophy (CE) is an extensive congenital abdominal wall defect which encompasses:

Epidemiology

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

Pathology

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

Recognized wider associations include:

Markers

Radiographic features

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 omphalocele
  • presence of lumbosacral anomalies

Treatment and prognosis

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

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Kloakenekstrophie:
angeborene
Wirbelanomalien
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Myelomeningozele
 
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