Bladder exstrophy

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A bladderBladder exstrophy (also known as Ectopia vesicae) refers to a herniation of the urinary bladder through an anterior abdominal wall defect. It can occur toThe severity of these defects is widely variable severity.

Epidemiology

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

Pathology

It 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 like the mass like lesion.

Genetics

Most cases are sporadic

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

Amniotic fluid volumes are often normal

Complications 

Treatment and prognosis

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

See also

  • -<p>A<strong> bladder exstrophy</strong> (also known as <strong>Ectopia vesicae</strong>) refers to a herniation of the urinary bladder through an anterior abdominal wall defect. It can occur to variable severity.</p><h4>Epidemiology</h4><p>The estimated Incidence of bladder exstrophy is at  ~ 1 : 10,000 - 50,000 live births <sup>4,6</sup>. There is a recognised male predilection with a M : F ratio of  ~ 3 : 1 <sup>6</sup> . Most cases are sporadic.</p><h4>Pathology</h4><p>It 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 like the mass like lesion.</p><h5>Genetics</h5><p>Most cases are sporadic</p><h5>Associations</h5><h6><strong>General</strong></h6><ul>
  • -<li>extension of the bladder defect into the urethra </li>
  • -<li><a href="/articles/cryptorchidism" title="cryptorchidism">cryptorchidism </a></li>
  • -<li>bilateral <a href="/articles/inguinal-hernia" title="Inguinal herniation">inguinal herniation</a>
  • -</li>
  • -<li><a href="/articles/oeis-complex" title="OEIS complex">OEIS complex </a></li>
  • -<li><a href="/articles/epispadias" title="Epispadias" style="color: rgb(63, 117, 216); text-decoration: none; ">epispadia</a></li>
  • +<p><strong>Bladder exstrophy</strong> (also known as <strong>Ectopia vesicae</strong>) refers to a herniation of the urinary bladder through an anterior abdominal wall defect. The severity of these defects is widely variable.</p><h4>Epidemiology</h4><p>The estimated incidence of bladder exstrophy is  1:10,000-50,000 live births <sup>4,6</sup>. There is a recognised male predilection with a male to female ratio of  ~ 3:1 <sup>6</sup> . Most cases are sporadic.</p><h4>Pathology</h4><p>It 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 like the mass like lesion.</p><h5>Associations</h5><h6><strong>General</strong></h6><ul>
  • +<li>extension of the bladder defect into the urethra</li>
  • +<li><a href="/articles/cryptorchidism">cryptorchidism </a></li>
  • +<li>bilateral <a href="/articles/inguinal-hernia">inguinal herniation</a>
  • +</li>
  • +<li><a href="/articles/oeis-complex">OEIS complex </a></li>
  • +<li><a href="/articles/epispadias">epispadia</a></li>
  • -<li>vaginal duplication</li>
  • -<li>clitoral cleft</li>
  • -</ul><h5>Serological markers</h5><ul><li><a href="/articles/altered-alpha-fetoprotein-levels" title="Raised alpha fetoprotein">raised maternal alpha feto protein levels</a></li></ul><h4>Radiographic features</h4><p>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 <sup>5</sup> may also indicate the diagnosis</p><p>Amniotic fluid volumes are often normal</p><h4>Complications  </h4><ul>
  • -<li><a href="/articles/urinary-incontinence" title="urinary incontinence ">urinary incontinence </a></li>
  • -<li>
  • -<a href="/articles/infertility" title="infertility">infertility</a> </li>
  • -<li><a href="/articles/acute-bacterial-pyelonephritis" title="Acute pyelonephritis">acute pyelonephritis </a></li>
  • -<li>increased incidence of malignancy in the extruded bladder</li>
  • +<li>vaginal duplication</li>
  • +<li>clitoral cleft</li>
  • +</ul><h5>Serological markers</h5><ul><li><a href="/articles/afp-elevation">raised maternal alpha feto protein levels</a></li></ul><h4>Radiographic features</h4><p>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 <sup>5</sup> may also indicate the diagnosis</p><p>Amniotic fluid volumes are often normal</p><h4>Complications </h4><ul>
  • +<li><a href="/articles/urinary-incontinence">urinary incontinence </a></li>
  • +<li><a href="/articles/infertility">infertility</a></li>
  • +<li><a href="/articles/acute-bacterial-pyelonephritis">acute pyelonephritis </a></li>
  • +<li>increased incidence of malignancy in the extruded bladder</li>
  • -<li>
  • -<a href="/articles/cloacal-exstrophy" title="cloacal exstrophy">cloacal exstrophy</a> : more severe anomaly</li>
  • -<li><a href="/articles/fetal-anterior-abdominal-wall-defects" title="Fetal anterior abdominal wall defects">congenital anterior abdominal wall defects</a></li>
  • +<li>
  • +<a href="/articles/cloacal-exstrophy">cloacal exstrophy</a>: more severe anomaly</li>
  • +<li><a href="/articles/fetal-anterior-abdominal-wall-defects">congenital anterior abdominal wall defects</a></li>

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