Bladder exstrophy

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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 ^3,5. There is a recognised male predilection with a male ~~to female~~-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~~, resulting in a subsequent eversion of the bladder mucosa. This then protrudes out as a mass-like lesion.

Associations

General

extension of the bladder defect into the urethra
cryptorchidism
bilateral inguinal herniation
OEIS complex (omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects)
epispadias

In females

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 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 sign) ⁷. Hurley stick appearance of distal ureters has been described in excretory urogram ⁸.

Amniotic fluid volumes are often normal.

Treatment and prognosis

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

Complications

urinary incontinence
infertility
acute pyelonephritis
increased incidence of malignancy in the extruded bladder

-<p><strong>Bladder exstrophy</strong> (also known as <strong>ectopia vesicae</strong>) refers to a herniation of the <a href="/articles/urinary-bladder">urinary bladder</a> 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>3,5</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>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.</p><h5>Associations</h5><h6>General</h6><ul>
+<p><strong>Bladder exstrophy</strong> (also known as <strong>ectopia vesicae</strong>) refers to a herniation of the <a href="/articles/urinary-bladder">urinary bladder</a> 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>3,5</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>Bladder exstrophy is thought to be caused by a developmental defect of the cloacal membrane, resulting in a subsequent eversion of the bladder mucosa. This then protrudes out as a mass-like lesion.</p><h5>Associations</h5><h6>General</h6><ul>
~~-<li><p><a href="/articles/oeis-complex">OEIS complex</a></p></li>~~
+<li><p><a href="/articles/oeis-complex">OEIS complex</a> (<a href="/articles/omphalocele-1" title="Omphalocele">omphalocele</a>, <a href="/articles/cloacal-exstrophy" title="Exstrophy of the cloaca">exstrophy of the cloaca</a>, <a href="/articles/anal-atresia-2" title="Imperforate anus">imperforate anus</a>, and spinal defects)</p></li>
~~-<li><p>vaginal duplication</p></li>~~
~~-<li><p>clitoral cleft</p></li>~~
-</ul><h5>Serological markers</h5><ul><li><p><a href="/articles/alpha-fetoprotein-elevation">raised maternal alpha-fetoprotein levels</a></p></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>4</sup> may also indicate the diagnosis. </p><p>Failure of the pubic bones to meet in the midline (widened <a href="/articles/pubic-symphysis">pubic symphysis</a>). 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 (<a href="/articles/manta-ray-sign-bladder">manta ray sign</a>) <sup>7</sup>. <a href="/articles/hurley-stick-ureters">Hurley stick</a> appearance of distal ureters has been described in <a href="/articles/intravenous-urography">excretory urogram</a> <sup>8</sup>.</p><p><a href="/articles/amniotic-fluid-volume-1">Amniotic fluid volumes</a> are often normal.</p><h4>Treatment and prognosis</h4><p>Treatment is with surgical intervention - staged multidisciplinary reconstruction and the prognosis is generally good. </p><h5>Complications </h5><ul>
+<li><p><a href="/articles/vaginal-duplication" title="vaginal duplication">vaginal duplication</a></p></li>
+<li><p><a href="/articles/clitoral-cleft" title="clitoral cleft">clitoral cleft</a></p></li>
+</ul><h5>Serological markers</h5><ul><li><p><a href="/articles/alpha-fetoprotein-elevation">raised maternal alpha-fetoprotein levels</a></p></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>4</sup> may also indicate the diagnosis. </p><p>Failure of the pubic bones to meet in the midline (widened <a href="/articles/pubic-symphysis">pubic symphysis</a>). 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 (<a href="/articles/manta-ray-sign-bladder">manta ray sign</a>) <sup>7</sup>. <a href="/articles/hurley-stick-ureters">Hurley stick</a> appearance of distal ureters has been described in <a href="/articles/intravenous-urography">excretory urogram</a> <sup>8</sup>.</p><p><a href="/articles/amniotic-fluid-volume-1">Amniotic fluid volumes</a> are often normal.</p><h4>Treatment and prognosis</h4><p>Treatment is with surgical intervention - staged multidisciplinary reconstruction and the prognosis is generally good.</p><h5>Complications</h5><ul>

References changed:

1. Daltro P, Fricke B, Kline-Fath B et al. Prenatal MRI of Congenital Abdominal and Chest Wall Defects. AJR Am J Roentgenol. 2005;184(3):1010-6. <a href="https://doi.org/10.2214/ajr.184.3.01841010">doi:10.2214/ajr.184.3.01841010</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/15728634">Pubmed</a>
2. Emanuel P, Garcia G, Angtuaco T. Prenatal Detection of Anterior Abdominal Wall Defects with US. Radiographics. 1995;15(3):517-30. <a href="https://doi.org/10.1148/radiographics.15.3.7624560">doi:10.1148/radiographics.15.3.7624560</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/7624560">Pubmed</a>
3. Ben-Chaim J, Docimo S, Jeffs R, Gearhart J. Bladder Exstrophy from Childhood into Adult Life. J R Soc Med. 1996;89(1):39P-46P. <a href="https://doi.org/10.1177/014107689608900112">doi:10.1177/014107689608900112</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/8709084">Pubmed</a>
4. Michael Entezami, Ursula Knoll, Matthias Albig et al. Ultrasound Diagnosis of Fetal Anomalies. (2004) ISBN: 9781588902122 - <a href="http://books.google.com/books?vid=ISBN9781588902122">Google Books</a>
5. Richard Fotter. Pediatric Uroradiology. (2008) ISBN: 9783540330042 - <a href="http://books.google.com/books?vid=ISBN9783540330042">Google Books</a>
6. Michael Entezami, Ursula Knoll, Matthias Albig et al. Ultrasound Diagnosis of Fetal Anomalies. (2004) ISBN: 9781588902122 - <a href="http://books.google.com/books?vid=ISBN9781588902122">Google Books</a>
7. Roller B & Dyer R. The "Manta Ray" Pelvis. Abdom Radiol (NY). 2016;41(8):1666-7. <a href="https://doi.org/10.1007/s00261-016-0706-y">doi:10.1007/s00261-016-0706-y</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26971339">Pubmed</a>
8. Syed Z.H. Jafri, Marco A. Amendola, Ananias C. Diokno. Lower Genitourinary Radiology. (2012) ISBN: 9781461216483 - <a href="http://books.google.com/books?vid=ISBN9781461216483">Google Books</a>
1. Daltro P, Fricke BL, Kline-fath BM et-al. Prenatal MRI of congenital abdominal and chest wall defects. AJR Am J Roentgenol. 2005;184 (3): 1010-6. <a href="http://www.ajronline.org/cgi/content/full/184/3/1010">AJR Am J Roentgenol (full text)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/15728634">Pubmed citation</a><div class="ref_v2"></div>
2. Emanuel PG, Garcia GI, Angtuaco TL. Prenatal detection of anterior abdominal wall defects with US. Radiographics. 1995;15 (3): 517-30. <a href="http://radiographics.rsna.org/content/15/3/517.abstract">Radiographics (abstract)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/7624560">Pubmed citation</a><div class="ref_v2"></div>
3. Ben-chaim J, Docimo SG, Jeffs RD et-al. Bladder exstrophy from childhood into adult life. J R Soc Med. 1996;89 (1): 39P-46P. - <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1295642">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/8709084">Pubmed citation</a><div class="ref_v2"></div>
4. Entezami M, Albig M, Knoll U et-al. Ultrasound Diagnosis of Fetal Anomalies. Thieme. (2003) ISBN:1588902129. <a href="http://books.google.com/books?vid=ISBN1588902129">Read it at Google Books</a> - <a href="http://www.amazon.com/gp/product/1588902129?ie=UTF8&tag=radiopaediaor-20&linkCode=as2&camp=1789&creative=9325&creativeASIN=1588902129">Find it at Amazon</a><div class="ref_v2"></div>
5. Fotter R, Baert AL. Pediatric Uroradiology. Springer Verlag. (2008) ISBN:3540330046. <a href="http://books.google.com/books?vid=ISBN3540330046">Read it at Google Books</a> - <a href="http://www.amazon.com/gp/product/3540330046?ie=UTF8&tag=radiopaediaor-20&linkCode=as2&camp=1789&creative=9325&creativeASIN=3540330046">Find it at Amazon</a><div class="ref_v2"></div>
6. Entezami M, Albig M, Knoll U et-al. Ultrasound Diagnosis of Fetal Anomalies. Thieme. (2003) ISBN:1588902129. <a href="http://books.google.com/books?vid=ISBN1588902129">Read it at Google Books</a> - <a href="http://www.amazon.com/gp/product/1588902129?ie=UTF8&tag=radiopaediaor-20&linkCode=as2&camp=1789&creative=9325&creativeASIN=1588902129">Find it at Amazon</a><div class="ref_v2"></div>
7. Roller BL, Dyer RB. The "manta ray" pelvis. Abdominal radiology (New York). 41 (8): 1666-7. <a href="https://doi.org/10.1007/s00261-016-0706-y">doi:10.1007/s00261-016-0706-y</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26971339">Pubmed</a> <span class="ref_v4"></span>
8. Syed Z.H. Jafri, Marco A. Amendola, Ananias C. Diokno. Lower Genitourinary Radiology. (2012) <a href="https://books.google.co.uk/books?vid=ISBN9781461216483">ISBN: 9781461216483</a><span class="ref_v4"></span>

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