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<span id="openzim-page-title" class="mw-page-title-main"><span class="mw-page-title-main">Bladder exstrophy</span></span>
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</style><table class="infobox infobox-has-images-with-white-backgrounds"><tbody><tr><th colspan="2" class="infobox-above" style="background:#ccc">Bladder exstrophy</th></tr><tr><th scope="row" class="infobox-label">Other names</th><td class="infobox-data"><b>Ectopia vesicae</b></td></tr><tr><td colspan="2" class="infobox-full-data"></td></tr><tr><td colspan="2" class="infobox-full-data">Female baby with classical bladder exstrophy</td></tr><tr><th scope="row" class="infobox-label"><a href="Medical_specialty" title="Medical specialty">Specialty</a></th><td class="infobox-data"><a href="Medical_genetics" title="Medical genetics">Medical genetics</a> <span class="penicon autoconfirmed-show"></span></td></tr></tbody></table><style data-mw-deduplicate="TemplateStyles:r1236303919">
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<p><b>Bladder exstrophy</b> is a <a href="Congenital" class="mw-redirect" title="Congenital">congenital</a> anomaly that exists along the spectrum of the exstrophy-epispadias complex, and most notably involves protrusion of the <a href="Urinary_bladder" class="mw-redirect" title="Urinary bladder">urinary bladder</a> through a defect in the abdominal wall. Its presentation is variable, often including abnormalities of the <a href="Human_pelvis" class="mw-redirect" title="Human pelvis">bony pelvis</a>, <a href="Pelvic_floor" title="Pelvic floor">pelvic floor</a>, and genitalia. The underlying embryologic mechanism leading to bladder exstrophy is unknown, though it is thought to be in part due to failed reinforcement of the <a href="Cloacal_membrane" title="Cloacal membrane">cloacal membrane</a> by underlying <a href="Mesoderm" title="Mesoderm">mesoderm</a>.<sup id="cite_ref-1" class="reference"><a href="#cite_note-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup>
Exstrophy means the inversion of a hollow organ.<sup id="cite_ref-2" class="reference"><a href="#cite_note-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup>
</p>
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<div class="mw-heading mw-heading2"><h2 id="Signs_and_symptoms">Signs and symptoms</h2></div>
<p>The classic manifestation of bladder exstrophy presents with:
</p>
<ul><li>A defect in the abdominal wall occupied by both the exstrophied bladder as well as a portion of the <a href="Urethra" title="Urethra">urethra</a></li>
<li>A flattened <a href="Puborectal_sling" class="mw-redirect" title="Puborectal sling">puborectal sling</a></li>
<li>Separation of the <a href="Pubic_symphysis" title="Pubic symphysis">pubic symphysis</a></li>
<li>Shortening of a pubic rami</li>
<li>External rotation of the pelvis.</li></ul>
<p>Females frequently have a displaced and narrowed <a href="Vagina#Vaginal_opening_and_hymen" title="Vagina">vaginal orifice</a>, a <a href="https://en.wiktionary.org/wiki/bifid" class="extiw external" title="wikt:bifid">bifid</a> <a href="Clitoris" title="Clitoris">clitoris</a>, and divergent <a href="Labia" title="Labia">labia</a>.<sup id="cite_ref-Campbell-Walsh_3-0" class="reference"><a href="#cite_note-Campbell-Walsh-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup>
</p>
<div class="mw-heading mw-heading2"><h2 id="Cause">Cause</h2></div>
<p>The cause is not yet clinically established but is thought to be in part due to failed reinforcement of the cloacal membrane by underlying mesoderm.<sup id="cite_ref-Bladder_Exstrophy_4-0" class="reference"><a href="#cite_note-Bladder_Exstrophy-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup>
</p>
<div class="mw-heading mw-heading2"><h2 id="Diagnosis">Diagnosis</h2></div>
<p>In a small retrospective study of 25 pregnancies, five factors were found to be strongly associated with a prenatal diagnosis of bladder exstrophy:<sup id="cite_ref-Bladder_Exstrophy_4-1" class="reference"><a href="#cite_note-Bladder_Exstrophy-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup>
</p>
<ul><li>Inability to visualize the bladder on ultrasound</li>
<li>A lower abdominal bulge</li>
<li>A small penis with anteriorly displaced scrotum</li>
<li>A low set umbilical insertion</li>
<li>Abnormal widening of the iliac crests</li></ul>
<p>While a diagnosis of bladder exstrophy was made retrospectively in a majority of pregnancies, in only three cases was a prenatal diagnosis made.<sup id="cite_ref-pmid7770267_5-0" class="reference"><a href="#cite_note-pmid7770267-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup>
</p>
<div class="mw-heading mw-heading2"><h2 id="Management">Management</h2></div>
<p>The extreme rarity of the disease limits the surgical opportunities to practice the complex closure required in these patients. For this reason, patients have the best outcomes when the bladder closures are performed at high volume centers where surgical and nursing teams have extensive experience in caring for the disease.<sup id="cite_ref-pmid16094068_6-0" class="reference"><a href="#cite_note-pmid16094068-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> The highest volume center in the United States, and the world, is the Johns Hopkins Hospital in Baltimore, Maryland; they have seen over 1300 exstrophy patients in the past 50 years.<sup id="cite_ref-pmid30292419_7-0" class="reference"><a href="#cite_note-pmid30292419-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup>
</p><p>Upon delivery, the exposed bladder is irrigated and a non-adherent film is placed to prevent as much contact with the external environment as possible. In the event the child was not born at a medical center with an appropriate exstrophy support team then transfer will likely follow. Upon transfer, or for those infants born at a medical center able to care for bladder exstrophy, imaging may take place in the first few hours of life prior to the child undergoing surgery.<sup id="cite_ref-Campbell-Walsh_3-1" class="reference"><a href="#cite_note-Campbell-Walsh-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup>
</p><p>Primary (immediate) closure is indicated only in those patients with a bladder of appropriate size, elasticity, and contractility as those patients are most likely to develop a bladder of adequate capacity after early surgical intervention.<sup id="cite_ref-Gearhart-Jeffs-1998_8-0" class="reference"><a href="#cite_note-Gearhart-Jeffs-1998-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup>
</p><p>Conditions that are absolute contraindications despite bladder adequacy include duplication of the penis or scrotum and significant bilateral <a href="Hydronephrosis" title="Hydronephrosis">hydronephrosis</a>.<sup id="cite_ref-9" class="reference"><a href="#cite_note-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup>
</p>
<div class="mw-heading mw-heading3"><h3 id="Surgery">Surgery</h3></div>
<p>Modern therapy is aimed at surgical reconstruction of the bladder and genitalia. Both males and females are born with this anomaly. Treatment is similar.
</p><p>In males treatments have been:
In the modern staged repair of exstrophy (MSRE) the initial step is closure of the abdominal wall, often requiring a pelvic <a href="Osteotomy" title="Osteotomy">osteotomy</a>. This leaves the patient with penile epispadias and <a href="Urinary_incontinence" title="Urinary incontinence">urinary incontinence</a>. At approximately 2–3 years of age, the patient then undergoes repair of the epispadias after <a href="Testosterone_(medication)" title="Testosterone (medication)">testosterone</a> stimulation. Finally, bladder neck repair usually occurs around the age of 4–5 years, though this is dependent upon a bladder with adequate capacity and, most importantly, an indication that the child is interested in becoming continent. In some of the bladder reconstructions, the bladder is augmented with the addition of a segment of the large intestines to increase the volume capacity of the reconstructed bladder.
In the complete primary repair of exstrophy (CPRE) the bladder closure is combined with an epispadias repair, in an effort to decrease costs and morbidity.<sup id="cite_ref-pmid10492227_10-0" class="reference"><a href="#cite_note-pmid10492227-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> This technique has, however, led to significant loss of penile and corporal tissue, particularly in younger patients.<sup id="cite_ref-pmid15371793_11-0" class="reference"><a href="#cite_note-pmid15371793-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup>
</p><p>In females treatment has included:
Surgical reconstruction of the clitoris, which is separated into two distinct bodies. Surgical reconstruction to correct the split of the mons, redefine the structure of the bladder neck and urethra. <a href="Vaginoplasty" title="Vaginoplasty">Vaginoplasty</a> will correct the anteriorly displaced vagina. If the anus is involved, it is also repaired. Fertility remains and women who were born with bladder exstrophy usually develop prolapse due to the weaker muscles of the pelvic floor.<sup id="cite_ref-12" class="reference"><a href="#cite_note-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup>
</p>
<div class="mw-heading mw-heading2"><h2 id="Bladder_exstrophy_males_reassigned_and_raised_as_females">Bladder exstrophy males reassigned and raised as females</h2></div>
<p>Between 1960 and 2000,<sup id="cite_ref-Bailey_2016_13-0" class="reference"><a href="#cite_note-Bailey_2016-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> doctors believed XY males with <a href="Cloacal_exstrophy" title="Cloacal exstrophy">cloacal exstrophy</a> (the most extreme form of bladder exstrophy) would be happier socially as females, and surgically reassigned them. This occurred under the belief that humans were born psychosexually neutral. The practice proved controversial, after follow-ups by <a href="William_Reiner" title="William Reiner">William Reiner</a><sup id="cite_ref-14" class="reference"><a href="#cite_note-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> found that many struggled to adhere to a female gender identity, had masculine interests, were attracted to females, or reverted to the male sex spontaneously or upon learning they were genetic males.<sup id="cite_ref-Bailey_2003_15-0" class="reference"><a href="#cite_note-Bailey_2003-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup>
</p><p>According to <a href="J._Michael_Bailey" title="J. Michael Bailey">J. Michael Bailey</a>:<sup id="cite_ref-Bailey_2003_15-1" class="reference"><a href="#cite_note-Bailey_2003-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup>
</p>
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</style><blockquote class="templatequote"><p>Reiner thinks that all the cloacal cases born as boys would be happier as boys rather than girls, because their brains have been biologically prepared for the male role. He thinks that those who remain girls are at best missing out, and at worst are experiencing great inner torment. He thinks their parents should tell them and, essentially, let them choose their sex.</p></blockquote>
<p>In terms of their sexual orientations, a 2016 academic review by <a href="J._Michael_Bailey" title="J. Michael Bailey">J. Michael Bailey</a> found 7 published cases of boys reassigned female at birth and raised as girls, of which 5 were cloacal exstrophy cases. All were strongly attracted to females.<sup id="cite_ref-Bailey_2016_13-1" class="reference"><a href="#cite_note-Bailey_2016-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> According to a 2015 article in <i><a href="The_Boston_Globe" title="The Boston Globe">The Boston Globe</a></i>, Wiliam Reiner has followed up on the sexual orientations of approximately 70 genetic males who were raised as females. Only one reported having sexual attractions to males, "I’m more convinced than ever that sexual orientation is built in... certainly for males" he said.<sup id="cite_ref-16" class="reference"><a href="#cite_note-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> A 2023 review by Swift-Gallant et al. also cited two additional cases from media reports, both of whom were gynephilic.<sup id="cite_ref-Swift-Gallant_2023_17-0" class="reference"><a href="#cite_note-Swift-Gallant_2023-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup>
</p><p>Reiner's results have caused some doctors to reconsider the practice of sex reassigning genetic males.<sup id="cite_ref-Bailey_2003_15-2" class="reference"><a href="#cite_note-Bailey_2003-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> A 2011 study found that 79% of paediatric urologists surveyed favoured male assignment for genetic males with cloacal exstrophy.<sup id="cite_ref-18" class="reference"><a href="#cite_note-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup>
</p>
<div class="mw-heading mw-heading3"><h3 id="Cases_in_the_media">Cases in the media</h3></div>
<ul><li>Joe Holliday. British cloacal exstrophy male who was raised female. Masculine interests and attracted to females. Found out born male at age 25, and reverted to male sex.<sup id="cite_ref-19" class="reference"><a href="#cite_note-19"><span class="cite-bracket">[</span>19<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Swift-Gallant_2023_17-1" class="reference"><a href="#cite_note-Swift-Gallant_2023-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup></li>
<li>Sophie Ottaway. British cloacal exstrophy male raised as a female. Found out about birth sex at age 22. Retained female identity, although believes the reassignment was incorrect. Attracted to women.<sup id="cite_ref-20" class="reference"><a href="#cite_note-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Swift-Gallant_2023_17-2" class="reference"><a href="#cite_note-Swift-Gallant_2023-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup></li></ul>
<div class="mw-heading mw-heading2"><h2 id="Prognosis">Prognosis</h2></div>
<p>The most important criterion for improving long-term prognosis is success of the initial closure.<sup id="cite_ref-pmid3806816_21-0" class="reference"><a href="#cite_note-pmid3806816-21"><span class="cite-bracket">[</span>21<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-pmid2746771_22-0" class="reference"><a href="#cite_note-pmid2746771-22"><span class="cite-bracket">[</span>22<span class="cite-bracket">]</span></a></sup> If a patient requires more than one closure their chance of continence drops off precipitously with each additional closure - at just two closures the chance of voiding continence is just 17%.<sup id="cite_ref-pmid19906564_23-0" class="reference"><a href="#cite_note-pmid19906564-23"><span class="cite-bracket">[</span>23<span class="cite-bracket">]</span></a></sup>
</p><p>Even with successful surgery, people may have long-term complications.<sup id="cite_ref-pmid17558272_24-0" class="reference"><a href="#cite_note-pmid17558272-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup> Some of the most common include:
</p>
<ul><li><a href="Vesicoureteral_reflux" title="Vesicoureteral reflux">Vesicoureteral reflux</a></li>
<li><a href="Bladder_spasm" class="mw-redirect" title="Bladder spasm">Bladder spasm</a></li>
<li><a href="Bladder_calculus" class="mw-redirect" title="Bladder calculus">Bladder calculus</a></li>
<li><a href="Urinary_tract_infections" class="mw-redirect" title="Urinary tract infections">Urinary tract infections</a></li></ul>
<div class="mw-heading mw-heading2"><h2 id="Epidemiology">Epidemiology</h2></div>
<p>Occurring at a rate between 1 in 10,000 to 1 in 50,000 <sup id="cite_ref-25" class="reference"><a href="#cite_note-25"><span class="cite-bracket">[</span>25<span class="cite-bracket">]</span></a></sup> with a male-to-female ratio of 2.3–6:1,<sup id="cite_ref-Shapiro_26-0" class="reference"><a href="#cite_note-Shapiro-26"><span class="cite-bracket">[</span>26<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Ives_27-0" class="reference"><a href="#cite_note-Ives-27"><span class="cite-bracket">[</span>27<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-28" class="reference"><a href="#cite_note-28"><span class="cite-bracket">[</span>28<span class="cite-bracket">]</span></a></sup> bladder exstrophy is relatively rare. For those individuals with bladder exstrophy who maintain their ability to reproduce, the risk of bladder exstrophy in their children is approximately 500-fold greater than the general population.<sup id="cite_ref-Shapiro_26-1" class="reference"><a href="#cite_note-Shapiro-26"><span class="cite-bracket">[</span>26<span class="cite-bracket">]</span></a></sup>
</p>
<div class="mw-heading mw-heading2"><h2 id="References">References</h2></div>
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<li id="cite_note-1"><span class="mw-cite-backlink"><b><a href="#cite_ref-1">^</a></b></span> <span class="reference-text">Muecke EC: The role of the cloacal membrane in exstrophy: the first successful experimental study. J Urol 1964; 92:659.</span>
</li>
<li id="cite_note-2"><span class="mw-cite-backlink"><b><a href="#cite_ref-2">^</a></b></span> <span class="reference-text"><style data-mw-deduplicate="TemplateStyles:r1238218222">
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</ol></div></div>
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<div class="side-box-text plainlist">Wikimedia Commons has media related to <span style="font-weight: bold; font-style: italic;"><a href="https://commons.wikimedia.org/wiki/Category:Bladder_exstrophy" class="extiw external" title="commons:Category:Bladder exstrophy">Bladder exstrophy</a></span>.</div></div>
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</style><div id="Congenital_malformations_and_deformations_of_urinary_system136" style="font-size:114%;margin:0 4em"><a href="Congenital_disorder" class="mw-redirect" title="Congenital disorder">Congenital</a> <a href="Urologic_disease" title="Urologic disease">malformations and deformations</a> of <a href="Urinary_system" title="Urinary system">urinary system</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%">Abdominal</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="Kidney_disease" title="Kidney disease">Kidney</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em">
<ul><li><a href="Renal_agenesis" title="Renal agenesis">Renal agenesis</a></li>
<li><a href="Renal_hypoplasia" title="Renal hypoplasia">Renal hypoplasia</a></li>
<li><a href="Potter_sequence" title="Potter sequence">Potter sequence</a></li>
<li><a href="Papillorenal_syndrome" title="Papillorenal syndrome">Papillorenal syndrome</a></li></ul>
<ul><li><i><a href="Cystic_kidney_disease" title="Cystic kidney disease">cystic</a></i>
<ul><li><a href="Polycystic_kidney_disease" title="Polycystic kidney disease">Polycystic kidney disease</a></li>
<li><a href="Meckel_syndrome" class="mw-redirect" title="Meckel syndrome">Meckel syndrome</a></li>
<li><a href="Multicystic_dysplastic_kidney" title="Multicystic dysplastic kidney">Multicystic dysplastic kidney</a></li>
<li><a href="Medullary_sponge_kidney" title="Medullary sponge kidney">Medullary sponge kidney</a></li></ul></li></ul>
<ul><li><a href="Horseshoe_kidney" title="Horseshoe kidney">Horseshoe kidney</a></li>
<li><a href="Renal_ectopia" class="mw-redirect" title="Renal ectopia">Renal ectopia</a></li>
<li><a href="Nephronophthisis" title="Nephronophthisis">Nephronophthisis</a></li>
<li><a href="Supernumerary_kidney" title="Supernumerary kidney">Supernumerary kidney</a></li>
<li><a href="Pelvic_kidney" title="Pelvic kidney">Pelvic kidney</a></li></ul>
<ul><li><a href="Dent's_disease" title="Dent's disease">Dent's disease</a></li>
<li><a href="Alport_syndrome" title="Alport syndrome">Alport syndrome</a></li></ul>
</div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="Ureter" title="Ureter">Ureter</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em">
<ul><li><a href="Ectopic_ureter" title="Ectopic ureter">Ectopic ureter</a></li>
<li><a href="Megaureter" title="Megaureter">Megaureter</a></li>
<li><a href="Duplicated_ureter" title="Duplicated ureter">Duplicated ureter</a></li></ul>
</div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Pelvic</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th id="Bladder46" scope="row" class="navbox-group" style="width:1%"><a href="Urinary_bladder_disease" title="Urinary bladder disease">Bladder</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em">
<ul></ul>
</div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="Urethra" title="Urethra">Urethra</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em">
<ul><li><a href="Epispadias" title="Epispadias">Epispadias</a></li>
<li><a href="Hypospadias" title="Hypospadias">Hypospadias</a></li>
<li><a href="Posterior_urethral_valve" title="Posterior urethral valve">Posterior urethral valves</a></li>
<li><a href="Penoscrotal_transposition" title="Penoscrotal transposition">Penoscrotal transposition</a></li></ul>
</div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Both</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em">
<ul><li><a href="Prune_belly_syndrome" title="Prune belly syndrome">Prune belly syndrome</a></li></ul>
</div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Vestigial</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th id="Urachus11" scope="row" class="navbox-group" style="width:1%"><a href="Urachus" title="Urachus">Urachus</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em">
<ul><li><a href="Urachal_cyst" title="Urachal cyst">Urachal cyst</a></li>
<li><a href="Urachal_fistula" title="Urachal fistula">Urachal fistula</a></li>
<li><a href="Umbilical-urachal_sinus" title="Umbilical-urachal sinus">Urachal sinus</a></li></ul>
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This article is issued from <a class="external text" title="Last edited on 2025-07-19" href="https://en.wikipedia.org/wiki/?title=Bladder_exstrophy&oldid=1301315234">Wikipedia</a>. The text is available under <a class="external text" href="https://creativecommons.org/licenses/by-sa/4.0/deed.en">Creative Commons Attribution-Share Alike 4.0</a> unless otherwise noted. Additional terms may apply for the media files.
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