Editing Cloacal exstrophy

{{Infobox medical condition (new)
| synonyms      = [[Omphalocele]]-cloacal exstrophy-[[imperforate anus]]-spinal defect syndrome
| name          = Cloacal exstrophy
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| complications = Limb deformities, open neural tube defects<ref>Ben‐Neriah, Z.; Withers, S.; Thomas, M.; Toi, A.; Chong, K.; Pai, A.; Velscher, L.; Vero, S.; Keating, S.; Taylor, G; and Chitayat, D. (2007)." OEIS complex: prenatal ultrasound and autopsy findings". ''Ultrasound Obstet Gynecol'', 29: 170-177. https://doi.org/10.1002/uog.3874 </ref>
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'''Cloacal exstrophy''' ('''CE''') is a severe [[birth defect]] wherein much of the abdominal organs (the [[urinary bladder|bladder]] and [[intestines]]) are exposed. It often causes the splitting of the bladder, [[genitalia]], and the [[anus]]. It is sometimes called '''OEIS complex'''.<ref>{{Cite web|url=https://omim.org/entry/258040|title=OMIM Entry - 258040 - OEIS COMPLEX|website=omim.org|language=en-us|access-date=2018-01-29}}</ref>

Diagnostic tests can include ultrasound, [[Voiding cystourethrography|voiding cystourethrogram]] (VCUG), [[intravenous pyelogram]] (IVP), nuclear renogram, computerized axial tomography ([[CT scan]]), and [[magnetic resonance imaging]] (MRI).<ref>{{Cite web|url=http://www.meddean.luc.edu/Lumen/MedEd/urology/guimaghm.htm|title=G/U Imaging:Home|website=www.meddean.luc.edu|access-date=2018-01-29}}</ref> Cloacal exstrophy is a rare birth defect, present in 1/200,000 pregnancies and 1/400,000 live births. It is associated with a defect of the [[ventral]] body wall and can be caused by inhibited [[mesoderm]]al migration.<ref>{{cite journal |last1= Hassan|first1=Moaied A. |date=May 2018|title=OEIS complex with a vesico-enteric fistula |journal=Journal of Pediatric Surgery Case Reports |volume= 35|pages= 45–47|doi= 10.1016/j.epsc.2018.05.016|doi-access= free}}</ref> The defect can often be comorbid with [[spinal bifida]] and kidney abnormalities.<ref>{{cite web|url= https://rarediseases.info.nih.gov/diseases/4080/omphalocele-exstrophy-of-the-cloaca-imperforate-anus-and-spinal-defects-complex|title= Omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects complex|author= <!--Not stated-->|website= Genetic and Rare Diseases Information Center|publisher= US Department of Health & Human Services|quote= There is a high association with other birth defects, especially spina bifida, which occurs in up to 75% of cases. Omphalocele, a defect of the abdominal wall in the region of the umbilicus, is also common, as are kidney abnormalities.|access-date= 2021-01-14|archive-date= 2021-01-16|archive-url= https://web.archive.org/web/20210116123424/https://rarediseases.info.nih.gov/diseases/4080/omphalocele-exstrophy-of-the-cloaca-imperforate-anus-and-spinal-defects-complex|url-status= dead}}</ref> Many individuals who are born with [[XY sex-determination system|XY]] cloacal exstrophy are [[assigned female at birth]] due to the appearance of the external genitalia.<ref>{{Cite journal|url=https://www.degruyter.com/document/doi/10.1515/JPEM.2004.17.3.273/html|title=Communicating with Parents with Full Disclosure: A Case of Cloacal Extrophy with Genital Ambiguity|date=March 1, 2004|journal=Journal of Pediatric Endocrinology and Metabolism|volume=17|issue=3|pages=273–280|via=www.degruyter.com|doi=10.1515/JPEM.2004.17.3.273 |last1=Myers |first1=C. |last2=Lee |first2=P.A. |pmid=15112903 |s2cid=23729036 |url-access=subscription}}</ref><ref>{{cite web|title=''Progress and Politics in the intersex rights movement, Feminist theory in action''|url=http://www.aisia.org/wp-content/uploads/2016/11/Dreger__Herndon_2009.pdf|author1=Alice D. Dreger |author2=April M. Herndon }}</ref> Performing genital [[Intersex medical interventions|surgery on intersex infants]] for cosmetic purposes is considered a human rights violation by the [[World Health Organization]].<ref>{{Cite journal|last=Carpenter|first=Morgan|date=December 2018|title=Intersex Variations, Human Rights, and the International Classification of Diseases|journal=Health and Human Rights|volume=20|issue=2|pages=205–214|pmid=30568414|pmc=6293350}}</ref><ref>{{Cite journal|last=Greenberg|first=Julie A.|date=June 2017|title=Legal, ethical, and human rights considerations for physicians treating children with atypical or ambiguous genitalia|journal=Seminars in Perinatology|volume=41|issue=4|pages=252–255|doi=10.1053/j.semperi.2017.03.012|pmid=28478089}}</ref>

== Cloacal exstrophy males reassigned and raised as females ==
Between 1960 and 2000,<ref name=":1" /> doctors believed XY males with cloacal 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 [[William Reiner]] 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.<ref name=":0">{{Cite book |last=Bailey |first=J. Michael |url=https://www.researchgate.net/publication/281747420 |title=The Man Who Would Be Queen |date=2003 |publisher=Joseph Henry Press |isbn=978-0309084185 |pages=48–52 |format=PDF}}</ref>

According to [[J. Michael Bailey]]:<ref name=":0" />{{Blockquote|text=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.}}In terms of their sexual orientations, a 2016 academic review by [[J. Michael Bailey]] found 7 published cases of genetic males who were reassigned as females at birth and raised as girls, 5 of whom were cloacal exstrophy cases. All were strongly attracted to females.<ref name=":1">{{Cite journal |last1=Bailey |first1=J. Michael |last2=Vasey |first2=Paul L. |last3=Diamond |first3=Lisa M. |last4=Breedlove |first4=S. Marc |last5=Vilain |first5=Eric |last6=Epprecht |first6=Marc |date=2016 |title=Sexual Orientation, Controversy, and Science |journal=Psychological Science in the Public Interest |language=en |volume=17 |issue=2 |pages=72–74 |doi=10.1177/1529100616637616 |issn=1529-1006 |doi-access=free|pmid=27113562 }}</ref> According to a 2015 article in ''[[The Boston Globe]]'', 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.<ref>{{Cite web |last=Swidey |first=Neil |date=23 August 2015 |title=What makes people gay? (An update) |url=http://www.bostonglobe.com/magazine/2015/08/22/what-makes-people-gay-update/8Mos2MXHvX5JsxP7AzW9RJ/story.html |access-date= |website=[[The Boston Globe]] |archive-date=20 August 2015 |archive-url=https://web.archive.org/web/20150820112932/http://www.bostonglobe.com/magazine/2015/08/22/what-makes-people-gay-update/8Mos2MXHvX5JsxP7AzW9RJ/story.html |url-status=bot: unknown }}</ref> A 2023 review by Swift-Gallant et al. cited an additional two cases from media reports, both of whom were [[gynephilic]] (attracted to women).<ref name=":2">{{Cite journal |last1=Swift-Gallant |first1=Ashlyn |last2=Aung |first2=Toe |last3=Rosenfield |first3=Kevin |last4=Dawood |first4=Khytam |last5=Puts |first5=David |date=2023-09-20 |title=Organizational Effects of Gonadal Hormones on Human Sexual Orientation |url=https://www.researchgate.net/publication/374055009 |journal=Adaptive Human Behavior and Physiology |language=en |volume=9 |issue=4 |pages=347 |doi=10.1007/s40750-023-00226-x |issn=2198-7335 |url-access= |via=Springer}}</ref>

Reiner's results have caused some doctors to reconsider the practice of sex reassigning genetic males.<ref name=":0" /> A 2011 study found that 79% of paediatric urologists surveyed favoured male assignment for genetic males with cloacal exstrophy.<ref>{{Cite journal |last1=Diamond |first1=David A. |last2=Burns |first2=Jeffrey P. |last3=Huang |first3=Lin |last4=Rosoklija |first4=Ilina |last5=Retik |first5=Alan B. |date=2011 |title=Gender Assignment for Newborns With 46XY Cloacal Exstrophy: A 6-Year Followup Survey of Pediatric Urologists |url=http://www.jurology.com/doi/10.1016/j.juro.2011.03.101 |journal=Journal of Urology |language=en |volume=186 |issue=4S |pages=1642–1648 |doi=10.1016/j.juro.2011.03.101 |pmid=21862059 |issn=0022-5347 |url-access=subscription}}</ref>

=== Cases in the media ===

* Joe Holliday, a British cloacal exstrophy male who was reassigned and raised as a female. Holliday had masculine interests and is attracted to females. He found out he was a genetic male at age 25, and reverted to living as a male.<ref>{{Cite web |last=Chapman |first=Nigel |date=10 August 2015 |title=Joe (27) was raised a female on medical advice. He feels his identity was stolen as a baby and wants it back |url=https://www.spaldingvoice.co.uk/joe-27-was-raised-a-female-on-medical-advice-he-feels-his-identity-was-stolen-as-a-baby-and-wants-it-back/ |access-date= |website=The Voice of Spalding and South Holland |language=en-GB}}</ref><ref name=":2" />
* Sophie Ottaway, a British cloacal exstrophy male who was reassigned and raised as a female. Ottaway found out about her genetic sex at age 22. She retained female identity, although believes the reassignment was incorrect. She is attracted to women.<ref>{{Cite news |last=Calkin |first=Jessamy |date=2023-08-25 |title='The doctors changed my gender at two days old – 22 years later, I discovered the truth' |url=https://www.telegraph.co.uk/family/life/sophie-ottaway-rare-birth-defect-gender-surgery/ |access-date=2024-06-03 |work=The Telegraph |language=en-GB |issn=0307-1235}}</ref><ref name=":2" />

==See also==
* [[Bladder exstrophy]]

==References==
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== External links ==
{{Medical resources
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|  Orphanet        = 93929
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[[Category:Congenital disorders of urinary system]]
[[Category:Rare diseases]]
[[Category:Intersex variations]]