Cloacal exstrophy explained

Synonyms:Omphalocele-cloacal exstrophy-imperforate anus-spinal defect syndrome
Cloacal exstrophy
Complications:Limb deformities, open neural tube defects[1]
Treatment:Surgical intervention

Cloacal exstrophy (CE) is a severe birth defect wherein much of the abdominal organs (the bladder and intestines) are exposed. It often causes the splitting of the bladder, genitalia, and the anus. It is sometimes called OEIS complex.[2]

Diagnostic tests can include ultrasound, voiding cystourethrogram (VCUG), intravenous pyelogram (IVP), nuclear renogram, computerized axial tomography (CT scan), and magnetic resonance imaging (MRI).[3] 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 mesodermal migration.[4] The defect can often be comorbid with spinal bifida and kidney abnormalities.[5] Many individuals who are born with XY cloacal exstrophy are assigned female at birth due to the appearance of the external genitalia.[6] [7] Performing genital surgery on intersex infants for cosmetic purposes is considered a human rights violation by the World Health Organization.[8] [9]

Cloacal exstrophy males reassigned and raised as females

Between 1960 and 2000, 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.[10]

According to J. Michael Bailey:In terms of their sexual orientations, a 2016 academic review by J. Michael Bailey 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.[11] According 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.[12] A 2023 review by Swift-Gallant et al. also cited two additional cases from media reports, both of whom were gynephilic.[13]

Reiner's results have caused some doctors to reconsider the practice of sex reassigning genetic males. A 2011 study found that 79% of paediatric urologists surveyed favoured male assignment for genetic males with cloacal exstrophy.[14]

Cases in the media

See also

Notes and References

  1. 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
  2. Web site: OMIM Entry - 258040 - OEIS COMPLEX. omim.org. en-us. 2018-01-29.
  3. Web site: G/U Imaging:Home. www.meddean.luc.edu. 2018-01-29.
  4. Hassan. Moaied A. . May 2018. OEIS complex with a vesico-enteric fistula . Journal of Pediatric Surgery Case Reports . 35. 45–47. 10.1016/j.epsc.2018.05.016. free.
  5. Web site: Omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects complex. . Genetic and Rare Diseases Information Center. US Department of Health & Human Services. 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.. 2021-01-14. 2021-01-16. https://web.archive.org/web/20210116123424/https://rarediseases.info.nih.gov/diseases/4080/omphalocele-exstrophy-of-the-cloaca-imperforate-anus-and-spinal-defects-complex. dead.
  6. Communicating with Parents with Full Disclosure: A Case of Cloacal Extrophy with Genital Ambiguity. March 1, 2004. Journal of Pediatric Endocrinology and Metabolism. 17. 3. 273–280. www.degruyter.com. 10.1515/JPEM.2004.17.3.273 . Myers . C. . Lee . P.A. . 15112903 . 23729036 .
  7. Web site: Progress and Politics in the intersex rights movement, Feminist theory in action. Alice D. Dreger . April M. Herndon .
  8. Carpenter. Morgan. December 2018. Intersex Variations, Human Rights, and the International Classification of Diseases. Health and Human Rights. 20. 2. 205–214. 30568414. 6293350.
  9. Greenberg. Julie A.. June 2017. Legal, ethical, and human rights considerations for physicians treating children with atypical or ambiguous genitalia. Seminars in Perinatology. 41. 4. 252–255. 10.1053/j.semperi.2017.03.012. 28478089.
  10. Book: Bailey, J. Michael . The Man Who Would Be Queen . 2003 . Joseph Henry Press . 978-0309084185 . 48–52 . PDF.
  11. Bailey . J. Michael . Vasey . Paul L. . Diamond . Lisa M. . Breedlove . S. Marc . Vilain . Eric . Epprecht . Marc . 2016 . Sexual Orientation, Controversy, and Science . Psychological Science in the Public Interest . en . 17 . 2 . 72–74 . 10.1177/1529100616637616 . 1529-1006 . free. 27113562 .
  12. Web site: Swidey . Neil . 23 August 2015 . What makes people gay? (An update) . . 20 August 2015 . https://web.archive.org/web/20150820112932/http://www.bostonglobe.com/magazine/2015/08/22/what-makes-people-gay-update/8Mos2MXHvX5JsxP7AzW9RJ/story.html . bot: unknown .
  13. Swift-Gallant . Ashlyn . Aung . Toe . Rosenfield . Kevin . Dawood . Khytam . Puts . David . 2023-09-20 . Organizational Effects of Gonadal Hormones on Human Sexual Orientation . Adaptive Human Behavior and Physiology . en . 9 . 4 . 347 . 10.1007/s40750-023-00226-x . 2198-7335 . limited . Springer.
  14. Diamond . David A. . Burns . Jeffrey P. . Huang . Lin . Rosoklija . Ilina . Retik . Alan B. . 2011 . Gender Assignment for Newborns With 46XY Cloacal Exstrophy: A 6-Year Followup Survey of Pediatric Urologists . Journal of Urology . en . 186 . 4S . 1642–1648 . 10.1016/j.juro.2011.03.101 . 21862059 . 0022-5347 . subscription.
  15. Web site: Chapman . Nigel . 10 August 2015 . Joe (27) was raised a female on medical advice. He feels his identity was stolen as a baby and wants it back . The Voice of Spalding and South Holland . en-GB.
  16. News: Calkin . Jessamy . 2023-08-25 . 'The doctors changed my gender at two days old – 22 years later, I discovered the truth' . 2024-06-03 . The Telegraph . en-GB . 0307-1235.

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