Cloacal exstrophy
| Cloacal exstrophy | |
|---|---|
| Other names | Omphalocele-cloacal exstrophy-imperforate anus-spinal defect syndrome |
| Specialty | Medical genetics  |
| Complications | Limb deformities, open neural tube defects[1] |
| Treatment | Surgical intervention |
Cloacal exstrophy (EC) 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[edit]
Between 1960 and 2000,[10] 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.[11]
According to J. Michael Bailey:[11]
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 boys reassigned female at birth and raised as girls, of which 5 were cloacal exstrophy cases. All were strongly attracted to females.[10] 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]
Reiner's results have caused some doctors to reconsider the practice of sex reassigning genetic males.[11] A 2011 study found that 79% of paediatric urologists surveyed favoured male assignment for cloacal exstrophy.[13]
Cases in the media[edit]
- Joe Holiday. 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.[14]
- 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.[15]
See also[edit]
References[edit]
- ^ 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
- ^ "OMIM Entry - 258040 - OEIS COMPLEX". omim.org. Retrieved 2018-01-29.
- ^ "G/U Imaging:Home". www.meddean.luc.edu. Retrieved 2018-01-29.
- ^ Hassan, Moaied A. (May 2018). "OEIS complex with a vesico-enteric fistula". Journal of Pediatric Surgery Case Reports. 35: 45–47. doi:10.1016/j.epsc.2018.05.016.
- ^ "Omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects complex". Genetic and Rare Diseases Information Center. US Department of Health & Human Services. Archived from the original on 2021-01-16. Retrieved 2021-01-14.
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.
- ^ Myers, C.; Lee, P.A. (March 1, 2004). "Communicating with Parents with Full Disclosure: A Case of Cloacal Extrophy with Genital Ambiguity". Journal of Pediatric Endocrinology and Metabolism. 17 (3): 273–280. doi:10.1515/JPEM.2004.17.3.273. S2CID 23729036 – via www.degruyter.com.
- ^ Alice D. Dreger; April M. Herndon. "Progress and Politics in the intersex rights movement, Feminist theory in action" (PDF).
- ^ Carpenter, Morgan (December 2018). "Intersex Variations, Human Rights, and the International Classification of Diseases". Health and Human Rights. 20 (2): 205–214. PMC 6293350. PMID 30568414.
- ^ 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. doi:10.1053/j.semperi.2017.03.012. PMID 28478089.
- ^ a b 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. 17 (2): 72–74. doi:10.1177/1529100616637616. ISSN 1529-1006.
- ^ a b c Bailey, J. Michael (2003). The Man Who Would Be Queen (PDF). Joseph Henry Press. pp. 48–52. ISBN 978-0309084185.
- ^ Swidey, Neil (23 August 2015). "What makes people gay? (An update)". The Boston Globe.
- ^ 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. 186 (4S): 1642–1648. doi:10.1016/j.juro.2011.03.101. ISSN 0022-5347.
- ^ 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.
- ^ Calkin, Jessamy (2023-08-25). "'The doctors changed my gender at two days old – 22 years later, I discovered the truth'". The Telegraph. ISSN 0307-1235. Retrieved 2024-06-03.