Medicine:Cloacal exstrophy
| Cloacal exstrophy | |
|---|---|
| Other names | Omphalocele-cloacal exstrophy-imperforate anus-spinal defect syndrome |
| 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]
See also
References
- ↑ 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" (in en-us). https://omim.org/entry/258040.
- ↑ "G/U Imaging:Home". http://www.meddean.luc.edu/Lumen/MedEd/urology/guimaghm.htm.
- ↑ 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". US Department of Health & Human Services. https://rarediseases.info.nih.gov/diseases/4080/omphalocele-exstrophy-of-the-cloaca-imperforate-anus-and-spinal-defects-complex. "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. https://www.degruyter.com/document/doi/10.1515/JPEM.2004.17.3.273/html.
- ↑ "Progress and Politics in the intersex rights movement, Feminist theory in action". http://www.aisia.org/wp-content/uploads/2016/11/Dreger__Herndon_2009.pdf.
- ↑ Carpenter, Morgan (December 2018). "Intersex Variations, Human Rights, and the International Classification of Diseases". Health and Human Rights 20 (2): 205–214. 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.
External links
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