Cloacal exstrophy

Cloacal exstrophy
Other namesOmphalocele-cloacal exstrophy-imperforate anus-spinal defect syndrome
SpecialtyMedical genetics 
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Complicationslimb deformities, open neural tube defects [1]
TreatmentSurgical 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]

See also

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. ↑ "OMIM Entry - 258040 - OEIS COMPLEX". omim.org. Retrieved 2018-01-29.
  3. ↑ "G/U Imaging:Home". www.meddean.luc.edu. Retrieved 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. doi:10.1016/j.epsc.2018.05.016.
  5. ↑ "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.
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