Learn About Cloacal Exstrophy

What is the definition of Cloacal Exstrophy?
OEIS complex, also known as cloacal exstrophy, is the most severe birth defect within the exstrophy-epispadias complex. It is characterized by Omphalocele, Exstrophy, Imperforate anus and Spinal defects. A child with this condition will have the bladder and a portion of the intestines exposed outside the abdomen with the bony pelvis open like a book. In males, the penis is either flat and short or sometimes split. In females, the clitoris is split and there may be two vaginal openings. Also, frequently the intestine is short and the anus is not open (anus imperforate). 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. The defect occurs when the baby is developing inside the uterus during the first trimester of pregnancy, and seems to be due to the rupture of a tissue known as the cloacal membrane which results in the abnormal development of the abdominal wall and other malformations. The timing of the rupture determines the severity of the disorder. The cause for this malformation is unknown.
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What are the alternative names for Cloacal Exstrophy?
  • Omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects complex
  • Cloacal exstrophy
  • Cloacal exstrophy sequence
  • OEIS complex
  • Omphalocele - cloacal exstrophy - imperforate anus - spinal defect
  • Omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects
  • Omphalocele-cloacal exstrophy-imperforate anus-spinal defect syndrome
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What are the latest Cloacal Exstrophy Clinical Trials?
Long Term Assessment and Outcome of Adult Patients With Congenital Genitourinary Abnormalities.

Summary: A transitional Urology database was created in parallel with National Spina Bifida registry to follow patients with complex congenital urogenital anomalies and be able to prospectively evaluate them. The investigators obtained the standardized questionnaires to collect long-term data regarding patients' genitourinary status including urine and fecal continence, sexuality, fertility, and pelvic hea...

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Who are the sources who wrote this article ?

Published Date: May 02, 2022
Published By: Genetic and Rare Diseases Informnation Center

What are the Latest Advances for Cloacal Exstrophy?
Paediatric cystolitholapaxy through the Mitrofanoff/Monti channel.
Pelvic and lower extremity immobilization for cloacal exstrophy bladder and abdominal closure in neonates and older children.
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