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Learn About Exstrophy-Epispadias Complex

What is the definition of Exstrophy-Epispadias Complex?
Exstrophy-epispadias complex (EEC) refers to a spectrum of birth defects that includes epispadias, classical bladder exstrophy, and exstrophy of the cloaca and several variants. EEC is characterized by a visible defect of the lower abdominal wall and other problems. In normal development, the cloacal membrane temporarily separates the cloaca (final part of the intestine) into urogenital and anal regions, and it ruptures after fusing with a structure known as the urogenital septum, which is made up of the tissue that will form the abdominal muscles (mesoderm). If the cloacal membrane does not fuse correctly with the urogenital septum, it does not form the mesoderm and, as a result, the abdominal muscles do not form. The exact timing of the rupture determines whether the child is born with isolated epispadias, classic bladder exstrophy, or cloacal exstrophy. Depending on severity, EEC may involve the urinary system, musculoskeletal system, pelvis, pelvic floor, abdominal wall, genitalia, and sometimes the spine and anus. There is no known cause for EEC. The University of Michigan has a webpage about the development of the embryo and its parts, including the formation of the cloaca.
What are the alternative names for Exstrophy-Epispadias Complex?
  • Exstrophy-epispadias complex
  • Exstrophy of the bladder
  • BEEC
  • Bladder exstrophy-epispadias-cloacal extrophy complex
  • EEC
  • Bladder exstrophy
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What are the latest Exstrophy-Epispadias Complex Clinical Trials?
PROSPECTIVE STUDY EVALUATING THE SAFETY AND EFFECTIVENESS OF ADJUSTABLE CONTINENCE THERAPY (ACT) BALLOONS FOR THE MANAGEMENT OF URINARY INCONTINENCE IN CHILDREN WITH BLADDER EXSTROPHY OR INCONTINENT EPISPADIAS

Summary: The purpose of this study is to evaluate the effectiveness and safety of a minimally invasive surgical procedure with ACT (Adjustable Continence Therapy) balloons implantation for the treatment of urinary incontinence in children with bladder exstrophy or isolated epispadias. The ACT therapy consists of two small adjustable silicone balloons connected with a tubing to a port, surgically placed aro...

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