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The Bladder Exstrophy-Epispadias Complex (BEEC)

  • Geoffroy de Sallmard
  • Omar Alhadeedi
  • Delphine Demède
  • Pierre MouriquandEmail author
Chapter

Abstract

The BEEC complex is the result of a failed cavitation and separation of the pelvic organs and a failed closure of the pelvic ring. During the first 2 months of gestation, the embryo (germinal disc) is subjected to a complex process (delimitation) [1] of cranio-caudal tubularization resulting in the cavitation and connection of the pelvic organs to the pelvic floor. This leads to the creation of separated urinary, genital and intestinal cavities independently connected to the perineal surface by distinct conduits and their corresponding sphincter. If the delimitation process is interrupted early, the distal bowels, the bladder and the urethra are not individualized and appear as contiguous and often duplicated plates. This is a cloacal exstrophy and is often associated with other abnormalities. If the process halts later in the gestation, the bowels are properly formed and connected, but the bladder and urethra are both widely open, presenting as a classical bladder exstrophy. Finally, when the process stops late, only the urethra and the urethral sphincter are open and incompetent, presenting as an epispadias. Depending on the timing of the cavitation failure, one should distinguish posterior epispadias where the sphincteric mechanisms are involved and the child incontinent at various degrees and anterior epispadias where the continence mechanisms are completely or partially respected but the genital tubercle remains abnormal. Epispadias exists both in males and females.

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Geoffroy de Sallmard
    • 1
  • Omar Alhadeedi
    • 1
  • Delphine Demède
    • 1
  • Pierre Mouriquand
    • 1
    Email author
  1. 1.Service de Chirurgie Uro-génitale de l’Enfant, Hospices Civils de LyonUniversité Claude Bernard, Lyon1, Hôpital Mère-EnfantBronFrance

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