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Bladder Extrophy

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Topic updated on 12/08/14 1:57pm
Introduction
  • A congenital disorder that involves the musculoskeletal and genitourinary systems
  • Epidemiology
    • 1/40,000 infants diagnosed with this condition
  • Pathology
    • abnormal anterior rupture of the cloacal membrane early in the embryonic period
    • mesenchymal ingrowth into abdominal wall is also inhibited
    • altered migration of sclerotomes that comprise the anterior elements (pubis)
  • Associated conditions
    • family history should be sought out as often associated with other conditions
Presentation
  • Symptoms
    • a thorough history and a complete examination are essential
    • urinary system infection(s)
  • Physical exam
    • genitourinary system
      • exposed bladder
    • musculoskeletal
      • acetabuli are ~12 degrees externally rotated  
        • without pubis to tether the anterior ring, the posterior elements externally rotate
      • gait shows an external foot progression
Imaging
  • Radiographs
    • recommended views
      • obtain  AP pelvic radiograph
Treatment
  • Operative
    • staged multidisciplinary reconstruction
      • management should be multidisciplinary and involve pediatric urologist and general surgeon
      • technique
        • stage I: primary closure of bladder (newborn)
        • stage II: epispadias repair in males (1-2 y/o)
        • stage III: bladder neck reconstructions (4 y/o)
        • pelvic osteotomies may be performed at any stage of process

 

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