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Updated: Jun 14 2021

Bladder Exstrophy

  • summary
    • Bladder Exstrophy is a rare congenital disorder that involves the musculoskeletal and genitourinary systems and may present with several pelvic abnormalities.
    • Diagnosis can be confirmed with pelvic radiographs revealing pubic rami diastasis, shortened pubi rami, and acetabular retroversion.
    • Treatment is surgical with staged multidisciplinary reconstruction.
  • Epidemiology
    • Incidence
      • classic exstrophy
        • 1/40,000 infants diagnosed with this condition
      • cloacal exstrophy
        • 1/200,000 infants with intestinal track involved as well
  • Etiology
    • Pathology
      • abnormal anterior rupture of the cloacal membrane early in the embryonic period
      • mesenchymal ingrowth into the 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 retroverted
          • without pubis to tether the anterior ring, the posterior elements retrovert
        • waddling gait with external foot progression
  • Imaging
    • Radiographs
      • recommended views
        • obtain AP pelvic radiograph
      • findings
        • pubic rami diastasis
        • shortened pubic rami
        • acetabular retroversion
  • Treatment
    • Goal of treatment
      • close abdominal wall
      • achieve urinary continence
      • normal renal function
    • Operative
      • staged multidisciplinary reconstruction
        • indications
          • all cases require surgical treatment
        • multidisciplinary approach
          • management should be multidisciplinary and involve pediatric urologist and general surgeon
          • reconstruction sequence may vary by the preference of urologist
        • components
          • primary closure of bladder (newborn)
            • usually the first stage
          • epispadias repair in males (1-2 y/o)
            • usually 2nd stage
          • bladder neck reconstructions (4 y/o)
            • usually 3rd stage
          • pelvic osteotomies
            • performed in order to decrease tension on the bladder and repaired abdominal wall to decrease dehiscence
  • Technique
    • Staged multidisciplinary reconstruction
      • 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
          • in order to decrease tension on the bladder and repaired abdominal wall to decrease dehiscence
          • timing
            • closure of pelvic ring may be performed at any stage of the process
          • fixation
            • pelvic osteotomy fixation depends on the age
              • newborns
                • not required in newborns (skin traction and hips flexed 90 degrees)
              • younger patients
                • external fixation in younger patients
              • age > 8 years
                • augment correction of diastasis with plate fixation in > 8 y/o
  • Complications
    • Recurrent pubic diastasis
      • Common whether or not osteotomy was performed
      • Does not appear to impact activity level
    • Complications of anterior innominate osteotomy
      • wound dehiscence
      • transient femoral nerve palsy
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