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Introduction
  • A congenital disorder that involves the musculoskeletal and genitourinary systems
  • Epidemiology
    • classic exstrophy
      • 1/40,000 infants diagnosed with this condition
    • cloacal exstrophy
      • 1/200,000 infants with intestinal track involved as well
  • 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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