| 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
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| Presentation |
- Symptoms
- a thorough history and a complete examination are essential
- urinary system infection(s)
- Physical exam
- genitourinary system
- 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
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| Imaging |
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| 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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