Pelvic inlet reconstruction for obstruction associated with lumbosacral agenesisutilizing distraction osteogenesisand circular external fixation

Lumbosacral agenesis is a severe anomaly characterized by the absence of some portion of the lumbosacral spine, paralysis of the bowel and bladder, and variable degree of lower extremity paralysis with intact sensation. A 15-year-old female with severe lumbosacral agenesis was presented with a history of pelvic inlet obstruction requiring emergency colostomy 3 years previously. Following vertical midline osteotomy of the fused ileum, two olive wires were inserted through each half of the ileum and secured to proper halves of the specialized external fixator. Gradual distraction was continued for 8 weeks. At that time, reestablishment of adequate pelvic inlet was confirmed by CT scan. Patient lastly was examined at 34 years of age, 19 years after frame removal. X-rays show a fairly remarkable preservation of the pelvic distraction gap and a pseudoarticulation between the lumbar spine and the pelvis allowing both sitting and standing. The patient is very happy with her social life, has been married twice, and has conceived three children naturally.