Abstract

Patients with lumbosacral agenesis characteristically sit on their iliac wings with their torsos bent forward, which yields an increase in intra-abdominal pressure and, subsequently, negative effects on their diaphragm. The sacrum is not available as an anchor point for instrumentation. Dunn-McCarthy rods or Galveston fixations cannot be performed due to these limited anatomic properties. On the other hand, the absence of necessary bone mass for fusion anteriorly limits the fusion interventions to the posterior. Therefore, a secure and rigid fixation is essential to preclude the need for an external support. There are limited publications discussing different techniques due to the relatively rare incidence of the disease. We report the clinical and radiological results of a new technique applied to three patients in which previously recommended methods are modified. Two 6-year-old female patients and one 5-year-old male patient with lumbosacral agenesis underwent posterior lumbopelvic instrumentation and fusion. Together with standard pedicle screw spinal instrumentation, pelvic fixation is obtained with a combination of supero-inferior directed rod and/or screw to overcome deforming forces created at the flexion-extension pivot points of the lumbopelvic junction. Autogenic anterior tibial cortical structural graft is used for laminopelvic bridging, and demineralized bone matrix is used for the augmentation of osteoinduction. A single leg hip spica is applied for 4months to protect the fixation. Total correction yielded an aligned spine with a posture that allows for sitting on the ischial spines for all three patients. Solid fusion was observed to maintain this correction at the final follow-up. The use of new-generation pediatric spinal instrumentation systems with a new technique without knee disarticulation provides a safe and effective fixation and fusion in lumbosacral agenesis.

Highlights

  • Surgery has great challenges in lumbosacral agenesis due to the pathognomonic pelvic anatomy, together with the absence of a big bone segment

  • Together with standard pedicle screw spinal instrumentation, pelvic fixation is obtained with a combination of superoinferior directed rod and/or screw to overcome deforming forces created at the flexion–extension pivot points of the lumbopelvic junction

  • Together with standard pedicle screw spinal instrumentation in the thoracic 12th vertebrae, lumbar 1st and 2nd in Case 1, and thoracic 8th to 12th vertebrae in Case 2, thoracic 11th to lumbar 1 in Case 3 pelvic fixation was obtained with a combination of supero-inferior directed divergent rod and/or screw to overcome deforming forces created at the flexion–extension pivot points of the lumbopelvic junction

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Summary

Introduction

Surgery has great challenges in lumbosacral agenesis due to the pathognomonic pelvic anatomy, together with the absence of a big bone segment. There are limited publications discussing different techniques due to the relatively rare incidence of the disease. Some distal lumbar segments are absent together with the sacrum in lumbosacral agenesis; this leads to trunk shortening and sitting problems due to the hypermobile lumbopelvic junction. The sacrum is not available as an anchor point for instrumentation. DunnMcCarthy rods or Galveston fixations cannot be performed due to these anatomic properties. The absence of anterior bone mass for fusion obligates a posterior fusion procedure. A secure and rigid fixation is necessary to maintain correction and to achieve solid fusion and preclude the need for a long-term external

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