Surgical Treatment of Lumbosacral Pseudarthrosis and Spondyloptosis in a Patient with Neurofibromatosis Type 1 and a Large Lumbar Anterior Meningocele. Case Report and Review of the Literature

Benjamin Brokinkel; Juliane Schroeteler; Bernd Kasprzak; Angela Brentrup; Christian Ewelt; Walter Stummer; Mark Klingenhoefer

doi:10.1055/s-0034-1372439

Journal of Neurological Surgery Part A: Central European Neurosurgery, Table of Contents

J Neurol Surg A Cent Eur Neurosurg 2015; 76(02): 172-175
DOI: 10.1055/s-0034-1372439

Case Report

Georg Thieme Verlag KG Stuttgart · New York

Surgical Treatment of Lumbosacral Pseudarthrosis and Spondyloptosis in a Patient with Neurofibromatosis Type 1 and a Large Lumbar Anterior Meningocele. Case Report and Review of the Literature

Authors

Benjamin Brokinkel

¹Department of Neurosurgery, Universitätsklinikum Münster, Münster, Germany
Juliane Schroeteler

¹Department of Neurosurgery, Universitätsklinikum Münster, Münster, Germany
Bernd Kasprzak

²Clinic for Vascular and Endovascular Surgery, University Hospital Münster, Münster, Germany
Angela Brentrup

¹Department of Neurosurgery, Universitätsklinikum Münster, Münster, Germany
Christian Ewelt

¹Department of Neurosurgery, Universitätsklinikum Münster, Münster, Germany
Walter Stummer

¹Department of Neurosurgery, Universitätsklinikum Münster, Münster, Germany
Mark Klingenhoefer

¹Department of Neurosurgery, Universitätsklinikum Münster, Münster, Germany

Abstract

Background Lumbar anterior meningocele (MC) is rare in patients with neurofibromatosis type 1 (NF1). Although spinal fusion with maximum possible resection of the cele might be indicated in these special cases, reports describing operative procedures are lacking.

Clinical Presentation We present a young woman with NF1 and lower back pain due to lumbar anterior MC, extensive osteolysis, and deformation of the lumbar spine. After Harrington spondylodesis from T12 to S1 in 1989, she developed lumbosacral pseudarthrosis and transcutaneous migration of the spondylodesis. Thus the left pole of the spondylodesis was shortened below the level of L5 in 2005. Years later, the patient presented with lower back pain and wound infection. Imaging revealed the MC and avulsion of the right S1 screw with lumbosacral pseudarthrosis and spondyloptosis, and transcutaneous migration of the right spondylodesis pole. Three-stage surgery was indicated with explantation of the Harrington system in the first step and posterior fusion from T10 to S2 in the second step. Finally, vertebral resection, gathering of the cele, and implantation of a customized titanium cage was achieved through an anterior transabdominal approach. Postoperatively, the patient reported sufficient reduction of lower back pain.

Conclusions Lumbar anterior MC is rare in patients with NF1 and may cause spinal instability. When surgery is indicated, long-range spinal fusion and reduction of the cele should be considered. Surgery may be challenging requiring a staged operative procedure and an interdisciplinary collaboration.

Keywords

meningocele - neurofibromatoses - spinal fusion - von Recklinghausen disease - spinal instability

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References

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