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European Spine Journal

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01-05-2013 | Case Report

Surgical treatment of Klippel–Feil syndrome with basilar invagination

Authors: Nobuhide Ogihara, Jun Takahashi, Hiroki Hirabayashi, Keijoro Mukaiyama, Hiroyuki Kato

Published in: European Spine Journal | Special Issue 3/2013

Abstract

Introduction

Klippel–Feil syndrome (KFS) is a congenital cervical vertebral union caused by a failure of segmentation during abnormal development and frequently accompanies conditions such as basicranial malformation, atlas assimilation, or dens malformation. Especially in basilar invagination (BI), which is a dislocation of the dens in an upper direction, compression of the spinomedullary junction from the ventral side results in paralysis, and treatment is required.

Clinical presentation

We present the case of a 38-year-old male patient with KFS and severe BI. Plane radiographs and computed tomography (CT) images showed severe BI, and magnetic resonance image (MRI) revealed spinal cord compression caused by invagination of the dens into the foramen magnum and atlantoaxial subluxation. Reduction by halo vest and skeletal traction were not successful. Occipitocervical fusion along with decompression of the foramen magnum, C1 laminectomy, and reduction using instruments were performed. Paralysis was temporarily aggravated and then gradually improved. Unsupported walking was achieved 24 months after surgery, and activities of daily life could be independently performed at the same time. CT and MRI revealed that dramatic reduction of vertical subluxation and spinal cord decompression were achieved.

Conclusion

Reduction and internal fixation using instrumentation are effective techniques for KFS with BI; however, caution should be exercised because of the possibility of paralysis caused by intraoperative reduction.

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Title: Surgical treatment of Klippel–Feil syndrome with basilar invagination
Authors: Nobuhide Ogihara
Jun Takahashi
Hiroki Hirabayashi
Keijoro Mukaiyama
Hiroyuki Kato
Publication date: 01-05-2013
Publisher: Springer-Verlag
Published in: European Spine Journal / Issue Special Issue 3/2013
Print ISSN: 0940-6719
Electronic ISSN: 1432-0932
DOI: https://doi.org/10.1007/s00586-012-2489-3

At a glance: The STEP trials

Springer Medicine

Surgical treatment of Klippel–Feil syndrome with basilar invagination

Abstract

Introduction

Clinical presentation

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Introduction

Clinical presentation

Conclusion

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