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Surgical treatment of Klippel–Feil syndrome with basilar invagination

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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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Acknowledgments

The authors would like to thank the staff members of the Department of Orthopaedic Surgery, Shinshu University, School of Medicine.

Conflict of interest

The authors have no competing or financial interests to disclose in the preparation of this manuscript.

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Authors and Affiliations

  1. Department of Orthopaedic Surgery, School of Medicine, Shinshu University, 3-1-1 Asahi, Matsumoto-city, Nagano, 390-8621, Japan

    Nobuhide Ogihara, Jun Takahashi, Hiroki Hirabayashi, Keijoro Mukaiyama & Hiroyuki Kato

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  1. Nobuhide Ogihara
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  2. Jun Takahashi
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  3. Hiroki Hirabayashi
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  4. Keijoro Mukaiyama
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  5. Hiroyuki Kato
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Correspondence to Nobuhide Ogihara.

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Ogihara, N., Takahashi, J., Hirabayashi, H. et al. Surgical treatment of Klippel–Feil syndrome with basilar invagination. Eur Spine J 22 (Suppl 3), 380–387 (2013). https://doi.org/10.1007/s00586-012-2489-3

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  • DOI: https://doi.org/10.1007/s00586-012-2489-3

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