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Surgical treatment of Klippel-Feil syndrome with basilar invagination.
Eur Spine J 2013; 22 Suppl 3:S380-7ES

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.

Authors+Show Affiliations

Department of Orthopaedic Surgery, School of Medicine, Shinshu University, 3-1-1 Asahi, Matsumoto-city, Nagano, 390-8621, Japan. ogiogi@kif.biglobe.ne.jpNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

22926486

Citation

Ogihara, Nobuhide, et al. "Surgical Treatment of Klippel-Feil Syndrome With Basilar Invagination." European Spine Journal : Official Publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society, vol. 22 Suppl 3, 2013, pp. S380-7.
Ogihara N, Takahashi J, Hirabayashi H, et al. Surgical treatment of Klippel-Feil syndrome with basilar invagination. Eur Spine J. 2013;22 Suppl 3:S380-7.
Ogihara, N., Takahashi, J., Hirabayashi, H., Mukaiyama, K., & Kato, H. (2013). Surgical treatment of Klippel-Feil syndrome with basilar invagination. European Spine Journal : Official Publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society, 22 Suppl 3, pp. S380-7. doi:10.1007/s00586-012-2489-3.
Ogihara N, et al. Surgical Treatment of Klippel-Feil Syndrome With Basilar Invagination. Eur Spine J. 2013;22 Suppl 3:S380-7. PubMed PMID: 22926486.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Surgical treatment of Klippel-Feil syndrome with basilar invagination. AU - Ogihara,Nobuhide, AU - Takahashi,Jun, AU - Hirabayashi,Hiroki, AU - Mukaiyama,Keijoro, AU - Kato,Hiroyuki, Y1 - 2012/08/28/ PY - 2011/12/26/received PY - 2012/08/15/accepted PY - 2012/06/18/revised PY - 2012/8/29/entrez PY - 2012/8/29/pubmed PY - 2013/11/20/medline SP - S380 EP - 7 JF - European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society JO - Eur Spine J VL - 22 Suppl 3 N2 - 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. SN - 1432-0932 UR - https://www.unboundmedicine.com/medline/citation/22926486/Surgical_treatment_of_Klippel_Feil_syndrome_with_basilar_invagination_ L2 - https://doi.org/10.1007/s00586-012-2489-3 DB - PRIME DP - Unbound Medicine ER -