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Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia: case report.
Surg Neurol 2006; 65(5):497-502; discussion 502SN

Abstract

BACKGROUND

Chiari I malformation, accompanied by superposed bony anomaly of the craniovertebral junction, is comparatively rare. We report a case of Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia.

CASE DESCRIPTION

The patient was a 61-year-old woman demonstrating numbness of the extremities, sensory impairment, muscular weakness, and tendon hyper-reflexia. X-ray images and CT scans demonstrated assimilation of the atlas to the occipital bone, C2 and C3 fusion, abnormal passage of the vertebral arteries, and an anomalous bony mass on the right lateral mass of the atlas protruding into the spinal column. The odontoid process was also deviated to the left. Magnetic resonance images demonstrated bilateral descent of the cerebellar tonsils and syringomyelia extending from C6 to T8. Computed tomographic scans with the head rotated to the right demonstrated increased narrowing of the vertebral column caused by the right lateral mass of the atlas, and MR images confirmed exaggerated deformation of the spinal cord at the same region. This deformation manifested no neurologic symptoms, and we therefore performed foramen magnum decompression and duraplasty using Gore-Tex (W.L. Gore & Associates, Inc., Flagstaff, AZ). In the early postoperative period, neurologic symptoms improved.

CONCLUSION

We believe it is important that a treatment plan for Chiari I malformation accompanied by bony anomaly of the craniovertebral junction be determined based on morphologic investigation of the region supplemented by dynamic imaging-based evaluation of instability, or a careful inspection for atypical passage of the vertebral arteries, a frequent site of complication.

Authors+Show Affiliations

Department of Neurological Surgery, Kagawa University, Miki-cho, Kagawa, 761-0793, Japan. mku@oak.dti.ne.jpNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

16630916

Citation

Kagawa, Masahiro, et al. "Chiari I Malformation Accompanied By Assimilation of the Atlas, Klippel-Feil Syndrome, and Syringomyelia: Case Report." Surgical Neurology, vol. 65, no. 5, 2006, pp. 497-502; discussion 502.
Kagawa M, Jinnai T, Matsumoto Y, et al. Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia: case report. Surg Neurol. 2006;65(5):497-502; discussion 502.
Kagawa, M., Jinnai, T., Matsumoto, Y., Kawai, N., Kunishio, K., Tamiya, T., & Nagao, S. (2006). Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia: case report. Surgical Neurology, 65(5), pp. 497-502; discussion 502.
Kagawa M, et al. Chiari I Malformation Accompanied By Assimilation of the Atlas, Klippel-Feil Syndrome, and Syringomyelia: Case Report. Surg Neurol. 2006;65(5):497-502; discussion 502. PubMed PMID: 16630916.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia: case report. AU - Kagawa,Masahiro, AU - Jinnai,Takahiro, AU - Matsumoto,Yoshihito, AU - Kawai,Nobuyuki, AU - Kunishio,Katsuzo, AU - Tamiya,Takashi, AU - Nagao,Seigo, PY - 2005/05/02/received PY - 2005/06/27/accepted PY - 2006/4/25/pubmed PY - 2006/5/26/medline PY - 2006/4/25/entrez SP - 497-502; discussion 502 JF - Surgical neurology JO - Surg Neurol VL - 65 IS - 5 N2 - BACKGROUND: Chiari I malformation, accompanied by superposed bony anomaly of the craniovertebral junction, is comparatively rare. We report a case of Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia. CASE DESCRIPTION: The patient was a 61-year-old woman demonstrating numbness of the extremities, sensory impairment, muscular weakness, and tendon hyper-reflexia. X-ray images and CT scans demonstrated assimilation of the atlas to the occipital bone, C2 and C3 fusion, abnormal passage of the vertebral arteries, and an anomalous bony mass on the right lateral mass of the atlas protruding into the spinal column. The odontoid process was also deviated to the left. Magnetic resonance images demonstrated bilateral descent of the cerebellar tonsils and syringomyelia extending from C6 to T8. Computed tomographic scans with the head rotated to the right demonstrated increased narrowing of the vertebral column caused by the right lateral mass of the atlas, and MR images confirmed exaggerated deformation of the spinal cord at the same region. This deformation manifested no neurologic symptoms, and we therefore performed foramen magnum decompression and duraplasty using Gore-Tex (W.L. Gore & Associates, Inc., Flagstaff, AZ). In the early postoperative period, neurologic symptoms improved. CONCLUSION: We believe it is important that a treatment plan for Chiari I malformation accompanied by bony anomaly of the craniovertebral junction be determined based on morphologic investigation of the region supplemented by dynamic imaging-based evaluation of instability, or a careful inspection for atypical passage of the vertebral arteries, a frequent site of complication. SN - 0090-3019 UR - https://www.unboundmedicine.com/medline/citation/16630916/Chiari_I_malformation_accompanied_by_assimilation_of_the_atlas_Klippel_Feil_syndrome_and_syringomyelia:_case_report_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0090-3019(05)00468-4 DB - PRIME DP - Unbound Medicine ER -