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Neuropediatrics 1988; 19(2): 105-108
DOI: 10.1055/s-2008-1052411
Case report

© Georg Thieme Verlag KG Stuttgart · New York

Surgical Correction of Cervical Hyperextension in Rigid Spine Syndrome

S.  Giannini1 , F.  Ceccarelli1 , C.  Granata2 , T.  Capelli3 , L.  Merlini3
  • 1Department of Orthopaedic Surgery, Istituto Ortopedico Rizzoli, University of Bologna, via G. C. Pupilli, 1, I-40136 Bologna, Italy
  • 2Rehabilitation Unit, Istituto Ortopedico Rizzoli, University of Bologna, via G. C. Pupilli, 1, I-40136 Bologna, Italy
  • 3Neuropathology, Istituto Ortopedico Rizzoli, University of Bologna, via G. C. Pupilli, 1, I-40136 Bologna, Italy
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Publication History

Publication Date:
19 May 2008 (online)

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Abstract

The rigid spine syndrome is a condition characterized by a mild axial and proximal myopathy in which the fibrous shortening of spinal extensor muscles causes a marked limitation in flexion of the spine. This 10-year-old boy with rigid spine syndrome showed a progressive limitation in flexion of the cervical spine with fixed hyperextension of the neck. To look forward he had to bend forward his trunk flexing hips and knees. An X-ray study demonstrated a progressive limitation in flexion but also in extension of the cervical spine. A particular radiologic aspect named "Alligator sign" was demonstrated.

The hyperextension of the neck was corrected surgically by opening the interspinous spaces from C2 to C7 through a capsulotomy. The correction was stabilized with bone grafts fixed to the spinous processes.

This boy with the straightened cervical spine was then able to look forward without any compensatory posture.

Key words

Rigid spine syndrome - Surgery - Cervical spine