Hypoplasia of the atlas causing cervical myelopathy with situs inversus totalis.

STUDY

DESIGN

A case report of myelopathic hypoplasia of the atlas with situs inversus totalis.

OBJECTIVES

To describe a case of cervical myelopathy caused by hypoplasia of the atlas with situs inversus totalis, and to briefly review the pertinent literature.

SETTING

Department of Orthopaedic Surgery, Kagoshima, Japan.

METHODS

The history, results of examination, and findings of radiographic imaging studies for a 56-year-old man with a 10-year history of progressive myelopathy who presented to our hospital are described.

RESULTS

Imaging studies revealed congenital hypoplasia of the atlas, ossification of the posterior longitudinal ligament at the levels of C3-C4, and situs inversus totalis. He underwent laminectomy of the atlas and laminoplasty of C3-C7 for decompression of the spinal cord. Operative intervention resulted in significant neurological improvement and relief of occipital neuralgia.

CONCLUSION

To our knowledge, no case of myelopathic hypoplasia of the atlas with situs inversus totalis has previously been described. When encountering inherited disorders such as situs inversus totalis, a thorough search must be made for anomalies of the craniovertebral junction.