The Journal of Bone and Joint Surgery (American). 2007;89:2732-2736.
doi:10.2106/JBJS.F.01322
© 2007 The Journal of Bone and Joint Surgery, Inc.
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Crowned Dens Syndrome

Shinichi Goto, MD1, Jutaro Umehara, MD1, Toshimi Aizawa, MD2 and Shoichi Kokubun, MD2

1 Department of Orthopaedic Surgery, Senboku Kumiai General Hospital, 1-30 Omagari-torimati, Daisen, Akita 014-0027, Japan. E-mail address for S. Goto: cfq40980{at}par.odn.ne.jp
2 Department of Orthopaedic Surgery, Tohoku University School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980, Japan
Investigation performed at the Department of Orthopaedic Surgery, Senboku Kumiai General Hospital, Akita, and the Department of Orthopaedic Surgery, Tohoku University School of Medicine, Sendai, Japan

Disclosure: The authors did not receive any outside funding or grants in support of their research for or preparation of this work. Neither they nor a member of their immediate families received payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, division, center, clinical practice, or other charitable or nonprofit organization with which the authors, or a member of their immediate families, are affiliated or associated.

Background: Patients with crowned dens syndrome typically present with severe neck pain and have calcium deposits around the odontoid process of the axis on radiographs. To our knowledge, the cases of only thirty-five patients have been reported in the English-language literature and the clinical features remain unclear. The purposes of this study were to examine the clinical features of crowned dens syndrome, determine treatment outcomes, and propose diagnostic criteria.

Methods: Forty patients with severe neck pain had calcium deposition around the odontoid process on computed tomography scans, and they were thus diagnosed as having crowned dens syndrome. Data were collected in relation to these patients, including the date of onset of neck pain, the presence of inflammatory indicators (increased body temperature, C-reactive protein levels, and white blood-cell count), and treatment outcomes.

Results: The male-to-female ratio was 0.6, and two-thirds of the patients were more than seventy years of age. All patients had markedly restricted neck motion, particularly in rotation, and all had one or more positive inflammatory indicators. Calcium deposition was detected in all areas around the odontoid process, but chiefly behind the process. Pain was typically relieved by nonsteroidal anti-inflammatory drugs, prednisolone, or both. A combination of both appeared to be the most effective.

Conclusions: We believe that crowned dens syndrome is more common than previously recognized, especially in elderly patients. It is diagnosed on the basis of acute and severe neck pain; marked restriction of neck motion, particularly in rotation; the presence of inflammatory indicators, such as an elevated C-reactive protein level; calcium deposition around the odontoid process detected by computed tomography; no history of trauma; and the exclusion of other inflammatory diseases and tumors. Prednisolone and nonsteroidal anti-inflammatory drugs in combination are the recommended treatment for symptom relief.

Level of Evidence: Therapeutic Level IV. See Instructions to Authors for a complete description of levels of evidence.


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