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Recovery After Cervical Decompression Surgery for the Treatment of Crowned Dens Syndrome Causing Progressive Neurological Decline: A Case Report

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Traumatic injury to the spinal cord can be divided into complete and incomplete subtypes, based on complete or partial absence of neurologic function below the level of injury. Incomplete spinal cord injury has been shown to occur following cervical hyperextension or flexion injury in the setting of preexisting cervical spinal stenosis. The broad spectrum of symptoms from incomplete spinal cord injury includes weakness and sensory deficits potentially affecting upper and lower extremities, as well as bowel, bladder, and sexual dysfunction in severe cases. The most common type of incomplete spinal cord injury is central cord syndrome, which is characterized by a more severely impaired motor function in the upper than the lower extremities [1, 8, 10, 13, 16]. Progressive neurological deficit has also been reported to occur due to C1–C2 pannus formation in the setting of rheumatic disease [4, 12, 17, 19, 20].

Crowned dens syndrome (CDS) is a rare but known cause of decreased cervical range of motion and severe axial neck pain, in the setting of calcium deposition around the odontoid, for which medical treatment with nonsteroidal anti-inflammatory drugs and/or steroids has been reported [2, 7]. Patients with CDS may present with symptoms of cervical myelopathy caused by a mass containing calcium pyrophosphate dehydrate (CPPD) at the atlantoaxial articulation and/or in the transverse ligament and/or in the ligamentum flavum [3, 9, 11, 14, 15]. According to the findings of Goto et al. evaluating 2,023 patients with neck pain as the chief complaint, 40 patients were identified with CDS, indicating a prevalence of 2.0% [7].

Chang et al. reported an overall prevalence of radiographically evident atlantoaxial CPPD deposition of 12.5%, which was increasing with age [5]. However, severe and gradually progressive neurological deterioration due to an isolated large mass consisting of calcium pyrophosphate at the C1–C2 level causing functional quadriplegia is rare, and, therefore, the optimal treatment remains to be determined. Surgical decompression and stabilization may alleviate the compression on the cervical spinal cord [11], yet the potential for neurological recovery remains to be further elucidated. We report the case of an 87-year-old male diagnosed with quadriplegia resulting from CDS who made an excellent recovery following surgical decompression. .

This article appears in HSS Journal: Volume 10, Issue 1.
View the full article at springerlink.com.

About the HSS Journal

HSS Journal, an academic peer-reviewed journal published three times a year, February, July and October. The Journal accepts and publishes peer reviewed articles from around the world that contribute to the advancement of the knowledge of musculoskeletal diseases and disorders.


Alexander Aichmair, MD

Hospital for Special Surgery

Headshot of Richard J. Herzog, MD, FACR
Richard J. Herzog, MD, FACR
Director, Spinal Imaging, Chief, Division of Teleradiology, Hospital for Special Surgery
Attending Radiologist, Hospital for Special Surgery
Headshot of Giorgio Perino, MD
Giorgio Perino, MD

Assistant Attending Pathologist, Hospital for Special Surgery
Assistant Professor of Pathology, Weill Cornell Medical College

Headshot of Darren R. Lebl, MD, MBA
Darren R. Lebl, MD, MBA
Associate Attending Orthopedic Surgeon, Hospital for Special Surgery
Assistant Scientist, Research Division, Hospital for Special Surgery

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