Involvement of the Spine in Patients with Multiple Hereditary Exostoses

http://www.ejbjs.org/cgi/content/abstract/91/8/1942

The Journal of Bone and Joint Surgery (American). 2009;91:1942-1948. doi:10.2106/JBJS.H.00762© 2009
The Journal of Bone and Joint Surgery, Inc.

Involvement of the Spine in Patients with Multiple Hereditary Exostoses.
James W. Roach, MD1, Joshua W.B. Klatt, MD1 and Nathan D. Faulkner, MD1
1 Salt Lake City Shriners Hospital,
Fairfax Road at Virginia Street,
Salt Lake City,
UT 84103.
E-mail address for J.W. Roach: jameswroach@msn.com


Investigation performed at Salt Lake City Shriners Hospital, Salt Lake City, Utah
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: Extension of a vertebral exostosis into the spinal canal is rare, but many isolated cases have been reported in the literature.
Three existing patients with multiple hereditary exostoses at our institution had development of neurologic findings and were found to have exostoses in the spinal canal.
These findings led us to perform magnetic resonance imaging or computed tomographic scans for the remaining patients with multiple hereditary exostoses at our institution.

Methods: Forty-four patients at our institution (including twenty-six male patients and eighteen female patients) had multiple hereditary exostoses.
Forty-three patients were evaluated with magnetic resonance imaging and one was evaluated with computed tomography to look for spinal column involvement.

Results: Thirty (68%) of the forty-four patients had exostoses arising from the spinal column, and twelve (27%) had lesions encroaching into the spinal canal.
Thirty-six of the forty-four patients also had plain radiographs, but only six had radiographs that accurately identified the lesions and another six had radiographs that mistakenly identified lesions that were not confirmed with magnetic resonance imaging or computed tomography.

Patients with lesions inside the spinal canal were typically asymptomatic and neurologically normal, with radiographs that did not demonstrate the lesion.
Compared with female patients, male patients were more likely to have spinal lesions and more likely to have lesions encroaching into the spinal canal (p = 0.014).

Conclusions: The risk that a patient with multiple hereditary exostoses has a lesion within the spinal canal is much higher than previously suspected (27%).
Because the potential exists for serious neurologic injury to occur, we have begun to use magnetic resonance imaging to screen all patients who have multiple hereditary exostoses at least once during the growing years