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The Incidence of Vertebral Exostoses in Multiple Hereditary Exostoses and Recommendations for Spinal Screening
Austin E Wininger1, Barkha N Chhabra, Richard E Haigler
1Shriners Hospitals for Children, Houston, TX.
Background:
Multiple hereditary exostoses (MHE) lead to the development of pedunculated or sessile osteocartilaginous lesions. Vertebral involvement occurs in MHE and encroaching intracanal exostoses can result in devastating consequences. Magnetic resonance imaging (MRI) of the entire spine has been used to screen for vertebral exostoses to detect high-risk patients. The primary purpose of this investigation is to determine the incidence of vertebral and encroaching intracanal exostoses in patients with MHE. A secondary purpose is to determine if pelvis and rib exostoses serve as "harbinger" lesions of vertebral involvement in MHE.
Methods:
A retrospective chart review was performed on 39 patients (21 male and 18 female individuals) with MHE who underwent routine spinal screening with noncontrast entire spine MRI. The average age at screening was 12.3 years (range, 3 to 17 y). Screening was ordered consecutively on patients seen during the study period who were between ages 8 and 18 years or had complaints that could be related to encroaching intracanal exostoses.
Results:
The incidence of vertebral exostoses in this cohort of 39 patients with MHE was 28% (11 total). An encroaching intracanal exostosis was seen in 3 patients (2 cervical, 1 thoracic). Nonencroaching vertebral exostoses were discovered in 8 patients. Sufficient pelvis and rib imaging to determine the presence of pelvis and rib exostoses was available in 8 of those with vertebral exostoses and 19 of those with no vertebral exostoses on screening MRI. In this cohort, the sensitivity and specificity of the presence of both pelvis and rib exostoses for determining the presence of spinal involvement in MHE are 88% and 5%, respectively.
Conclusions:
Based on the results of this cohort, vertebral exostoses are common in MHE, and screening MRI of the entire spine can be used to determine which patients need close observation. If a more selective screening protocol is utilized, an entire spine MRI could be obtained for patients who desire increased physical activity levels or for patients with both pelvis and rib exostoses. At a minimum, treating physicians should monitor patients with MHE closely for neurological symptoms and have a low threshold to obtain advanced spinal imaging.
Level Of Evidence:
Level III-diagnostic.
Insights
Vertebral exostoses are common in multiple hereditary exostoses (MHE). Entire spine MRI screening identifies patients needing close observation for MHE-related spinal issues.
Area of Science:
- Orthopedics
- Radiology
- Genetics
Background:
- Multiple hereditary exostoses (MHE) can cause osteocartilaginous lesions, including vertebral involvement.
- Intracanal exostoses in MHE can lead to severe neurological complications.
- Whole-spine MRI is used to screen for vertebral exostoses in high-risk MHE patients.
Purpose of the Study:
- Determine the incidence of vertebral and encroaching intracanal exostoses in MHE patients.
- Investigate if pelvis and rib exostoses predict vertebral involvement in MHE.
Main Methods:
- Retrospective chart review of 39 MHE patients (ages 3-17) who underwent non-contrast entire spine MRI.
- Screening included patients aged 8-18 or those with symptoms potentially related to spinal exostoses.
Main Results:
- 28% of MHE patients had vertebral exostoses (11 total).
- 3 patients had encroaching intracanal exostoses (2 cervical, 1 thoracic).
- Pelvis and rib exostoses had 88% sensitivity but only 5% specificity for spinal involvement.
Conclusions:
- Vertebral exostoses are frequent in MHE, justifying entire spine MRI screening.
- Selective MRI screening may be suitable for active patients or those with both pelvis and rib exostoses.
- Close monitoring for neurological symptoms and prompt advanced spinal imaging are crucial for MHE patients.
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