Syringomyelia and vertebral osteochondromas in patients with multiple hereditary exostosis

Area of Science:

• Orthopedics
• Neurosurgery
• Radiology

Background:

• Multiple hereditary exostosis (MHE) is a genetic disorder characterized by the development of osteochondromas.
• Spinal canal involvement by osteochondromas in MHE patients is a known complication, but its prevalence is not well-established.
• Previous studies reported higher incidences of spinal osteochondromas in MHE patients.

Purpose of the Study:

• To determine the prevalence of spinal osteochondromas and spinal canal encroachment in MHE patients.
• To investigate the association between spinal osteochondromas and syringomyelia in MHE patients.
• To correlate MRI findings with clinical symptoms in MHE patients with spinal involvement.

Main Methods:

• Retrospective analysis of MRI findings in 27 patients diagnosed with MHE.
• Evaluation for osteochondromas arising from the spinal column and their encroachment into the spinal canal.
• Assessment for incidental findings such as syringomyelia.
• Correlation of imaging findings with patient symptomatology and need for surgical intervention.

Main Results:

• Six patients (23.1%) had osteochondromas originating from the spinal column.
• Three patients (11.5%) exhibited osteochondromas encroaching into the spinal canal.
• Three patients (11.5%) were found to have incidental syringomyelia.
• Of the nine patients with positive spinal findings, only five (55.6%) were symptomatic, and two required surgery.

Conclusions:

• The incidence of spinal osteochondromas in MHE patients may be lower than previously reported.
• A significant association was observed between MHE and incidental syringomyelia, a finding not previously highlighted.
• Clinical correlation is essential, as many spinal osteochondromas in MHE patients may be asymptomatic.