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Published on: September 16, 2022
[Spinal cord compression by a cervical oseteocartilaginous exostosis: surgical strategy aspects]
1Neurochirurgische Klinik und Institut für Neuropathologie (BS), Universitätsklinik Aachen. vrohde@ukaachen.de
Insights
A unique case of spinal cord compression in a boy with hereditary multiple exostosis (HME) was successfully managed. A novel surgical approach involving lateral hemilaminectomy at C2 led to spontaneous exostosis extrusion and neurological recovery.
Area of Science:
- Neurosurgery
- Orthopedic Surgery
- Genetics
Background:
- Hereditary multiple exostosis (HME) is a genetic disorder characterized by the formation of multiple bone tumors.
- Spinal cord compression is a rare but serious complication of HME, particularly when occurring at the cervical level.
- Delayed diagnosis of spinal cord compression can lead to significant neurological deficits.
Observation:
- A 12-year-old boy with HME presented with symptoms suggestive of spinal cord compression due to a C2 lamina exostosis.
- A pre-existing perinatal brain lesion causing tetraparesis complicated the initial assessment and diagnosis of spinal cord compression.
- The exostosis exhibited an unusual, extensive spur-like growth pattern.
Findings:
- Surgical management involved a modified laminectomy with initial lateral cutting of the C2 hemilaminae.
- Intraoperative electrophysiological monitoring was crucial for preventing spinal cord injury during patient positioning.
- Lateral cutting facilitated spontaneous extrusion of the C2 exostosis, leading to immediate improvement in electrophysiological findings and prompt neurological recovery.
Implications:
- This case highlights a unique growth pattern of exostosis in HME.
- The modified surgical technique, including lateral hemilaminectomy and electrophysiological monitoring, proved effective in managing cervical spinal cord compression.
- The successful outcome underscores the importance of vigilant monitoring and tailored surgical strategies in complex HME cases.
Aim:
The authors present the therapeutic management of a 12-year-old boy with known hereditary multiple exostosis syndrome (HME), who developed spinal cord compression symptoms caused by an exostosis of the C2 lamina. A perinatal brain lesion with tetraparesis delayed the recognition of the spinal cord compression substantially, which resulted in an extensive spur-like growth of the exostosis.
Method:
In comparison with already published cases, this growth pattern was rather unique and required consideration on the best surgical management. We decided to monitor the spinal cord function from positioning of the patient to skin closure and to modify the surgical steps of the laminectomy with initial lateral cutting of both hemilaminae.
Results:
Electrophysiological monitoring helped to avoid spinal cord compression by inadequate head anteflexion during positioning. Lateral cutting of the hemilaminae C2 resulted in spontaneous extrusion of the exostosis with immediate improvement of the electrophysiological findings. The boy experienced a prompt improvement of his neurological deficits.
Conclusion:
The good surgical and clinical result confirm the value of the applied management concept.