Rückenmarkskompression durch eine zervikale osteokartilaginäre Exostose: Operationsstrategische Aspekte

Zusammenfassung

Studienziel: Die Autoren präsentieren das operative Management eines 12-jährigen Jungen, der im Rahmen eines bekannten hereditären multiplen Exostosen-Syndroms (HME) eine symptomatische Rückenmarkskompression durch eine Exostose der Lamina des 2. Halswirbels entwickelt hat. Die lange Verkennung der Rückenmarkskompression infolge einer perinatalen Hirnschädigung mit nachfolgender Tetraparese führte zu einem extensiven Wachstum der Exostose, die sich schließlich spornartig in das Zervikalmark eingegraben hat. Methode: Wegen des ausgeprägten Befundes erfolgte die elektrophysiologische Überwachung der Rückenmarksfunktion ab Patientenlagerung und die Modifikation der operativen Schritte der Laminektomie mit initialer lateraler Durchtrennung der Halbbogen. Ergebnisse: Das elektrophysiologische Monitoring bei Lagerung ließ eine rückenmarkskomprimierende Kopfanteflexion vermeiden. Die Durchtrennung der Halbbogen führte zu einer spontanen Expression des Sporns mit sofortiger Besserung der Potentialbefunde. Korrespondierend kam es zur sofortigen Besserung der neurologischen Defizite. Schlussfolgerung: Das gute operative Ergebnis sowohl in bildgebender als auch in klinischer Hinsicht lassen die Wertigkeit des Managementkonzepts erkennen.

Abstract

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.

Schlüsselwörter

Exostose - hereditäres multiples Exostose-Syndrom - Osteochondrom - diaphyseale Aklasie - Halswirbelsäule

Key words

Exostosis - hereditary multiple exostosis syndrome - osteochondroma - diaphyseal aclasis - cervical spine

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