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Pediatric Craniovertebral Junction Diseases pp 273–293Cite as

Os Odontoideum Syndrome: Pathogenesis, Clinical Patterns and Indication for Surgical Strategies in Childhood

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Part of the book series: Advances and Technical Standards in Neurosurgery ((NEUROSURGERY,volume 40))

Abstract

Os odontoideum is a rare condition with a controversial pathogenesis and poorly understood natural history. Hypoplasia of the odontoid associated with an independent oval ossicle, with smooth margins widely separated from C2 and well above the superior facets of the axis, is termed “os odontoideum”. The neurological manifestations arise from bulbospinal compression both at rest and during motion, due to the craniovertebral junction (CVJ) instability itself. Consequently, the surgical management of os odontoideum should aim at achieving both neural decompression and stabilization of the CVJ. The aims of this paper are to introduce the embryological steps involved in the CVJ development, to underline the updated theories propounded to interpret developmental and congenital disorders of the os odontoideum, to introduce the most updated surgical techniques and to discuss some exemplary cases selected from our personal experience.

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  1. Department of Head Neck Diseases, Institute of Neurosurgery, Catholic University of Rome, Largo Gemelli, 8, Rome, 0068, Italy

    Massimiliano Visocchi & Concezio Di Rocco

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  1. Massimiliano Visocchi
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  2. Concezio Di Rocco
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Correspondence to Massimiliano Visocchi .

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  1. INI-International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

  2. Department of Neurosurgery, Hacettepe University, Sihhiye, Ankara, Turkey

    Nejat Akalan

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Visocchi, M., Di Rocco, C. (2014). Os Odontoideum Syndrome: Pathogenesis, Clinical Patterns and Indication for Surgical Strategies in Childhood. In: Di Rocco, C., Akalan, N. (eds) Pediatric Craniovertebral Junction Diseases. Advances and Technical Standards in Neurosurgery, vol 40. Springer, Cham. https://doi.org/10.1007/978-3-319-01065-6_9

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