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Orthotopic os odontoideum

MedGen UID:: 1390495
•Concept ID:: C4476740
•: Anatomical Abnormality

HPO:	HP:0025375

Definition

Os odontoideum is classified into two anatomic types (orthotopic and dystopic). Os odontoideum is defined as an ossicle that consists of smooth and separate caudal portions of the odontoid process.With dystopic os odontoideum, the ossicle is located near the basion or is fused with the clivus. [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVOrthotopic os odontoideum

Abnormality of the skeletal system
- Abnormal skeletal morphology
  - Abnormal axial skeleton morphology
    - Abnormality of the vertebral column
      - Abnormal odontoid process morphology
        Os odontoideum
        Orthotopic os odontoideum

Recent clinical studies

Diagnosis

Fatal cervical myelopathy in a child with glutaric aciduria type 1.

Chauvet E, Ribeiro D, Kern I, Fluss J
J Inherit Metab Dis 2024 Mar;47(2):217-219. Epub 2024 Feb 7 doi: 10.1002/jimd.12716. PMID: 38326670

A delayed diagnosis of an unstable os odontoideum.

Surov A, Hess S, Spielmann RP, Kunze C
Eur J Med Res 2008 Mar 31;13(3):136-8. PMID: 18499561

Atlantoaxial instability associated with an orthotopic os odontoideum: a multimodality imaging assessment.

Vargas TM, Rybicki FJ, Ledbetter SM, MacKenzie JD
Emerg Radiol 2005 Jun;11(4):223-5. doi: 10.1007/s10140-005-0406-3. PMID: 16133608

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