Os odontoideum is a rare entity of the second cervical vertebra, characterized by a circumferentially corticated ossicle separated from the body of C2. The ossicle is a distinct entity from an odontoid fracture or a persistent ossiculum terminale. The diagnosis may be made incidentally on imaging obtained for the workup of neck pain or neurologic signs and symptoms. Diagnosis usually can be made with plain radiographs. MRI and CT can assess spinal cord integrity and C1-C2 instability. The etiology of os odontoideum is a topic of debate, with investigative studies supporting both congenital and traumatic origins. A wide clinical range of symptoms exists. Symptoms may present as nondescript pain or include occipital-cervical pain, myelopathy, or vertebrobasilar ischemia. Asymptomatic cases without evidence of radiologic instability are typically managed with periodic observation and serial imaging. The presence of atlantoaxial instability or neurological dysfunction necessitates surgical intervention with instrumentation and fusion for stability.
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http://dx.doi.org/10.5435/JAAOS-D-18-00637 | DOI Listing |
Clin Neurol Neurosurg
December 2024
Clinic of Radiology, Bahçeşehir University Göztepe Medical Park Hospital, Istanbul, Turkey.
Introduction: Os odontoideum (OO) is a rare anatomic variant of the axis characterized by the separation of a part of the axis. It may cause cervical instability, atlantoaxial dislocation, myelopathy, or permanent paralysis. We present an extremely rare case of an OO with posterior atlantoaxial dislocation in a child.
View Article and Find Full Text PDFChilds Nerv Syst
November 2024
Department of Neurosurgery, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK.
Purpose: Pseudoachondroplasia is a rare skeletal dysplasia caused by a mutation in the COMP gene. Infants with pseudoachondroplasia present with rhizomelic dwarfism. Pseudoachondroplasia can resemble achondroplasia, which also presents with a phenotype of rhizomelic dwarfism.
View Article and Find Full Text PDFJ Pediatr Orthop
November 2024
Department of Orthopaedic Surgery, Nihon University, Tokyo, Japan.
Background: Although 10% to 60% of patients with Down syndrome (DS) develop atlantoaxial instability (AAI), clarifying the course of asymptomatic AAI may prevent unnecessary clinical interactions and investigations. This study investigates the radiographic changes observed in asymptomatic AAI associated with DS in Japanese children as they grow from infancy to adolescence over a minimum of 10 years.
Methods: A retrospective analysis of cervical radiographs acquired from asymptomatic patients with DS in both infancy and adolescence was carried out.
Mol Syndromol
October 2024
Division of Pediatric Intensive Care, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey.
Introduction: Christianson syndrome is a rare neurodevelopmental disorder associated with mutations in the gene located on the chromosome X. It is characterized by intellectual disability, developmental delay, speech and language impairments, dysmorphic features, seizures, ataxia, and neurobehavioral problems.
Case Presentation: A 5-year-old boy was presented with respiratory failure and then progressive muscle weakness in all four extremities.
Adv Tech Stand Neurosurg
September 2024
Department of Neurosurgery, University of Iowa Hospitals and Clinics, Stead Family Children's Hospital, Iowa, USA.
Objective: Database review (1978-2019) is to identify the cause of os odontoideum, its presentation, associated abnormalities, and management recommendations.
Methods And Materials: Review of referral database of 514 patients and 258 surgically treated patients ages 4-64 years. Detailed history of early childhood trauma and initial encounter record retrieval were made.
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