Congenital Osseous Anomalies of the Upper Cervical Spine

Hosalkar, Harish S.; Sankar, Wudbhav N.; Wills, Brian P. D.; Goebel, Jennifer; Dormans, John P.; Drummond, Denis S.
February 2008
Journal of Bone & Joint Surgery, American Volume;Feb2008, Vol. 90-A Issue 2, p337
Academic Journal
Background: The developmental anatomy and biomechanics of the upper cervical spine are unique in children. Congenital osseous anomalies in this region may be associated with an increased risk for subsequent neurological compromise from instability and/or spinal cord encroachment. We performed a double-cohort study evaluating congenital osseous anomalies of the upper cervical spine in children who presented with one or more clinical problems, and we attempted to outline the risk of possible neurological compromise. Methods: We reviewed the medical records and imaging studies of all children seen and treated for osseous anomalies of the upper cervical spine at our institution between 1988 and 2003. Patients were divided into two cohorts on the basis of the presence or absence of associated syndromes. Parameters reviewed included demographic data, clinical presentation, and imaging features. All anomalies involving the central nervous system, the occipitocervical junction, and the upper cervical osseous canal were included. Complicating sequelae such as canal stenosis, segmental instability, and other anomalies of the central nervous system and spine were identified. Results: Sixty-eight consecutive children were identified. Twenty-one patients had an underlying described syndrome. There were 234 osseous anomalies (average, 3.4 per patient). Three or more anomalies were noted in 79% of the patients. There was no significant difference in the mean number of anomalies (p = 0.80) or in the frequency of any specific anomaly (p > 0.20 for all) between syndromic and nonsyndromic patients. The variety of clinical presentations included neck pain (twenty-six patients), neurological changes (twenty-one patients), and torticollis and/or stiffness (twenty-one patients). Twenty-three patients had more than one complaint. Six patients had isolated spinal instability, twenty-eight had isolated spinal cord encroachment, and six had a combination of both. Forty-four (65%) of the sixty-eight patients underwent surgical decompression and/or arthrodesis principally focused from the foramen magnum to the second cervical vertebra. Conclusions: As a result of these findings, we recommend a thorough evaluation and advanced imaging of the upper cervical spine in all children who present with symptoms related to the upper cervical spine, to identify associated anomalies and further define the nature of canal encroachment including any potential for neurologic compromise. Level of Evidence: Prognostic Level III. See Instructions to Authors for a complete description of levels of evidence.


Related Articles

  • Duplicated Pituitary Gland and Odontoid Process. Usta, Y.; Sakha, F.; White, W. L.; Little, A. S.; Knecht, L. // Neuroradiology Journal;Jun2012, Vol. 25 Issue 3, p360 

    The development of the pituitary gland is not well understood, but duplication of the gland, a rare embryonic anomaly, may shed some light on the process. Since 1880 only about 40 cases have been described. A 56-year-old woman complained of chronic bilateral upper extremity paresthesia and...

  • Midline Cervical Cleft: A Rare Congenital Anomaly. Renukasvvamy, Gayathri Mandya; Soma, Marlene A.; Hartley, Benjamin E. J. // Annals of Otology, Rhinology & Laryngology;Nov2009, Vol. 118 Issue 11, p786 

    Objectives: A midline cervical cleft (MCC) is a rare congenital anomaly due to failure of fusion of the first and second branchial arches during embryogenesis. It may present as a midline defect of the anterior neck skin with a skin projection or sinus, or as a subcutaneous fibrous cord. This...

  • Atlanto-axial segmentation defects and os odontoideum in two male siblings with opsismodysplasia. Farid Chehida; Maher Ghachem; Franz Grill; Klaus Klaushofer // Skeletal Radiology;Mar2009, Vol. 38 Issue 3, p293 

    Abstract  We report two siblings aged 11 and 7 years, respectively, who presented with the clinical and radiographic features of opsismodysplasia (non-lethal type). 3D computed tomography scans of the craniocervical region revealed a split atlas and os odontoideum in both siblings. To...

  • Varied clinical presentation of os odontoideum: a case report. Chrobak, Karen; Larson, Ryan; Stern, Paula J. // Journal of the Canadian Chiropractic Association;Sep2014, Vol. 58 Issue 3, p268 

    Objective: To present a case of an os odontoideum and to provide insight into the varied clinical presentations. Clinical Features: A 54 year old man presented with chronic neck pain without headache. A clinical examination was performed and the chiropractor viewed his AP and lateral...

  • A giant hypertrophy of C5 spinous process in Klippel-Feil syndrome. METE, Mesut; ÜNSAL, Ülkün Ünlü; DURANSOY, Yusuf Kurtuluş; UMUR, Ahmet Şükrü // Journal of Neurological Sciences;2015, Vol. 32 Issue 3, p575 

    Congenital cervical spinal anomalies are relatively common and can be seen in upper and lower cervical regions. However, hypertrophy of the lamina and spinous process of cervical vertebra is extremely rare. Here we reported an 11-year-old girl with unilateral hypertrophy of the lamina and...

  • cervical rib. Peters, Michael // BMA A-Z Family Medical Encyclopedia;2004, p157 

    An encyclopedia entry for "cervical rib" is presented. The condition refers to a congenital abnormality in which the lowest of the seven cervical vertebrae has overdeveloped to form an additional rib located parallel to and above the first normal rib. A person with cervical rib may experience...

  • The Epidemiological, Morphological, and Clinical Aspects of the Cervical Ribs in Humans. Spadliński, Łukasz; Cecot, Tomasz; Majos, Agata; Stefańczyk, Ludomir; Pietruszewska, Wioletta; Wysiadecki, Grzegorz; Topol, Mirosław; Polguj, Michał // BioMed Research International;11/15/2016, Vol. 2016, p1 

    A familiarity with the anatomy of some types of bone anomalies is necessary for clinicians involved in many medical areas. The aim of this paper is to review the newest literature concerning the morphology, embryology, clinical image, and therapeutic methods of the cervical ribs in the humans....

  • Cervical Spine Anomaly. ANGWAFO, NIMAE N.; SHORT, MATTHEW W. // American Family Physician;6/15/2009, Vol. 79 Issue 12, p1101 

    A quiz about the clinical diagnosis of a 32-year-old male patient who presented for an evaluation of his cervical spine to determine if he had congenital anomaly similar to what had been diagnosed in his 3-year-old daughter is presented.

  • Pancreatic divisum and intestinal nonrotation diagnosed with magnetic resonance imaging: Case... Bloom; Hickey, Carl // Canadian Association of Radiologists Journal;Oct99, Vol. 50 Issue 5, p310 

    Presents the findings of a case report involving a patient suffering from congenital anomalies. Potential causes of pancreatic divisum and intestinal nonrotation; Efficiency of magnetic resonance imaging in diagnosing congenital anomalies.


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics