Congenital Anomalies of the Cervical Spine

88th SGOS/SGCXN, Wright-Patterson Air Force Base, OH 45433, USA. <>
Neurosurgery clinics of North America (Impact Factor: 1.44). 08/2007; 18(3):463-78. DOI: 10.1016/
Source: PubMed


There are numerous congenital anomalies of the cervical spine. They can be simple and clinically inconsequential to complex with serious neurologic and structural implications. They can occur in isolation or as one of several maldeveloped organs in the patients. Many are discovered incidentally. The more common anomalies seen by pediatric spine surgeons include defects of the anterior or posterior arches of C1, occipital assimilation of the atlas, basilar invagination or impression, os odontoideum, and Klippel-Feil syndrome. Management begins with a detailed history, physical examination, and imaging studies. In general, those lesions that are causing or have caused neurologic injury, chronic pain, or spinal deformity or place the patient at high risk for developing these require treatment.

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    • "Klippel-Feil syndrome is a congenital disorder highlighted by vertebral column and skeletal anomalies, deafness, genitourinary and cardiovascular abnormalities.10 Short neck, autofused spinal segments, neurovascular variability and atlanto-occipital instability are only a few examples of why cervical spine disease represents a surgical challenge in patients with KFS.11–13 Various classification systems have been proposed.14–15 According to Samartzis14 classification, our patient would fit into type II category, which is characterised by non-contiguous fused segments and intermediate symptoms severity. "
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    ABSTRACT: Cervical arthroplasty may be justified in patients with Klippel-Feil syndrome (KFS) in order to preserve cervical motion. The aim of this paper is to report an arthroplasty failure in a patient with KFS. A 36-year-old woman with KFS underwent two-level arthroplasty for adjacent segment disc degeneration. Anterior migration of the cranial prosthesis was encountered 5 months postoperatively and was successfully revised with anterior cervical fusion. Cervical arthroplasty in an extensively stiff and fused neck is challenging and may lead to catastrophic failure. Although motion preservation is desirable in KFS, the special biomechanical features may hinder arthroplasty. Fusion or hybrid constructs may represent more reasonable options, especially when multiple fused segments are present.
    No preview · Article · Mar 2011 · Indian Journal of Orthopaedics
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    ABSTRACT: Purpose To evaluate the effect of a vertical expandable prosthetic titanium rib (VEPTR) on head tilt in patients with congenital scoliosis. Methods Twelve Patients with fused ribs, congenital scoliosis (CS), thoracic insufficiency syndrome (TIS), and cervical tilt were treated with VEPTR open wedge thoracostomy. The Cobb angles of the primary thoracic curves were measured. Preoperative measurements of cervical tilt angle, first thoracic (T1) oblique take-off angle, head decompensation, and shoulder horizontal angle were all measured. All the patients were treated with VEPTR open wedge thoracostomy. Result There was statistically significant improvement in thoracic Cobb angle. At the end of follow-up there was significant improvement in the shoulder horizontal angle from 14.4° to 6.7°. Head decompensation had improved to ≤2 cm in nine patients. Both thoracic oblique take-off angle and cervical tilt angle had improved from 35.1° to 33.9° preoperatively to 30° and 27.2° at the end of follow-up. Complications included device migration (one patient) and pedicle screw loosing (one patient). Conclusion VEPTR is able to stabilize head tilt in patients with congenital scoliosis.
    No preview · Article · Jan 2011 · European Journal of Orthopaedic Surgery & Traumatology
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    ABSTRACT: Cervical spine radiographs of 33 patients with Klippel-Feil syndrome were studied for patterns of bony fusion and presence of wasp-waist sign. Five patients were found to have the classic features of massive cervical fusion. Two patients with two adjacent levels of bony fusion showed a wasp-waist sign. Fusion at one level of the cervical spine accounted for 26 remaining cases. The wasp-waist sign was observed in 14 of the cases in which there was complete vertebral interbody fusion, making this finding a valuable radiologic sign. Partial anterior or posterior vertebral interbody fusion, or isolated fusion of the neural arch, however, may or may not be associated with the wasp-waist sign. Klippel-Feil syndrome, easy to recognize when presenting with classic features or when associated with the wasp-waist sign, may be confused with a variety of other entities.
    No preview · Article · Nov 1993 · Skeletal Radiology
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