Klippel-Feil Syndrome With Cervical Cord Compression Resulting From Cervical Subluxation in Association With an Omo-Vertebral Bone

Southwell, Richard B.; Reynolds, Arden F.; Badger, Virginia M.; Sherman, Frederick C.

doi:10.1097/00007632-198009000-00014

Cited by 18 publications

(6 citation statements)

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“…KFS represents a relatively rare kind of congenital malformation of the cervical spine where appropriate segmentation of vertebrae during the second to eighth week of gestational development is interrupted. 1 4 13 14 Sprengel's deformity and the presence of omovertebral bone may be accompanied associations. 14 In addition to this, several reports have described KFS along with other congenital pathology affecting visceral, musculoskeletal, otolaryngologic, and neurologic systems.…”

Section: Discussionmentioning

confidence: 99%

Prevalence of Klippel-Feil Syndrome in a Surgical Series of Patients with Cervical Spondylotic Myelopathy: Analysis of the Prospective, Multicenter AOSpine North America Study

Nouri

Tetreault

Zamorano

et al. 2015

Global Spine Journal

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Study Design Prospective study. Objective To evaluate the prevalence of Klippel-Feil syndrome (KFS) in a prospective data set of patients undergoing surgical treatment for cervical spondylotic myelopathy (CSM) and to evaluate if magnetic resonance imaging (MRI) features in patients with KFS are more pronounced than those of non-KFS patients with CSM. Methods A retrospective analysis of baseline MRI data from the AOSpine prospective and multicenter CSM-North American study was conducted. All the patients presented with at least one clinical sign of myelopathy and underwent decompression surgery. The MRIs and radiographs were reviewed by three investigators. The clinical and imaging findings were compared with patients without KFS but with CSM. Results Imaging analysis discovered 5 of 131 patients with CSM (∼3.82%) had single-level congenital fusion of the cervical spine. The site of fusion differed for all the patients. One patient underwent posterior surgery and four patients received anterior surgery. Postoperative follow-up was available for four of the five patients with KFS and indicated stable or improved functional status. All five patients demonstrated pathologic changes of adjacent segments and hyperintensity signal changes in the spinal cord on T2-weighted MRI. Multiple MRI features, most notably maximum canal compromise (p = 0.05) and T2 signal hyperintensity area (p = 0.05), were worse in patients with CSM and KFS. Conclusions The high prevalence of KFS in our surgical series of patients with CSM may serve as an indication that these patients are prone to increased biomechanical use of segments adjacent to fused vertebra. This supposition is supported by a tendency of patients with KFS to present with more extensive MRI evidence of degeneration than non-KFS patients with CSM.

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Section: Discussionmentioning

confidence: 99%

Prevalence of Klippel-Feil Syndrome in a Surgical Series of Patients with Cervical Spondylotic Myelopathy: Analysis of the Prospective, Multicenter AOSpine North America Study

Nouri

Tetreault

Zamorano

et al. 2015

Global Spine Journal

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“…A number of hypotheses exist, and the most commonly accepted postulates that degenerative changes within the cord are caused by hypermobility of the cervical spine at the interspace levels adjacent to the fused vertebrae leading to mechanical abnormality [1,5,6]. The instability created by the fused vertebrae is believed to produce degenerative change and symptoms by two methods.…”

Section: Discussionmentioning

confidence: 99%

“…Originally described by Klippel and Feil in 1912, Klippel-Feil syndrome is characterized by congenital fusion of the cervical spine secondary to failure of segmentation of the cervical vertebrae in the 3rd to 8th week of life in utero, affecting 1:42,000 live births [4,5].…”

Section: Introductionmentioning

confidence: 99%

“…The Klippel-Feil syndrome may also be associated with multiple other organ developmental defects, including the ear, with deafness noted in 30%, congenital heart defects, and ventriculo-septal defects in particular, genitourinary tract abnormalities, especially unilateral renal agenesis, and musculoskeletal disorders, with a 60% preponderance of scoliosis in those affected [4,5,6]. Both symptomatic and asymptomatic primary or secondary neurological disorders may occur, and are well documented in the adult population [1,3].…”

Section: Introductionmentioning

confidence: 99%

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Cervical disc prolapse in childhood associated with Klippel-Feil syndrome

Allsopp

Griffiths

Sgouros³

2001

Child's Nervous System

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Klippel-Feil syndrome is characterized by a variable degree of congenital fusion of the cervical spine. It can exist in association with other mesodermal deformities affecting several systems. Symptomatic cervical disc prolapse in the context of the syndrome is well documented in young adults. We present a case of a 5-year-old girl with the syndrome, who presented with mild motor developmental delay and cervical cord compression from a prolapsed C3/4 intervertebral disc, seen on MR scan. She also had posterior elements fused from C-2 to C-4. She improved after cervical discectomy. The presence of degenerative disc disease at such a young age and at a level mechanically protected by posterior element fusion indicates a congenital primary defect, rather than mechanical stress fatigue.

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“…The syndrome is believed to result from faulty segmentation along the developing axis of the embryo during the 3rd to 8th gestational weeks [12]. In this condition, hypermobility and degenerative spondylosis at the level adjacent to the fused segments may result in mechanical pressure on the spinal cord.…”

Section: Introductionmentioning

confidence: 99%

Cervical arthroplasty in a patient with Klippel-Feil syndrome

Yi¹,

Kim

Shin

et al. 2007

Acta Neurochir (Wien)

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SummaryThis is the first published report of a patient with KlippelFeil syndrome treated with cervical arthroplasty. A 36-year-old man presented with posterior neck pain and myelopathic symptoms. A radiograph demonstrated congenital fusion of the vertebral bodies at C2-3, C4-5 and C5-6. On MRI, the spinal cord was compressed by a protruding cervical disc and bony spurs at C6-7. After anterior discectomy and decompression of the spinal cord at the C6-7 level, the disc was replaced with the Bryan cervical disc system (Medtronic Sofamor Danek, Memphis, TN, USA) to restore normal motion. The absence of adjacent segment degeneration and the preservation of cervical motion were noted 2 years after surgery. Arthroplasty may be performed in selected patients with Klippel-Feil syndrome in order to restore motion and to prevent degeneration of the adjacent segment by reducing hypermobility.

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Klippel-Feil Syndrome With Cervical Cord Compression Resulting From Cervical Subluxation in Association With an Omo-Vertebral Bone

Cited by 18 publications

References 0 publications

Prevalence of Klippel-Feil Syndrome in a Surgical Series of Patients with Cervical Spondylotic Myelopathy: Analysis of the Prospective, Multicenter AOSpine North America Study

Prevalence of Klippel-Feil Syndrome in a Surgical Series of Patients with Cervical Spondylotic Myelopathy: Analysis of the Prospective, Multicenter AOSpine North America Study

Cervical disc prolapse in childhood associated with Klippel-Feil syndrome

Cervical arthroplasty in a patient with Klippel-Feil syndrome

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