The cervical spine in the Klippel-Feil syndrome

Baba, H.; Maezawa, Yasuhisa; Furusawa, Nobuaki; Chen, Q.; Imura, Satoru; Tomita, Katsuro

doi:10.1007/bf00185222

Cited by 54 publications

(34 citation statements)

References 24 publications

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“…Various spinal and extraspinal anomalies have been associated with KFS, but vary between individuals [10,23,25,36,37,44,46,53]. Moreover, up to 68% of KFS patients, primarily noted in adulthood, report symptoms related to their syndrome [2,20,43,44,46]. However, the development of SOM is a tremendous concern for the KFS patient, which left unnoticed could potentially complicate the complex course and management associated with this syndrome.…”

Section: Introductionmentioning

confidence: 99%

Superior odontoid migration in the Klippel–Feil patient

Samartzis

Kalluri²,

Herman

et al. 2006

Eur Spine J

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Klippel-Feil syndrome (KFS) is an uncommon condition noted primarily as congenital fusion of two or more cervical vertebrae. Superior odontoid migration (SOM) has been noted in various skeletal deformities and entails an upward/vertical migration of the odontoid process into the foramen magnum with depression of the cranium. Excessive SOM could potentially threaten neurologic integrity. Risk factors associated with the amount of SOM in the KFS patient are based on conjecture and have not been addressed in the literature. Therefore, this study evaluated the presence and extent of SOM and the various risk factors and clinical manifestations associated therein in patients with KFS. Twenty-seven KFS patients with no prior history of surgical intervention of the cervical spine were included for a prospective radiographic and retrospective clinical review. Radiographically, McGregor's line was utilized to evaluate the degree of SOM. Anterior and posterior atlantodens intervals (AADI/PADI), number of fused segments (C1-T1), presence of occipitalization, classification-type, and lateral and coronal cervical alignments were also evaluated. Clinically, patient demographics and presence of cervical symptoms were assessed. Radiographic and clinical evaluations were conducted by two independent blinded observers. There were 8 males and 19 females with a mean age of 13.5 years at the time of radiographic and clinical assessment. An overall mean SOM of 5.0 mm (range = -1.0 to 19.0 mm) was noted. C2-C3 (74.1%) was the most commonly fused segment. A statistically significant difference was not found between the amount of SOM to age, sex-type, classification-type, AADI, PADI, and lateral cervical alignment (P > 0.05). A statistically significant greater amount of SOM was found as the number of fused segments increased (r = 0.589; P = 0.001) and if such levels included occipitalization (r = 0.616; P = 0.001). A statistically significant greater amount of SOM was also found with an increase in coronal cervical alignment (r = 0.413; P = 0.036). Linear regression modeling further supported these findings as the strongest predictive variables contributing to an increase in SOM. A 7.20 crude relative risk (RR) ratio [95% confidence interval (CI) = 1.05-49.18; risk differences (RD) = 0.52] was noted in contributing to a SOM greater than 4.5 mm if four or more segments were fused. Adjusting for coronal cervical alignment greater than 10°, five or more fused segments were found to significantly increase the RR of a SOM greater than 4.5 mm (RR = 4.54; 95% CI = 1.07-19.50; RD = 0.48). The RR of a SOM greater than 4.5 mm was more pronounced in females -006-0280-z (RR = 1.68; 95% CI = 0.45-6.25; RD = 0.17) than in males. Eight patients (29.6%) were symptomatic, of which symptoms in two of these patients stemmed from a traumatic event. However, a statistically significant difference was not found between the presence of symptoms to the amount of SOM and other exploratory variables (P > 0.05). A mean SOM of 5.0 mm was found in our ...

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Section: Introductionmentioning

confidence: 99%

Superior odontoid migration in the Klippel–Feil patient

Samartzis

Kalluri²,

Herman

et al. 2006

Eur Spine J

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“…Those with hyper mobility of the upper cervical segment are at risk of developing neurologic impairment. Affected individuals with hyper mobility of the lower cervical segment are at increased risk for degenerative disk diseases and should be treated symptomatically (15,16).…”

Section: Discussionmentioning

confidence: 99%

Klippel - Feil Syndrome Associated with Atrial Septal Defect

Bejiqi

Retkoceri

Bejiqi

et al. 2013

Med Arh

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“…1) Hypermobility of the cervical spine at the adjacent non-fused segments in Klippel-Feil syndrome would lead to excessive stress and the development of cervical disc degeneration. 2,11,22) Thus, the presence of either congenital or acquired cervical fusion may lead to the development of adjacent disc degeneration in the young. A 24-year-old man with congenital insensitivity to pain developed a herniated cervical disc at the C6-7 intervertebral level.…”

Section: H Ikeda Et Almentioning

confidence: 99%

Nontraumatic Cervical Disc Herniation in a 21-Year-Old Patient With No Other Underlying Disease

Ikeda

Hanakita

Takahashi

et al. 2012

Neurol. Med. Chir.(Tokyo)

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A 21-year-old woman presented with cervical myelopathy due to nontraumatic cervical disc herniation associated with cervical canal stenosis. The patient underwent removal of the herniated disc and anterior fusion with an autogenous iliac crest bone graft. After surgery, the patient showed satisfactory improvement. Cases of cervical disc herniation in our center and reported cases without cervical trauma in either young adults or in childhood were reviewed retrospectively. We discuss the pathogenesis of cervical disc herniation in our young patient in the context of these other cases. Cervical disc herniation rarely occurs before the age of 30 years. A history of cervical trauma and preexisting fusion of the cervical spine are risk factors for cervical disc herniation. The present case is the youngest known of nontraumatic disc herniation without other underlying disease. Hypermobility due to neck cracking and a relatively narrow spinal canal might have been important in causing cervical myelopathy by disc herniation.

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The cervical spine in the Klippel-Feil syndrome

Cited by 54 publications

References 24 publications

Superior odontoid migration in the Klippel–Feil patient

Superior odontoid migration in the Klippel–Feil patient

Klippel - Feil Syndrome Associated with Atrial Septal Defect

Nontraumatic Cervical Disc Herniation in a 21-Year-Old Patient With No Other Underlying Disease

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