Risk Factors in Klippel-Feil Syndrome

Pizzutillo, Peter D.; Woods, Marion L.; Nicholson, Lisa; Gd, MacEwen

doi:10.1097/00007632-199409150-00020

Cited by 120 publications

(99 citation statements)

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“…Like several previous reports, our study was comprised of mainly females (70.4%) [19,20,23,38,53]. Evaluating the role of sex-type in association with a historically established marker of 4.5 mm or greater considered as symptomatic SOM, our study had noted that females were associated with a 1.68 greater probability than males in developing such a SOM; however, this may be attributed to the higher incidence of the condition with relation to sex-type in the sample or population.…”

Section: Resultssupporting

confidence: 50%

“…Since the presence of congenital scoliosis has been noted to occur in up to 78% of patients with KFS [25,38,53], this study attempted to evaluate the role of the coronal and lateral cervical alignment and its relationship to the amount of SOM. Although the study did not note a statistically significant association between lateral cervical alignment and the amount of SOM, a statistically significant correlation was noted between the increase in coronal cervical alignment and the increase in SOM.…”

Section: Resultsmentioning

confidence: 99%

“…Various classification systems have attempted to elaborate upon the epidemiology and risk of neurologic injury in the KFS patient [8,15,20,22,37,38,45]. In our study, the authors utilized a classification system specific to the cervical spine as proposed by Guille et al [20] and Samartzis et al [45] in the adult and developing child, respectively.…”

Section: Resultsmentioning

confidence: 99%

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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: Resultssupporting

confidence: 50%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Superior odontoid migration in the Klippel–Feil patient

Samartzis

Kalluri²,

Herman

et al. 2006

Eur Spine J

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“…However, there are other authors who do not agree that the asymptomatic patient with Klippel-Feil syndrome should undergo early prophylactic arthrodesis because of instability [4,9]. Furthermore, patients with Klippel-Feil syndrome have fewer motion segments in the cervical spine than normal people, and therefore, it is likely that other segment problems will occur as they live longer after arthrodesis [6].…”

Section: Discussionmentioning

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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“…Patients with mild cases may not show any severe clinical symptoms at presentation but may develop neurological symptoms secondary to degenerative disc disease of the adjacent mobile segments, spinal instability from hypermobility or from trauma, or spinal stenosis in later decades of life. [13][14][15] The age at which neurological symptoms appear depends on the level of fusion of the cervical vertebrae. 6 Fusion of the first and the second cervical vertebrae (C1 and C2) tends to produce symptoms in the first decade of life, while fusion of the second and third cervical vertebrae (C2 and C3) is associated with neurological symptoms in the third decade.…”

Section: Test) Ross and Londsaymentioning

confidence: 99%

Anomaly of cervical vertebrae found on orthodontic examination: 8-year-old boy with cleft lip and palate diagnosed with Klippel-Feil syndrome

Yoshihara¹,

Suzuki²,

Yawaka³

2010

The Angle Orthodontist

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We incidentally encountered an anomaly of the cervical vertebrae during orthodontic examination of an 8-year-old boy with cleft lip and palate. Klippel-Feil syndrome was diagnosed by the orthopedic specialist to whom he had been referred for detailed examination. This case report shows the results of axial multislice-helical computed tomography with three-dimensional reconstructions of the cervical vertebrae. Furthermore, we evaluated the frequency of congenital anomalies on lateral cephalogram in patients with cleft lip and palate or cleft palate and non-cleft controls from among all patients in our orthodontic clinic. (Angle Orthod. 2010;80:975-980.)

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Risk Factors in Klippel-Feil Syndrome

Cited by 120 publications

References 0 publications

Superior odontoid migration in the Klippel–Feil patient

Superior odontoid migration in the Klippel–Feil patient

Cervical arthroplasty in a patient with Klippel-Feil syndrome

Anomaly of cervical vertebrae found on orthodontic examination: 8-year-old boy with cleft lip and palate diagnosed with Klippel-Feil syndrome

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