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Article: Classification of congenitally fused cervical patterns in Klippel-Feil patients: Epidemiology and role in the development of cervical spine-related symptoms

TitleClassification of congenitally fused cervical patterns in Klippel-Feil patients: Epidemiology and role in the development of cervical spine-related symptoms
Authors
KeywordsBlock vertebrae
Cervical spine
Classification
Congenital
Developmental
Epidemiology
Fusion
Klippel-Feil
Radiographic
Risk
Symptoms
Issue Date2006
PublisherLippincott, Williams & Wilkins. The Journal's web site is located at http://www.spinejournal.com
Citation
Spine, 2006, v. 31 n. 21, p. E798-E804 How to Cite?
AbstractSTUDY DESIGN. A retrospective cohort and series review. OBJECTIVES. To determine the role of cervical spine fusion patterns on the development of cervical spine-related symptoms (CSS) in patients with Klippel-Feil syndrome (KFS) and evaluate age- and time-dependent factors that may contribute to fused cervical patterns and the development of the CSS. SUMMARY OF BACKGROUND DATA. Although the "hallmark" of KFS is the presence of congenitally fused cervical vertebrae, the epidemiology and role of specific cervical fused patterns are limited. In addition, the incidence of symptoms and various age- and time-dependent factors that are directly attributed to the congenitally fused cervical segments in KFS patients is unknown. METHODS. A radiographic and clinical review of 28 KFS patients at a single institution. Radiographically, Type I patients were defined as having a single congenitally fused cervical segment. Type II patients demonstrated multiple noncontiguous, congenitally fused segments, and Type III patients had multiple contiguous, congenitally fused cervical segments. Clinical records were reviewed for patient demographics, presence and type of symptoms, and clinical course. RESULTS. Twelve males and 16 females were reviewed for clinical follow-up (mean, 8.5 years) and radiographic assessment (mean, 8.0 years). The mean age at presentation was 7.1 years; mean age of onset of CSS was 11.9 years. Clinically, 64% had no complaints referable to their cervical spine. Radiographically, 25%, 50%, and 25% were Type I, Type II, and Type III, respectively. At final clinical follow-up, 2 patients were myelopathic (Type II and Type III) and 2 were radiculopathic (Type II and Type III). Type III patients were largely asymptomatic but were associated with the highest risk in developing radiculopathy or myelopathy than Type I or Type II patients. Axial symptoms were predominantly associated with Type I patients. Myelopathic patients developed initial CSS earlier (meanage, 10.6 years) than patients with predominant axial (mean age, 13.0 years) or radiculopathic symptoms (mean age, 18.6 years) (P > 0.05). Patients with radiculopathy or myelopathy were diagnosed at a mean age of 17.9 years. Type I patients were predominantly females, while males were largely Type III. Surgery entailed 11% of patients, composed of 2 myelopathic patients (Type II and Type III) and 1 radiculopathic patient (Type II). CONCLUSIONS. In our review, 36% of KFS patients had CSS and the majority had axial symptoms. Axial neck symptoms were highly associated with Type I patients, whereas predominant radicular and myelopathic symptoms occurred in Type II and Type III patients. This classification system has promise for early detection for CSS. Activity modification should be stressed in KFS patients at high risk for neurologic compromise. © 2006 Lippincott Williams & Wilkins, Inc.
Persistent Identifierhttp://hdl.handle.net/10722/92901
ISSN
2015 Impact Factor: 2.439
2015 SCImago Journal Rankings: 1.459
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorSamartzis, Den_HK
dc.contributor.authorHerman, Jen_HK
dc.contributor.authorLubicky, JPen_HK
dc.contributor.authorShen, FHen_HK
dc.date.accessioned2010-09-22T05:03:13Z-
dc.date.available2010-09-22T05:03:13Z-
dc.date.issued2006en_HK
dc.identifier.citationSpine, 2006, v. 31 n. 21, p. E798-E804en_HK
dc.identifier.issn0362-2436en_HK
dc.identifier.urihttp://hdl.handle.net/10722/92901-
dc.description.abstractSTUDY DESIGN. A retrospective cohort and series review. OBJECTIVES. To determine the role of cervical spine fusion patterns on the development of cervical spine-related symptoms (CSS) in patients with Klippel-Feil syndrome (KFS) and evaluate age- and time-dependent factors that may contribute to fused cervical patterns and the development of the CSS. SUMMARY OF BACKGROUND DATA. Although the "hallmark" of KFS is the presence of congenitally fused cervical vertebrae, the epidemiology and role of specific cervical fused patterns are limited. In addition, the incidence of symptoms and various age- and time-dependent factors that are directly attributed to the congenitally fused cervical segments in KFS patients is unknown. METHODS. A radiographic and clinical review of 28 KFS patients at a single institution. Radiographically, Type I patients were defined as having a single congenitally fused cervical segment. Type II patients demonstrated multiple noncontiguous, congenitally fused segments, and Type III patients had multiple contiguous, congenitally fused cervical segments. Clinical records were reviewed for patient demographics, presence and type of symptoms, and clinical course. RESULTS. Twelve males and 16 females were reviewed for clinical follow-up (mean, 8.5 years) and radiographic assessment (mean, 8.0 years). The mean age at presentation was 7.1 years; mean age of onset of CSS was 11.9 years. Clinically, 64% had no complaints referable to their cervical spine. Radiographically, 25%, 50%, and 25% were Type I, Type II, and Type III, respectively. At final clinical follow-up, 2 patients were myelopathic (Type II and Type III) and 2 were radiculopathic (Type II and Type III). Type III patients were largely asymptomatic but were associated with the highest risk in developing radiculopathy or myelopathy than Type I or Type II patients. Axial symptoms were predominantly associated with Type I patients. Myelopathic patients developed initial CSS earlier (meanage, 10.6 years) than patients with predominant axial (mean age, 13.0 years) or radiculopathic symptoms (mean age, 18.6 years) (P > 0.05). Patients with radiculopathy or myelopathy were diagnosed at a mean age of 17.9 years. Type I patients were predominantly females, while males were largely Type III. Surgery entailed 11% of patients, composed of 2 myelopathic patients (Type II and Type III) and 1 radiculopathic patient (Type II). CONCLUSIONS. In our review, 36% of KFS patients had CSS and the majority had axial symptoms. Axial neck symptoms were highly associated with Type I patients, whereas predominant radicular and myelopathic symptoms occurred in Type II and Type III patients. This classification system has promise for early detection for CSS. Activity modification should be stressed in KFS patients at high risk for neurologic compromise. © 2006 Lippincott Williams & Wilkins, Inc.en_HK
dc.languageengen_HK
dc.publisherLippincott, Williams & Wilkins. The Journal's web site is located at http://www.spinejournal.comen_HK
dc.relation.ispartofSpineen_HK
dc.subjectBlock vertebraeen_HK
dc.subjectCervical spineen_HK
dc.subjectClassificationen_HK
dc.subjectCongenitalen_HK
dc.subjectDevelopmentalen_HK
dc.subjectEpidemiologyen_HK
dc.subjectFusionen_HK
dc.subjectKlippel-Feilen_HK
dc.subjectRadiographicen_HK
dc.subjectRisken_HK
dc.subjectSymptomsen_HK
dc.titleClassification of congenitally fused cervical patterns in Klippel-Feil patients: Epidemiology and role in the development of cervical spine-related symptomsen_HK
dc.typeArticleen_HK
dc.identifier.emailSamartzis, D:dspine@hku.hken_HK
dc.identifier.authoritySamartzis, D=rp01430en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1097/01.brs.0000239222.36505.46en_HK
dc.identifier.pmid17023841-
dc.identifier.scopuseid_2-s2.0-33749532523en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-33749532523&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume31en_HK
dc.identifier.issue21en_HK
dc.identifier.spageE798en_HK
dc.identifier.epageE804en_HK
dc.identifier.eissn1528-1159-
dc.identifier.isiWOS:000241077400024-
dc.publisher.placeUnited Statesen_HK
dc.identifier.scopusauthoridSamartzis, D=34572771100en_HK
dc.identifier.scopusauthoridHerman, J=7403275959en_HK
dc.identifier.scopusauthoridLubicky, JP=7004313450en_HK
dc.identifier.scopusauthoridShen, FH=7201583245en_HK

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