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Article: Sprengel's deformity in Klippel-Feil syndrome

TitleSprengel's deformity in Klippel-Feil syndrome
Authors
KeywordsCervical
Deformity
Fusion
Klippel-Feil
Risk factors
Scoliosis
Sprengel's
Issue Date2007
PublisherLippincott, Williams & Wilkins. The Journal's web site is located at http://www.spinejournal.com
Citation
Spine, 2007, v. 32 n. 18, p. E512-E516 How to Cite?
AbstractSTUDY DESIGN. A retrospective study. OBJECTIVES. To address the role of congenitally fused cervical segments, the degree of cervical scoliosis, and other risk factors on the presence of Sprengel's deformity (SD) in young patients with Klippel-Feil syndrome (KFS). SUMMARY OF BACKGROUND DATA. Numerous abnormalities are associated with KFS, one of the most common being SD. It has been postulated that more severe forms of KFS may be more associated with extraspinal manifestations, such as SD. METHODS. Thirty KFS patients from a single institution were reviewed. Cervical neutral lateral/dynamic/ anteroposterior and thoracic anteroposterior plain radiographs were assessed. Radiographically, occipitalization (O-C1), number of congenitally fused segments (C1-T1), classification type (Types I-III), degree of cervical scoliosis, and the presence of SD was assessed. Clinical chart review entailed patient demographics and evidence of the clinical assessment of SD. The threshold for statistical significance was P < 0.05. RESULTS. There were 11 males (36.7%) and 19 females (63.3%) with a mean age of 13.5 years (range, 2.7-26.3 years). Occipitalization was present in 10 (33.3%) individuals and C2-C3 was the most common level fused (70.0%). The mean number of congenitally fused segments was 3.3 (range, 1-6 levels). The mean degree of cervical scoliosis was 17.3° (range, 0°-67°). There were 6 (20%) Type I, 15 Type II (50.0%), and 9 Type III (30%) patients. SD was noted in 5 (16.7%) of the patients. Four patients had unilateral, whereas 1 patient had bilateral SD. There was 4.0 and 3.1 mean number of congenitally fused segments in patients with or without SD, respectively. SD did not occur in Type I patients (single fused block). The presence of SD was found to be nonsignificant regarding sex type (P = 0.327), presence of occipitalization (P = 0.300), number of congenitally fused segments (P = 0.246), specific congenitally fused segments (P > 0.05), classification type (P > 0.05), and scoliosis (P = 0.702). CONCLUSION. SD occurred in 16.7% of KFS patients. Sex type, number of congenitally fused segments, specific fused patterns, occipitalization, classification type, and the degree of cervical scoliosis did not seem to be significantly associated with the presence of SD in KFS patients in our series. Thorough examination for the presence and degree of SD in KFS is necessary, irrespective of the extent of cervical abnormalities. Alternatively, the treating physician should not dismiss a thorough cervical spine examination in patients with SD, evaluating factors that may predispose the KFS patient to an increased risk of neurologic injury. © 2007 Lippincott Williams & Wilkins, Inc.
Persistent Identifierhttp://hdl.handle.net/10722/92944
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.accessioned2010-09-22T05:04:32Z-
dc.date.available2010-09-22T05:04:32Z-
dc.date.issued2007en_HK
dc.identifier.citationSpine, 2007, v. 32 n. 18, p. E512-E516en_HK
dc.identifier.issn0362-2436en_HK
dc.identifier.urihttp://hdl.handle.net/10722/92944-
dc.description.abstractSTUDY DESIGN. A retrospective study. OBJECTIVES. To address the role of congenitally fused cervical segments, the degree of cervical scoliosis, and other risk factors on the presence of Sprengel's deformity (SD) in young patients with Klippel-Feil syndrome (KFS). SUMMARY OF BACKGROUND DATA. Numerous abnormalities are associated with KFS, one of the most common being SD. It has been postulated that more severe forms of KFS may be more associated with extraspinal manifestations, such as SD. METHODS. Thirty KFS patients from a single institution were reviewed. Cervical neutral lateral/dynamic/ anteroposterior and thoracic anteroposterior plain radiographs were assessed. Radiographically, occipitalization (O-C1), number of congenitally fused segments (C1-T1), classification type (Types I-III), degree of cervical scoliosis, and the presence of SD was assessed. Clinical chart review entailed patient demographics and evidence of the clinical assessment of SD. The threshold for statistical significance was P < 0.05. RESULTS. There were 11 males (36.7%) and 19 females (63.3%) with a mean age of 13.5 years (range, 2.7-26.3 years). Occipitalization was present in 10 (33.3%) individuals and C2-C3 was the most common level fused (70.0%). The mean number of congenitally fused segments was 3.3 (range, 1-6 levels). The mean degree of cervical scoliosis was 17.3° (range, 0°-67°). There were 6 (20%) Type I, 15 Type II (50.0%), and 9 Type III (30%) patients. SD was noted in 5 (16.7%) of the patients. Four patients had unilateral, whereas 1 patient had bilateral SD. There was 4.0 and 3.1 mean number of congenitally fused segments in patients with or without SD, respectively. SD did not occur in Type I patients (single fused block). The presence of SD was found to be nonsignificant regarding sex type (P = 0.327), presence of occipitalization (P = 0.300), number of congenitally fused segments (P = 0.246), specific congenitally fused segments (P > 0.05), classification type (P > 0.05), and scoliosis (P = 0.702). CONCLUSION. SD occurred in 16.7% of KFS patients. Sex type, number of congenitally fused segments, specific fused patterns, occipitalization, classification type, and the degree of cervical scoliosis did not seem to be significantly associated with the presence of SD in KFS patients in our series. Thorough examination for the presence and degree of SD in KFS is necessary, irrespective of the extent of cervical abnormalities. Alternatively, the treating physician should not dismiss a thorough cervical spine examination in patients with SD, evaluating factors that may predispose the KFS patient to an increased risk of neurologic injury. © 2007 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.subjectCervicalen_HK
dc.subjectDeformityen_HK
dc.subjectFusionen_HK
dc.subjectKlippel-Feilen_HK
dc.subjectRisk factorsen_HK
dc.subjectScoliosisen_HK
dc.subjectSprengel'sen_HK
dc.titleSprengel's deformity in Klippel-Feil syndromeen_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/BRS.0b013e318133fcf8en_HK
dc.identifier.pmid17700431en_HK
dc.identifier.scopuseid_2-s2.0-34548142682en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-34548142682&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume32en_HK
dc.identifier.issue18en_HK
dc.identifier.spageE512en_HK
dc.identifier.epageE516en_HK
dc.identifier.isiWOS:000248809800024-
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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