| Congenital limb deficiences associated with Klippel-Feil syndrome: a survey of 57 subjects. | |
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MedLine Citation:
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PMID: 11186401 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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We reviewed data from 57 patients (40 women) with Klippel-Feil syndrome to identify and characterize limb deficiencies. The cervical synostosis was classified according to the description of Feil (1919). Limb deficiencies were classified according to Henkel et al. (1978) and compared with the sclerotome theory of McCredie and Willert (1999). In a wide variety of combinations of Klippel-Feil syndrome (types I-III) and other anomalies, only 5 patients had a longitudinal upper limb deficiency (one arm or both arms). 4 patients had Klippel-Feil syndrome type II and 1 had type I. 4 patients had "longitudinal distal radial deficiencies", Henkel types 1, 2, 4 and 5 and 1 patient showed "longitudinal combined humero-ulna deficiencies" types 2 and 3 of both arms. The lower limb was not affected. The combination of Klippel-Feil syndrome and upper limb deficiency shows that the defect occurred between the 4th and 5th week of gestation. Sclerotome 6 was mainly affected in our study. In 4 of the 5 patients, the cervical fusion level and sclerotome level of the limb deficiency clearly or partly matched, whereas there was no agreement in 1 patient. |
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Authors:
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M Thomsen; M Kröber; U Schneider; C Carstens |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Acta orthopaedica Scandinavica Volume: 71 ISSN: 0001-6470 ISO Abbreviation: Acta Orthop Scand Publication Date: 2000 Oct |
Date Detail:
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Created Date: 2001-01-19 Completed Date: 2001-02-01 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 0370352 Medline TA: Acta Orthop Scand Country: Norway |
Other Details:
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Languages: eng Pagination: 461-4 Citation Subset: IM |
Affiliation:
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Department of Pediatric Orthopedics at the Orthopedic Surgery Hospital, University of Heidelberg, Germany. marc.thomsen@ok.uni-heidelberg.de |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Arm / abnormalities* Child Child, Preschool Female Fingers / abnormalities Gestational Age Humans Infant Klippel-Feil Syndrome / complications* Male Metacarpus / abnormalities Middle Aged Radius / abnormalities |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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