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  • Title: Scoliosis and congenital anomalies associated with Klippel-Feil syndrome types I-III.
    Author: Thomsen MN, Schneider U, Weber M, Johannisson R, Niethard FU.
    Journal: Spine (Phila Pa 1976); 1997 Feb 15; 22(4):396-401. PubMed ID: 9055366.
    Abstract:
    STUDY DESIGN: This investigation was aimed at characterizing anomalies and syndromes associated with Klippel-Feil syndrome in a large group of patients. The authors evaluated the clinical and radiographic features, documented the associated anomalies, and registered the type of treatment. OBJECTIVE: The anomalies or syndromes and the development of scoliosis were correlated to the type of Klippel-Feil syndrome. MATERIAL AND METHODS: In a cross-sectional study, the authors reviewed data from 57 patients with Klippel-Feil syndrome treated over 25 years at the Department for Orthopedics of the University of Heidelberg. The patients (17 males and 40 females; average age of the first contact, 12 years) were classified into three types according to the description of Feil in 1919. RESULTS: Klippel-Feil syndrome Type I (fusion of cervical and upper thoracic vertebra with synostosis) and Type II (isolated cervical spine) corresponded to 40% and 47% of patients, respectively. Type III (cervical vertebra associated with lower thoracic or upper lumbar fusion) was displayed in 13% of the patients only. The authors found a variety of combinations of Klippel-Feil syndrome and other anomalies in the patients examined in this study, with 67% of the patients characterized by an association with other disorders or syndromes. Of the patients, 70% showed scoliosis. Its degree depended on the type of Klippel-Feil syndrome. Scoliosis in Type I correlated with 31 degrees (Cobb angle), in Type III with 23 degrees, and in Type II with 9 degrees only. Thus, Type II, with isolated cervical fusion, shows a low risk for scoliosis. CONCLUSION: This study increases knowledge of a wide range of anomalies and syndromes identified in association with Klippel-Feil syndrome. A special finding of the study was a correlation between the degree of scoliosis and Klippel-Feil syndrome Types I, II, and III.
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