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Scoliosis and Congenital Anomalies Associated With Klippel-Feil Syndrome Types I-III

Thomsen, Marc N., MD, PhD*; Schneider, Ulrich, MD, PhD*; Weber, Michael, MD, PhD*; Johannisson, Reiner, PhD; Niethard, Fritz U., MD, PhD

Cervical Spine

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 1919.9

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° (Cobb angle), in Type III with 23°, and in Type II with 9° 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.

From the *Department for Pediatric Orthopedics at the Orthopedic Surgery Hospital, University of Heidelberg, Germany, the Institute of Pathology, University of Lübeck, Germany, and the ‡Department for Orthopedics at the RWTH Aachen, Germany.

Acknowledgment date: October 31, 1995.

First revision date: February 26, 1996.

Acceptance date: May 7, 1996.

Device status category: 1.

Address reprint requests to: Marc N. Thomsen, MD, PhD; Stiftung Orthopädische Universitätsklinik Heidelberg; Schlierbacher Landstr. 200 a; D-69118 Heidelberg; Germany

© Lippincott-Raven Publishers.