SCOLIOSIS AND CONGENITAL-ANOMALIES ASSOCIATED WITH KLIPPEL-FEIL SYNDROME TYPES I-III

Citation
Mn. Thomsen et al., SCOLIOSIS AND CONGENITAL-ANOMALIES ASSOCIATED WITH KLIPPEL-FEIL SYNDROME TYPES I-III, Spine (Philadelphia, Pa. 1976), 22(4), 1997, pp. 396-401
Citations number
26
Categorie Soggetti
Orthopedics,"Clinical Neurology
ISSN journal
03622436
Volume
22
Issue
4
Year of publication
1997
Pages
396 - 401
Database
ISI
SICI code
0362-2436(1997)22:4<396:SACAWK>2.0.ZU;2-H
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 featu res, documented the associated anomalies, and registered the type of t reatment. Objective. The anomalies or syndromes and the development of scoliosis were correlated to the type of Klippel-Feil syndrome. Mater ial 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 p atients (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 cerv ical and upper thoracic vertebra with synostosis) and Type II (isolate d cervical spine) corresponded to 40% and 47% of patients, respectivel y. 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 an omalies in the patients examined in this study, with 67% of the patien ts 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 degr ees (Cobb angle), in Type III with 23 degrees, and in Type II with 9 d egrees only. Thus, Type II, with isolated cervical fusion, shows a low risk for scoliosis. Conclusion. This study increases knowledge of a w ide range of anomalies and syndromes identified in association with Kl ippel-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.