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.