7/25/2019 The Spine Journal Volume 7 Issue 3 2007 [Doi 10.1016%2Fj.spinee.2006.02.031] -- 95 Years Ago in Spine- Klippel-Feil Syndrome

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95 Years

Ago in Spine:

Klippel-Feil

Syndrome

In 1912, Maurice Klippel and Andre

Feil published a case report of a 46-year-old French

tailor who strikingly appeared to have no neck [1].

After careful evaluation of their patient who was short

in stature, the authors noted a low posterior hairline,

short neck, and limited range of neck motion. Radio-

graphic and postmortem (the patient died 1 month after

his initial physical examination) anatomical evaluation

noted complete fusion of the patients cervical spine

with additional spinal anomalies. In fact, the authors

noted only 12 differentiated vertebrae, a thoracolumbar

scoliotic curve, and remarked on the potential heredi-

tary nature of the patients manifestations. In light of

their case report, the term Klippel-Feil syndrome

(KFS) was coined and since then referred to patientspresenting with failure of vertebral segmentation of

two or more cervical segments with or without associ-

ated anomalies.

At the time of this seminal report, Maurice Klippel

was an attending neurologist at the Hopital Tenon in

Paris, France and Andre Feil was his resident. In 1919,

Feil proposed a three-tiered classification system of

KFS based on various fusion patterns and abnormalities

of the spine[2]. In the years to follow, numerous reports

have noted the spinal and extraspinal heterogeneous

manifestations associated with KFS as well as variations

in classification schemas of this condition. Nonetheless,

the initial reports by Klippel and Feil during the earlytwentieth century raised awareness of potential develop-

mental and genetic factors contributing to the formation

of the spinal column and, as such, contributed to the

growing interest as well as understanding of congenital

spinal deformities.

References

[1] Klippel M, Feil A. [Un cas dabsence des vertebres cervicales.

Avec cage thoracique remontant jusqua la base du crane (cage

thoracique cervicale)]. Nouv Iconog Salpetriere 1912;25:22350.

[2] Feil A. [Labsebce et la diminuation des vertebres cervicales (etude

cliniqueet pathogenique); le syndrome dereduction numerique cer-

vicales]. Theses de Paris; 1919.

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