[The so-called Klippel-Feil syndrome and its orthopedic incidences]

Eighteen cases presenting Klippel-Feil syndrome were reviewed. Clinical and radiological criteria were analyzed: short neck, severe restriction of motion, and low posterior hairline which make up the classical clinical triad. Radiological abnormalities of the cervical spine included: a reduction in the number of cervical vertebrae, fused vertebral blocks (16 cases), cervical spine-bifida occulta, spinal dysraphism (12 cases). Other cervical vertebral disorders have been added which complete the original description: cervico-thoracic abnormalities (12 cases), craniocervical junction abnormalities (7 cases) and also thoraco lumbar abnormalities (5 cases). Although this syndrome is essentially descriptive, orthopaedic complications may occur: scoliosis (9 cases) of which only two had an increasing deformity and required posterior spine stabilisation; cervico-occipital instabilities (2 cases) which although rare, should be sought out with care because they can have serious consequences; Sprengel's deformities (7 cases) which did not warrant operative intervention. The Klippel-Feil syndrome, Wilderwanck syndrome, and Goldenhar syndrome are all close descriptive entities, and have limited surgical consequences.