[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.
Chaumien
J P
JP
Hôpital des Enfants Malades.
Rigault
P
P
Maroteaux
P
P
Padovani
J P
JP
Touzet
P
P
D001269:Atlanto-Occipital Joint; D002574:Cervical Vertebrae / Q000002:abnormalities* / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007593:Joint Instability / Q000209:etiology*; D007714:Klippel-Feil Syndrome / Q000150:complications* / Q000000981:diagnostic imaging; D011859:Radiography; D012189:Retrospective Studies; D012540:Scapula / Q000002:abnormalities; D012600:Scoliosis / Q000209:etiology*
Le soi-disant syndrome de Klippel-Feil et ses incidences orthopédiques.