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Klippel-Feil syndrome was first described in 1912 by Maurice Klippel and André Feil [1] and is characterized by a congenital synostosis of some or all of the cervical vertebrae. This fusion results from defective segmentation during embryo’s development in the first 3 to 8 weeks of gestation. Diagnosis is based on a clinical triad associating a short neck, a low hairline and limited cervical amplitude. Less than 50% of patients present this clinical triad, and a wide spectrum of associated disorders may be present. This heterogeneity makes it difficult to estimate the incidence and prevalence of this syndrome, as many patients remain undiagnosed due to their asymptomatic state. Cervicalgia, neurological manifestations and decreased cervical amplitude of movement are the most common symptoms leading to the diagnosis of this syndrome. In cases of Klippel Feil syndrome, trauma can have disastrous consequences, such as spinal cord injury or facet joint dislocation. This syndrome also tends to accelerate degeneration of the adjacent segment of the fused segments.

Le syndrome de Klippel-Feil est une synostose congénitale de certaines ou de toutes les vertèbres cervicales. Le diagnostic est basé sur une triade clinique associant un cou court, une ligne d’implantation basse des cheveux et une limitation des amplitudes cervicales, qui est cependant variablement présente chez les patients, expliquant que le syndrome ne soit parfois identifié qu’à l’âge adulte. Nous présentons ici un cas de découverte d’un syndrome de Klippel-Feil associée à un syndrome de Chiari de type I chez une patiente adulte de 33 ans suivie pour un incidentalome hypophysaire au décours d’un accident de la voie publique ayant aggravé une cervicalgie mécanique chronique.