[Atlanto-axial, Atlanto-occipital dislocations, developmental cervical canal stenosis in the Ehlers-Danlos syndrome (author's transl)]

Abstract

Clinical, biological, histopathological and laboratory data are reported on a 13-year-old male with atlanto-axial, atlanto-occipital hypermobilities, cervical canal stenosis, hypoplastic atlas and hypoplastic first rib in the Ehlers-Danlos syndrome. He developed frequent attacks of sudden tetraplegia or tetraparesis and numbness below C2 due to trivial neck injuries. Examination revealed marked hypermobilities of all joints the cutis elastica (Fig. 1), spastic tetraparesis and fasciculation of the tongue. Occipito-atlantal hypermobility measured by Wiesel and Rhothmans' method revealed 5 mm horizontal translation (Fig. 2). Anterior and lateral dislocations of atlanto-axial joints were marked due to laxicity of the joint. Posterior decompression of C1, laminotomy of C2,3, occipito-C2 fixation with autogenous iliac "board (3 X 6 cm, rectangular board) graft" yielded favorable results with 15 months follow-up. To our knowledge, this is the first patient reported with this unusual combinations.