[Ehlers-Danlos syndrome--clinico-pathology and orthopedic treatment]

Eight cases of Ehlers-Danlos syndrome (EDS) were surveyed for clinical manifestations and orthopaedic treatment. Cases of EDS were clinically subdivided into three groups. The first group was characterized by multiple congenital contractures in the extremities, so that it was very difficult to distinguish it from arthrogryposis multiplex congenita in early infancy. Since laxity of the soft tissue coexists with contracture in this group, over-correction of deformity caused much more disability than under-correction, especially in clubfoot. Care should therefore be taken to avoid this. The third group was characterized by marked laxity of the soft tissue from birth. Diagnosis in this group was easily made by the obvious triad of EDS. Lengthy immobilization in an appropriate position was necessary to maintain a corrected position for the treatment of any deformity in the hip, foot or hand. In the second group, deformity and disability were clinically so mild that no orthopaedic management was generally required.