EHLERS-DANLOS-SYNDROME TYPE-VI (EDS-VI) - PROBLEMS OF DIAGNOSIS AND MANAGEMENT

Ehlers-Danlos Syndrome Type VI (EDS VI) is a rare autosomal recessivel y inherited connective tissue disorder, which poses several problems o f diagnosis and management. We report on a patient who developed sever e kyphoscoliosis long before the diagnosis was reached. We conclude th at early biochemical diagnosis and a timely operative procedure by ext ensive posterior instrumentation is; the basis for successful manageme nt of this disorder.