The orthopaedic manifestations and management of children with Stuve-Wiedemann syndrome.

Stüve-Wiedemann syndrome is an autosomal-recessive disorder characterised by bowing of the long bones, progressive scoliosis, episodic hyperthermia and respiratory distress, usually resulting in death in infancy. We reviewed five children with the condition who had been followed since birth and who survived into childhood with a mean age at operation of 7.8 years (5 to 14). There was marked functional impairment with dysplasia of the long bones and scoliosis. Treatment of the triplanar deformities of the femora involved the use of the Ilizarov technique with the Taylor Spatial Frame. Walking was preserved and improved in three children along with considerable enhancement of the appearance. Early insertion of a growing rod to control the progressive juvenile scoliosis was beneficial. The use of the Taylor Spatial Frame is strongly recommended to address the major complex deformities of the lower limbs which are encountered in this condition and to prevent their progression.