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.

We studied the effects of the timing of tourniquet release in 88 patients randomly allocated for release after wound closure and bandaging (group A), or before the quadriceps layer had been closed allowing control of bleeding before suture (group B). The groups were similar in mean age, weight, gender, preoperative knee score, radiographic grading, and prosthesis implanted.

Patients in group B had less postoperative pain, achieved earlier straight-leg raising, and had fewer wound complications. Five patients in group A had to return to theatre, three for manipulation under anaesthesia, one for secondary closure of wound dehiscence, and one for drainage of a haematoma. The last patient later developed a deep infection, which was treated by a two-stage revision. There were no significant differences between the two groups in operating time, or the decrease in haemoglobin concentration at 48 hours postoperatively.

Some of the adverse effects of the use of a tourniquet for knee surgery can be significantly reduced by early tourniquet release, with haemostasis before the quadriceps mechanism and the wound are closed.