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.
The use of prolonged halo stabilisation in a child is increasingly indicated for trauma and congenital instability of the cervical spine, but complications of pin fixation in this age group are frequent. We have analysed four aspects of the mechanics of the halo pin: the forces applied by each of six surgeons was shown to vary widely, penetration of the inner table occurred relatively easily, friction at the pin-halo interface influenced forces, and the skull thickness measured by CT scan varied from 1.1 mm to 4.3 mm in children under six years of age. We recommend CT scanning of the skull before elective halo application in young children to ascertain the safest pin sites.
The existence of pulmonary metastases in patients presenting with osteosarcoma is known to indicate a poor prognosis. Lung resection of solitary lesions is now a standard treatment approach, but with limited successful long-term results. We report the progress of a 16-year-old girl with osteosarcoma of the proximal tibia who underwent 11 lung resections for pulmonary metastases over a two-year period, and who has since then been free of disease for seven years.
The term "floating knee" describes the flail joint resulting from fractures of the shaft or adjacent metaphyseal region of the ipsilateral femur and tibia; we have reviewed 15 children with this combined injury seen at the Winnipeg Children's Hospital. All had been involved in motor vehicle accidents, eight having been struck while cycling; the biomechanics of such an injury have also been studied. Treatment is difficult and the regimes varied. Results were poor when both fractures were treated nonoperatively and we recommend that at least one of the fractures should be rigidly fixed.
Thirty-three Monteggia fracture-dislocations occurring in patients aged 2 to 15 years were reviewed. A follow-up of 2 to 7 years in 25 patients revealed that 88% had good to excellent results and 12% had results which were fair or poor. Closed reduction was successful in 24 of 28 cases and appeared to be very effective. Open reduction was required only for older children or when treatment was begun late. A mild hyperextension deformity at the elbow noted on follow-up of patients with anterior dislocation of the radial head seemed to support the theory that the injury is caused by hyperextension. A new classification of Monteggia fracture-dislocations in children is proposed.
