Aim. To assess the efficacy of combined medical and surgical management in obtaining normal lower limb mechanical alignment in a patient cohort with genotypically similar hypophosphataemic rickets. Methods. A notes and radiograph audit was performed of all patients attending our institution with hypophosphataemic rickets: a subset with PHEX gene anomalies was studied further. Lower limb radiographs were assessed at two points during childhood and note made of treatment start, compliance; indication, timing and result of surgery. Standing leg alignment radiographs were measured at skeletal maturity or at latest review. Results. 35 patients (16 females, 18 skeletally mature) were identified. 10 commenced treatment at <12m. 11 patients (5 female) underwent 24 surgical procedures (13 for varus deformities). Surgery was bilateral in 10/11 patients. 5/14 osteotomies were performed after skeletal maturity. Malalignment was common: with NSA (neck-shaft angle) abnormalities in 20%, abnormal angles at distal femur and proximal tibia in 58% and 60% respectively and ankle abnormalities in 24%, prior to surgery. Surgical management led to normal mechanical alignment at skeletal maturity. At latest review, no patient had a leg length difference. 40% of non-operated, skeletally immature limbs have significant malalignment despite
Our aim was to review the efficacy of the wound vacuum-assisted closure (VAC) system in the treatment of deep infection after extensive instrumentation and fusion for spinal deformity in children and adolescents. A total of 14 patients with early deep spinal infection were treated using this technique. Of these, 12 had neuromuscular or syndromic problems. Clinical and laboratory data were reviewed. The mean follow-up was 44 months (24 to 72). All wounds healed. Two patients required plastic surgery to speed up the process. In no patient was the hardware removed and there was no loss of correction or recurrent infection. We believe that the wound VAC system is a useful tool in the armamentarium of the spinal surgeon dealing with patients susceptible to wound infections, especially those with neuromuscular diseases. It allows for the retention of the instrumentation and the maintenance of spinal correction. It is reliable and easy to use.