Introduction and Purpose of Study. Osteogenesis imperfecta (OI) is a bone metabolic disorder that results in multiple fractures and deformities in children. The management of these patients should be in highly specialised units were multi-disciplinary management is mandatory. The aims of this study were twofold: 1. To determine the incidence and pattern of fractures in this population. 2. To determine the type, outcomes and complications of surgical treatment in the same population. Methods. A retrospective audit of patients treated for OI at a tertiary academic Hospital, from January 2002 to December 2011 was done. Results. Fifty three patients with OI were seen in the period under review. The patients came from six South African provinces including two other African countries. The male to female ratio was 1:1. The majority of patients were classified as type III and type IV, 19 (36%) and 14 (26%) respectively. Twelve patients (23%) had a first degree relative with OI. All patients received bisphosphonate therapy intravenously except two who were on oral medication. Seventeen patients (33%) had associated kyphoscoliosis – none were treated surgically. The most common long bone fractures were of the midshaft femur (61 fractures) and tibia (35 fractures). Seventeen patients (32%) received intramedullary rodding of either femur or tibia. Surgery had to be repeated due to rod migration in nine long bones (29%). The most common complication of surgery was rod migration and peri-implant fracture. Conclusion. Long bone fractures of the femur and tibia were most common cause of morbidity. Intramedullary rodding is a safe and effective means of long bone fracture management in patients with OI

Objective. Posterior vertebral column resection (PVCR) is indicated in the management of severe rigid spine deformities. It is a complex surgical procedure and is only performed in a few spine centres due to the technical expertise required and associated risk. The purpose of this study is to review the indications, surgical challenges and outcomes of patients undergoing PVCR. Methods. 12 patients with severe spinal deformities who underwent PVCR were retrospectively reviewed after a follow-up of 2 years. Surgery was performed with the aid of motor evoked spinal cord monitoring and cellsaver when available. The average surgical duration was 310 minutes (100–490). The average blood loss was 1491 ml (0–3500). The indication for PVCR was gross deformity and myelopathy which was due to congenital spinal deformities and one case of old tuberculosis. Clinical records and the radiographic parameters were reviewed. Results. Kyphosis of an average of 72 degrees was corrected to 28 degrees. The associated scoliosis was corrected from an average of 49.2 to 21.2 degrees. Ten patients improved neurologically to ASIA D and E. One patient deteriorated markedly, required revision with no initial improvement but reached ASIA E at 6 months after surgery. Four patients had associated syringomyelia. All were re-scanned at 1 year. The three with small syrinx's demonstrated no progression on MRI and the large syrinx resolved completely. In addition to the neurological deterioration, complications included 1 right lower lobe pneumonia. Conclusion. PVCR is an effective option to correct complex rigid kyphoscoliosis. In addition it allows excellent circumferential decompression of the cord and neurological recovery. When the congenital scoliosis is associated with syringomyelia with no other cause evident, it may allow resolution of the syrinx. Key words: Posterior vertebral column resection, severe spinal deformities, myelopathy, syringomyelia. NO DISCLOSURES

Purpose of study. The aim is to assess the use of non-fusion instrumentation “growth rods” in early onset scoliosis (EOS). Methods. A retrospective review of 12 consecutive patients who had undergone a growth rod procedure for EOS was performed. Six patients had neuromuscular scoliosis, 5 had juvenile idiopathic scoliosis while one had a congenital aetiology. Growth constructs were predominately constructed from modular commercially available sets using hooks, screws and connection blocks. One VEPTR was used in a severe kyphoscoliosis. Patients returned to theatre at 6 monthly intervals for a lengthening procedure. Patients were assessed with regards to age at presentation, age at surgery, indications for surgery, initial Cobb angle, post- operative Cobb angle, number of lengthening's done, instrumentation used, amount of spine growth achieved and complications. Results. The average age at presentation was 3 yrs 8 months (birth – 7 years 5 months). The average Cobb angle was 55 (38–90). Age at index surgery ranged from 2yrs 9 months to 8 years 2 months. The Cobb angle after the first procedure averaged 37 (range 20–90). The average lengthening over 51 lengthening procedures was 8 mm. Four patients have reached the end of the process and under gone a definitive fusion with pedicle screws and growth rods. Their final Cobb angle averaged 32 (26–48). Definitive surgery was performed earlier than planned in one patient due to repetitive rod breakage. Lengthening was abandoned in one patient whose implants became septic and required removal. Two patients required revision for superior construct failure. Conclusion. The growth rod procedure allows spinal deformity correction and control as well as on-going growth in trunk height. It is a labour intensive process with a significant incidence of complications. There is however very little choice in these patients due to concerns of fusion restricting pulmonary development. NO DISCLOSURES

Introduction. Pedicle Subtraction osteotomy (PRO) in correction of severe spinal deformities is well established. Prospective analysis of its efficacy in complex spinal deformities is sparse in literature. Aims and objectives. To assess the role of PRO in correction of uniplanar and multiplanar spinal deformity and to assess the role of revision PRO in failed corrections. Material and methods. 50 patients were operated between 1996-2007 and followed up for 2 years (2-6). 27 had uniplanar kyphosis (60-128 degrees) and kyphoscoliosis was seen in 10. Failed corrections were seen in 11 uniplanar and 2 multiplanar deformities. The average pre-operative kyphosis and sagittal balance was 78.7 degrees and 22 mm (7-30) respectively. Scoliotic deformity ranged from 97-138 (average 108 degrees) and the coronal imbalance from 10-55 (average 24mm). Deformity distribution was upper dorsal 5, mid dorsal 22, dorso-lumbar 18 and lumbar 5. A single posterior approach sufficed in 47 cases while 3 required an anterior approach for reconstruction. 13 patients had pre-operative neurological deficit (bedridden 10, ambulatory 3). The average surgical time required was 300 minutes and blood loss was 800cc. The anterior defect reconstructed averaged 16.5mm (5-28). Results. Pulmonary complications occurred in 8 (21%), (embolism 1, pneumonia 2, hypoxia 5). Wound infection required debridement in 3 (8%). Failed corrections were seen in 10 (3 out of 37 in our series, 8%) due to failure of construct 2, severe disease 2, infection (active 2, quiescent 4). Neurological deterioration occurred in 1(2%), medial pedicle wall perforation. 12 patients regained ambulation (independent 7, support 5). Post-operative kyphosis and sagittal balance was 36.5 (10-108) and 10mm (5-20) respectively. Average correction was: sagittal 46.4%, coronal 37.5% and revisions 58%. The correction of kyphosis and sagittal balance was statistically comparable between primary and revision cases (p >0.05). Conclusions. PRO offers an excellent single stage decompression and controlled correction of kyphosis