Abstract
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.
