123 Management of scoliosis in children with severe, complex and atypical osteogenesis imperfecta

Background Osteogenesis Imperfecta (OI) is a heterogeneous bone fragility disorder most commonly caused by mutations in the genes encoding type 1 procollagen. The prevalence of OI is 1:15,000 and is characterised by low trauma fractures of appendicular and axial skeleton. Children can present with long bone and spinal deformities. GOSH is one of four Highly Specialised Services for OI in England with a cohort of over 300 children; 106 patients are designated as Severe, Complex and Atypical (SCA) on criteria agreed across all centres. Children with Complex OI include those with a scoliosis of Cobb angle >45°and are reportable to NHS England. Methods To look at the numbers of children with Cobb angle >45° and those children who have had surgical intervention. Results Total number of children with SCA OI at GOSH: 106/327 (32.4%). 12/106 (11.3%) children had scoliosis with Cobb angle >30°; 5 (4.72%) had Cobb angle >45°. Since April 2011, 10 children have had spinal surgery: Age range at onset of scoliosis 0.25-12 years (mean 6.5). Age range at surgery 2-16 years (mean 11.3). Cobb angle prior to surgery 60 to 90° (mean 74). Surgery: posterior spinal fusion n=8; magnetic growth rods n=2. Outcomes: no metalwork complications n=8; revision surgeries n=2 both of whom had early onset scoliosis. All patients treated with bisphosphonates. All patients had multidisciplinary team assessment and discussion. There appeared to be three subtypes of curve progression: Early onset ( Non–ambulant type (5 to 12 years), gradual progression. Adolescent ambulant type (>10 years), variable progression. Conclusion Spinal surgery can be a successful intervention for children with OI, including those with severe bone fragility. Multidisciplinary assessment to inform decision-making around surgery is paramount. Our experience will facilitate the development of a standardised national spinal pathway for children with complex OI.