Spinal deformity in metabolic disorders

Abstract

Children with metabolic bone disease and congenital problems such as osteogenesisimperfecta may develop spinal deformity, particularly scoliosis, unlike in adults where kyphosis is more common. Bracing is unlikely to be effective in these disorders since it must push on the ribs which suffer from the same disorder as the vertebra and cannot bear corrective force without deformation. Treating the underlying disease to strengthen the bone is important both for the deformity and any necessary surgical treatment. Patients with osteogenesisimperfecta may develop very severe early onset scoliosis and basilar invagination. Children with storage disease are prone to developing odontoid hypoplasia, atlantoaxial instability, and thoracolumbar kyphosis similar to patients with spondyloepiphyseal dysplasia but with the added problem of soft tissue hypertrophy from the storage products.