The health-related quality of life (HRQoL) of paediatric patients with orthopaedic conditions and spinal deformity is important, but existing generic tools have their shortcomings. We aim to evaluate the use of Paediatric Quality of Life Inventory (PedsQL) 4.0 generic core scales in the paediatric population with specific comparisons between those with spinal and limb pathologies, and to explore the feasibility of using PedsQL for studying scoliosis patients’ HRQoL. Paediatric patients attending a speciality outpatient clinic were recruited through consecutive sampling. Two groups of patients were included: idiopathic scoliosis, and paediatric orthopaedic upper and lower limb condition without scoliosis. Patients were asked to complete PedsQL 4.0 generic core scales, Youth version of 5-level EuroQol-5-dimension questionnaire, and Refined Scoliosis Research Society 22-item (SRS-22r) questionnaire. Statistical analyses included scores comparison between scoliosis and limb pathology patients using independent-samples Aims
Methods
We report five children who presented at the mean age of 1.5 years (1.1 to 1.9) with a progressive thoracolumbar kyphosis associated with segmental instability and subluxation of the spine at the level above an anteriorly-wedged hypoplastic vertebra at L1 or L2. The spinal deformity appeared to be developmental and not congenital in origin. The anterior wedging of the vertebra may have been secondary to localised segmental instability and subsequent kyphotic deformity. We suggest the term ‘infantile developmental thoracolumbar kyphosis with segmental subluxation of the spine’ to differentiate this type of deformity from congenital displacement of the spine in which the congenital vertebral anomaly does not resolve. Infantile developmental kyphosis with segmental subluxation of the spine, if progressive, may carry the risk of neurological compromise. In all of our patients the kyphotic deformity progressed over a period of three months and all were treated by localised posterior spinal fusion. At a mean follow-up of 6.6 years (5.0 to 9.0), gradual correction of the kyphosis was seen on serial radiographs as well as reconstitution of the hypoplastic wedged vertebra to normality. Exploration of the arthrodesis was necessary at nine months in one patient who developed a pseudarthrosis.
