Purpose. To define the orthopaedic problems associated with pseudoachondroplasia (PSACH) and their functional impact. Methods. We reviewed the medical records of 12 consecutive patients presenting to our unit. Radiographic analysis of deformity included assessment of mechanical axis and dysplasia at hip, knee and ankle measured by acetabular index (AI), Reimer's migration percentage (MP), neck-shaft angle, distal lateral femoral (aDFLA) and proximal (mMPTA) and distal tibial angles. The paediatric/adolescent PODCI questionnaires and the SF36 were used to assess quality of life issues. Results. 12 patients (9 female) were reviewed at median age 18yrs (range 12-43yrs). Most symptoms related to walking tolerance, joint discomfort and deformity: 9 patients had genu varum, 7 tibial torsion, 2 patella instability and 3 significant low back pain. All patients had hip dysplasia. 10 had medial displacement of the mechanical axis, with a mean mDFLA 105 deg (88 -128) and mMPTA 75deg (51-90). 2 patients have been treated only with growth hormone; 10 patients have undergone a total of 9 distal femoral, 19 proximal tibial and 2 supramalleolar osteotomies. 6 procedures were performed using an external fixator. 7 limb segments have been treated by guided growth and in all these cases alignment has improved. One patient has had
Osteonecrosis (ON) can cause considerable morbidity in young people who undergo treatment for acute lymphoblastic leukaemia (ALL). The aims of this study were to determine the operations undertaken for ON in this population in the UK, along with the timing of these operations and any sequential procedures that are used in different joints. We also explored the outcomes of those patients treated by core decompression (CD), and compared this with conservative management, in both the pre- or post-collapse stages of ON. UK treatment centres were contacted to obtain details regarding surgical interventions and long-term outcomes for patients who were treated for ALL and who developed ON in UKALL 2003 (the national leukaemia study which recruited patients aged 1 to 24 years at diagnosis of ALL between 2003 and 2011). Imaging of patients with ON affecting the femoral head was requested and was used to score all lesions, with subsequent imaging used to determine the final grade. Kaplan-Meier failure time plots were used to compare the use of CD with non surgical management.Aims
Methods