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
To monitor the performance of services for developmental dysplasia of the hip (DDH) in Northern Ireland and identify potential improvements to enhance quality of service and plan for the future. This was a prospective observational study, involving all infants treated for DDH between 2011 and 2017. Children underwent clinical assessment and radiological investigation as per the regional surveillance policy. The regional radiology data was interrogated to quantify the use of ultrasound and ionizing radiation for this population.Aims
Methods
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
To explore the of age of onset distribution for Perthes’ disease
of the hip, with particular reference to gender, laterality and
conformity to the lognormal distribution. A total of 1082 patients were identified from the Liverpool Perthes’
Disease Register between 1976 and 2010, of which 992 had the date
of diagnosis recorded. In total, 682 patients came from the geographical
area exclusively served by Alder Hey Hospital, of which 673 had
a date of diagnosis. Age of onset curves were analysed, with respect to
the predefined subgroups.Aims
Patients and Methods
The accurate assessment of skeletal maturity
is essential in the management of orthopaedic conditions in the growing
child. In order to identify the time of peak height velocity (PHV)
in adolescents, two systems for assessing skeletal maturity have
been described recently; the calcaneal apophyseal ossification method
and the Sanders hand scores. The purpose of this study was to compare these methods in assessing
skeletal maturity relative to PHV. We studied the radiographs of
a historical group of 94 healthy children (49 females and 45 males),
who had been followed longitudinally between the ages of three and
18 years with serial radiographs and physical examination. Radiographs
of the foot and hand were undertaken in these children at least
annually between the ages of ten and 15 years. We reviewed 738 radiographs
of the foot and 694 radiographs of the hand. PHV was calculated
from measurements of height taken at the time of the radiographs. Prior to PHV we observed four of six stages of calcaneal apophyseal
ossification and two of eight Sanders stages. Calcaneal stage 3
and Sanders stage 2 was seen to occur about 0.9 years before PHV,
while calcaneal stage 4 and Sanders stage 3 occurred approximately
0.5 years after PHV. The stages of the calcaneal and Sanders systems can be used in
combination, offering better assessment of skeletal maturity with
respect to PHV than either system alone. Cite this article:
The role of heritable thrombophilic risk factors in the pathogenesis of the Perthes’ disease is controversial. The clinical and radiological findings of Perthes’ disease may be indistinguishable from those of Gaucher’s disease, and the most common Jewish N370S Gaucher mutation is threefold greater in patients with Perthes’ disease. Familial osteonecrosis of the femoral head is associated with variant mutations of collagen type II (COL2A1 mutations). We therefore studied the potential role of genetic thrombophilia and the Gaucher and COL2A1 mutations in children with Perthes’ disease. Genomic DNA of 119 children with radiologically-confirmed Perthes’ disease diagnosed between 1986 and 2005 was analysed for the thrombophilic polymorphisms Factor V Leiden, 677T-MTHFR and FIIG20210A. The results were compared with those of a group of 276 children without Perthes’ disease. DNA was also analysed for the Gaucher mutations N370S, G insertion (84GG), L444P, Intron 2 (IVS2+1G>
A) and R496H. Enzymic assays confirmed the Gaucher disease status. Collagen (COL2A1) mutations of the 12q13 gene were also analysed. The prevalence of thrombophilic markers was similar among the 119 patients with Perthes’ disease and the 276 control subjects. The prevalence of the Gaucher mutation was consistent with Israeli population carriership data and did not confirm an earlier-claimed association with Perthes’ disease. All 199 patients were negative for the studied COL2A1 mutations. We found no genetic association between Perthes’ disease and either Gaucher’s disease or COL2A1 mutations or increased genetic thrombophilia among our patients compared with the control group. A systematic review of case-control studies suggested that there was a positive association between Perthes’ disease and Factor V Leiden. The impact of this association upon the disease, although not consistent across the studies, remains unclear.
