Children with spinal dysraphism can develop various musculoskeletal deformities, necessitating a range of orthopaedic interventions, causing significant morbidity, and making considerable demands on resources. This systematic review aimed to identify what outcome measures have been reported in the literature for children with spinal dysraphism who undergo orthopaedic interventions involving the lower limbs. A PROSPERO-registered systematic literature review was performed following PRISMA guidelines. All relevant studies published until January 2023 were identified. Individual outcomes and outcome measurement tools were extracted verbatim. The measurement tools were assessed for reliability and validity, and all outcomes were grouped according to the Outcome Measures Recommended for use in Randomized Clinical Trials (OMERACT) filters.Aims
Methods
This study compares outcomes in patients with complete congenital fibula absence, associated with severe lower limb deformity, treated with an amputation protocol to those using an extension prosthesis. 32 patients were identified. 9 patients (2M: 7F, median age at presentation of 22 yrs) utilized an extension prosthesis. 23 patients (16M: 7F, median age at presentation of 10 months) underwent 25 amputations during childhood: only two underwent tibial kyphus correction to facilitate prosthetic wear. Mobility was assessed using the SIGAM and K scores. Quality of life was assessed using the PedsQL inventory questionnaire; pain by a verbal severity score. Patients undergoing amputation were further subdivided by age, below and above 2 yrs at the time of surgery.Purpose
Method
This study aims to evaluate the development of deformity in patients with hypophosphataemic rickets and the evolution of the orthopaedic management thereof. Fifty-four patients had undergone treatment for hypophosphataemic rickets at our institution since 1995. Clinical records for all patients were obtained. Forty-one patients had long leg radiographs available that were analysed using Traumacad™ software. Statistical analysis was performed using SPSS 23 (SPSS Inc., Chicago, Illinois, USA). Of the 41 patients, 18 (43%) had no radiographic deformity. 20 have undergone bilateral lower limb surgery for persistent deformity (Mechanical Axis ≥ Zone 2). A further 3 patients are awaiting surgery. Six patients (12 limbs, 14 segments) had osteotomies and internal fixation as primary intervention: only one limb developed recurrent deformity. There were no major complications. Fourteen patients (28 limbs) had 8-plates (Orthofix, Verona) applied. In 5 limbs correction is on-going. Neutral alignment (central Zone 1) was achieved in 14/20 (70%) patients. Two patients required osteotomy and external fixation for resistant deformity. The mean rate of angular correction following 8-plate application was 0.3 and 0.7 degrees/month for the tibia and femur respectively. The mean age at 8-plate insertion was 10.25y (5–15y). Patients with more than 3 years of growth remaining responded significantly better than older patients (Fisher Exact Test, p=0.024). Guided growth was more successful in correcting valgus deformity than varus deformity (Fisher Exact Test, p=0.04). In the younger patients, diaphyseal deformity corrected as the mechanical axis improved at the rate of 0.2 and 0.7 degrees /month for the tibial and femoral shafts. Serum phosphate and alkaline phosphatase levels did not affect response to surgery or complication rate. Guided growth by means of 8-plates is a successful in addressing deformity in hypophosphataemic rickets. Surgery is best performed in patients with more than 3 years of growth remaining.
This study aimed to define the rates of lower limb angular correction using temporary hemiepiphysiodesis in differing skeletal pathologies. A retrospective review of 61 children (36M:25F) with angular deformities about the knee who underwent 8-plate hemiepiphysiodesis (mean age 10.8y) was undertaken. The children were divided into 9 groups based on their underlying pathology (lower limb hypoplasia, Blount's disease, skeletal dysplasia, rickets, metabolic disease, acquired growth disturbance, vascular malformation, steroid use and complex genetic disorders). Radiographic measurements of each limb segment was undertaken using the TraumaCad® digital templating software based on standing long-leg radiographs - mechanical lateral distal femoral angle (mLDFA) and mechanical medial proximal tibial angle (mMPTA). The rate of correction of each parameter was calculated as a function of the time lapse between the operation date and first radiographic evidence of full correction of the mechanical axis (zone 1). A total of 144 limb segments (80 distal femoral, 64 proximal tibial physes) were analysed. 62.5% of children had mechanical axes outside the knee joint at the time of operation; 63.2% achieved full correction. The rate of angular correction at the distal femur (mLDFA) was quickest in those with acquired growth disturbance (1.15°/month), complex genetic disorders (1.12°/month) and rickets (0.93°/month). It was slowest in those with vascular malformation (0.40°/month), lower extremity hypoplasia (0.44°/month) and metabolic disease (0.49°/month). At the proximal tibia, mMPTA correction was quickest in those with acquired growth disturbance (0.77°/month) and skeletal dysplasia (0.57°/month); whilst being slowest in those with metabolic disease (0.22°/month) and Blount's disease (0.29°/month). The rate of angular correction about the knee varies with the underlying pathology with correction rates varying up to 3-fold. This study demonstrated the differential rate of correction of angular deformities in children with different skeletal pathologies, which would help guide the timing of hemiepiphysiodesis.
To review the natural history of upper limb osteochondromas and assess their functional effect. We performed a retrospective casenote review of a consecutive patient cohort presenting between 1997–2012 with upper limb osteochondromas. Indications for surgical intervention were noted and considered to be cosmetic, functional (including pain relief) and ‘prophylactic’ in terms of deformity prevention. All patients were invited to complete questionnaires for the PODCI, DASH, OSS and MHS scores.Aim
Materials
To assess the efficacy of combined medical and surgical management in obtaining normal lower limb mechanical alignment in a patient cohort with genotypically similar hypophosphataemic rickets. A notes and radiograph audit was performed of all patients attending our institution with hypophosphataemic rickets: a subset with PHEX gene anomalies was studied further. Lower limb radiographs were assessed at two points during childhood and note made of treatment start, compliance; indication, timing and result of surgery. Standing leg alignment radiographs were measured at skeletal maturity or at latest review.Aim
Methods
We hypothesised that Vitamin D deficiency could be related to SUFE in children without endocrinological abnormalities. We therefore sought to examine prevalence and severity of Vitamin D insufficiency in a cross-section of SUFE patients. Vitamin D levels were tested for at time of hospital admission for operative treatment of SUFE. Seven patients, between the months of July 2011 to November 2011, presented to our institution. All were chronic, stable slips treated with in-situ screw fixation. All patients presented in the summer months and were operated on within 3 weeks of presentation.Purpose of study
Methods
To define the orthopaedic problems associated with pseudoachondroplasia (PSACH) and their functional impact. 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.Purpose
Methods
To document the success rate of closed reduction and soft tissue release in the treatment of bilateral true dislocation in developmental dysplasia of the hip (DDH). Case-note review of 22 children (44 hips) with idiopathic bilateral hip dislocation referred to a tertiary centre before walking age. The management protocol was as follows:
Examination under general anaesthesia, arthrogram, closed reduction and appropriate soft tissue release (adductors/psoas), application of a ‘frog’ cast. CT scan at 2 weeks to confirm reduction. Change of cast and arthrogram at 6 weeks to confirm improving position and stability. Cast removal at 12 weeks, and application of an abduction brace for 6 weeks. Treatment failure could occur on day 1 (failure of reduction), at week 2 (failure to maintain reduction), at week 6, or after cast removal.Purpose
Methods
To determine whether radiographically demonstrated femoral neck irregularities (pistolgrip-deformity, focal prominences or lytic defects) are associated with positive clinical impingement tests. The 1989 Bergen birth cohort (n=4004) was invited to a population-based follow-up including clinical examination and two pelvic radiographs. 2081 (52%) were enrolled. Associations between clinical and radiographic findings were examined using chi-squared or Fischer's exact test.Purpose
Methods
To investigate the effect of soft tissue release (STR) and the length of postoperative immobilisation on the long term outcomes of closed reduction (CR) of the hip for developmental dysplasia of the hip. 77 hips (72 patients) who had undergone closed reduction (CR) between 1977-2005 were studied retrospectively to review their outcome (Severin grade), identify the reasons for failure and to assess factors associated with residual dysplasia. Particular attention was paid to the use of a STR at the time of CR (to improve initial hip stability) and the duration of postoperative immobilisation.Purpose
Materials
To document the incidence and nature of complications associated with hemiepiphysiodesis using a screw and plate device (8-plate, Orthofix). We reviewed case notes and radiographs of 71 children (130 segments) with lower limb deformities treated with temporary hemiepiphyseal arrest using the 8-plate. 96% of deformities were in the coronal plane, 4% sagittal. 72% of coronal deformities demonstrated valgus malalignment. We defined three types of complications: complications Complications were related to variables of patient age, gender, diagnosis, location of deformity and associated surgery.Purpose
Methods
