Elongating rods have been used in the management of Osteogenesis Imperfecta (OI) for the last 50 years; complication rates have been high in many reviews of available techniques. The functional outcomes and complications of a cohort of 22 Osteogenesis Imperfecta patients treated with 66 Sheffield Telescopic Intramedullary Rods at an average of 19 years post-initial surgery are analysed. The revision rate was 35% for any reason, 20% excluding revisions for rods separating due to growth. Re-operation other than revisions occurred in 10 rods (15%). Mobility was significantly better in the initial post-operative period (p=0.0015), this difference maintained in adulthood (p=0.0077). Back pain was the most frequent symptom. Symptoms related to the insertion technique across the knee and ankle were rare but those related to femoral trochanteric entry were common. Physeal damage following surgery was not experienced and all rods elongated. All patients were satisfied with the outcome of their surgeries. SF-36 scores were significantly different for physical functioning domains, social functioning and vitality in comparison to normal population values, but comparable to other studies of OI. The outcomes of this technique are satisfactory in adulthood; re-operation rates are high but related mainly to outgrowing the rods. Concerns regarding insertion with this fixed device at the knee and ankle are not founded, although proximal femoral fixation remains a problem

Purpose

Developmental exposure to estrogens has been shown to affect a number of organ systems, including long and short bones. Epigenetic effects of DES exposure have been shown to affect the third generation of progeny. Furthermore, recent studies have shown that environmental exposure to estrogen-like compounds is much higher than originally anticipated. This study aims to discover the effect of in utero exposure to a well-known estrogen agonist, diethylstilbestrol (DES), on lumbar bone, intervertebral disc (IVD), and articular cartilage. Femoral bone was studied to determine the specificity of the effect.

Proximal femoral focal deficiency is a congenital disorder of malformation of the proximal femur and/or the acetabulum. Patients present with limb length discrepancy and clinical features along a spectrum of severity. As these patients progress through to skeletal maturity and on to adulthood, altered biomechanical demands lead to progression of arthropathy in any joint within the lower limb. Abnormal anatomy presents a challenge to surgeons and conventional approaches and implants may not necessarily be applicable. We present a case of a 62-year-old lady with unilateral proximal femoral focal deficiency (suspected Aitken Class A) who ambulated with an equinus prosthesis for her entire life. She presented with ipsilateral knee pain and instability due to knee arthritis but could not tolerate a total knee arthroplasty due to poor quadriceps control. A custom osteointegration prosthesis was inserted with a view to converting to the proximal segment to a total hip replacement if required. The patient went on to develop ipsilateral symptomatic hip arthritis but altered acetabular anatomy required a custom tri-flange component (Ossis, Christchurch, New Zealand) and a custom proximal femoral component to link with the existing osseointegration component (Osseointegration Group of Australia, Sydney, Australia) were designed and implanted. The 18 month follow up of the custom hip components showed that the patient had Oxford hip scores that were markedly improved from pre-operatively. Knee joint heights were successfully restored to equal when the patient's prosthesis was attached. The patient describes feeling like “a normal person”, walks unaided for short distances and can ambulate longer distances with crutches. Advances in design and manufacture of implants have empowered surgeons to offer life improving treatments to patients with challenging anatomy. Using a custom acetabular tri-flange and osseointegration components is one possible solution to address symptomatic ipsilateral hip and knee arthropathy in the context of PFFD in adulthood

Aims. A flexed knee gait is common in patients with bilateral spastic cerebral palsy and occurs with increased age. There is a risk for the recurrence of a flexed knee gait when treated in childhood, and the aim of this study was to investigate whether multilevel procedures might also be undertaken in adulthood. Patients and Methods. At a mean of 22.9 months (standard deviation 12.9), after single event multi level surgery, 3D gait analysis was undertaken pre- and post-operatively for 37 adult patients with bilateral cerebral palsy and a fixed knee gait. Results. There was a significant improvement of indices and clinical and kinematic parameters including extension of the hip and knee, reduction of knee flexion at initial contact, reduction of minimum and mean knee flexion in the stance phase of gait, improved range of movement of the knee and a reduction of mean flexion of the hip in the stance phase. Genu recurvatum occurred in two patients (n = 3 legs, 4%) and an increase of pelvic tilt (> 5°) was found in 12 patients (n = 23 legs, 31%). Conclusion. Adult patients with bilateral cerebral palsy and a flexed knee gait benefit from multilevel surgery including hamstring lengthening. The risk of the occurence of genu recurvatum and increased pelvic tilt is lower than has been previously reported in children. Cite this article: Bone Joint J 2017;99-B:1256–64

Dynamic 2D sonography of the infant hip is a commonly used clinical procedure for developmental dysplasia of the hip (DDH) screening. It however has been found to be unreliable with some studies reporting associated misdiagnosis rates of up to 29%. In a recent systematic review, Charlton et al. examined dynamic ultrasound (US) screening for hip instability in the first six weeks after birth and found current best practices for such early screening techniques to be divergent between international institutions in terms of clinical scanning protocols. Such protocols include: the appropriate scanning plane and US probe position (e.g. coronal, transverse, lateral, anterior), DDH diagnostic metrics (e.g. femoral head coverage, alpha angle), appropriate patient age when scanning, and follow up procedures. To improve reliability of diagnosis and to help in standardizing diagnosis across different raters and health-centers, we propose an automated method for dynamically assessing hip instability using 3D US. 38 infant hips from 19 patients were scanned with B-mode 3D US by a paediatric orthopaedic surgeon and two technologists from the radiology department at a paediatric tertiary care centre. To quantify hip assessment, we proposed the use of femoral head coverage variability (ΔFHC3D) within 3D US volumes collected during a sequence of US scans (one at rest, and another with posterior stress applied to the joint as maneuvered during a dynamic assessment). We used phase symmetry image features to localize the ilium's vertical cortex and a random forest classifier to identify the location of the femoral head. The proposed ΔFHC3D provided good repeatability with an average test-retest ICC measure of 0.70 (95% confidence interval: 0.35 to 0.87, F(21,21) = 7.738, p<.001). The mean difference of ΔFHC3D measurements was 0.61% with a SD of 4.05%. Since the observed changes in ΔFHC3D start near 0% and range up to about 18% from stable to mildly unstable hips in this cohort, the mean difference and standard deviation of ΔFHC3D measurements observed suggest that the proposed metric and technique likely have sufficient resolution and repeatability to quantify differences in hip laxity. The long-term significance of this approach to evaluating dynamic assessments may lie in increasing early diagnostic accuracy in order to prevent dysplasia remaining undetected prior to manifesting itself in early adulthood joint disease

Although physical and ultrasound (US)-based screening for congenital deformities of the hip (developmental dysplasia of the hip, or DDH) is routinely performed in most countries, one of the most commonly performed manoeuvres done under ultrasound observation - dynamic assessment - has been shown to be relatively unreliable and is associated with significant misdiagnosis rates, on the order of 29%. Our overall research objective is to develop a quantitative method of assessing hip instability, which we hope will standardise diagnosis across different raters and health-centres, and may perhaps improve reliability of diagnosis. To quantify dynamic assessment, we propose to use the variability in femoral head coverage (FHC) measurements within multiple US scans collected during a dynamic assessment. In every US scan, we use our recently-developed automatic FHC measuring tool which leverages phase symmetry features to approximate vertical cortex of ilium and a random forest classifier to identify approximate location of the femoral head. Having estimated FHC in each scan, we estimate the change in FHC across all the US scans during a dynamic assessment and compare this change with variability of FHC found in previous studies. Our findings - in a dynamic assessment on an infant done by an orthopaedic surgeon, the femoral centre moved by up to 19% of its diameter during distraction, from 55% FHC to 74% FHC. This variability is similar to the variability of FHC in static US scans reported in previous studies, so the variability in FHC readings we found are not indicative of any subluxation or dislocation of the infant's femoral head. Our clinician's qualitative assessment concluded the hip to be normal and not indicative of instability. This suggests that our technique likely has sufficient resolution and repeatability to quantify differences in laxity between stable and unstable hips, although this presumption will have to be confirmed in a subsequent study with additional subjects. The long-term significance of this approach to evaluating dynamic assessments may lie in increasing early diagnostic sensitivity in order to prevent dysplasia remaining undetected prior to manifesting itself in early adulthood joint disease

Purpose. Factors contributing to chronic postoperative pain (CPOP) are poorly defined in young people and developmental considerations are poorly understood. With over 5 million children undergoing surgery yearly and 25% of adults referred to chronic pain clinics identifying surgery as the antecedent, there is a need to elucidate factors that contribute to CPOP in young people. The present study includes patients undergoing hip preservation surgery at a children's hospital. Methods. The HOOS and the SF-12 Health Survey were administered to 614 patients prior to surgery with 422 patients completing follow-up data (6-months, 1-year, and 2-years post-surgery). Examining baseline characteristics for those who completed follow-up versus those who did not, the only significant difference was that patients with more than one surgery were less likely to complete follow-up measures. Pain, quality of life, and functioning across time were examined using SAS PROC TRAJ procedure, a mixture model that estimates a regression model for each discrete group within the population. Longitudinal pain trajectories were empirically grouped. Baseline preoperative characteristics of age, gender, preoperative pain, quality of life, functioning, and mental health that could potentially distinguish trajectory groups were examined. Results. A three trajectory model (low pain, pain improvement, and high pain) emerged indicating three different treatment responders. Pain trajectory groups did not differ significantly on gender, presurgical age, BMI, prior hip surgery, surgical type, joint congruence, or Tönnis grade. The pain trajectory groups differed significantly from each other on presurgical pain, pain chronicity, quality of life, and functioning. Those in the high pain group and pain improvement group endorsed having presurgical depression at significantly higher rates and lower presurgical quality of life compared to those in the low pain group (p<.01). Similarly, those in the high pain group reported significantly worse functioning prior to surgery compared to the pain improvement group (p<.0001) and those in the low pain group (p<.0001). Conclusions. Chronic pain is a common problem and surgery can be a trigger. There may be a subset of hip preservation surgery patients who are predisposed to chronic pain independent of hip health. The results demonstrate the need for preoperative screening prior to hip preservation surgery. A prospective, interventional study to identify patients at risk for a poor pain trajectory is underway. Significance. This is the largest study to date to examine longitudinal pain, quality of life, and functioning trajectories for adolescent and young adults undergoing hip preservation surgery. Chronic pain is a serious public health concern, with the United States spending $19 billion annually on pain-related disability. Identifying predictors of poor long-term outcomes in adolescents and young adult samples with postoperative pain may prevent the development of chronic pain into adulthood as well as inform presurgical preparation and postsurgical care

The childhood hip conditions of Developmental Dysplasia, Legg-Calve-Perthes Disease and Slipped Capital Femoral Epiphysis have a wide spectrum of anatomical outcomes following childhood treatment; ranging from morphologies, which result in normal hip function throughout life, to severely deranged morphologies, which result in pain and disability during childhood and adolescence. Some of these outcomes are as a result of well-intentioned interventions that result in catastrophic complications. In 2003, after years of working with impingement complicating periacetabular osteotomies and building on the work of William Harris, Reinhold Ganz published his concepts of ‘cam’ and ‘pincer’ hip impingement, and how these anatomical morphologies resulted in hip arthritis in adulthood. These concepts of impingement were added to his previous published work on hip instability to provide a comprehensive theory describing how hip arthritis develops on the basis of anatomical abnormalities. Surgical techniques have been developed to address each of these morphological pathologies. Ganz's concepts of hip impingement and instability may be applied to severe paediatric hip deformities to direct reconstructive joint preserving surgery to both the femur and the acetabulum. Ganz's surgical approaches have also been refined for use in paediatric hip surgery to allow radical reshaping salvage osteotomies to be performed on the developing femoral head with minimal risk of the devastating consequence of vascular Necrosis

This study addresses the evolution of the orthopaedic management of patients with hypophosphatemic rickets, with the aim of providing skeletal mature aligned lower limbs, with minimal surgical insult. Methods. We describe a case series of 8 patients with hypophosphatemic rickets that highlight an evolution in practice over the last 8 years. Our initial treatment involved external fixation with circular frames, addressing both axial deformity and length. Two contralateral long bones were addressed simultaneously and surgery was conducted in early adolescence. Problems encountered were poor quality regenerate, requiring prolonged periods in external fixation, and often recurrence of deformity following frame removal. Minor deformity recurrence made planning for the often inevitable knee replacement difficulty. Our current management is that patients only start surgical correction once skeletal maturity has been reached, two contralateral axial long bone corrections are performed using CHAOS (computer hexapod-assisted orthopaedic surgery) procedures with IM nails and multiple osteotomies (occasionally locking plates are required). Patients are able to fully weight bear immediately post operatively, after a suitable recovery period the remaining bones can be corrected. Then lengthening can be considered once the osteotomies have fully consolidated by exchange nailing of the femur, for an IM lengthening nail. At this stage the patient is able to fully consider the risks and benefits of this final and potentially unnecessary procedure. Conclusions. Hypophosphotaemic rickets is a rare condition, both the disease and its management can have severe effects on patients both physically and psychologically, at Bristol we have developed an treatment pathway that we feel helps to minimize the physical and psychological effects of treatment, with the end result of aligned lower limbs, that should provide a good basis for arthroplasty surgery if required in adulthood

Aims

The aetiologies of common degenerative spine, hip, and knee pathologies are still not completely understood. Mechanical theories have suggested that those diseases are related to sagittal pelvic morphology and spinopelvic-femoral dynamics. The link between the most widely used parameter for sagittal pelvic morphology, pelvic incidence (PI), and the onset of degenerative lumbar, hip, and knee pathologies has not been studied in a large-scale setting.

Aims

To assess if older symptomatic children with club foot deformity differ in perceived disability and foot function during gait, depending on initial treatment with Ponseti or surgery, compared to a control group. Second aim was to investigate correlations between foot function during gait and perceived disability in this population.

Aims

Single-event multilevel surgery (SEMLS) has been used as an effective intervention in children with bilateral spastic cerebral palsy (BSCP) for 30 years. To date there is no evidence for SEMLS in adults with BSCP and the intervention remains focus of debate.

Adult presentation of neglected congenital muscular torticollis is rare. We report 12 patients with this condition who underwent a modified Ferkel’s release comprising a bipolar release of sternocleidomastoid with Z-lengthening. They had a mean age of 24 years (17 to 31) and were followed up for a minimum of two years. Post-operatively a cervical collar was applied for three weeks with intermittent supervised active assisted exercises for six weeks. Outcome was assessed using a modified Lee score and a Cheng and Tang score. The mean pre-operative rotational deficit was 8.25° (0° to 15°) and mean lateral flexion deficit was 20.42° (15° to 30°), which improved after treatment to a mean of 1.67° (0° to 5°) and 7.0° (4° to 14°) after treatment, respectively. According to the modified Lee scoring system, six patients had excellent results, two had good results and four had fair results, and using the Cheng and Tang score, eight patients had excellent results and four had good results.

Surgical management of adult patients with neglected congenital muscular torticollis using a modified Ferkel’s bipolar release gives excellent results. The range of neck movement and head tilt improved in all 12 patients and cosmesis improved in 11, despite the long-standing nature of the deformity.