Paediatric tibial shaft fractures: an instructional review for the FRCS exam.

This instructional review presents the literature and guidelines relevant to the classification, management and prognosis of paediatric tibial shaft fractures at a level appropriate for the FRCS exit examination in Trauma and Orthopaedic surgery.

Paediatric distal radius fractures: an instructional review for the FRCS examination.

An instructional review of the literature and guidelines relevant for the classification, management and prognosis of paediatric distal radius fractures. Aimed at the knowledge level required for the trauma and orthopaedic FRCS examination.

Comparison between a multicentre, collaborative, closed-loop audit assessing management of supracondylar fractures and the British Orthopaedic Association Standard for Trauma 11 (BOAST 11) guidelines.

Aims: Supracondylar fractures are the most frequently occurring paediatric fractures about the elbow and may be associated with a neurovascular injury. The British Orthopaedic Association Standards for Trauma 11 (BOAST 11) guidelines describe best practice for supracondylar fracture management. This study aimed to assess whether emergency departments in the United Kingdom adhere to BOAST 11 standard 1: a documented assessment, performed on presentation, must include the status of the radial pulse, digital capillary refill time, and the individual function of the radial, median (including the anterior interosseous), and ulnar nerves. Materials and Methods: Stage 1: We conducted a multicentre, retrospective audit of adherence to BOAST 11 standard 1. Data were collected from eight hospitals in the United Kingdom. A total of 433 children with Gartland type 2 or 3 supracondylar fractures were eligible for inclusion. A centrally created data collection sheet was used to guide objective analysis of whether BOAST 11 standard 1 was adhered to. Stage 2: We created a quality improvement proforma for use in emergency departments. This was piloted in one of the hospitals used in the primary audit and was re-audited using equivalent methodology. In all, 102 patients presenting between January 2016 and July 2017 were eligible for inclusion in the re-audit. Results: Stage 1: Of 433 patient notes audited, adherence to BOAST 11 standard 1 was between 201 (46%) and 232 (54%) for the motor and sensory function of the individual nerves specified, 318 (73%) for radial pulse, and 247 (57%) for digital capillary refill time. Stage 2: Of 102 patient notes audited, adherence to BOAST 11 standard 1 improved to between 72 (71%) and 80 (78%) for motor and sensory function of the nerves, to 84 (82%) for radial pulse, and to 82 (80%) for digital capillary refill time. Of the 102 case notes reviewed in stage 2, only 44 (43%) used the quality improvement proforma; when the proforma was used, adherence improved to between 40 (91%) and 43 (98%) throughout. Conclusion: Adherence to BOAST 11 standard 1 is poor in hospitals across the country. This is concerning as neurovascular deficit may be an indication for emergent surgery, and missed neurovascular injury can cause long-term, or even permanent, functional impairment. We present a simple proforma that improves adherence to this standard, can easily be implemented into emergency departments, and may improve patient safety. Cite this article: Bone Joint J 2018;100-B:346-51.

Is the E133K allele of VG5Q associated with Klippel-Trenaunay and other overgrowth syndromes?

BACKGROUND: It has been reported that the activating mutation, E133K, in the angiogenic factor VG5Q (formally named AGGF1) causes Klippel-Trenaunay Syndrome (KTS), a rare vascular disease associated with asymmetric overgrowth. This proposal followed from the observation that five out of 130 KTS patients were constitutionally heterozygous for VG5Q, E133K. OBJECTIVE: To explore the possibility that VG5Q, and specifically E133K, is implicated in other mosaic overgrowth syndromes. RESULTS: 24 patients were analysed for this sequence change. One patient was constitutionally heterozygous for E133K. Analysis of both parents revealed that the patient's mother, who was healthy, also carried E133K. An analysis of 275 healthy controls showed that 3.3% (9/275) of the population were carriers of E133K. CONCLUSIONS: The findings bring into question the assertion that VG5Q, E133K is a mutation and that it causes KTS.

Bilateral hip reconstruction in severe whole-body cerebral palsy: ten-year follow-up results.

The results of a functional, clinical and radiological study of 30 children (60 hips) with whole-body cerebral palsy were reviewed at a mean follow-up of 10.2 years (9.5 to 11). Correction of windsweep deformity of the hips was performed by bilateral simultaneous combined soft-tissue and bony surgery at a mean age of 7.7 years (3.1 to 12.2). We were able to recall 22 patients; five had died of unrelated causes and three were lost to follow-up. Evaluation involved interviews with patients/carers and clinical and radiological examination. The gross motor functional classification system was used to assess overall motor function and showed improvement in seven patients. Of the 12 patients thought to have pain pre-operatively, only one had pain post-operatively. Improved handling was reported in 18 of 22 patients (82%). Those with handling problems were attributed by the carers to growth of the patients. All patients/carers considered the procedure worthwhile. The range of hip movements improved, and the mean windsweep index improved from 50 pre-operatively to 36 at follow-up. The migration percentage and centre-edge angle were assessed on plain radiographs. Radiological containment improved, the mean migration percentage improved from 50 pre-operatively to 20 at follow-up and the mean centre-edge angle improved from -5 degrees to 29 degrees . No statistical difference was noted between the three-year and ten-year follow-up results, indicating that the improvements in clinical and radiological outcome had been maintained.

The challenges of a comprehensive surgical approach to Blount's disease.

PURPOSE: The purpose of this study is to evaluate a surgical technique that aims to address the multi-planar deformities occurring in early onset Blount's disease. METHODS: Seven patients (eight lower limbs) with early onset Blount's disease were treated with a surgical technique that used an external fixator to simultaneously address all aspects of deformity. Presenting radiographic parameters including mechanical axis deviation (MAD), lateral distal femoral angle (LDFA), medial proximal tibial angle (MPTA) and medial plateau angle (MPA) were compared with final follow-up and the unaffected limb. Data was also collected on surgical complications and functional outcomes. RESULTS: After a mean interval of 4.6 years (2.2 to 9), the MAD of the affected limb improved from 4.85 cm to 1.88 cm, MPTA from 43.58° to 75.46° and MPA from 43.41° to 20.71°. The mean time in the external fixator was 5.6 months (3.5 to 10.4). Recurrence was noted in three patients; two had evidence of nerve injury and one patient had premature fibular consolidation. CONCLUSION: The surgical technique described in this study has attempted to address the complex multiaxial deformity that is encountered in Blount's disease. It identifies difficulties and limitations in developing a comprehensive surgical protocol and highlights complications associated with this approach.

Analysis of a paediatric orthopaedic network: a six-year experience in the South West of the United Kingdom.

PURPOSE: We report our experience of a paediatric orthopaedic network, based on a 'hub and spoke' model, covering the South West of the United Kingdom. We identify the areas of most clinical concern, the effect of the network on stream-lining patient management and the benefits of the network to the clinician. METHODS: Prospective data were collected from the minutes of the bi-annual meetings of the South West Paediatric Network (UK) between November 2006 and May 2012. Data collected included details of the condition, previous treatment, problems, complications and advice given. Cases continue to be followed up in subsequent meetings. RESULTS: In total 131 cases were included and hip conditions were discussed most frequently (35.1%). The most common indication for discussion was to support and confirm the local management plan. In total, a mean average of 8.75 cases in total were presented per consultant during the study period, with those within ten to 12 years of starting independent practice presenting the majority. The clinical outcome for patients discussed in this forum was local provision of care in 74%, with transfer to the regional centre in 15.7%. Following advice, 14% of direct referrals were given appropriate advice and avoided a journey to the tertiary centre. CONCLUSION: The network has enabled local provision of care, reduced the burden of travel on patients and prevented unnecessary referrals to the tertiary centre. Additionally, it provides a mechanism to reassure and educate clinicians.

Validation of the multiplier method for leg-length predictions on a large European cohort and an assessment of the effect of physiological age on predictions.

PURPOSE: The Avon Longitudinal Study of Parents and Children (ALSPAC) prospective cohort was used to determine the accuracy of the Paley multiplier method for predicting leg length. Using menarche as a proxy, physiological age was then used to increase the accuracy of the multiplier. METHODS: Chronological age was corrected in female patients over the age of eight years with documented date of first menses. Final sub-ischial leg length and predicted final leg length were predicted for all data points. RESULTS: Good correlation was demonstrated between the Paley and ALSPAC data. The average error in prediction depended on the time of assessment, tending to improve as the child got older. It varied from 2.2 cm at the age of seven years to 1.8 cm at the age of 14 years. When chronological age was corrected, the accuracy of multiplier increased. Age correction of 50% improved multiplier predictions by up to 28%. CONCLUSION: There appears to have been no significant change in growth trajectories of the two populations who were chronologically separated by 40 years. While the Paley data were based on extracting trends from averaged data, the ALSPAC dataset provides descriptive statistics from which it is possible to compare populations and assess the accuracy of the multiplier method. The data suggest that the accuracy improves as the patient gets close to the average skeletal maturity but that results need to be interpreted in conjunction with a radiological assessment of the growth plates. The magnitude of the errors in prediction suggest that when using the multiplier, the clinician must remain vigilant and prepared to perform a contralateral epiphyseodisis if the prediction proves to be wrong. The data suggest a relationship between the multiplier and menarche. There appears to be a factorisation and when accounting for physiological age, one needs to correct by 50% of the difference between chronological and physiological age.

Computer hexapod-assisted orthopaedic surgery provides a predictable and safe method of femoral deformity correction.

AIMS: Computer hexapod assisted orthopaedic surgery (CHAOS), is a method to achieve the intra-operative correction of long bone deformities using a hexapod external fixator before definitive internal fixation with minimally invasive stabilisation techniques. The aims of this study were to determine the reliability of this method in a consecutive case series of patients undergoing femoral deformity correction, with a minimum six-month follow-up, to assess the complications and to define the ideal group of patients for whom this treatment is appropriate. PATIENTS AND METHODS: The medical records and radiographs of all patients who underwent CHAOS for femoral deformity at our institution between 2005 and 2011 were retrospectively reviewed. Records were available for all 55 consecutive procedures undertaken in 49 patients with a mean age of 35.6 years (10.9 to 75.3) at the time of surgery. RESULTS: Patients were assessed at a mean interval of 44 months (6 to 90) following surgery. The indications were broad; the most common were vitamin D resistant rickets (n = 10), growth plate arrest (n = 6) and post-traumatic deformity (n = 20). Multi-planar correction was required in 33 cases. A single level osteotomy was performed in 43 cases. Locking plates were used to stabilise the osteotomy in 33 cases and intramedullary nails in the remainder. Complications included two nonunions, one death, one below-knee deep vein thrombosis, one deep infection and one revision procedure due to initial under-correction. There were no neurovascular injuries or incidence of compartment syndrome. CONCLUSION: This is the largest reported series of femoral deformity corrections using the CHAOS technique. This series demonstrates that precise intra-operative realignment is possible with a hexapod external fixator prior to definitive stabilisation with contemporary internal fixation. This combination allows reproducible correction of complex femoral deformity from a wide variety of diagnoses and age range with a low complication rate. Cite this article: Bone Joint J 2017;99-B:283-8.

Improving patient outcomes in fibrous dysplasia/McCune-Albright syndrome: an international multidisciplinary workshop to inform an international partnership.

To develop consensus on improving the management of patients, we convened an international workshop involving patients, clinicians, and researchers. Key findings included the diagnostic delay and variability in subsequent management with agreement to develop an international natural history study. We now invite other stakeholders to join the partnership. PURPOSE: The aim of this study was develop a consensus on how to improve the management of patients with fibrous dysplasia and prioritize areas for research METHODS: An international workshop was held over 3 days involving patients, clinicians, and researchers. Each day had a combination of formal presentations and facilitated discussions that focused on clinical pathways and research. RESULTS: The patient workshop day highlighted the variability of patients' experience in getting a diagnosis, the knowledge of general clinical staff, and understanding long-term outcomes. The research workshop prioritized collaborations that improved understanding of the contemporary natural history of fibrous dysplasia/McCune-Albright syndrome (FD/MAS). The clinical workshop outlined the key issues around diagnostics, assessment of severity, treatment and monitoring of patients. CONCLUSIONS: In spite of advances in understanding the genetic and molecular underpinnings of fibrous dysplasia/McCune-Albright syndrome, clinical management remains a challenge. From the workshop, a consensus was reached to create an international, multi-stakeholder partnership to advance research and clinical care in FD/MAS. We invite other stakeholders to join the partnership.

Osteofibrous dysplasia of the tibia. Is there a need for a radical surgical approach?

Osteofibrous dysplasia is an unusual developmental condition of childhood, which almost exclusively affects the tibia. It is thought to follow a slowly progressive course and to stabilise after skeletal maturity. The possible link with adamantinoma is controversial and some authors believe that they are part of one histological process. We retrospectively reviewed 16 patients who were diagnosed as having osteofibrous dysplasia initially or on the final histological examination. Their management was diverse, depending on the severity of symptoms and the extent of the lesion. Definitive (extraperiosteal) surgery was localised "shark-bite" excision for small lesions in five patients. Extensive lesions were treated by segmental excision and fibular autograft in six patients, external fixation and bone transport in four and proximal tibial replacement in one. One patient who had a fibular autograft required further excision and bone transport for recurrence. Six initially underwent curettage and all had recurrence. There were no recurrences after localised extraperiosteal excision or bone transport. There were three confirmed cases of adamantinoma. The relevant literature is reviewed. We recommend extraperiosteal excision in all cases of osteofibrous dysplasia, with segmental excision and reconstruction in more extensive lesions.

Quantifying the kinematics of natural and prosthetic knee flexion.

A new method has been developed for quantifying knee kinematics during flexion. This method was used to measure knee kinematics from lateral radiographs taken at different angles of flexion with the two femoral condyles superimposed in each image, thus standardizing the plane of flexion-extension. When applied to the radiogaphs of five healthy male volunteers (age range 21-26 years), it showed that flexion was accompanied by translation between the articular surfaces. Knee kinematics were also measured in five patients after total knee replacement (TKR) surgery with a Kinemax Modular Total Knee prosthesis (Howmedica, Warsaw, Indiana). In the TKR patients, a translation was detected in three out of the five patients. This indicates that the prosthesis is capable of restoring normal kinematics, but does not always do so.

Osteomyelitis of the scaphoid with sequestration of the primary ossification centre.

An 8-year-old boy presented with Staphylococcus aureus osteomyelitis affecting the left (non-dominant) scaphoid. Surgical drainage resulted in the expulsion of the primary ossification centre as a sequestrum. Seven years later the wrist function was minimally impaired and X-rays showed complete ossification of the cartilage remnant with a relatively normal scaphoid.

Failure of trochanteric osteotomy in total hip replacement: a comparison of two methods of reattachment.

A retrospective study was undertaken to assess the rate of trochanteric union after a primary Charnley total hip replacement. In one group the trochanter was reattached with Wrobleski spring wire, and in the second group with a Dall-Miles clamp. Non-union occurred in 29% of each group. The high rate of failure may have implications for morbidity and function. Alternative surgical approaches for total hip replacement should be considered.

A method for the kinematic evaluation of the knee following anterior cruciate ligament injury and reconstruction.

A quantitative method for assessing the kinematics of the knee in the sagittal plane has been developed in order to evaluate the role of the anterior cruciate ligament following injury and reconstruction. Measurements were made on a series of lateral radiographs obtained at different angles of flexion with the limb weight-bearing and the foot and ankle rotated so that the condyles of the femur overlapped. The kinematics of the joint were then defined by recording the path of the tip of the medial tibial spine as flexion proceeded, using a coordinate system based on the femur. This method overcomes the problems inherent in quantifying knee kinematics by using the pathway of the centre of rotation. In an amputated knee, tibial positions could be specified to within approximately 1.2 mm. There were no significant differences between results obtained at the beginning and end of a six month period for the normal knees of two patients; the standard deviation of the measured tibial positions was approximately 1.6 mm.

Walking problems in young children.

This article attempts to define normal development throughout childhood and provide straightforward advice in the physical assessment of the child in this situation. It also attempts to outline the groups of conditions that lead to walking abnormalities and highlight the salient physical features and special investigations that are required to make these diagnoses with confidence.

A prejudiced view.

The contemporary practice of orthopaedic surgery requires an evidence-based approach to support all medical and surgical interventions. In this essay, the author expresses a forthright, personal and somewhat prejudiced appeal to retain the legitimacy of clinical decision making in conditions that are rare, contain multiple variables, have a solution that generally works or has an unpredictable course.

Management of congenital femoral deficiency.

Abnormalities that affect the developing human limb range from subtle differences that are of no functional or cosmetic consequence, to complete absence of all limbs. Advances in non-invasive imaging have improved antenatal detection of these conditions and has broadened the options available to prospective parents. This paper considers congenital femoral deficiency and includes an overview of limb bud development and the mechanisms involved in normal growth. The use of ultrasound in antenatal screening and the clinical and radiological features in childhood will be discussed in addition to management including surgical reconstruction and prosthetic use. This should be one component of a multidisciplinary approach to provide the parents of an affected child with balanced information on functional prognosis, management choices and implications for future pregnancies.

High-energy open tibial fractures in children: treatment with a programmable circular external fixator.

Between 2005 and 2010 ten consecutive children with high-energy open diaphyseal tibial fractures were treated by early reduction and application of a programmable circular external fixator. They were all male with a mean age of 11.5 years (5.2 to 15.4), and they were followed for a mean of 34.5 months (6 to 77). Full weight-bearing was allowed immediately post-operatively. The mean time from application to removal of the frame was 16 weeks (12 to 21). The mean deformity following removal of the frame was 0.15° (0° to 1.5°) of coronal angulation, 0.2° (0° to 2°) sagittal angulation, 1.1 mm (0 to 10) coronal translation, and 0.5 mm (0 to 2) sagittal translation. All patients achieved consolidated bony union and satisfactory wound healing. There were no cases of delayed or nonunion, compartment syndrome or neurovascular injury. Four patients had a mild superficial pin site infection; all settled with a single course of oral antibiotics. No patient had a deep infection or re-fracture following removal of the frame. The time to union was comparable with, or better than, other published methods of stabilisation for these injuries. The stable fixator configuration not only facilitates management of the accompanying soft-tissue injury but enables anatomical post-injury alignment, which is important in view of the limited remodelling potential of the tibia in children aged > ten years. Where appropriate expertise exists, we recommend this technique for the management of high-energy open tibial fractures in children.

Angular deformity of the ankle with sparing of the distal fibula following meningococcal septicaemia: a case series involving 14 ankles in ten children.

Progressive angular deformity of an extremity due to differential physeal arrest is the most common late orthopaedic sequela following meningococcal septicaemia in childhood. A total of ten patients (14 ankles) with distal tibial physeal arrest as a consequence of meningococcal septicaemia have been reviewed. Radiological analysis of their ankles has demonstrated a distinct pattern of deformity. In 13 of 14 cases the distal fibular physis was unaffected and continued distal fibular growth contributed to a varus deformity. We recommend that surgical management should take account of this consistent finding during the correction of these deformities.