Femur Fractures in 5 Individuals With Pantothenate Kinase-associated Neurodegeneration: The Role of Dystonia and Suggested Management.

BACKGROUND: Pantothenate kinase-associated neurodegeneration (PKAN) is a rare, neurodegenerative disorder that manifests with progressive loss of ambulation and refractory dystonia, especially in the early-onset classic form. This leads to osteopenia and stress on long bones, which pose an increased risk of atraumatic femur fractures. The purpose of this study is to describe the unique challenges in managing femur fractures in PKAN and the effect of disease manifestations on surgical outcomes. METHODS: A retrospective case review was conducted on 5 patients (ages 10 to 20 y) with PKAN with a femur fracture requiring surgical intervention. Data regarding initial presentation, surgical treatment, complications, and outcomes were obtained. RESULTS: All patients were non-ambulatory, with 4 of 5 patients sustaining an atraumatic femur fracture in the setting of dystonia episode. One patient had an additional contralateral acetabular fracture. Postoperatively, 4 of the 5 patients sustained orthopaedic complications requiring surgical revision, with 3 of these secondary to dystonia. Overall, 4 required prolonged hospitalization in the setting of refractory dystonia. CONCLUSION: Femur fractures in PKAN present distinct challenges for successful outcomes. A rigid intramedullary rod with proximal and distal interlocking screws is most protective against surgical complications associated with refractory dystonia occurring during the postoperative period. Multidisciplinary planning for postoperative care is essential and may include aggressive sedation and pain management to decrease the risk of subsequent injuries or complications. LEVEL OF EVIDENCE: Level IV.

Quality of life of children during distraction osteogenesis: a comparison between intramedullary magnetic lengthening nails and external fixators.

BACKGROUND: Distraction osteogenesis is a very demanding process. For decades, external fixation was the only reliable option for gradual deformity correction. Recently, intramedullary magnetic nails have gained popularity. This research aimed to assess the quality of life in children during gradual deformity correction using intramedullary lengthening nails compared to external fixation. METHOD: Prospective analysis included children who had gradual lower limb deformity correction between 2017 and 2019. Group A included children who had magnetic lengthening nails; patients in group B had external fixation devices. Child health utility 9D (CHU- 9D) and EuroQol 5D youth (EQ- 5D-Y) were used to measure the quality of life at fixed points during the distraction osteogenesis process. The results were used to calculate the utility at each milestone and the overall quality of life adjusted years (QALYs). RESULTS: Thirty-four children were recruited, group A had 16 patients, whilst group B had 18 patients. The average ages were 16.0 years and 14.7 years for groups A and B, respectively. Group A patients reported significantly better utility compared to group B. This was observed during all stages of treatment (P = 0.00016). QALYs were better for group A (0.44) compared to group B (0.34) (P < 0.0001). CONCLUSION: The quality of life was generally better in group A compared to group B. In most patients, the health utility progressively improved throughout treatment. In the same way, QALYs were better with the lengthening nails compared to external fixators. The magnetic lengthening devices (PRECICE nails) which were used in this research were recently relabelled to restrict their applications in children; this study was conducted before these restrictions.

The (extended) achondroplasia foramen magnum score has good observer reliability.

BACKGROUND: Achondroplasia is the most common skeletal dysplasia. A significant complication is foramen magnum stenosis. When severe, compression of the spinal cord may result in sleep apnea, sudden respiratory arrest and death. To avoid complications, surgical decompression of the craniocervical junction is offered in at-risk cases. However, practice varies among centres. To standardize magnetic resonance (MR) reporting, the achondroplasia foramen magnum score was recently developed. The reliability of the score has not been assessed. OBJECTIVE: To assess the interobserver reliability of the achondroplasia foramen magnum score. MATERIALS AND METHODS: Base of skull imaging of children with achondroplasia under the care of Sheffield Children's Hospital was retrospectively and independently reviewed by four observers using the achondroplasia foramen magnum score. Two-way random-effects intraclass coefficient (ICC) was used to assess inter- and intra-observer reliability. RESULTS: Forty-nine eligible cases and five controls were included. Of these, 10 were scored normal, 17 had a median score of 1 (mild narrowing), 11 had a median score of 2 (effacement of cerebral spinal fluid), 10 had a score of 3 (compression of cord) and 6 had a median score of 4 (cord myelopathic change). Interobserver ICC was 0.72 (95% confidence interval = 0.62-0.81). Intra-observer ICC ranged from 0.60 to 0.86. Reasons for reader disagreement included flow void artefact, subtle T2 cord signal and myelopathic T2 cord change disproportionate to canal narrowing. CONCLUSION: The achondroplasia foramen magnum score has good interobserver reliability. Imaging features leading to interobserver disagreement have been identified. Further research is required to prospectively validate the score against clinical outcomes.

Femoral Lengthening in Children-A Comparison Between Magnetic Intramedullary Lengthening Nails and External Fixators.

BACKGROUND: Femoral lengthening can be achieved using external fixators or intramedullary lengthening nails. The purpose of this research was to compare the outcome of femoral lengthening in children using PRECICE magnetic lengthening nails with lengthening external fixators. METHODS: Retrospective analysis of 50 children who had femoral lengthening. Group A included patients who had lengthening with external fixation, patients in Group B had lengthening with PRECICE intramedullary lengthening nails. Each group included 25 patients. The sample strictly included children aged between 11 and 17 years. Patients in each group were matched according to age and indication for lengthening whether congenital or acquired conditions. The outcomes focused on the ability to achieve target length, healing index, residual malalignment, length of hospitalization following the osteotomy surgery, and encountered complications. RESULTS: Mean patient age was 14.7 years for each group. The length gain was 42±12 mm for Group A and 41.6±8 mm for Group B (P=0.84). Lengthening nails achieved the target length more accurately compared with external fixation (P=0.017). The healing index was significantly higher in group A with 53.2±19 days/cm compared with 40.2±14 days/cm in group B (P=0.03). Group A had significantly higher complications than group B (P<0.0001). There was no statistically significant difference in the final coronal malalignment between the 2 groups (P=0.2). The mean length of stay was 9.2±5.8 days for group A and 4.2±3.3 days for group B (P=0.0005). CONCLUSION: Magnetic lengthening nails are clinically effective for femoral lengthening in the pediatric population. Compared with external fixation, healing index and complications were more favorable with PRECICE nails. Further research is required to study the cost-effectiveness of this technique. LEVEL OF EVIDENCE: Level IV-case series.

Femoral lengthening in young patients: An evidence-based comparison between motorized lengthening nails and external fixation.

BACKGROUND: Femoral lengthening is a procedure of great importance in the treatment of congenital and acquired limb deficiencies. Technological advances have led to the latest designs of fully implantable motorized intramedullary lengthening nails. The use of these nails has increased over the last few years. AIM: To review and critically appraise the literature comparing the outcome of femoral lengthening in children using intramedullary motorized lengthening nails to external fixation. METHODS: Electronic databases (MEDLINE, CINAHL, EMBASE, Cochrane) were systematically searched in November 2019 for studies comparing the outcome of femoral lengthening in children using magnetic lengthening nails and external fixation. The outcomes included amount of gained length, healing index, complications and patient reported outcomes. RESULTS: Of the 452 identified studies, only two (retrospective and non-randomized) met the inclusion criteria. A total of 91 femora were included. In both studies, the age of patients treated with nails ranged from 15 to 21 years compared to 9 to 15 years for patients in the external fixation group. Both devices achieved the target length. Prevalence of adverse events was less in the nail (60%-73%) than in the external fixation (81%-100%) group. None of the studies presented patient reported outcomes. CONCLUSION: The clinical effectiveness of motorized nails is equivalent or superior to external fixation for femoral lengthening in young patients. The available literature is limited and does not provide evidence on patient quality of life or cost effectiveness of the interventions.

Deformity Correction, Surgical Stabilisation and Limb Length Equalisation in Patients with Fibrous Dysplasia: A 20-year Experience.

INTRODUCTION: Fibrous dysplasia (FD) of bone can be present with pain, deformity and pathological fractures. Management is both medical and surgical. Little literature exists on the surgical management of both monostotic and polyostotic FD. We present our experience of limb reconstruction surgery in this pathological group of bone disease. MATERIALS AND METHODS: A retrospective cohort of children who underwent limb reconstruction surgery at a single high-volume paediatric centre was identified from a prospective database. Case notes and radiographs were reviewed. Surgical techniques, outcomes and difficulties were explored. RESULTS: Twenty-one patients were identified aged between 7 and 13 at presentation to the limb reconstruction unit. Eleven were female, nine had McCune-Albright syndrome, seven had polyostotic FD and five had monostotic. Proximal femoral varus procurvatum deformity was the most common site requiring surgical intervention. The distal femur, tibia, humerus and forearm were also treated.Methods include deformity correction with intramedullary fixation including endo-exo-endo techniques, elastic nailing, guided growth, circular fixator technique and fixator-assisted plating. Correction of deformity and leg length discrepancies was common.The osteotomies went on to heal with no nonunions or delayed healing. We encountered secondary deformity at distal end of nails as the children grew as expected. These were managed with revision nailing techniques and in some cases external fixation. There was one implant failure, which did not require revision surgery. CONCLUSION: The surgical management of pathological bone disease is challenging. Corrective osteotomies with intramedullary fixation can be very successful if appropriate limb reconstruction principles are adhered to. Deformity correction, guided growth and lengthening can all be successfully achieved in bone affected by FD. Polyostotic FD can be present with secondary deformities, and these can be difficult to manage. HOW TO CITE THIS ARTICLE: Hampton MJ, Weston-Simmons S, Giles SN, et al. Deformity Correction, Surgical Stabilisation and Limb Length Equalisation in Patients with Fibrous Dysplasia: A 20-year Experience. Strategies Trauma Limb Reconstr 2021;16(1):41-45.

Mechanical Analysis of Explanted Telescopic Rods in the Management of Osteogenesis Imperfecta: A Multicenter Study.

BACKGROUND: Telescopic rods in the management of osteogenesis imperfecta fail frequently. This could be attributed to technical errors, rod design, and rod structure. We aimed to analyze the mechanical properties and tribology of explanted male and female components to identify effects of in vivo telescoping that may relate to observed patterns of successful telescoping or failure. METHODS: Recruitment took place at 3 of the 4 English centers for osteogenesis imperfecta. Twenty-five rods explanted for growth or failure during revision to a new rod were analyzed in terms of clinical indication and prerevision imaging to identify if there was a technical mode of failure. Laboratory analysis was performed using optical and scanning electrical microscopy, radiograph diffraction analysis, hardness test, bending test, and energy-dispersive x-ray spectroscopy. RESULTS: All implants tested were of high-grade stainless steel. Female components had inferior strength [mean Vickers hardness property (HV0.3) at 0.3 to 313 kg] in comparison to male components (HV0.3 406) due to different techniques of manufacture. Female rods also had a higher wear coefficient (7.89×10-12 m3/N/m3) than the male rods (6.46×10-12 m3/N/m3). Abrasive wear, shear deformation, scratches, and wear debris were identified in all rods. Male and female components displayed corrosion contributing to adhesive wear. Intraoperatively cut rods, particularly the female components, had irregular ends leading to more wear. CONCLUSIONS: Current manufacturing techniques result in inferior material strength in female components compared with males, which combined with wear patterns is likely to lead to implant failure. Intraoperative cutting of rods may increase risk of failure due to wear. Considering techniques to improve strength as well as design in new implants may lead to better outcomes. LEVELS OF EVIDENCE: Level IV-cross-sectional study.

Pelvic Support Hip Reconstruction with Internal Devices: An Alternative to Ilizarov Hip Reconstruction.

AIM AND OBJECTIVE: Ilizarov hip reconstruction (IHR) is a traditional method of salvaging chronic adolescent problem hips but faces practical issues from external fixators leading to reduced compliance. We present the same reconstruction procedure using only internal devices with a modification in the technique and review early results. MATERIALS AND METHODS: We retrospectively evaluated eight patients between 2014 and 2017 with chronic painful hips treated by two-stage reconstruction; stage I included femoral head resection and pelvic support osteotomy using double plating, whereas stage II comprised distal femoral osteotomy avoiding varus followed by the insertion of a retrograde magnetic nail for postoperative lengthening. Patients continued physiotherapy postoperatively while protecting from early weight-bearing. RESULTS: At a mean follow-up of 19 months (range, 6-36), all osteotomies healed with a bone healing index of 47 days/cm (range, 30-72). Pain improved from 8.3 (range, 7-9) to 2 (range, 0-6) while the limb length discrepancy got corrected from 4.3 cm (range, 3-5) to 1.4 cm (range, 0-2.5) at the final follow-up. Trendelenburg sign was eliminated in three patients and delayed in five patients. No examples of infection or permanent knee stiffness were noted. One patient had plate breakage due to mechanical fall, and another patient had 35 mm of lateral mechanical axis deviation (MAD) requiring corrective osteotomy. CONCLUSION: Pelvic support hip reconstruction with exclusive internal devices is a technique in evolution with encouraging early results. It avoids common complications of external fixators and facilitates quick rehabilitation of joints. Refraining from distal varus can effectively eliminate Trendelenburg gait, although with some degree of lateral MAD. Unlike external fixation where there is a possibility of gradual correction, this staged procedure of internal fixation is technically demanding with a learning curve. CLINICAL SIGNIFICANCE: Pelvic support hip reconstruction performed by internal implants is a viable alternative to Ilizarov hip reconstruction with potential benefits. HOW TO CITE THIS ARTICLE: Metikala S, Kurian BT, Madan SS, et al. Pelvic Support Hip Reconstruction with Internal Devices: An Alternative to Ilizarov Hip Reconstruction. Strategies Trauma Limb Reconstr 2020;15(1):34-40.

Pelvic Support Hip Reconstruction with Internal Devices: An Alternative to Ilizarov Hip Reconstruction.

AIM AND OBJECTIVE: Ilizarov hip reconstruction (IHR) is a traditional method of salvaging chronic adolescent problem hips but faces practical problems from external fixators leading to reduced compliance. We present the same reconstruction utilising only internal devices with a modification in technique and aim to review early results. MATERIALS AND METHODS: We retrospectively evaluated eight patients between 2014 and 2017 with chronic painful hips treated by a two-stage reconstruction; stage 1 included femoral head resection and pelvic support osteotomy using double plating, while stage 2 comprised distal femoral osteotomy avoiding varus followed by insertion of retrograde magnetic nail for postoperative lengthening. Patients continued physiotherapy postoperatively while protecting from early weight-bearing. RESULTS: At mean follow-up of 19 months (range 6-36), all osteotomies healed with bone healing index of 47 days/cm (range 30-72). Pain improved from 8.3 (range 7-9) to 2 (range 0-6), while limb length discrepancy got corrected from 4.3 cm (range 3-5) to 1.4 cm (range 0-2.5) at final follow-up. Trendelenburg sign was eliminated in three and delayed in five. No examples of infection or permanent knee stiffness were noted. One patient had plates breakage due to mechanical fall and one had 35 mm of lateral mechanical axis deviation requiring corrective osteotomy. CONCLUSION: Pelvic support hip reconstruction with exclusive internal devices is a technique in evolution with encouraging early results. It avoids common complications of external fixators and facilitates quick rehabilitation of joints. Refraining from distal varus can effectively eliminate Trendelenburg gait, albeit with some degree of lateral mechanical axis deviation. Unlike external fixation where there is a possibility of gradual correction, this staged procedure of internal fixation is technically demanding with a learning curve. CLINICAL SIGNIFICANCE: Pelvic support hip reconstruction performed by internal implants is a viable alternative to IHR with potential benefits. HOW TO CITE THIS ARTICLE: Metikala S, Kurian BT, Madan SS, et al. Pelvic Support Hip Reconstruction with Internal Devices: An Alternative to Ilizarov Hip Reconstruction. Strategies Trauma Limb Reconstr 2020;15(2):91-97.

Correction of Bowleg Deformity in Achondroplasia through Combined Bony Realignment and Lateral Collateral Ligament Tightening.

INTRODUCTION: Achondroplasia is one of the most common osteochondrodysplasias with an incidence of 1 in 26,000 live births. Bowing of lower limbs can cause significant morbidity in this population. The use of the Ilizarov external fixator to tighten collateral ligaments of the knee in children has not been reported in the literature. We report the technique and early results of lateral collateral ligament (LCL) tightening with correction of genu varum in children with achondroplasia. MATERIALS AND METHODS: A retrospective review of children with achondroplasia presenting with bowleg deformity who were treated by corrective osteotomy and LCL tightening was conducted. Between 1998 and 2003, 12 patients (24 limb segments) underwent this procedure and were included in the study. All patients had grade III LCL laxity preoperatively. Pre- and postoperative anteroposterior standing mechanical axis radiographs were evaluated. The final outcome was graded using the grading system of Paley et al. RESULTS: All patients had bilateral corrections. The Ilizarov external fixator was used in 10 patients and the Orthofix limb reconstruction system in 2 patients. The bony realignment was achieved through monofocal or bifocal tibial osteotomies. The LCL was tightened in all limb segments using the Paley's type II strategy. The final result was graded as excellent in 20 limb segments and good in 4 limb segments. One patient developed transient common peroneal nerve palsy, four developed grade II pin site infections, and there was premature consolidation of the tibial regenerate in one patient. HOW TO CITE THIS ARTICLE: Kurian BT, Belthur MV, Jones S, et al. Correction of Bowleg Deformity in Achondroplasia through Combined Bony Realignment and Lateral Collateral Ligament Tightening. Strategies Trauma Limb Reconstr 2019;14(3):132-138.

Geroderma osteodysplasticum: Histological features and the role of panel-based exome sequencing in diagnosis.

Geroderma osteodysplasticum (GO) has clinical and histological features that overlap with other causes of wrinkly skin. Here we present the case of a child diagnosed with GO following exome sequencing of a panel of genes covering the wide differential diagnosis. The histological features of the overlapping conditions are presented, highlighting the utility of panel testing for conditions of this type. This is relevant to many genetic conditions and can influence ongoing management as exemplified by this case.

The use of recombinant morphogenic protein-2(rhBMP-2) in children undergoing revision surgery for persistent non-union.

The purpose of the study was to evaluate the safety and efficacy with the use of BMP-2 for treating persistent non-unions in children with underlying complex conditions. Between October 2006 and November 2010 in our unit, 15 patients were treated with rhBMP-2 to enhance bone union. There were nine females and six males with a mean age of 9.5 years (range 4-15) at time of surgery. Seventy-five per cent of the patients required revision of internal fixation with insertion of rhBMP-2 to the non-union site, and the reminder had freshening of the non-union site with rhBMP-2 application. Patients had undergone a mean of 2 (1-5) operations prior to implantation of rhBMP-2. All the patients in the study group were available for review with mean follow-up of 44 months (range 21-70). The mean time to union was 16 weeks (range 10-28 weeks). No adverse events related to BMP-2 application were noted in our study group. Healing occurred clinically and radiographically in 16 of the 17 sites. Our study demonstrates that BMP-2 enhances healing of the persistent non-unions without any adverse events.

Zoledronate Attenuates Accumulation of DNA Damage in Mesenchymal Stem Cells and Protects Their Function.

Mesenchymal stem cells (MSCs) undergo a decline in function following ex vivo expansion and exposure to irradiation. This has been associated with accumulation of DNA damage and has important implications for tissue engineering approaches or in patients receiving radiotherapy. Therefore, interventions, which limit accumulation of DNA damage in MSC, are of clinical significance. We were intrigued by findings showing that zoledronate (ZOL), an anti-resorptive nitrogen containing bisphosphonate, significantly extended survival in patients affected by osteoporosis. The effect was too large to be simply due to the prevention of fractures. Moreover, in combination with statins, it extended the lifespan in a mouse model of Hutchinson Gilford Progeria Syndrome. Therefore, we asked whether ZOL was able to extend the lifespan of human MSC and whether this was due to reduced accumulation of DNA damage, one of the important mechanisms of aging. Here, we show that this was the case both following expansion and irradiation, preserving their ability to proliferate and differentiate in vitro. In addition, administration of ZOL before irradiation protected the survival of mesenchymal progenitors in mice. Through mechanistic studies, we were able to show that inhibition of mTOR signaling, a pathway involved in longevity and cancer, was responsible for these effects. Our data open up new opportunities to protect MSC from the side effects of radiotherapy in cancer patients and during ex vivo expansion for regenerative medicine approaches. Given that ZOL is already in clinical use with a good safety profile, these opportunities can be readily translated for patient benefit.

Osteochondroma arising from a lumbar facet joint in a 16-year-old.

Osteochondromas are benign tumours of bony or cartilaginous origin, which may be solitary or multiple. They are rare in the axial skeleton and have previously been reported to arise from facet joints only in four cases in the English literature. We report the case of a 16-year-old girl who presented with a palpable bony lump and a short history of back pain. Imaging showed a bony lesion arising from a left-sided facet joint in the lumbar spine. Following excision biopsy, there was complete resolution of symptoms. The literature on the origin and management of spinal osteochondromas is discussed.

Management of paediatric tibial fractures using two types of circular external fixator: Taylor spatial frame and Ilizarov circular fixator.

BACKGROUND: The use of circular fixators for the treatment of tibial fractures is well established in the literature. The aim of this study was to compare the Ilizarov circular fixator (ICF) with the Taylor spatial frame (TSF) in terms of treatment results in consecutive patients with tibial fractures that required operative management. METHOD: A retrospective analysis of patient records and radiographs was performed to obtain patient data, information on injury sustained, the operative technique used, time duration in frame, healing time and complications of treatment. The minimum follow-up was 24 months. RESULTS: Ten patients were treated with ICF between 2000 and 2005, while 15 patients have been treated with TSF since 2005. Two of the 10 treated with ICF and 5 of the 15 treated with TSF were open fractures. All patients went on to achieve complete union. Mean duration in the frame was 12.7 weeks for ICF and 14.8 weeks for the TSF group. Two patients in the TSF group had delayed union and required additional procedures including adjustment of fixator and bone grafting. There was one malunion in the TSF group that required osteotomy and reapplication of frame. There were seven and nine pin-site infections in the ICF and TSF groups, respectively, all of which responded to antibiotics. There were no refractures in either group. CONCLUSION: In an appropriate patient, both types of circular fixator are equally effective but have different characteristics, with TSF allowing for postoperative deformity correction. Of concern are the two cases of delayed union in the TSF group, all in patients with high-energy injuries. We feel another larger study is required to provide further clarity in this matter. LEVEL OF EVIDENCE: Level II-comparative study.

The oblique plane deformity in slipped capital femoral epiphysis.

BACKGROUND: Slipped capital femoral epiphysis (SCFE) is commonly treated with in situ pinning. However, a severe slip may not be suitable for in situ pinning because the required screw trajectory is such that it risks perforating the posterior cortex and damaging the remaining blood supply to the capital epiphysis. In such cases, an anteriorly placed screw may also cause impingement. It is also possible to underestimate the severity of the slip using conventional radiographs. The aim of this study was to describe and evaluate a novel method for calculating the true deformity in SCFE and to assess the interobserver and intraobserver reliability of this technique. METHODS: We selected 20 patients with varying severity of SCFE who presented to our institution. Cross-sectional imaging [either axial computed tomography (CT) scans or magnetic resonance imaging (MRI) scans] and anteroposterior (AP) pelvis radiographs were assessed by four reviewers with varying levels of experience on two occasions. The degree of slip on the axial image and on the AP pelvis radiographs were measured and, from this, the oblique plane deformity was calculated using the method as popularised by Paley. The intraclass correlation coefficient (ICC) was calculated to determine the interobserver and intraobserver reliabilities between and amongst the raters. RESULTS: The interobserver reliability for the calculated oblique plane deformity in SCFE ICC was 0.947 [95 % confidence interval (CI) 0.90-0.98] and the intraobserver reliability for the calculated oblique plane deformity of individual raters ranged from 0.81 to 0.94. The deformity in the oblique plane was always greater than the deformity measured in the axial or the coronal plane alone. CONCLUSION: This method for calculating the true deformity in SCFE has excellent interobserver and intraobserver reliability and can be used to guide treatment options. This technique is a reliable and reproducible method for assessing the degree of deformity in SCFE. It may help orthopaedic surgeons with varying degrees of experience to identify which hips are suitable for in situ pinning and those which require surgical dislocation and anatomical reduction, given that plain radiographs in a single plane will underestimate the true deformity in the oblique plane. LEVEL OF EVIDENCE: Level II diagnostic study.

Type II Monteggia injury in a toddler: an unusual presentation.

Monteggia type fracture dislocations of the elbow are a common occurrence in children. Bado types I and III are the most common types comprising 70 and 23%, respectively, and type II injuries account only for 6%. We present an interesting case of Monteggia type II fracture dislocation in an 11-month-old child, which has not been described before in a child younger than 11 years of age. Any ulnar fracture, especially in a child, should be evaluated carefully. Closed reduction is the mainstay of treatment in Monteggia type II fracture dislocation in children.

Screening for spinal stenosis in achondroplastic patients undergoing limb lengthening.

The need for a screening programme for spinal stenosis in children with achondroplasia undergoing limb lengthening was identified in a tertiary limb reconstruction service. The aim of this study was to evaluate whether screening would identify the 'at risk' group. A total of 26 achondroplastic patients underwent our screening programme. Canal diameters were measured by MRI. Neurosurgical interventions were recorded. Of the patients, 13 had severe foramen magnum narrowing. Six patients required single or multiple surgical decompressions. We identified female sex, delayed milestones and a tight cervicomedullary junction as high risks. We stress upon the importance of developing a nationalized screening programme with guidelines to identify a high-risk group.

Complications of Elastic Stable Intramedullary Nailing for treating paediatric long bone fractures.

This study reports the complications observed in children with long bone fractures treated using Elastic Stable Intramedullary Nailing (ESIN). One hundred and sixty-four (n = 164) fractures in 160 patients under the age of 16 years formed the basis of our review. This included 108 boys and 52 girls with the median age of 11 years and median follow up of 7.5 months. The analysis included fractures of the radius/ulna, humerus, femur and tibia. All pathological fractures were excluded. In this series 54 patients (34%) had complications however majority of these were minor complications with irritation due to prominent nail ends being the commonest complication. No long-term sequelae were encountered in our patients.