Remodeling of Rotational Malunion of Femoral Shaft in Children.

Background: The degree of expected rotational remodeling in femoral shaft fractures is poorly understood, partly because of the difficulty in accurately measuring rotational alignment radiographically before and after treatment. This study aimed to assess the degree of rotational remodeling in the short-term following post-traumatic fracture shaft of the femur with > 10° rotational malunion in children under 15 years. Methodology: We carried out an observational study with a prospective follow-up on 18 children aged < 15 years with isolated, unilateral, oblique, or spiral, closed femur shaft fracture treated with closed reduction and immobilization with hip spica or internal fixation with elastic stable intramedullary nails or minimal invasive plate osteosynthesis. Only those with an initial axial malalignment measured on postoperative reduction radiographs of more than 10° by Ozel et al. technique were included. We evaluated the residual rotational malunion outcome using the ultrasound technique of Terjesen et al. by assessing the difference in femoral torsion as a guide to rotational malunion. The difference between the initial malreduction and the torsional difference between the hips at follow-up was deemed the extent of rotational change or remodeling. Results and Conclusion: The mean axial malalignment was 21.6° (10°-32°). The mean rotational remodeling was 13.6° (range 7°-21°), with an SD of 3.9. This study shows that a significant amount of rotational remodeling occurs in children up to 14 years of age within 2 years of injury. It is higher in younger patients (r = 0.786; p < 0.001), and the extent of remodeling is proportional to the magnitude of the initial deformity (r = 0.81, p < 0.001).

Diagnostic exome identifies a novel PRKG2 mutation in a proband with skeletal dysplasia.

This graphic abstract combines pedigree, dysmorphology features, radiographs, and the PRKG2 protein domain, specifically the CNB-A regulatory domain, which harbors a mutation resulting in premature protein termination.

Pediatric floating knee injuries: Clinical modifiers affecting sports and transfer outcomes.

AIM: This study assessed the functional outcome, and the clinical modifiers that influence them with the aim to assist the clinician plan a better management strategy in Paediatric Floating Knee (PFK) injuries. METHODS: A quasi-prospective, single-center observational study was designed to determine the functional and radiological outcomes in children ( 4 cm of bone loss (p < 0.01) but poor correlation with age at injury (p = 0.5), open fracture (p = 0.17), comminuted femoral and/or tibial fracture patterns (p > 0.05) and loss of soft tissue cover (p = 0.08). CONCLUSIONS: Early recognition of clinical modifiers such as high ISS and bone loss > 4 cm warrants targeted limb reconstruction strategy and can help to prognosticate outcome.

Clinical Spectrum and Microbial Etiology of Bone and Joint Infections in Children: A Retrospective Analysis from South India.

Acute infections of bone and joints are medical emergencies. Early diagnosis and treatment are essential for limb salvage and prevention of deformities. Data from developing countries are essential to develop region-specific treatment guidelines including choice of empiric antibiotics. We reviewed electronic medical records of children (≤ 12 years old) admitted to the pediatrics or orthopedics department of a tertiary care hospital in South India from 2013 to 2017 with a diagnosis of septic arthritis and/or osteomyelitis. Clinical, microbiological, and follow-up data were collected and analyzed. The median (interquartile range, IQR) age of the children (N = 207) was 48 (7.5-105) months. Acute infections were more common in infants, whereas chronic cases were common in children > 5 years of age. Staphylococcus aureus (71%) was the most common organism identified. Gram-negative organisms were more frequently isolated in infants compared with older children. Blood and/or wound culture positivity was 78% (N = 161) overall and 78% (N = 31) in chronic cases. The median (IQR) duration of antibiotics was 7 (5-8) weeks. Sequelae and readmissions occurred in 47% (N = 81) of the 172 patients followed for a year. Culture positivity rates especially of wound were high even after receiving antibiotics.

Case report of a rare purine synthesis disorder due to 5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase (AICAR) deficiency.

BACKGROUND: AICA (5-aminoimidazole-4-carboxamide) ribosiduria is an inborn error in purine biosynthesis caused due to biallelic pathogenic variants in the 5-aminoimidazole-4-carboxamide ribonucleotide-formyltransferase/imp cyclohydrolase (ATIC) gene located on chromosome 2q35. ATIC codes for a bifunctional enzyme, AICAR transformylase and inosine monophosphate (IMP) cyclohydrolase, which catalyse the last two steps of de novo purine synthesis. This disorder has been previously reported in only 4 cases worldwide, and herein, we report the first from India. CASE REPORT: The proband presented with global developmental delay, developmental hip dysplasia (DDH), acyanotic heart disease and nystagmoid eye movements. Whole exome sequencing (WES) identified compound heterozygous pathogenic variants in the ATIC. A novel splice site variant; c.1321-2A > G and a previously reported missense variant; c.1277A > G (p.Lys426Arg) were identified. Segregation analysis of parents showed the father to be a heterozygous carrier for the splice site variant and the mother, a heterozygous carrier for the missense variant. CONCLUSION: This case of a rare genetic disorder of purine biosynthesis of ATIC deficiency is the first case reported from India. Early diagnosis lead to early interventional therapy and genetic counselling.

Elastic stable intramedullary nailing versus submuscular plating in pediatric diaphyseal femur fractures: a randomized controlled trial among children in South India.

Elastic stable intramedullary nailing (ESIN) is the current preferred method for treating diaphyseal femur fractures in children. Introduction of the submuscular locked plate (SMP) fixation construct has opened the debate on treatment options for pediatric diaphyseal femur fractures in the older children and adolescents. A randomized controlled trial (RCT) protocol was designed to compare ESIN and SMP for diaphyseal femur fractures in children. An open-labelled RCT comparing SMP with ESIN was conducted from January 2013 to June 2016, for children aged 6-15 years with closed, acute femoral diaphyseal fractures. Randomization was done through computer-generated randomization sequence and opaque-sealed envelopes. Rate of adverse surgical events including unplanned re-operations was assessed as the primary outcome and secondary analysis was done for time to union, degree of malunion, limb length discrepancy, functional outcome at 2 years, surgical duration and blood loss, radiation exposure, hospital stay, cost incurred and secondary implant removal procedure. Forty children were randomized with allocation concealment. There were three adverse events in the SMP arm and five in the ESIN arm. Fifteen children with SMP underwent routine implant removal compared to only three children with ESIN (P < 0.001). Both ESIN and SMP are equally safe, viable and effective options for treating pediatric diaphyseal femoral fractures. However, the additional cost of secondary surgery for implant removal in the SMP group proved to be a deterrent factor, which led to ESIN being the preferred option in our resource-limited setting.

Modified Woodward's procedure confers functional improvement in Sprengel's deformity.

Modified Woodward's procedure improves shoulder movement and cosmesis. There is a paucity of literature reporting objective assessment of functional outcomes. We report cosmetic and functional outcomes in Sprengel's deformity treated with modified Woodward's procedure. Children aged 12 years and under, who underwent Modified Woodward's procedure from January 2006 to December 2014 were included in the prospective study. Improvement in Cavendish grade, Rigault scale, and shoulder movements was noted. Pediatric outcomes data collection instrument (PODCI) and simple shoulder test (SST) scores were assigned at the final follow-up. Statistical analysis was conducted with paired T-test and Wilcoxon signed-rank tests. Fourteen patients (one bilateral) with a mean follow-up of 4.5 years (1-8 years) were analyzed. The mean age at surgery was 5.7 years (3-12 years). Ten (71%) patients had omovertebral bars. Six patients had Klippel-Feil syndrome including one with cervical hemivertebrae with anterior chest wall deficiency. Cavendish grade and Rigault scale improved significantly (P < 0.05). Mean shoulder abduction and forward flexion improved by 37.3° (P < 0.001) and 38.7° (P < 0.001), respectively. The mean normative PODCI score was 24.07 (reported normative score ranges from -146 to 53) and the SST score was 9.64 (reported score ranges from 0 to 12). SST scores demonstrated a moderate correlation with shoulder function. Patients without associated anomalies had better cosmetic (P = 0.057) and functional (P = 0.029) improvement. Modified Woodward's procedure improved cosmesis and provided near-normal functional outcomes in children irrespective of age and sex. Better improvement was noted in children without anomalies.

A rare presentation of primary lymphoma of bone with aneurysmal bone cyst-like changes.

Primary lymphoma of bone occurs rarely in children with variable imaging findings ranging from a lytic lesion to an aggressive permeative lesion. We detail a case report with review of literature of a 14-year-old boy with a rare presentation of primary lymphoma of bone with aneurysmal bone cyst-like changes. The lesion was surgically excised as management for aneurysmal bone cyst but histopathological examination of the excised tissue revealed a non-Hodgkin's type of lymphoma. This unique type of aneurysmal bone cyst-like presentation has not been well characterised until now in the imaging spectrum of primary lymphoma of bone. Clinicians need to be aware of this uncommon presentation for early intervention and appropriate management of this malignancy in children.

Optimizing the use of fibula in type II tibial hemimelia: early results.

We describe a technique for optimal use of fibula in reconstruction of type II tibial hemimelia. Six affected children with mean age of 1.4 years and treated over a 5-year period were reviewed. All underwent staged reconstruction by lowering the fibula to below knee level using Ilizarov soft tissue distraction, transfer of distal fibula under proximal tibia, and foot centralization. Mean follow-up period was 3.6 years (range: 1.6-6.05 years). Mean age at follow-up was 4.4 years, and increase in length was 4.08 cm. Tibiofibular union and foot centralization were universally achieved. Mild residual equinovarus deformity was present in three children and braced. Our technique allowed significant length gain and foot centralization in toddlers without distraction osteogenesis.

Femoral Neck Fractures in Children: A Review.

Paediatric femoral neck fractures are uncommon injuries and are usually caused by high-energy trauma. Low-energy trauma can result in pathologic neck fractures and stress fractures of the neck, due to repetitive activity. Surgical options can vary based on age, Delbet classification and displacement of the fracture. Treatment for displaced fractures is by closed or open reduction and smooth/cancellous screw fixation. Compression screw and side plate fixation is indicated for basal fractures. Fixation should be supplemented by spica cast immobilization in younger children. The high rate of complications occurs due to the vascular anatomy of the hip and proximal femur. Avascular necrosis, coxa vara, premature physeal closure, and nonunion are the most common and these often result in poor outcome.

Atypical osteomyelitis and concurrent septic arthritis due to Salmonella in immunocompetent children.

OBJECTIVE: Salmonella osteomyelitis in immunocompromised individuals with sickle cell anaemia is well documented. Its occurrence in immunocompetent children is rare. METHODS: All pus culture positive cases of salmonella typhi between the period 2009 to 2014 were reviewed and only those children without sickle cell disease or trait were considered further. RESULTS: Eighty five patients had positive cultures. Of these only three children had culture positive Salmonella septic arthritis in the absence of sickle cell disease. Two children had shoulder septic arthritis while one had hip septic arthritis. CONCLUSION: Our case series highlights the possibility of salmonella typhi osteomyelitis in immunocompetent individuals. Clinicians should be aware of this presentation which is usually delayed due to the atypical organism and lack of clinical response in the initial stages, as disastrous sequelae of septic arthritis may result if prompt treatment is not initiated in time.

Upper Cervical Fusion in Children With Spondyloepiphyseal Dysplasia Congenita.

BACKGROUND: Paraplegia or death secondary to upper cervical spine instability and spinal cord compression are known consequences of spondyloepiphyseal dysplasia congenita (SEDC). Stabilization and occasionally decompression of the upper cervical spine are indicated to treat upper cervical instability and stenosis. The purpose of this study was to report the results of upper cervical spine fusion in children with SEDC who had upper cervical instability. METHODS: Twenty children (17 females and 3 males) with SEDC who underwent upper cervical spine fusion at a mean age of 72 months were retrospectively analyzed. Three of these children were under the age of 2. Fifteen children had posterior instrumentation and fusion whereas 5 children had posterior in situ fusion without use of any implant. Thirteen of 20 children had iliac crest autograft. Radiographic and clinical results were reported. RESULTS: The average follow-up period was 8 years and 8 months. All children with instrumentation achieved fusion. Three of 5 children who had no instrumentation had nonunion (1 child had a stable nonunion and did not need revision; 1 had a single noninstrumented revision and ended up with a stable nonunion without further intervention; and the third one had a noninstrumented revision and had to have a second, instrumented, revision to achieve fusion). Six children had thoracolumbar scoliosis or kyphoscoliosis which required surgical management.No postoperative neurological deficits were observed. Two of the 3 children with a preoperative neurological deficit showed full recovery and the third one remained unchanged. Pseudarthrosis is the main complication for the noninstrumented group. Distal junctional instability after successful fusion is seen at long-term follow-up (average=6 y) for 13% of patients in instrumented group. CONCLUSIONS: Instrumentation and iliac bone grafting results in 100% upper cervical fusion for SEDC children who demonstrated instability before surgery. LEVEL OF EVIDENCE: Level IV-therapeutic.

Slipped upper femoral epiphysis: Outcome after in situ fixation and capital realignment technique.

BACKGROUND: Slipped upper femoral epiphysis (SUFE) is the gradually acquired malalignment of the upper femoral epiphysis (capital) and the proximal femoral metaphysis. SUFE is uncommon in India, and there are no previous studies on outcome and clinical characteristics of patients with SUFE from India. This study evaluates the presentation, disease associations and outcome of SUFE from a tertiary care centre in India. MATERIALS AND METHODS: Twenty six consecutive children with SUFE seen over a period of 4 years were reviewed. The clinical presentations, severity of the slip, surgical interventions (n=30) were assessed. Twenty one boys and five girls with a mean age 13.1 years (range 10-16 years) were included in the study. Four children had bilateral involvement. There were 4 rural and 22 urban children from the eastern and southern states of the country. The presentation was acute in 7, acute on chronic in 5, and chronic in 14, with a mean duration of symptoms of 51 days (range 3-120 days). Slips were stable in 16 and unstable in 10 children. Two children had adiposogenital syndrome. Body mass index was high in 12 out of 23 children. Vitamin D levels were low in 20 out of 21 children, with a mean vitamin D level of 12.61 ± 5 ng/ml. Eighteen children underwent in situ pinning. Eight children underwent capital realignment. RESULTS: Clinical outcome as assessed by Merle d' Aubigne score was excellent in 6, good in 10, fair in 6 and poor in 1. Half of the in situ fixation patients underwent osteoplasty procedure for femoroacetabular impingement and 5 more were symptomatic. The head neck offset and α angle after in situ pinning were -1.12 ± 3 mm and 66.05 ± 9.7°, respectively and this improved to 8.7 mm and 49°, respectively, after osteoplasty. One child in the pinning group had chondrolysis. Eight patients with severe slip underwent capital realignment. Mean followup was 20.15 months. The anterior head neck offset and α angle were corrected to 6.8 ± 1.72 mm and 44.6 ± 7.0° mm, respectively. Two children with unstable slip in the capital realignment group had avascular necrosis which was diagnosed at presentation by bone scan. CONCLUSION: High BMI, vitamin D deficiency and endocrine disorders are associated with SUFE in India and should be evaluated as some of these are amenable to prevention and treatment. Most patients treated with in situ pinning developed femoroacetabular impingement. The early results after capital realignment procedure are encouraging and help to avoid a second procedure which is needed in a majority of patients who underwent in situ pinning.

High failure rate of the Pavlik harness in dislocated hips: is it bilaterality?

BACKGROUND: The purpose of this study was to evaluate the efficacy of Pavlik harness treatment in patients with bilaterally dislocated Graf type IV hips and compare them to cases with unilaterally dislocated hips. METHODS: Twenty-one patients (42 hips) who presented with bilaterally dislocated hips with no prior treatment were studied. The comparison group consisted of 33 patients (33 hips) with unilateral hip dislocation treated with the same protocol. Successful treatment was defined as relocation of the hips within 3 weeks of Pavlik harness application. RESULTS: Twelve of 21 patients (57%) in the bilateral group (6 failed bilaterally, 6 unilaterally) and 18/33 patients (54.5%) in the unilateral group failed harness treatment. CONCLUSIONS: The use of the Pavlik harness in dislocated hips is associated with a high failure rate. Patients presenting with bilaterally dislocated hips however, are at no greater risk for failure than patients presenting with unilateral hip dislocation. LEVEL OF EVIDENCE: Level III.

Sjögren-Larsson syndrome--unusual presentation with pathological femoral neck fracture: a case report.

Patients with Sjögren-Larsson syndrome, an autosomal recessive syndrome characterized by ichthyosis, spastic diplegia/quadriplegia, mental retardation and ocular features that include pigmentary changes in the retina, rarely present to the orthopaedic surgeon. We detail a rare and unusual presentation of Sjögren-Larsson syndrome in an adolescent boy with bilateral femoral neck fractures.

Anterior distal femoral stapling for correcting knee flexion contracture in children with arthrogryposis--preliminary results.

BACKGROUND: Fixed flexion contractures of the knee are more common and disabling than extension contractures in children with arthrogryposis. For correcting these deformities, there are various surgical options such as soft tissue release, distal femoral osteotomy, and frame distraction. We sought in this study to examine the effectiveness of anterior distal femoral stapling using 8-plates for correcting knee flexion contracture in children with arthrogryposis. METHODS: We retrospectively assessed 16 knees in 10 children using clinical and radiographic measures. To determine the outcome, we assessed the Functional Mobility Scale (FMS) as well. Statistically, a paired t test, independent t test, and Wilcoxon signed-rank test were used to analyze the results. RESULTS: After anterior distal femoral stapling, there was a reduction in the flexion deformity of the knee in children with arthrogryposis, P<0.05. There was an estimated 18-degree correction comparing the mean preoperative flexion deformity and the mean postoperative flexion deformity. This correction was significant in children when the knee flexion deformity was less than 45 degrees. The FMS also improved in those patients where the residual flexion contracture was less than 30 degrees at follow-up, suggesting an improvement in their ambulatory capacity, P<0.05. CONCLUSION: Among children with arthrogryposis who present with knee flexion contractures, anterior distal femoral stapling with 8-plates improved their flexion deformity and ambulatory capacity. This technique is less invasive than soft tissue releases, distal femoral osteotomy, or frame distraction and is most rewarding in children with arthrogryposis whose flexion contractures is less than 45 degrees.

Outcome of in situ pinning in patients with unstable slipped capital femoral epiphysis: assessment of risk factors associated with avascular necrosis.

INTRODUCTION: Avascular necrosis (AVN) is a devastating complication following treatment of unstable slipped capital femoral epiphysis (SCFE). The advent of newer methods such as open reduction and surgical dislocation of the hip has increased the debate on the optimal method of treatment. However, the risk or predisposing factors for AVN remain unclear. We aimed to assess the outcome of in situ fixation and the risk factors associated with AVN. METHODS: We retrospectively reviewed the records of 27 patients (27 hips) with unstable SCFE out of the 280 children managed for SCFE from 1995 to 2006. The mean age in years of the patients at surgery was 12.2+/-1.58, and our sample comprised 70.4% males, and 29.6% females, with a mean follow-up of 3.1+/-1.9 years. Univariable and multivariable binomial regression models were used to assess factors predisposing to AVN. RESULTS: AVN occurred in 22.2% (6/27) of the children with unstable SCFE. After adjustment for age, race, and time to surgery, sex and preoperative slip angle were the only 2 significant factors related to an increased risk of AVN, risk ratio (RR)=4.15, 95% confidence interval=1.00-17.19, P=0.05 and RR=1.04, 95% confidence interval=1.00-1.07, P=0.03, respectively. Female children constitute a high-risk group for AVN in this subgroup. CONCLUSIONS: AVN is still prevalent among patients with unstable SCFE who underwent in situ pinning. Female sex and slip magnitude are potential predisposing factors for developing AVN.

The anteromedial approach to the psoas tendon in patients with cerebral palsy.

PURPOSE: Release of the psoas tendon for flexion deformity of the hip in children with cerebral palsy has traditionally been performed at the pelvic brim, lateral to the neurovascular bundle, or at its insertion into the lesser trochanter. As the psoas tendon is lateral to the pectineus, the traditional exposure of the tendon through an approach medial to the pectineus is limited by the extent to which the pectineus can be retracted proximally. TECHNICAL NOTE: We describe the use of the anteromedial approach used for the developmentally dislocated hip to expose the psoas tendon between the pectineus and the neurovascular bundle. This provides a much better visualisation of the tendon as it crosses the superior pubic ramus to its insertion. The use of this approach has not been described in cerebral palsy.