Epidemiology of spinal fractures in children: Cross-sectional study.

INTRODUCTION: Epidemiological studies of fractures of the spine in children are all old, mostly single-centre, with series spanning periods of 5 to 20 years. HYPOTHESIS: As lifestyle is constantly changing, notably with an increase in sports activities and improvements in the prevention of road and household accidents, epidemiology has likely changed. OBJECTIVE: To update the description of spinal trauma in children and adolescents compared to the existing literature. MATERIAL AND METHOD: A multicentre cross-sectional study of spinal fracture, dislocation and spinal cord injury without radiological abnormality (SCIWORA) in children was carried out in 15 French university hospital centres, for a period of one year (2016). RESULTS: One hundred and sixty-five children were identified: 85 girls, 80 boys; mean age 11 years (range, 10 months-17 years); median, 12 years 6 months. One hundred and fifty-two children (92%) had fracture, 8 (5%) dislocation (including 7 C1-C2 rotary dislocations), and 5 (3%) SCIWORA. Fractures were multiple in 80 cases (49%), contiguous in 73 cases (91%) and non-contiguous in 7 (9%). Locations were cervical in 25 cases (15%), thoracic in 85 (52%), lumbar in 75 and sacral in 4 (2%). Fracture types comprised 234 vertebral compactions (78%), 25 burst fractures (8%), 5 chance fractures (2%), 2 odontoid fractures, and 33 other lesions. Causes comprised fall in 77 cases (47%), sports accidents in 56 (34%), road accidents in 29 (18%), and others in 3. In 52 cases (32%), there was ≥1 associated lesion: appendicular in 35 cases (67%), thoracic or abdominal in 31 (60%), and head in 16 (31%). Twenty-one cases had multiple lesions (40%). Eighteen cases showed neurological involvement (11%) including 5 SCIWORAs. Neurological complications were more frequent before 9 years of age. CONCLUSION: The epidemiology of spine fractures in children has slightly changed. There are now fewer cervical lesions. Causes are less often road accidents and more often sports accidents. Multi-level lesions remain frequent and the rate of neurological complications is around 10%. Compaction fracture is the most common type.

FAM46A mutations are responsible for autosomal recessive osteogenesis imperfecta.

BACKGROUND: Stüve-Wiedemann syndrome (SWS) is characterised by bowing of the lower limbs, respiratory distress and hyperthermia that are often responsible for early death. Survivors develop progressive scoliosis and spontaneous fractures. We previously identified LIFR mutations in most SWS cases, but absence of LIFR pathogenic changes in five patients led us to perform exome sequencing and to identify homozygosity for a FAM46A mutation in one case [p.Ser205Tyrfs*13]. The follow-up of this case supported a final diagnosis of osteogenesis imperfecta (OI), based on vertebral collapses and blue sclerae. METHODS AND RESULTS: This prompted us to screen FAM46A in 25 OI patients with no known mutations.We identified a homozygous deleterious variant in FAM46A in two affected sibs with typical OI [p.His127Arg]. Another homozygous variant, [p.Asp231Gly], also classed as deleterious, was detected in a patient with type III OI of consanguineous parents using homozygosity mapping and exome sequencing.FAM46A is a member of the superfamily of nucleotidyltransferase fold proteins but its exact function is presently unknown. Nevertheless, there are lines of evidence pointing to a relevant role of FAM46A in bone development. By RT-PCR analysis, we detected specific expression of FAM46A in human osteoblasts andinterestingly, a nonsense mutation in Fam46a has been recently identified in an ENU-derived (N-ethyl-N-nitrosourea) mouse model characterised by decreased body length, limb, rib, pelvis, and skull deformities and reduced cortical thickness in long bones. CONCLUSION: We conclude that FAM46A mutations are responsible for a severe form of OI with congenital bowing of the lower limbs and suggest screening this gene in unexplained OI forms.

Protein-altering MYH3 variants are associated with a spectrum of phenotypes extending to spondylocarpotarsal synostosis syndrome.

Spondylocarpotarsal synostosis syndrome (SCT) is a rare Mendelian disorder (OMIM #272460) characterized by prenatal vertebral fusion, scoliosis, short stature and carpal and tarsal synostosis. SCT is typically known as an autosomal recessive disease caused by variants in the FLNB gene. The genetic basis of the rarer cases of vertical transmissions remains unknown. In two independent families with symptoms related to autosomal dominant SCT, we identified - by exome sequencing - two protein-altering variants in the embryonic myosin heavy chain 3 (MYH3) gene. As MYH3 variants are also associated with distal arthrogryposis (DA1, DA2A, DA2B) and autosomal dominant multiple pterygium syndromes (MPS), the present study expands the phenotypic spectrum of MYH3 variants to autosomal dominant SCT. Vertebral, carpal and tarsal fusions observed in both families further confirm that MYH3 plays a key role in skeletal development.

Changes in Gait Variability From First Steps to Adulthood: Normative Data for the Gait Variability Index.

The process of learning to walk is ongoing throughout childhood. The Gait Variability Index (GVI; A. Gouelle et al., 2013) has been proposed to quantify the variability of spatiotemporal parameters (STP) during gait. The authors' aim was to evaluate the GVI and STP of healthy children and teenagers to (a) determine changes in the GVI with age and to derive normal values in children and (b) to evaluate the influence of STP on the GVI. A total of 140 typically developing children from 1 to 17 years old were categorized into 7 groups of 20 based on age. Spatiotemporal gait parameters were recorded using an electronic walkway. GVI increased and STP changed with age. In the children-teenagers group, the GVI was positively related to step length, speed, and negatively to cadence. Following normalization by lower limb length, correlations were no longer significant. In contrast, raw base of support was not correlated with the GVI but normalized base of support was. A multiple linear regression showed that only age had a direct impact on the GVI, indicating that gait continues to change after 6-7 years. These changes were only demonstrated by the GVI, highlighting its usefulness for the evaluation of gait in young populations.

Pyogenic Sacroiliitis in a 13-Month-Old Child: A Case Report and Literature Review.

Pyogenic sacroiliitis is exceptional in very young children. Diagnosis is difficult because clinical examination is misleading. FABER test is rarely helpful in very young children. Inflammatory syndrome is frequent. Bone scintigraphy and MRI are very sensitive for the diagnosis. Joint fluid aspiration and blood cultures are useful to identify the pathogen. Appropriate antibiotic therapy provides rapid regression of symptoms and healing. We report the case of pyogenic sacroiliitis in a 13-month-old child.Clinical, biological, and imaging data of this case were reviewed and reported retrospectively.A 13-month-old girl consulted for decreased weight bearing without fever or trauma. Clinical examination was not helpful. There was an inflammatory syndrome. Bone scintigraphy found a sacroiliitis, confirmed on MRI. Aspiration of the sacroiliac joint was performed. Empiric intravenous biantibiotic therapy was started. Patient rapidly recovered full weight bearing. On the 5th day, clinical examination and biological analysis returned to normal. Intravenous antibiotic therapy was switched for oral. One month later, clinical examination and biological analysis were normal and antibiotic therapy was stopped.Hematogenous osteoarticular infections are common in children but pyogenic sacroiliitis is rare and mainly affects older children. Diagnosis can be difficult because clinical examination is poor. Moreover, limping and decreased weight bearing are very common reasons for consultation. This may delay the diagnosis or refer misdiagnosis. Bone scintigraphy is useful to locate a bone or joint disease responsible for limping. In this observation, bone scintigraphy located the infection at the sacroiliac joint. Given the young age, MRI was performed to confirm the diagnosis. Despite the very young age of the patient, symptoms rapidly disappeared with appropriate antibiotic therapy.We report the case of pyogenic sacroiliitis in a 13-month-old child. It reminds the risk of misdiagnosing pyogenic sacroiliitis in children because it is exceptional and clinical examination is rarely helpful. It also highlights the usefulness of bone scintigraphy and MRI in osteoarticular infections in children.

MRI and suspected acute pyelonephritis in children: comparison of diffusion-weighted imaging with gadolinium-enhanced T1-weighted imaging.

OBJECTIVES: To evaluate the performance of diffusion-weighted imaging (DWI) against the reference standard of gadolinium-enhanced T1-weighted imaging (Gd-T1-WI) in children. METHODS: Thirty-nine consecutive patients (mean age 5.7 years) with suspected acute pyelonephritis underwent magnetic resonance imaging (MRI) including DWI and (the reference standard) Gd-T1-WI. Each study was read in double-blinded fashion by two radiologists. Each kidney was graded as normal or abnormal. Sensitivity and specificity of DWI were computed. Agreement between sequences and interobserver reproducibility were calculated (Cohen κ statistic and the McNemar tests). RESULTS: Thirty-two kidneys (41 %) had hypo-enhancing areas on Gd-T1-W images. The sensitivity and specificity of DWI were 100 % (32/32) and 93.5 % (43/46). DWI demonstrated excellent agreement (κ = 0.92,) with Gd-T1-W, with no significant difference (P = 0.25) in detection of abnormal lesions. Interobserver reproducibility was excellent with DWI (κ = 0.79). CONCLUSION: DWI enabled similar detection of abnormal areas to Gd-T1-WI and may provide an injection-free means of evaluation of acute pyelonephritis. KEY POINTS: • Diffusion weighted magnetic resonance imaging (DWI) can confirm acute pyelonepritis. • DWI provided comparable results to gadolinium enhanced T1-W MRI in acute pyelonepritis. • Contrast medium injection could be avoided for diagnosing acute pyelonephritis by MRI. • MRI with T2-WI and DWI provide a fast and comprehensive diagnostic tool.

CT analysis of C2 pedicles morphology and considerations of useful parameters for screwing.

INTRODUCTION: Surgery of cervical spine steadily requires realizing posterior osteosynthesis. The anchoring of instrumentation in C2 steadily constitutes an important stake of prognosis. Pedicle screwing is one of the best options and remains associated with a low morbidity. The aim of this CT study is to provide, from a wide population, the descriptive anatomical parameters of C2 pedicles. The data enable this analysis of feasibility of C2 pedicles screwing. MATERIALS AND METHODS: A continuous and retrospective series have explored 100 CT scans of the cervical spine without finding C2 fracture. The software OSIRIX v5.0.2 has been used. The dimensions of the pedicles in C2 as length, diameter, and distance from the vertebral foramens have been measured from the preset posterior entry point. Their orientation has been described in the axial and sagittal plan by the pedicle transverse angle, the sagittal angle and the pedicle-lamina angle used as a visible mark during the procedure. At least, the feasibility of pedicle screwing has been evaluated using a diameter criterion higher than 4 mm. RESULTS: The dimensions analysis of 200 studied pedicles has found an average length as 26.18 mm, an average diameter as 5.18 mm and an average distance between the entry point and the vertebral foramen as 9.06 mm. Their orientations have an average PTA as 36.6° and a SA as 25.8°. The average of the PLA was 81.3. The screwing feasibility has been evaluated as 92.5 % in the whole series. CONCLUSION: These morphological data come from a large series give some help for the C2 pedicle screwing preoperative planning. These lean on 3D measures but also on accessible mark during the procedure and despite the difference of the patient orientation. A CT preoperative planning of the pedicle screwing remains essential because more than 7 % of the pedicles have a diameter lower than 4 mm.

Percutaneous aspiration irrigation drainage technique in the management of septic arthritis in children.

BACKGROUND: Septic arthritis in childhood is a therapeutic emergency. The authors present their experience using an intermediate technique with the advantages of the percutaneous aspiration irrigation drainage: joint aspiration, irrigation, and declivious drainage. METHODS: All children were treated by joint aspiration under fluoroscopic control, large volume irrigation, and declivious nonsuction drainage associated with immobilization and intravenous antibiotics during 8 days to 10 days. The draining system was removed when clinical improvement (namely apyrexia) and the decrease of biological inflammatory response were obtained. A late follow-up phone interview was made for each patient. Fifty-two patients were included in this study, with a mean age of 4.3 years. The most common sites of arthritis were the hip (19 of 52, 36%) and the knee (17 of 52, 32%), but ankle (8 of 52, 15%), shoulder (12%), and elbow (3%) were also involved. RESULT: Apyrexia was obtained after a mean period of 2 days. The mean draining duration was 4.5 days. On the last follow-up visit (at 21 months on average; range, 12-56 months), all patients except one were totally painless and had no limitation of physical activity. CONCLUSION: Percutaneous aspiration irrigation drainage assured very good results in this study population, with rapid clinical and biological improvement and the absence of long-term sequelae. The advantages of this technique include permanent joint access and control of synovial effusions, with only one general anesthesia and minimal iatrogenic morbidity.

Lumbopelvic stabilization with external fixator in a patient with lumbosacral agenesis.

A case of caudal regression syndrome in which rehabilitation was obtained by lumbopelvic distraction and stabilization with external fixation Orthofix(®) is presented. The objective of the study is to describe the benefit of spine external fixator in caudal regression syndrome. Caudal regression syndrome is a rare and sporadic neural defect of distal spinal segments affecting the development of the spinal cord. It is characterized by vertebropelvic instability and essentially manifests as neurological deficit in the lower limbs and absence of bladder and bowel control. Pluridisciplinary management of this affection is complex. The clinical presentation and treatment of caudal regression syndromes are reviewed. A case of a boy for whom physical rehabilitation was obtained with external fixation Orthofix(®) is presented. Lumbopelvic stabilization was performed with autograft and allograft bone. A spine distraction was performed with external fixation with pedicular screws in L1 and L2 and in the pelvis bone. An elongation of 19 mm was obtained in 35 days. The external fixator was removed after 4 months and a spine cast was applied during 8 months. This treatment resulted in lumbopelvic nonunion of very low mobility. Lumbopelvic stability and the disappearance of pain were achieved. Sitting position was stable without hand support. At 14 years of follow-up, his condition is stable with possible deambulation at home with crutches. Lumbopelvic arthrodesis in caudal regression syndrome is difficult to obtain. The use of spine external fixator, however, allows trunk elongation and can achieve a stable sitting and upright position.

Elastic stable intramedullary nailing of tibial shaft fractures in children.

PURPOSE: Tibial fractures in the skeletally immature patient are usually treated without surgery. Elastic stable intramedullary nailing (ESIN) is commonly used for other diaphyseal fracture locations. Its advantages are minimally invasive surgery with a short hospitalisation duration, primary bone union and early weight bearing. The purpose of this study was to assess the use of ESIN in displaced tibial fractures in children over 6 years old and in cases of polytrauma. METHODS: This study was carried out over a 6-year period. The protocol consisted in ESIN of shaft tibial fractures in children over 6. Frontal and sagittal angulation, shortening and lengthening were measured on days 0, 2, 15, 30 and 45. At 6 months, 1 and 2 years, the femoro-tibial axis and eventual shortening or lengthening were assessed. RESULTS: The study involved 86 children (average age 11.8 years). As early as day 30, all patients had normal knee mobility and symmetrical foot progress angle. At 2-year follow-up, frontal angulation and leg length discrepancy had decreased and affected 2% of patients. Four patients (5%) suffered from superficial infections. There were no cases of osteomyelitis or refracture. CONCLUSIONS: The fixation of paediatric diaphyseal tibial fractures with ESIN is a rapid, well-codified and effective method for treating long-bone closed fractures in children. Advantages over other fixation techniques include a lower infection rate, a lower refracture rate, ease of management, and an aesthetically pleasing scar.

Early reduction versus skin traction in the orthopaedic treatment of femoral shaft fractures in children under 6 years old.

BACKGROUND: Femoral shaft fractures occur very frequently in children, and their prognosis usually is good. Nonoperative treatment is the gold standard for children under 6 years because of the excellent bony union and the remodelling qualities. PURPOSE: The aim of this study was to compare two orthopaedic therapeutic methods: skin traction versus immediate reduction. MATERIALS AND METHODS: The study involved 35 children, divided into two groups: in group 1, treatment consisted of skin traction for 21 days followed by hip spica casting; in group 2, an immediate reduction with early hip spica casting was performed. The ranges of motion, the delay before weight bearing, the hospitalisation duration and the required amount of painkillers were recorded. We compared initial shortening, axial, sagittal and rotational alignment, and femoral length discrepancy. We calculated the injured femoral diaphysal overgrowth and correlated it to the fracture type and location and to the initial shortening. Economical variables were also studied. RESULTS: The mean overgrowth was 8.9 mm in group 1 and 8.5 mm in group 2. Three years after the trauma, length discrepancy was 4 mm in group 1 and 1 mm in group 2. Hip spica casting leads to significant reductions in weight-bearing delay, hospitalisation duration and pain. The cost of treatment with skin traction was four times higher (24,472 euros) than that of immediate reduction (6,384 euros). DISCUSSION: Our results are in accordance with the literature. The femoral overgrowth was proportional to the initial shortening. Masculine gender, an oblique fracture and injury of the lower third of the femur were associated with the greatest femoral overgrowth. During the first year of follow-up, the femoral length discrepancy hardly varied after immediate reduction (4 mm), whereas the overgrowth reached 6 mm after skin traction. Overall, immediate hip spica casting leads to significant reductions in weight-bearing delay, hospitalisation duration, complications and costs, while having similar clinical results as traction.

Residual hip growth after pinning of slipped capital femoral epiphysis.

Screwing of slipped capital femoral epiphysis must prevent its further slipping by prematurely fusing the physis. Whichever material is used, persistent femoral growth has been described, thereby increasing the risk of bone deformation. The objective of this study is to evaluate the residual growth after screwing of slipped capital femoral epiphysis. This study concerned 26 children, among which 13 children have been included, and 13 children excluded because of an incomplete clinical or radiological follow-up, or treatment by another technique. The pathological hip was treated with one screw (in eight cases) or two screws (in five cases). The controlateral hip was fixed with one screw. The different measures were taken on anteroposterior radiographs done the days after surgery, and on the first radiograph on which the growth plate had fused. Growth plate fusion was obtained after an average of 20 months. Each patient had presented a residual growth of at least one hip, thus 85% of the 26 fixed screws. Among the four hips, which did not grow, three were pathological, and were fixed by one screw (in one case) or two screws (in two cases), in a central or medial position. There was not any statistical relationship between the growth persistence and the other studied criteria. These results, proving the growth persistency, suggest that the follow-up must be extremely careful, as the number of threads crossing the growth plate will decrease, with the risk of loss of mechanical stability and reappearance of the femoral epiphysis slippage.