Unravelling the etiology of sporadic late-onset cerebellar ataxia in a cohort of 205 patients: a prospective study.

BACKGROUND: Despite recent progress in the field of genetics, sporadic late-onset (> 40 years) cerebellar ataxia (SLOCA) etiology remains frequently elusive, while the optimal diagnostic workup still needs to be determined. We aimed to comprehensively describe the causes of SLOCA and to discuss the relevance of the investigations. METHODS: We included 205 consecutive patients with SLOCA seen in our referral center. Patients were prospectively investigated using exhaustive clinical assessment, biochemical, genetic, electrophysiological, and imaging explorations. RESULTS: We established a diagnosis in 135 (66%) patients and reported 26 different causes for SLOCA, the most frequent being multiple system atrophy cerebellar type (MSA-C) (41%). Fifty-one patients (25%) had various causes of SLOCA including immune-mediated diseases such as multiple sclerosis or anti-GAD antibody-mediated ataxia; and other causes, such as alcoholic cerebellar degeneration, superficial siderosis, or Creutzfeldt-Jakob disease. We also identified 11 genetic causes in 20 patients, including SPG7 (n = 4), RFC1-associated CANVAS (n = 3), SLC20A2 (n = 3), very-late-onset Friedreich's ataxia (n = 2), FXTAS (n = 2), SCA3 (n = 1), SCA17 (n = 1), DRPLA (n = 1), MYORG (n = 1), MELAS (n = 1), and a mitochondriopathy (n = 1) that were less severe than MSA-C (p < 0.001). Remaining patients (34%) had idiopathic late-onset cerebellar ataxia which was less severe than MSA-C (p < 0.01). CONCLUSION: Our prospective study provides an exhaustive picture of the etiology of SLOCA and clues regarding yield of investigations and diagnostic workup. Based on our observations, we established a diagnostic algorithm for SLOCA.

Clonidine GH stimulation test to differentiate MSA from idiopathic late onset cerebellar ataxia: a prospective, controlled study.

INTRODUCTION: Despite the consensus criteria for multiple system atrophy (MSA), the diagnosis of MSA of cerebellar type (MSA-C) may be difficult in the early stage of the disease. There are several differential diagnoses including idiopathic late-onset cerebellar ataxias (ILOCA) and it is often necessary to wait for clinical worsening so that the criteria can be met. Our aim was to assess the efficacy of clonidine growth hormone test (CGH test) to distinguish MSA-C from ILOCA in the early stage of the disease. METHODS: Within our cohort of late-onset sporadic, progressive cerebellar ataxia, the group of patients meeting the criteria for possible or probable MSA was compared to the ILOCA group. Clinical and paraclinical examination including CGH test were repeated during the prospective follow-up. RESULTS: Eighty-six patients were recruited, including 42 patients in the MSA group and 44 ILOCA patients with a mean follow-up of 33 months. At the inclusion visit, CHG test was pathological for 31% MSA of patients and 18.2% of ILOCA patients (p = 0.35). During the follow-up, 52.4% of MSA-C had a pathological CGH test, while only 20.5% of ILOCA (p < 0.01). CGH test had a sensitivity of 69.1% and a specificity of 68.2%, (p < 0.001) for MSA-C patients; CGH test allows in three quarters of cases, if negative, to rule out a probable MSA-C (negative predictive value of 75%, p = 0.0014). CONCLUSION: This prospective, controlled study showed that CGH test could be helpful in clinical practice to differentiate MSA-C from ILOCA in the early stage of the disease.

Compressive chylothorax after lumbar spine fracture.

A 14-year-old boy was admitted to the hospital after an episode of blunt trauma to the thorax, resulting in a Chance fracture of L1 and a compressive chylothorax 72h after admission. After initial drainage in the operating room, conservative management was successful. This case study documents one of the rare complications of spinal fractures in the context of high-energy blunt trauma. It is the first detailing a noniatrogenic post-traumatic compressive chylothorax in pediatrics responding positively to conservative management. Drainage should be considered the first-line procedure for both therapeutic and diagnostic purposes. Surgery is required if the leakage is still present after parenteral feeding and the implementation of a fat-free diet for 5-7 days.

Changes in qualitative and quantitative ultrasound assessment of the gastric antrum before and after elective caesarean section in term pregnant women: a prospective cohort study.

Ultrasound measurement of the antral cross-sectional area allows a quantitative estimate of gastric contents in non-pregnant adults, but this relationship may be affected by compression of the stomach exerted by the gravid uterus during pregnancy. This study aimed to assess differences in quantitative (Perlas score) and qualitative (antral cross-sectional area) ultrasound assessments of the gastric antrum performed immediately before and after caesarean section. Forty-three women having elective caesarean section performed under spinal anaesthesia were studied in the semirecumbent and semirecumbent-right lateral positions. Thirty-nine women showed no change in stomach contents using the Perlas score between the two measurement periods; four women showed a change, but by one grade only. The median (IQR [range]) antral cross-sectional area was 323 (243-495 [103-908]) mm2 before, and 237 (165-377 [112-762]) mm2 after, caesarean section in the semirecumbent position (p = 0.001); the comparable values in the semirecumbent-right lateral position were 418 (310-640 [161-1238]) mm2 and 362 (280-491 [137-1231]) mm2 (p = 0.09). The distance between the skin and the antrum, and the aorta and the antrum, decreased significantly in both positions after surgery. We suggest that our results indicate that stomach contents remain largely unchanged in women having elective caesarean section, but antral cross-sectional area decreases, especially in the semirecumbent position, related to a change in the position of the stomach within the abdomen. This implies that the relationship of antral cross-sectional area to volume of stomach contents, which has been determined for non-pregnant subjects, may not apply in term pregnant women.

Rare ACL enthesis tears treated by suture in children. A report of 14 cases after a mean 15 years follow-up.

INTRODUCTION: ACL enthesis tears are rare in children and there are very few reports in the literature. Characterized by avulsion of the ligament that tears off a very thin piece of cartilage or a bone fragment at the ACL epiphyseal insertion site, we hypothesize that conservative treatment by suture could reduce the number of patients requiring later ligament reconstruction. MATERIALS AND METHODS: Fourteen patients underwent surgery between 1986 and 2014 and were included in this retrospective study. Patients were selected according to the following criteria: suture failure requiring ligament reconstruction, reoperation-free survival for secondary injury and the subjective and objective IKDC scores by comparative laximetry. RESULTS: After almost 15 years of mean follow-up, reoperation-free survival was approximately 85%. Three patients required one or more additional surgeries; one for suture failure requiring 2 additional ligament reconstructions, one for a meniscal tear with a mechanical block requiring partial meniscectomy and one patient with secondary pain requiring arthroscopic surgery that was inconclusive. DISCUSSION: Rare and often misdiagnosed, ACL enthesis tears in children can be treated by suture with satisfactory results. Careful analysis of plain films and an X-ray centered on the intercondylar notch often provide the diagnosis. In case of doubt, MRI, which is now more accessible, can prevent missing this entity. Improved standard surgical techniques and careful patient selection could make conservative treatment an option once again while avoiding the risks associated with ligament reconstruction technique. LEVEL OF EVIDENCE: 4.

Results with all-inside meniscal suture in pediatrics.

INTRODUCTION: Incidence of meniscal lesions is increasing in the pediatric population. Given the harmful impact of meniscectomy, meniscal repair is attempted whenever possible. The present study sought to assess the efficacy of FasT-Fix (Smith and Nephew) all-inside arthroscopic meniscal repair devices in childhood meniscal lesions. The study hypothesis was that functional results would be equivalent to those of other suture techniques. MATERIAL AND METHODS: Nineteen patients were assessed following arthroscopic meniscal repair, at a mean 6 years (range: 3-9 years). Mean age was 14.8 years. There was associated ACL tear in 31% of cases. Functional assessment used Lysholm, subjective IKDC, Tegner activity level and KOOS scores. Anatomic assessment of healing used MRI, arthroscopy or CT-arthrography. RESULTS: At last follow-up, the meniscal survival rate was 89.5% (n=17/19). Functional results were good: Lysholm: 95.7/100; subjective IKDC: 90.7/100; Tegner: 7.64. Imaging found systematic healing. DISCUSSION: All-inside arthroscopic meniscal suture is increasingly used in adults, and can also be implemented in children or adolescents in pediatrics. FasT-Fix (Smith and Nephew) implants, although not always easy to use, ensure a perfectly functional knee and conserve long-term meniscal chondroprotection with a low risk of complications. LEVEL OF EVIDENCE: IV, retrospective study.

Intercondylar notch dysplasia in open-physis anterior cruciate ligament injuries: A case-control study.

BACKGROUND: The incidence of anterior cruciate ligament (ACL) tears in children is rising steadily due to a variety of factors including growing participation in sports. A narrow intercondylar notch is an intrinsic risk factor that is well documented in adults but rarely investigated in children. The objective of this study was to evaluate the potential association between a narrow intercondylar notch and ACL tears in children. HYPOTHESIS: A narrow intercondylar notch is associated with ACL tears. MATERIAL AND METHODS: In a paediatric case-control study, we compared intercondylar notch morphology as assessed by magnetic resonance imaging (MRI) in 49 patients with ACL tears (33 males and 16 females with a mean age of 13.6 years) and 50 controls with normal knees (18 boys and 32 girls with a mean age of 13.8 years). In each participant, posterior tibial slope was measured, as well as the notch width index (NWI) (width of the intercondylar notch over bicondylar width at the same level). In addition, to evaluate anterior impingement, the angle formed by Blumensaat's line and the axis of the tibia (α angle) was measured with the knee extended. RESULTS: The NWI was significantly lower in the cases than in the controls (0.244±0.02 and 0.263±0.02, respectively; P<0.05). The α angle was also significantly smaller in the cases (138.74°±4.6° vs. 141.30°±7.9° in the controls; P<0.05). DISCUSSION: ACL tears are associated with a small NWI in children. A narrow intercondylar notch is an established risk factor for ACL tears and should be sought routinely to determine whether notch-plasty should be performed during the ACL reconstruction procedure in order to decrease the risk of recurrent ACL tears. LEVEL OF EVIDENCE: III, case-control study.

Aneurysmal bone cyst: A 19-case series managed by percutaneous sclerotherapy.

INTRODUCTION: Sclerotherapy offers an alternative to surgery for the treatment of aneurysmal bone cyst (ABC). The main objective of the present study was to assess the radiological efficacy of sclerotherapy in terms of ossification on MRI. Secondary objectives were to assess clinical efficacy on pain evaluation and to analyze recurrence and complications according to type of sclerosing agent and intraoperative imaging technique. MATERIALS AND METHODS: Between 2006 and 2014, 19 patients (7 females, 12 males, aged 3 to 17 years) with ABC treated by sclerotherapy were included. Six received Ethibloc(®), 9 Aetoxisclerol(®), 2 liquid absolute alcohol, and 2 absolute alcohol gel. Assessment used fluoroscopy in 17 cases and CT in 2. Ossification was assessed on MRI and pain on a visual analog scale and HEDEN score. RESULTS: Ossification was complete in 11 cases (84.6%) and partial in 2 (15.4%). Eighteen patients (94.7%) were pain-free at 3 months. There was no recurrence, at a minimum 2 years' follow-up. One case of skin necrosis was observed, associated with use of liquid absolute alcohol; there was 1 case of arterial reflux of Ethibloc(®) under CT control. DISCUSSION: Sclerotherapy enables minimally invasive treatment of lesions that are deep, difficult of access to surgery and potentially damaging. Use of absolute alcohol gel and fluoroscopic control seems to improve the risk/benefit ratio, limiting complications by vascular extravasation of the sclerosing agent, thanks to real-time visualization of diffusion. Its clinical and radiological efficacy makes sclerotherapy and alternative primary treatment choice in ABC. LEVEL OF EVIDENCE: IV, retrospective study.

Arthroscopic treatment of septic arthritis of the knee in children.

INTRODUCTION: Childhood septic arthritis of the knee is a serious disease that can impair growth and cause serious functional sequelae. There are few data on arthroscopic treatment in children, and series were always less than 20 cases. HYPOTHESIS: The objective of this study was to assess clinical and radiographic results of arthroscopic drainage combined with antibiotic therapy for the treatment of childhood septic arthritis of the knee. The hypothesis was that arthroscopic treatment is also effective in children. MATERIALS AND METHODS: A retrospective study, conducted between January 2003 and December 2012, included patients under 15 years of age with septic arthritis of the knee treated by arthroscopic drainage with a minimum of 2 years' follow-up. RESULTS: Fifty-six patients, with a mean age at surgery of 3.4 years (range, 3 months to 12 years), were included. Staphylococcus aureus was the most common causative organism. Two patients (3.6%) had recurrence, successfully treated by repeat arthroscopic drainage. Mean Lysholm score was 96.9 (range, 70-100) and mean KOOS-Child pain, symptoms, daily life, sports and quality of life scores were respectively 97 (81-100), 95 (75-100), 98 (89-100), 93 (71-100) and 95 (70-100) at a mean 65 months' follow-up. Ranges of motion were normal. Radiology found no joint damage. DISCUSSION AND CONCLUSION: Arthroscopic drainage combined with antibiotic treatment is a simple and effective treatment for childhood septic arthritis of the knee and is for our reference attitude. It can also be indicated in case of recurrence. LEVEL OF EVIDENCE: IV, retrospective study.

Adolescent idiopathic scoliosis correction achieved by posteromedial translation using polyester bands: A comparative study of subtransverse process versus sublaminar fixation.

PURPOSE: Sublaminar polyester bands have been used in hybrid construct to achieve correction of adolescent idiopathic scoliosis since 2003. Despite the reported safety of the bands, some surgeons remain reluctant at the idea of approaching the canal because of the potential neurological complications reported with the Luque wiring. Sub transverse bands might be an alternative. The present study is the first to compare sublaminar polyester band fixation to fixation of polyester bands around the transverse processes in hybrid constructs used to treat AIS. METHODS: Two cohorts of consecutive patients treated for thoracic AIS were retrospectively reviewed, with a minimum 2-year follow-up. Posteromedial translation was used for main curve correction in all cases. Sublaminar polyester bands were used in group 1 (20 patients). In group 2 (20 patients), the same implant was used, but the bands were passed around the transverse process instead of the lamina. Radiographic analysis included frontal Cobb angle measurements for each curve, thoracic kyphosis and rotation of the apical vertebra (RVA). RESULTS: Mean operative time was similar in groups 1 and 2 (235±35 and 240±30minutes, respectively). Mean frontal correction achieved for the main curve was similar in both groups, 62.5±17.4% in group 1 and 54.1±19.4% in group 2. Sagittal correction was similar, with a final mean thoracic kyphosis of 30.9°±9.7° and 27.8°± 6.8° in group 1 and 2, respectively. Correction of RVA was similar in both groups postoperatively, 65.8% (±29.1) and 54.4% (±42.7) in group 1 and 2 respectively. No transverse process or lamina fracture was observed during insertion of the bands or curve correction in any of the groups. CONCLUSION: This study confirms that anchorage of Universal clamps (UCs) around transverse processes is a safe and efficacious technique in both the frontal and sagittal planes, providing a useful alternative for the correction of moderate AIS. UCs attached to transverse processes can achieve correction of moderate AIS similar to that obtained with sublaminar UCs while further reducing risks of vertebral canal complications. LEVEL OF EVIDENCE: Level IV.

The progression of lumbar curves in adolescent Lenke 1 scoliosis and the distal adding-on phenomenon.

INTRODUCTION: The postoperative deterioration of the curve below spinal fusion instrumentation resulting in a distal adding-on (AO) phenomenon in idiopathic adolescent scoliosis (IAS) frequently requires surgical revision with disappointing secondary clinical results. HYPOTHESIS: Analysis of AP (coronal) range of motion (cROM) and lateral (sagittal) range of motion (sROM) on dynamic (side-bending, flexion, extension) X-rays to determine the choice of the lowest instrumented vertebra (LIV) can help reduce distal adding-on. The goal of this study was to study the postoperative progression of the lumbar curve in Lenke 1 scoliosis operated on with a LIV based on dynamic X-ray results. MATERIALS AND METHODS: Right-sided Lenke 1 IAS that was treated surgically by posterior arthrodesis alone with a follow-up of at least 2 years was included in the study. The following radiographic parameters were evaluated: the Cobb angles of the curves, reducibility of the curves, the apex of the scoliosis, the central sacral vertical line, the stable vertebra (SV), the neutral vertebra (NV), the distances between the CSVL and the centroids of the LIV and of the first vertebra below instrumentation, as well as the tilt of the superior endplates. sROM and cROM were determined on dynamic X-rays. RESULTS: Fifty IAS were evaluated/185 files. Only three cases fulfilled the criteria for AO including two that were secondary to peri- or postoperative complications. The lumbar curve presented with a loss of correction of 0.9° at one year and 1.14° at the final follow-up. None of the parameters studied were correlated to the deterioration of the lumbar curve. DISCUSSION: The choice of the LIV has been shown to influence the deterioration of the lumbar curve and the development of AO. The choice of the LIV based on an analysis of AP (coronal) and lateral (sagittal) range of motion seems to prevent the development of AO. LEVEL OF EVIDENCE: 4, retrospective study.

Lumbo-sacral motion conserved after isthmic reconstruction: long-term results.

PURPOSE: The purpose of this study was to analyze the clinical and radiological results of repair of the interarticularis pars defect by a modified Buck's repair technique in patients with symptomatic spondylolysis with grade 1 spondylolisthesis. These patients with painful spondylolisthesis are the most eligible for direct repair of the defect rather than lumbo-sacral fusion in an attempt to save motion segments. METHODS: Forty-six patients with symptomatic spondylolysis with grade 1 spondylolisthesis and normal L4-L5 and L5-S1 disks, following the failure of conservative treatment, underwent surgery between 1988 and 2010. All interventions involved direct pars repair by a modified Buck's repair technique with internal fixation of the defect using screws and cancellous bone grafting. The Oswestry Disability Index (ODI) was used to evaluate the functional outcome. Healing of the pars defect was assessed by plain radiographs and computed tomography (CT) scanning. Motion of the L4-L5 and L5-S1 segments was measured with dynamic radiographs in flexion and extension. RESULTS: Thirty-five patients were evaluated. The mean follow-up period was 10 years. Functional outcome was excellent in 22 patients (ODI ≤ 10) and good for 8 patients (10 < ODI ≤ 20); five patients continued to have pain (ODI >20). Isthmus bone union occurred in 32 of 35 patients (91.4 %). L4-L5 motion was conserved with a mean angle of 11.8° (0-22); the mean lumbo-sacral angle was 9.9° (0-21). CONCLUSION: Direct repair of spondylolisthesis was described to avoid fusion in young patients with slight slipping and painful symptoms. A modified Buck's repair technique allows the conservation of L4-L5 motion with a rate of consolidation comparable to other series. The anatomy and stability of the spine were normalized by restoring the continuity of the loose posterior elements using this modified Buck's technique.

[Anaesthetic management of a child with hemoglobinopathy]. / Comment je prends en charge un enfant qui souffre d'une hémoglobinopathie.

Perioperative management of a child with sickle-cell disease requires close collaboration between hematologist, surgeon and anesthetist. The level of preoperative preparation must consider both the surgical risk and the impact of the disease. Preoperative hydration and blood transfusion are the most important part of preoperative management. Anesthetic technique is not as important as preoperative preparation, management of stress and anxiety, and optimization of intraoperative physiological parameters (oxygenation, acid-base balance, tissue perfusion, normothermia). Pain management must be optimal for these children with increased needs of analgesic. Preoperative assessment of a child with thalassemia must include evaluation of the impact of chronic hemolytic anemia and iron overload induced by repeated transfusions. The most important factor in the management of a child with glucose-6-phosphate dehydrogenase deficiency is to avoid exposure to oxidative stressors. Outpatients should be informed of the risk of hemolytic crisis, and free hemoglobin in the urine should lead to discontinuation of drugs associated with hemolysis and to maintenance of urine output to prevent acute renal failure.

Lumbosacral arthrodesis for neuromuscular scoliosis using a simplified Jackson technique.

UNLABELLED: Treating patients with severe neuromuscular scoliosis by long spinal fusion improves their quality of life and provides significant comfort for the patient and caregivers. But lumbosacral (L5-S1) fusion is challenging in these patients because of the significant deformities that result in poor bone anchoring quality and a risk of impingement between the skin and implants. In 1993, Jackson described a L5-S1 fusion technique using S1 pedicle screws and intrasacral rods (implanted under X-ray guidance) that are linked to the construct above with connectors. The goal of this study was to evaluate the clinical and radiological results and the postoperative complications of a simplified version of this technique, which does not require connectors or X-ray guidance. MATERIALS AND METHODS: Thirty-three patients were evaluated with a minimum follow-up of 4years (average 82months). Frontal balance, sagittal balance, Cobb angle, sacral slope, lumbar lordosis and lateral pelvic tilt in the frontal plane were assessed on preoperative, postoperative and follow-up X-rays. Intraoperative and postoperative complications were recorded. RESULTS: Complete fusion was obtained in 32 patients. The average Cobb angle was 62° initially and was reduced to 20° after surgery and 24° at the final follow-up. The average lateral pelvic tilt was 10.3° (0 to 26°) initially; it was surgically corrected to an average of 7.5° (0 to 24°); the average secondary loss of correction was 1.2° (0 to 9°). The sacral slope was corrected to an average of 11.2°; an average of 0.2° had been lost at the last follow-up (0 to 18°). Although the average for lumbar lordosis was unchanged, the standard deviation went from 29° to 16° after the corrective surgery and 17° at the last follow-up, with large cluster of measurements around the average value of 40°. The deformity correction was comparable to the results with other techniques (Galveston, sacroiliac screws); the complication rate was similar but the non-union rate was lower. This simplified Jackson technique appears to be an effective, simple method for L5-S1 fusion to correct neuromuscular scoliosis as it provides stable results over time. LEVEL OF EVIDENCE: Level IV, retrospective study.

Idiopathic congenital clubfoot: Initial treatment.

Clubfoot (talipes equinovarus) is a three-dimensional deformity of unknown etiology. Treatment aims at correction to obtain a functional, plantigrade pain-free foot. The "French" functional method involves specialized physiotherapists. Daily manipulation is associated to immobilization by adhesive bandages and pads. There are basically three approaches: the Saint-Vincent-de-Paul, the Robert-Debré and the Montpellier method. In the Ponseti method, on the other hand, the reduction phase using weekly casts usually ends with percutaneous tenotomy of the Achilles tendon to correct the equinus. Twenty-four hour then nighttime splinting in abduction is then maintained for a period of 3 to 4 years. Recurrence, mainly due to non-compliance with splinting, is usually managed by cast and/or anterior tibialis transfer. The good long-term results, with tolerance of some anatomical imperfections, in contrast with the poor results of extensive surgical release, have led to a change in clubfoot management, in favor of such minimally invasive attitudes. The functional and the Ponseti methods reported similar medium term results, but on scores that were not strictly comparable. A comparative clinical and 3D gait analysis with short follow-up found no real benefit with the increasingly frequent association of Achilles lengthening to the functional method (95% to 100% initial correction). Some authors actually suggest combining the functional and Ponseti techniques. The Ponseti method seems to have a slight advantage in severe clubfoot; if it is not properly performed, however, the risk of failure or recurrence may be greater. "Health economics" may prove decisive in the choice of therapy after cost-benefit study of each of these treatments.

The concordance of MRI and arthroscopy in traumatic meniscal lesions in children.

INTRODUCTION: Traumatic meniscal lesions in children must be diagnosed quickly and efficiently as a priority in order to conserve the meniscus and safeguard the future of the knee. They are often isolated and difficult to identify clinically. In the diagnostic work up stage, an excessive resort to diagnostic arthroscopy has given way to increasing use of MRI by radiologists without pediatric specialization. The present study examined the agreement between MRI aspect and arthroscopic exploration in traumatic meniscal lesions in children. PATIENTS AND METHODS: Sixty-nine knees in children aged 9 to 16 years having undergone MRI followed by arthroscopy for knee trauma between 1995 and 2008 were included in a retrospective design. Discoid meniscus was excluded. Files were reviewed by a single clinician and MRI scans by a radiologist specialized in pediatric pathology. Cases of epiphyseal fusion were excluded. All files were analyzable. Agreement with arthroscopic findings as reference was assessed for presence, location and type of meniscal lesion. RESULTS: Overall agreement with arthroscopy was respectively 78% and 82% on first and second MRI readings: 77% and 80% for the medial, and 78% and 84% for the lateral meniscus. On the first reading, there were 13 false positives for the medial and 5 for the lateral meniscus, versus 9 and 0 respectively on second reading. Overall sensitivity was 70% on first reading and 64% on second, and overall specificity 81% and 90%, respectively. DISCUSSION: The present results, in line with the literature, may appear encouraging, but hide considerable disparity between analysis of the medial and of the lateral meniscus: MRI overestimated medial and underestimated lateral meniscus lesions. CONCLUSION: MRI serves only as a support and does not provide sure diagnosis of meniscus lesion. Interpretation should take account of the clinical examination and the pediatric orthopedic specialist's experience.

Beyond glucose lowering: glucagon-like peptide-1 receptor agonists, body weight and the cardiovascular system.

AIM: Glucagon-like peptide-1 (GLP-1) belongs to the incretin hormone family: in the presence of elevated blood glucose, it stimulates insulin secretion and inhibits glucagon production. In addition, GLP-1 slows gastric emptying. GLP-1 secretion has also been reported to potentially affect patients with type 2 diabetes (T2DM) compared with non-diabetics and, as enzymatic inactivation by dipeptidyl peptidase-4 (DPP-4) shortens the GLP-1 half-life to a few minutes, GLP-1 receptor agonists such as exenatide twice daily (BID) and liraglutide have been developed, and have become part of the management of patients with T2DM. This review focuses on the potential beneficial effects of these compounds beyond those associated with improvements in blood glucose control and weight loss, including changes in the cardiovascular and central nervous systems. METHODS: This was a state-of-the-art review of the literature to evaluate the relationships between GLP-1, GLP-1 receptor agonists, weight and the cardiovascular system. RESULTS: GLP-1 receptor agonists improve glucose control and do not significantly increase the risk of hypoglycaemia. Also, this new class of antidiabetic drugs was shown to favour weight loss. Mechanisms may involve central action, direct action by reduction of food intake and probably indirect action through slowing of gastric emptying. The relative importance of each activity remains unclear. Weight loss may improve cardiovascular outcomes in patients with T2DM, although GLP-1 receptor agonists may have other direct and indirect effects on the cardiovascular system. Reductions in myocardial infarct size and improvements in cardiac function have been seen in animal models. Beneficial changes in cardiac function were also demonstrated in patients with myocardial infarcts or heart failure. Indirect effects could involve a reduction in blood pressure and potential effects on oxidation. However, the mechanisms involved in the pleiotropic effects of GLP-1 receptor agonists have yet to be completely elucidated and require further study. CONCLUSION: These compounds may play an important role in the treatment of patients with T2DM as their potential effects go beyond glucose-lowering (weight loss, potential improvement of cardiovascular risk factors). However, to better understand their place in the management of T2DM, further experimental and clinical prospective studies are required.

Physeal-sparing reconstruction of anterior cruciate ligament tears in children: results of 57 cases using patellar tendon.

This study evaluated the results of a physeal-sparing technique of intra-articular anterior cruciate ligament (ACL) reconstruction in skeletally immature patients, with particular reference to growth disturbance. Between 1992 and 2007, 57 children with a mean age of 12.2 years (6.8 to 14.5) underwent ACL reconstruction using the same technique. At a mean of 5.5 years (2 to 14) after surgery, 56 patients underwent clinical and radiological evaluation. At that time, 49 patients (87.5%) had reached bony maturity and 53 (95%) achieved A or B according to the IKDC 2000 classification. Four patients had stopped participation in sports because of knee symptoms, and three patients (5.4%) had a subsequent recurrent ACL injury. There was no clinical or radiological evidence of growth disturbance after a mean growth in stature of 20.0 cm (3 to 38). This study demonstrates that ACL reconstruction sparing the physes in children is a safe technique protecting against meniscal tears and giving better results than reconstruction in adults, without causing significant growth disturbance.

Congenital scoliosis: a frontal plane evaluation of 251 operated patients 14 years old or older at follow-up.

INTRODUCTION: Congenital scoliosis, carrying an incidence between 0.5 and 1 per 1000 births, raise the problem of their evolutive potential. HYPOTHESIS: Some predictive factors for the evolution of scoliotic curvature due to congenital vertebral malformation (CVM) can be found. MATERIAL AND METHODS: This was a retrospective multicenter study of 251 patients, at least 14 years old when evaluated at end of follow-up, with CVM and spinal deformity predominating in the frontal plane. RESULTS: 38.8% of patients showed associated neurologic, visceral or orthopedic abnormalities. CVM was single in 60.6%, double in 20.3%, triple in 6.4% and multiple in 12.7% of cases. 34.1% of CVMs were thoracic. Congenital scoliosis curvature was single in 88.8% of patients, double in 10% and triple in 1.2%. Mean curvature angle was 31.7° at diagnosis (range, 0-105°) and 41.3° preoperatively (range, 10-105°). Sixty-one patients showed associated kyphosis. Mean change in postoperative curvature angle over follow-up was 1.6° (range, -20° to 38°) in the 73 patients managed by arthrodesis, -0.4° (-24° to 30°) in the 64 managed by epiphysiodesis, and 0.4° (-18° to 35°) in the 49 managed by hemivertebral (HV) resection. Results were found to correlate significantly with age at surgery for patients managed by epiphysiodesis, but not for those managed by HV resection or arthrodesis. DISCUSSION: More than 30% of congenital scolioses involve associated intraspinal abnormality. All CVM patients should therefore undergo medullary and spinal MRI to assess the CVM in all three planes, and the medullary canal and its content. The evolution of scoliotic curvature induced by CVM is hard to predict. Several factors are to be taken into account: CVM type, number and location, and patient age. Curvature progression may be slow or very fast. It accelerates during the peak of puberty, stabilizing with bone maturity. Surgery is mandatory in evolutive scoliosis. Four procedures may be recommended, according to type of CVM and especially to patient age: arthrodesis, convex epiphysiodesis, HV resection or rib distraction. Surgery seeks to correct the spinal deformity induced by the CVM and prevent compensatory curvature and neurologic complications, while conserving sagittal and frontal spinal balance and sparing as many levels as possible. In case of HV involvement, the procedure of choice is CVM resection, which provides 87.5% good results in this indication; the procedure is relatively safe, conservative of spinal levels, and without age limit. LEVEL OF EVIDENCE: Level IV. Retrospective study.

New genetic associations detected in a host response study to hepatitis B vaccine.

The immune response to hepatitis B vaccination differs greatly among individuals, with 5-10% of healthy people failing to produce protective levels of antibodies. Several factors have been implicated in determining this response, chiefly individual genetic variation and age. Aiming to identify genes involved in the response to hepatitis B vaccination, a two-stage investigation of 6091 single-nucleotide polymorphisms (SNPs) in 914 immune genes was performed in an Indonesian cohort of 981 individuals showing normal levels of anti-HBs versus 665 individuals displaying undetectable levels of anti-HBs 18 months after initial dose of the vaccine. Of 275 SNPs identified in the first stage (476 normal/372 nonresponders) with P<0.05, significant associations were replicated for 25 polymorphisms in 15 genes (503 normal/295 nonresponders). We validated previous findings (HLA-DRA, rs5000563, P-value combined=5.57 x 10(-10); OR (95%CI)=0.61 (0.52-0.71)). In addition, we detected a new association outside of the human leukocyte antigen loci region that passed correction for multiple testing. This SNP is in the 3' downstream region of FOXP1, a transcription factor involved in B-cell development (P-value combined=9.2 x 10(-6); OR (95%CI)=1.38 (1.2-1.6)).These findings might help to understand the biological reasons behind vaccine failure and other aspects of variation in the immune responses of healthy individuals.