Takayasu arteritis: Prevalence and clinical presentation in Switzerland.

OBJECTIVE Takayasu arteritis (TAK) is a rare immune-mediated vasculitis of the aorta and its branches. Aims were to calculate prevalence and incidence in Switzerland, to assess disease activity and performance of MR-Angiography (MRA). METHODS 31 patients were recorded in a database, 27 were followed prospectively up to 3 years. Prevalence was calculated based on data of the national statistical bureau. Disease activity was defined using the revised EULAR criteria. MRA depicted stenotic changes and aortic wall enhancement. RESULTS A disease prevalence of 14.5/1.000.000 inhabitants and an incidence of 0.3/1.000.000 per year was calculated. Aortic wall enhancement was found in 10 patients while in clinical and serological remission. EULAR criteria missed 5 patients with disease activity with isolated elevations of ESR/CRP. Arterial stenosis did not change over time in 5 cases, it improved in 2 and increased in 7. At follow-up 16 patients were treated with tocilizumab, 11/16 in monotherapy, 5 patients were treatment-free, 25/27 stayed in remission. CONCLUSION In addition to prevalence and incidence, our data show that MRA qualifies to detect subclinical disease activity, but, on the other hand, that EULAR criteria may miss disease activity in case of isolated elevation of ESR/CRP

Magnetic resonance angiography in giant cell arteritis: results of a randomized controlled trial of tocilizumab in giant cell arteritis

Objective. To analyse magnetic resonance angiographic (MRA) vessel wall signals from a randomized controlled trial of tocilizumab (TCZ) to treat GCA. Methods. Participants were assigned in a 2:1 ratio to receive either TCZ + glucocorticoids (GCs) or placebo +GC infusions at 4-week intervals for 52 weeks. GCs were started at 1 mg/kg/day, then tapered to 0.1 mg/kg/day at week 12 and thereafter down to zero. Patients with initial positive MRA findings underwent control MRA at weeks 12 and 52. Vessel wall signals were scored from 0 (normal) to 3 (intense late enhancement). Outcomes were the number of patients with complete MRA remission at weeks 12 and 52, and changes in vasculitis score, vessel anatomy and atherosclerosis. Results. Of the 30 randomized participants, nine TCZ and two placebo patients had no vessel wall enhancement on initial MRA. At week 12, MRAs were performed in nine TCZ and four placebo patients (nine and three in clinical remission, respectively). Three (33%) TCZ patients showed normalization of vessel wall signals compared with one (25%) placebo patient. At week 52, there was additional MRA improvement in some TCZ patients, but one-third showed persistent or increased late vessel wall enhancement. There was no formation of aneurysms or stenosis and no increase in atherosclerosis. Conclusions. Although TCZ resulted in complete clinical and laboratory remission of GCA over 52 weeks, MRA signals in vessel walls normalized in only one-third of patients. Whether these signals are of prognostic importance remains to be determined

Pleomorphic rhabdomyosarcoma with an impressive response to chemotherapy: case report and review of the literature.

PURPOSE Pleomorphic rhabdomyosarcoma (RMS) represents a rare sarcoma subtype of the adult population. Due to its clinical characteristics, treatment is currently based on the guidelines for adult soft tissue sarcoma therapy. Hence, in the metastatic setting, doxorubicin-containing regimens are used in the sarcoma community, with limited treatment benefit. Scanty published data are available on the efficacy of systemic treatment. Whether treatment response and outcome of these patients could be improved by using pediatric protocols used typically in other RMS subtypes, like embryonal and alveolar RMS, is unclear. We report on an impressive effect of multiagent pediatric chemotherapy in an adult patient with metastatic pleomorphic RMS. METHODS We present the case of a 70-year-old man with metastatic pleomorphic RMS of his left thigh. Systemic chemotherapy according to the VAC regimen (vincristine, actinomycin, cyclophosphamide) was initiated. Follow-up clinical and radiologic assessment demonstrated an impressive treatment response. RESULTS Sixteen months after primary diagnosis, computed tomography scan shows no signs of tumor progression. CONCLUSIONS Our case report emphasizes that multiagent systemic therapy according to pediatric protocols should be considered in adult patients with pleomorphic RMS

The Influence of Various Cerebral and Extracerebral Pathologies on Apparent Diffusion Coefficient Values in the Fetal Brain

BACKGROUND AND PURPOSE: The changing MRI signal accompanying brain maturation in fetal brains can be quantified on apparent diffusion coefficient (ADC) maps. Deviations from the natural course of ADC values may reflect structural pathology. The purpose of this study was to determine the influence of fetal pathologies on the ADC values in different regions of the fetal brain and their evolution with increasing gestational age. METHODS: This was a retrospective study of 291 fetuses evaluated between the 14th and the 40th week of gestation using diffusion-weighted imaging (DWI). Fetuses with normal MRI findings but sonographically suspected pathology or fetuses with abnormalities not affecting the brain were analyzed in the control group and compared to fetuses suffering from different pathologies like hydrocephalus/ventriculomegaly, brain malformations, infections, ischemia/hemorrhage, diaphragmatic hernias, and congenital heart disease. Pairwise ADC measurements in each side of the white matter (WM) of the frontal, parietal, and occipital lobes, in the basal ganglia and the cerebellum, as well as a single measurement in the pons were performed and were plotted against gestational age. RESULTS: In the control group, brain maturation followed a defined gradient, resulting in lower ADC values in the most mature regions. Each disorder group experienced abnormal patterns of evolution of the ADC values over time deviating from the expected course. CONCLUSIONS: The ADC values in different regions of the fetal brain and their evolution with increasing gestational age are influenced by pathologies compromising the cerebral maturation

Surgical exposures and options for instrumentation in acetabular fracture fixation: Pararectus approach versus the modified Stoppa.

BACKGROUND As an alternative to the modified Stoppa approach, the Pararectus approach is used clinically for treatment of acetabular fractures involving the anterior column. The current study assessed the surgical exposure and the options for instrumentation using both of these approaches. METHODS Surgical dissections were conducted on five human cadavers (all male, mean age 88 years (82-97)) using the modified Stoppa and the Pararectus approach, with the same skin incision length (10cm). Distal boundaries of the exposed bony surfaces were marked using a chisel. After removal of all soft-tissues, distances from the boundaries in the false and true pelvis were measured with reference to the pelvic brim. The exposed bone was coloured and calibrated digital images of each inner hemipelvis were taken. The amount of exposed surface using both approaches was assessed and represented as a percentage of the total bony surface of each hemipelvis. For instrumentation, a suprapectineal quadrilateral buttress plate was used. Screw lengths were documented, and three-dimensional CT reconstructions were performed to assess screw trajectories qualitatively. Wilcoxon's signed rank test for paired groups was used (level of significance: p<0.05). RESULTS After utilization of the Pararectus approach, the distances from the farthest boundaries of exposed bone towards the pelvic brim were significantly higher in the false but not the true pelvis, compared to the modified Stoppa approach. The percentage (mean±SD) of exposed bone accessible after utilizing the Pararectus approach was 42±8%, compared to 29±6% using the modified Stoppa (p=0.011). In cadavers exposed by the Pararectus approach, screws placed for posterior fixation and as a posterior column screw were longer by factor 1.8 and 2.1, respectively (p<0.05), and screws could be placed more posteromedial towards the posterior inferior iliac spine or in line with the posterior column directed towards the ischial tuberosity. CONCLUSION Compared to the modified Stoppa, the Pararectus approach facilitates a greater surgical access in the false pelvis, provides versatility for fracture fixation in the posterior pelvic ring and allows for the option to extend the approach without a new incision

Transfer of osteochondral shell autografts to salvage femoral head impaction injuries in hip trauma patients.

INTRODUCTION Femoral head impaction defects are observed with variable severity, as a result of traumatic hip dislocations which can be caused by traffic accidents or seen in professional athletes amongst other mechanisms. Compression of the articular cartilage and the subchondral bone into the femoral head results in irregular articular surfaces influencing the outcome with predisposition to osteoarthritis, and being predictive for the need for delayed total hip replacement. This study reports the outcome after a minimum follow-up (FU) of five years in a consecutive series treated with transfer of osteochondral shell autografts in hips (TOSAH) from the head-neck junction into the defect using surgical hip dislocation. PATIENTS AND METHODS Between 06/2007 and 03/2014 a series of twelve consecutive patients (mean age: 35yrs, range 18-53; median Injury Severity Score: 12, range 9-27) sustained a traumatic posterior hip dislocation in combination with acetabular and/or Pipkin fractures and were inter alia treated using TOSAH using surgical hip dislocation. Conversion to total hip replacement (THR) during FU was noted as failure. Patients were clinically (Merle d'Aubigné score) and radiographically assessed for occurrence of osteoarthritis (OA), avascular necrosis (AVN) and/or heterotopic ossification (HO) at a minimal follow-up of five years. RESULTS Mean follow-up was 6.9 years (5.0-11.6). At five-year follow-up, we found a survivorship of 57.1% (95% Confidence interval {CI}, 46.7-100%). Four patients required conversion to a total hip replacement at 11, 16, 28 and 44 months respectively after the TOSAH procedure due to osteoarthritis progression. One patient required conversion to a total hip replacement 12 months after TOSAH procedure due to AVN. One patient was lost to follow-up after 2.7 years. The remaining six patients with preserved hips presented with a median Merle-d'Aubigné score of 16 points (range: 14-18) and no AVN. Two patients showed asymptomatic grade I osteoarthritis according to Tönnis at latest follow-up and three patients showed mild asymptomatic HO according to Brooker (Grade I-II). CONCLUSION The presented technique can be used as a salvage procedure for severely injured hip joints and to preserve the hip joint at midterm with satisfying clinical and radiological outcomes

Nonlinear three-dimensional noise filter with low-dose CT angiography: effect on the detection of small high-contrast objects in a phantom model

To study the effect of a nonlinear noise filter on the detection of simulated endoleaks in a phantom with 80- and 100-kVp multidetector computed tomographic (CT) angiography

Comparison of dual-energy subtraction and electronic bone suppression combined with computer-aided detection on chest radiographs: effect on human observers' performance in nodule detection

The objective of our study was to compare the effect of dual-energy subtraction and bone suppression software alone and in combination with computer-aided detection (CAD) on the performance of human observers in lung nodule detection

Magnetic resonance imaging to monitor disease activity in giant cell arteritis treated with ultra-short glucocorticoids and tocilizumab.

OBJECTIVES MRI is well established for diagnosing GCA. Its role in monitoring disease activity has yet to be determined. We investigated vascular and musculoskeletal inflammation using MRI in the patients of the GUSTO trial to assess the utility of MRI in monitoring disease activity. METHODS Eighteen patients with newly diagnosed GCA received 500 mg methylprednisolone intravenously for 3 consecutive days followed by tocilizumab monotherapy from day 3 until week 52. Cranial, thoracic and abdominal MRI exams were performed at baseline (active, new-onset disease), and at weeks 24, 52 (remission on-treatment), and 104 (remission off-treatment). MRI findings typical for PMR as well as extent and severity of vasculitic disease were rated. RESULTS In total, 673 vascular segments and 943 musculoskeletal regions in 55 thoracic/abdominal MRI and 490 vascular segments in 49 cranial MRI scans of 18 patients were analysed. Vasculitic vessels were still detectable in one in four cranial segments at week 24. At weeks 52 and 104, no cranial vascular segment showed a vasculitic manifestation. Large vessels, except for the ascending aorta, and PMR displayed little or no decrease in inflammatory findings over time. CONCLUSION Vasculitic manifestations in the cranial vessels normalised after 52 weeks of treatment, whereas large vessel and PMR findings persisted despite lasting full remission. The dynamics of cranial vessel signals suggest that MRI of these arteries might qualify as a potential diagnostic tool for monitoring disease activity and for detecting relapse after 52 weeks of treatment