Mucin-producing adenocarcinoma arising in an atrial myxoma.

We describe the unique autopsy findings of a patient who died of a metastasizing giant right atrial adenocarcinoma containing few areas of typical myxoma. That no mucin-producing extracardiac tumor was detected pointed to the atrial adenocarcinoma as being the primary. We hypothesize that the adenocarcinoma may have developed from coexistent bland glandular structures within the myxoma.

Aortic remodelling in Fabry disease.

AIMS: To evaluate thoracic aortic dilation in patients with Fabry disease (FD). METHODS AND RESULTS: A cohort of 106 patients with FD (52 males; 54 females) from three European centres were studied. The diameter of the thoracic aorta was assessed at three levels (sinus of Valsalva, ascending aorta, and descending aorta) using echocardiograms and cardiovascular magnetic resonance imaging. Aortic dilation at the sinus of Valsalva was found in 32.7% of males and 5.6% of females; aneurysms were present in 9.6% of males and 1.9% of females. No aortic dilation was observed in the descending aorta. There was no correlation between aortic diameter at the sinus of Valsalva and cardiovascular risk factors. CONCLUSION: Fabry disease should be considered as a cardiovascular disease that affects the heart and arterial vasculature, including the thoracic aorta. Thus, patients with FD should be closely monitored for the presence, and possible progression and complications of aortic dilation. CLINICAL TRIAL REGISTRATION: Protocol 101/01. Ethics committee, Faculty of Medicine, Lausanne.

Comparison of diffusion-weighted whole body MRI and skeletal scintigraphy for the detection of bone metastases in patients with prostate or breast carcinoma.

PURPOSE: To prospectively compare the diagnostic accuracy of diffusion-weighted whole body imaging with background whole body signal suppression (DWIBS) with skeletal scintigraphy for the diagnosis and differentiation of skeletal lesions in patients suffering from prostate or breast cancer. MATERIAL AND METHODS: A diagnostic cohort of 36 patients was included in skeletal scintigraphy and 1.5 T DWIBS MRI. Based on morphology and signal intensity patterns, two readers each identified and classified independently, under blinded conditions, all lesions into three groups: (1) malignant, (2) unclear if malignant or benign and (3) benign. Finally, for the definition of the gold standard all available imaging techniques and follow-up over a minimum of 6 months were considered. RESULTS: Overall, 45 circumscribed bone metastases and 107 benign lesions were found. DWIBS performed significantly better in detecting malignant skeletal lesions in patients with more than 10 lesions (sensitivity: 0.97/0.91) compared to skeletal scintigraphy (sensitivity: 0.48/0.42). No statistical difference could be found between DWIBS (0.58/0.33) and skeletal scintigraphy (0.67/0.58) in the sensitivity values for malignant skeletal lesions in patients with less than 5 lesions. For benign lesions, scintigraphy scored best with a sensitivity of 0.93/0.87 compared to 0.20/0.13 for DWIBS. Interobserver agreement with Cohen's kappa coefficient was calculated as 0.784 in the case of scintigraphy and 0.663 for DWIBS. CONCLUSION: With respect to staging, in prostate and breast carcinoma, the DWIBS technique is not superior to skeletal scintigraphy, but ranks equally. However, in the cases with many bone lesions, markedly more metastases could be discovered using the DWIBS technique than skeletal scintigraphy.

High resolution computerized tomography of the chest and pulmonary function testing in evaluating the effect of tobramycin solution for inhalation in cystic fibrosis patients.

To evaluate the sensitivity of high-resolution computerized tomography (HRCT) of the chest compared to spirometry measures in evaluating the effects of tobramycin solution for inhalation (TSI) in cystic fibrosis (CF) patients.Thirty-two subjects >/=6 years old with mild to moderate CF lung disease were enrolled in a randomized, double-blind, placebo-controlled pilot study. Duration was 28 days; 31 subjects completed the study.HRCT scores decreased 4.06 +/- 3.20 (mean +/- SD) for TSI and decreased 0.17 +/- 1.78 for placebo subjects (P = 0.13). Mean forced expiratory flow during middle half of forced vital capacity (FEF(25%-75%)) predicted increased 6.08 +/- 4.86 for TSI and decreased 0.60 +/- 2.34 for placebo (P = 0.23). Percentage forced expiratory volume in 1 s (FEV(1)) predicted increased slightly for both TSI and placebo (1.29 +/- 3.33 for TSI and 1.17 +/- 1.4 for placebo) (P = 0.97). Two of eight HRCT component scores (atelectasis and inhomogeneity) were observed to be highly discordant with observed HRCT global total score and other HRCT component scores. A modified total score was calculated by dropping them from the global total score. The modified HRCT total scores decreased 6.68 +/- 3.09 for TSI subjects and increased 0.02 +/- 2.0 for the placebo subjects (P = 0.07). Sample sizes were calculated to show statistical significance by differences in modified total HRCT scores, global total HRCT scores, FEF(25%-75%) predicted or FEV(1) % predicted. A total of 60, 100, 200, and over 800 patients would be necessary respectively.HRCT can be a useful measure of change in CF pulmonary disease, requiring a smaller sample size than that required to show treatment effect by pulmonary function testing (PFT) alone.

Arterial thromboembolic complications in critically ill children.

PURPOSE: To evaluate incidence and characteristics of arterial thromboembolic complications in critically ill children. MATERIALS AND METHODS: Hospital records of all consecutive patients with arterial thromboembolic events (ATEs) occurring in the pediatric intensive care unit (PICU) from January 1997 to August 2001 were reviewed. Data collected included demographics and location, treatment modalities and outcome of ATEs. RESULTS: Fifty-four ATEs in 51 children (median age, 14 days) were identified, reflecting an incidence of 1.2% of all PICU patients. Arterial thromboembolic events were located in peripheral arteries in 52 (96%) cases and were associated with indwelling arterial catheters (n=26) or cardiac catheterization (n=26). The remaining 2 ATEs were located in the left ventricle and cerebral arteries, respectively. Therapy consisted of heparin (n=51), thrombolysis (n=22), oral anticoagulation (n=12), and aspirin (n=34). Complete resolution was noted in 33 (70%), partial resolution in 10 (21%), and no resolution in 4 (8.5%) cases. Bleeding complications occurred in 1 patient treated with heparin and in 12 (54%) of the 22 patients receiving thrombolytic therapy. CONCLUSIONS: Arterial thromboembolic events are frequent complications of PICU, particularly affecting neonates, and mostly associated with catheters. Studies to determine safe and effective prophylactic and treatment modalities of ATEs in children are required.

The use of high resolution computerized tomography (HRCT) of the chest in evaluating the effect of tobramycin solution for inhalation in cystic fibrosis lung disease.

OBJECTIVES: To compare the usefulness of HRCT of the chest versus spirometric measures (PFTs) in evaluating the effect of tobramycin solution for inhalation (TSI) in cystic fibrosis (CF). METHODS: Thirty-two CF patients with mostly mild lung disease age > or = 6 years, were enrolled in a double-blind, placebo-controlled pilot study. Patients were chronically colonized with Pseudomonas aeruginosa for at least 6 months prior to and at enrollment. If patients were on TSI, they were taken off for at least 3 months prior to enrollment. Duration was 6 months; 31 subjects completed the study. HRCT and PFTs were evaluated at baseline, after 28 days of treatment and at the end of the study. Study medication was administered as 5 ml nebulized treatment twice a day for 28 days followed by 28 days off (one cycle). Study consisted of three cycles. Two radiologists scored all films using a validated system. A total HRCT score consists of the sum of subscores: linear opacities, hyperinflation, nodular opacities, peribronchial thickening, mucous plugging, and bronchiectasis; each subscore could range from 0 to 80, with potential total scores varying from 0 to 480. The percent of the maximum possible HRCT score was then calculated and used for all comparisons. RESULTS: Using two tailed paired t-test, the percent maximum HRCT score decreased by 1.4 +/- 2.6% (mean +/- SD) (P = 0.049) and 0.3 +/- 2.8% (P = 0.63) for the TSI group and decreased by 0.1 +/- 1.5% (P = 0.74) and increased by 0.6 +/- 1.8% (P = 0.23) for the placebo group between visits 1 and 2, and visits 1 and 3, respectively. The data were then analyzed using a mixed model utilizing changes in scores over the durations of the study for each group. The change of HRCT score for the TSI group was -0.24/day (P = 0.02) and -0.03/day (P = 0.22), and for the control group the change was -0.01 (P = 0.93) and 0.02 (P = 0.29) between visits 1 and 2, and visits 1 and 3 respectively. FEF(25-75)% and FEV(1)% changes were not statistically significant using both analyses. CONCLUSION: HRCT seems to be more sensitive in detecting treatment effect than PFT in CF patients with mild lung disease, especially following the first treatment period (visit 2). Total HRCT score showed some improvement at the end of the study, though not statistically significant. This is probably due to obtaining the HRCT an average of 30 days after completion of the TSI treatment, and selection of study population with mostly mild lung disease. This could indicate that the most significant improvement in the total HRCT score in this patient population occurs after the first treatment period with TSI.

Clinical experience in timed arterial compression contrast-enhanced magnetic resonance angiography of the hand.

PURPOSE: A retrospective analysis of the diagnostic performance of the timed arterial compression (TAC) technique, which allows freezing of the contrast bolus during first-pass contrast-enhanced (CE) magnetic resonance angiography (MRA) to diagnose vascular pathologies in the hand. MATERIAL AND METHODS: A total of 14 consecutive CE-MRAs of the hand were acquired by using the TAC technique. By inflating a blood pressure cuff up to 200 mm Hg triggered to the arterial contrast filling of the hand, prolonged measurement times up to 144 seconds, with a spatial resolution of 0.59 × 0.59 × 0.8 mm(3), could be realized. Overall image qualities, arterial signal-to-noise ratio (SNR), contrast-to-noise ratio (CNR), and vessel conspicuity besides the final diagnosis were evaluated. RESULTS: All 14 TAC-CE-MRAs of the hand were successfully accomplished without any adverse events and yielded, in all cases, a final diagnosis with a high total number of vascular pathologies (57). High arterial SNR and CNR values exceeding the soil of 85 and 60, respectively, resulted. Thus, overall vessel visibility (>90%), vessel conspicuity (mean Δ signal intensity [SI]/mm = 1,193) and image quality on a per patient level (>60%) were rated as excellent or good. CONCLUSIONS: TAC-CE-MRA of the hand offers high diagnostic performance because of its increased spatial resolution while preserving contrast, which allowed detection of tiny stenoses of the digital arteries.

Improved vessel delineation in keyhole time-resolved contrast-enhanced MR angiography using a gadolinium doped flush.

PURPOSE: To prospectively assess the influence of a gadolinium doped saline flush compared with a pure saline flush on the image quality of the supra-aortic vessels using time-resolved contrast-enhanced MR angiography (4D CE-MRA) in a randomized double blind clinical trial. MATERIALS AND METHODS: Twenty-two patients scheduled for contrast-enhanced craniocerebral MRI underwent a supplemental 4D CE-MRA covering the carotids to the superior sinus consisting of 30 dynamics of a T1-weighted 3D gradient-echo sequence (FFE) in sagittal direction. The temporal resolution of 1.1 s per dataset was achieved using the keyhole technique with the reference scan acquired at the end. Immediately after the intravenous (IV) injection of 0.1 mmol Gd/kg body weight of gadoterate, our patients received a 50-mL flush consisting either of a 0.9% saline solution (n = 11) or doped with 50 mM gadolinium (n = 11; total Gd: 0.11 mmol/kg) at a flow-rate of 2 mL/s. Vessel delineation, image quality, signal-to-noise- (SNR) and contrast-to-noise (CNR) ratios over time were compared. RESULTS: Both vessel delineation (internal carotid artery [ICA]: slope(saline) = 308.5; slope(Gd) = 528.9; P = 0.006; superior sagittal sinus [SSS]: slope(saline) = 505.3; slope(Gd) = 674.9; P = 0.007) and CNR (ICA: CNR(saline) = 57.3; CNR(Gd) = 80.55; P = 0.0417; SSS: CNR(saline) = 74.15; CNR(Gd) = 117.4; P = 0.0331) of the ICA and SSS were significantly increased using the gadolinium doped flush. CONCLUSION: A low concentrated gadolinium flush in comparison to a pure saline flush improves significantly vessel contrast and their delineation in time-resolved CE-MRA using the keyhole technique.

Comparison of neutral oral contrast versus positive oral contrast medium in abdominal multidetector CT.

To determine whether neutral contrast agents with water-equivalent intraluminal attenuation can improve delineation of the bowel wall and increase overall image quality for a non-selected patient population, a neutral oral contrast agent (3% mannitol) was administered to 100 patients referred for abdominal multidetector row computed tomography (MDCT). Their results were compared with those of 100 patients given a positive oral contrast agent. Qualitative and quantitative measurements were done on different levels of the gastrointestinal tract by three experienced readers. Patients given the neutral oral contrast agent showed significant better qualitative results for bowel distension (P < 0.001), homogeneity of the luminal content (P < 0.001), delineation of the bowel-wall to the lumen (P < 0.001) and to the mesentery (P < 0.001) and artifacts (P < 0.001), leading to a significant better overall image quality (P < 0.001) than patients receiving positive oral contrast medium. The quantitative measurements revealed significant better distension (P < 0.001) and wall to lumen delineation (P < 0.001) for the patients receiving neutral oral contrast medium. The present results show that the neutral oral contrast agent (mannitol) produced better distension, better homogeneity and better delineation of the bowel wall leading to a higher overall image quality than the positive oral contrast medium in a non-selected patient population.

Multicenter trial: comparison of two different formulations and application systems of low-dose nasal midazolam for routine magnetic resonance imaging of claustrophobic patients.

PURPOSE: To prospectively assess and compare two formulations and methods of administration of low-dose nasal midazolam for the treatment of claustrophobic patients undergoing magnetic resonance imaging (MRI) as part of a multicenter Phase III trial. MATERIALS AND METHODS: In all, 108 consecutive adult claustrophobic patients were randomly assigned to one of two treatment groups (multidose group: MDG, unit-dose group: UDG). MDG encompassed 55 patients who received intranasally a 0.5% midazolam formulation into each nostril (total dose, 1.0 mg), whereas the 53 patients in UDG received a 1% midazolam formulation into only one nostril (total dose, 1.0 mg). This initial dose could be repeated once. Patient tolerance and anxiety were assessed using a questionnaire and a visual analog scale immediately before and after MRI. Image quality was evaluated using a five-point scale. RESULTS: In all, 53/55 MR examinations (96%) with MDG and 52/53 (98%) with UDG were completed successfully. The dose of 1 mg had to be repeated significantly less often in UDG compared to MDG (4/53, 8% vs. 13/55, 24%; P = 0.003). The image quality of all MR examinations was rated good to excellent, and slightly better in UDG (P = 0.045). CONCLUSION: Nasally applied low-dose midazolam is a patient-friendly solution to facilitate MRI of claustrophobic patients. The nasal spray of UDG is superior to that of MDG with a necessity of additional dosing.

Altered myocardial motion pattern in Fabry patients assessed with CMR-tagging.

Progressive left ventricular hypertrophy is the hallmark of cardiac manifestations in patients with Fabry disease. Cardiovascular magnetic resonance with tissue tagging allows detailed assessment of the cardiac motion pattern. The aim was to test the hypothesis that not only Fabry patients with severe left ventricular hypertrophy exhibit changes in myocardial motion, but also Fabry patients with normal left ventricular mass. Magnetic resonance tagging using slice following complementary spatial modulation of magnetization (CSPAMM) combined with harmonic phase analysis (HARP) was applied to measure left ventricular shortening and contraction. Additionally, left ventricular rotation and global left ventricular torsion were examined. Twenty-nine Fabry patients grouped in patients with (n = 13) and without (n = 16) left ventricular hypertrophy were compared with 29 age and sex matched healthy volunteers. Long axis shortening and circumferential contraction showed reduced peak values with increasing left ventricular mass and were significantly reduced in Fabry patients with left ventricular hypertrophy (p < 0.001 and p < 0.05, respectively). Torsional deformation and apical rotation were increased both in Fabry patients with left ventricular hypertrophy as well as in patients with normal left ventricular mass (p < 0.001 for torsion) compared with controls. Applying the magnetic resonance tagging acquisition and analysis methods, myocardial motion abnormalities could not only be measured in Fabry patients with left ventricular hypertrophy but also in patients without macroscopic cardiac involvement.

Pigmented villonodular synovitis: MR imaging in pediatric patients.

OBJECTIVES: To describe the usefulness of gradient-echo imaging and contrast-enhanced MR imaging in diagnosing and assessing the extent of pigmented villonodular synovitis (PVNS) in pediatric patients. PATIENTS AND METHODS: Retrospective analysis of five pediatric patients (median age 14 years) with PVNS of the knee examined with a 1.5-T unit. Signal characteristics were obtained comparing T1- and proton-density (PD)-weighted imaging with gradient-echo imaging and enhanced imaging. The extent of the disease was assessed using the arthroscopic approach. Signal intensity ratios were statistically analyzed. RESULTS: Compared to PD- and T1-weighted imaging sequences, gradient-echo imaging provides superior depiction of the extent of the disease due to signal decay (T2*-effect) of hemosiderin-laden thickened synovium and masses. Inflamed synovium with low hemosiderin deposition was identified on enhanced imaging. Beside the suprapatellar bursa and Hoffa's fat pad, the area posterior to the cruciate ligaments is commonly involved. No bony abnormalities were present. CONCLUSION: Gradient-echo imaging together with enhanced imaging is useful in diagnosing and assessing the extent of PVNS in pediatric patients. A low signal mass behind the cruciate ligaments may represent an important diagnostic feature. Bony abnormalities were always absent.

Ultrasonography of the annular ligament partial tear and recurrent "pulled elbow".

A 2-year-old boy had multiple episodes of radial head subluxation, recurrent "pulled elbow." On initial ultrasonography, there was subluxation of the radial head in relation to the capitellum, and the annular ligament was partially disrupted. Following brace treatment, subluxation of the radial head was corrected and annular ligament disruption healed. As shown by ultrasonography, these findings correlated well with the clinical course and can be utilized to guide treatment.

Primary cerebral leiomyosarcoma in a child.

We present a 13-year-old boy with a low-grade primary intra-axial cerebral leiomyosarcoma. MRI showed a contrast-enhancing intra-axial mass with features of a cell-rich tumour. The diagnosis was established after neurosurgery and exclusion of an extracerebral primary tumour. The resection of local recurrence 6 months later confirmed the diagnosis, but a higher grade of malignancy was established. The patient died 15 months after the onset of symptoms from general intracranial and intraspinal spread.

Giant cardiac myxoma with malignant transformed glandular structures.

A case of a right-sided giant cardiac myxoma with malignant transformation of glandular structures causing systemic metastases is described. Plain chest radiography and computed tomography localized the tumor within the heart. Exact depiction of the origin of the tumor using subtracted 2D-projection MR angiography is documented. Radiologic findings and differential diagnosis of this unique tumor are discussed.