[Thoracic imaging in cystic fibrosis : Radiography]. / Thoraxbildgebung bei zystischer Fibrose : Radiographie.

CLINICAL ISSUE: Patients with cystic fibrosis (CF) require regular follow-up examinations, usually from birth onwards, using imaging techniques. METHODS: The conventional chest x­ray examination is the only technique recommended by the guidelines at this age. The examination can be performed at every age and is well standardized. The radiation dose to the patient is small and does not require any sedation. RADIOLOGICAL FINDINGS AND CLASSIFICATION: The typical structural changes of the bronchi and the lung parenchyma can be depicted. Typical findings are air-trapping, bronchiectasis, peribronchial cuffing, bronchial wall thickening, mucus plugging, nodular opacities, atelectasis and/or consolidations and hilar lymphadenopathy. Different scoring systems have been developed to allow for a relatively easy but reproducible assessment of the severity of the disease. The most important ones for daily clinical practice are the Chrispin-Norman score, Brasfield score, and the Wisconsin score. All of them show a good correlation with pulmonary function parameters and the clinical picture of the patients. PRACTICAL RECOMMENDATIONS: It is recommended to take an annual X­ray for patients with CF, and in addition in individual cases with clinical deterioration. Computed tomography (CT) can be performed if relevant information for treatment is expected to gained.

[Joint Statement of the German Respiratory Society and German Society of Thoracic Surgery in Cooperation with the German Radiological Society: Structural Prerequisites of Centers for Interventional Treatment of Emphysema]. / Positionspapier der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin und der Deutschen Gesellschaft für Thoraxchirurgie in Kooperation mit der Deutschen Röntgengesellschaft: Strukturvoraussetzungen von Zentren für die interventionelle Emphysemtherapie.

Interventional treatment of emphysema offers a wide range of surgical and endoscopic options for patientes with advanced disease. Multidsciplinary collaboration of pulmonology, thoracic surgery and imaging disciplines in patient selection, therapy and follow up ensures treatment quality. The present joint statement describes the required structural and quality prerequsites of treatment centres.

Novelties in imaging in pulmonary fibrosis and nodules. A narrative review.

In recent months two major fields of interest in pulmonary imaging have stood out: pulmonary fibrosis and pulmonary nodules. New guidelines have been released to define pulmonary fibrosis and subsequent studies have proved the value of these changes. In addition, new recommendations for classification of pulmonary nodules have been released. Radiological images are of major interest for automated and standardized analysis and so in both cases software tools using artificial intelligence were developed for visualization and quantification of the disease. These tools have been validated by human readers and demonstrated their capabilities. This review summarizes the new recommendations for classification of pulmonary fibrosis and nodules and reviews the capabilities of radiomics within these two entities.

Pulmonary interstitial glycogenosis - A systematic analysis of new cases.

BACKGROUND: Pulmonary interstitial glycogenosis (PIG) is a rare paediatric interstitial lung disease of unknown cause. The diagnosis can only be made by lung biopsy. Less than 100 cases have been reported. Clinical features, treatment and outcomes have rarely been assessed systematically in decent cohorts of patients. METHODS: In this retrospective multicentre study, the clinical presentation, radiologic findings, pattern of lung biopsy, extrapulmonary comorbidities, treatment and outcome of eleven children with PIG were collected systematically. RESULTS: 10/11 children presented with respiratory distress immediatly after birth and 8/11 needed invasive ventilation. In 8/11 children extrapulmonary comorbidities were present, congenital heart defects being the most common. 7/11 children received systemic glucocorticoids and of these four showed a clear favorable response. During a median follow-up of 3.0 years (range 0.42-12.0) one child died, while 10 patients improved. Chest CT-scans showed ground-glass opacities (7/10), consolidations (6/10), linear opacities (5/10) and mosaic attenuation (4/10) without uniform pattern. Besides interstitial thickening related to undifferentiated glycogen positive mesenchymal cells all tissue samples showed growth abnormalities with reduced alveolarization. CONCLUSIONS: PIG is associated with alveolar growth abnormalities and has to be considered in all newborns with unexplained respiratory distress. Apparent treatment benefit of glucocorticosteroids needs to be evaluated systematically.

[Cardiac and vascular malformations]. / Fehlbildungen von Herz und Gefäßen.

Malformations of the heart and great vessels show a high degree of variation. There are numerous variants and defects with only few clinical manifestations and are only detected by chance, such as a persistent left superior vena cava or a partial anomalous pulmonary venous connection. Other cardiovascular malformations are manifested directly after birth and need prompt mostly surgical interventions. At this point in time echocardiography is the diagnostic modality of choice for morphological and functional characterization of malformations. Additional imaging using computed tomography (CT) or magnetic resonance imaging (MRI) is only required in a minority of cases. If so, the small anatomical structures, the physiological tachycardia and tachypnea are a challenge for imaging modalities and strategies. This review article presents the most frequent vascular, cardiac and complex cardiovascular malformations independent of the first line diagnostic imaging modality.

[HRCT technique with low-dose protocols for interstitial lung diseases]. / HRCT-Technik mit Low-dose-Protokollen bei interstitiellen Lungenerkrankungen.

CLINICAL/METHODICAL ISSUE: For optimal visualization of the lung parenchyma a dedicated computed tomography (CT) examination protocol has to be selected. STANDARD RADIOLOGICAL METHODS: High resolution CT (HRCT) is the best technique for non-invasive evaluation of the lung parenchyma. ACHIEVEMENTS: Given the possibility to examine the complete lungs within one breath hold, assessment of disease severity and distribution is easily possible. Various parameters have to be considered to generate optimal image quality and best possible clinical results. PRACTICAL RECOMMENDATIONS: This review article discusses the various image acquisition parameters for HRCT as well as the issue of dose reduction.

Quantification of pulmonary microcirculation by dynamic contrast-enhanced magnetic resonance imaging: comparison of four regularization methods.

Tissue microcirculation can be quantified by a deconvolution analysis of concentration-time curves measured by dynamic contrast-enhanced magnetic resonance imaging. However, deconvolution is an ill-posed problem, which requires regularization of the solutions. In this work, four algebraic deconvolution/regularization methods were evaluated: truncated singular value decomposition and generalized Tikhonov regularization (GTR) in combination with the L-curve criterion, a modified LCC (GTR-MLCC), and a response function model that takes a-priori knowledge into account. To this end, dynamic contrast-enhanced magnetic resonance imaging data sets were simulated by an established physiologically reference model for different signal-to-noise ratios and measured on a 1.5-T system in the lung of 10 healthy volunteers and 20 patients. Analysis of both the simulated and measured dynamic contrast-enhanced magnetic resonance imaging datasets revealed that GTR in combination with the L-curve criterion does not yield reliable and clinically useful results. The three other deconvolution/regularization algorithms resulted in almost identical microcirculatory parameter estimates for signal-to-noise ratios > 10. At low signal-to-noise ratios levels (<10) typically occurring in pathological lung regions, GTR in combination with a modified L-curve criterion approximates the true response function much more accurately than truncated singular value decomposition and GTR in combination with response function model with a difference in accuracy of up to 76%. In conclusion, GTR in combination with a modified L-curve criterion is recommended for the deconvolution of dynamic contrast-enhanced magnetic resonance imaging curves measured in the lung parenchyma of patients with highly heterogeneous signal-to-noise ratios.

Computed tomographic examination of South American sea lions (Otaria flavescens) with suspected Mycobacterium pinnipedii infection.

Ten South American sea lions (Otaria flavescens) were presented for clinical evaluation and diagnosis of tuberculosis following known exposure to Mycobacterium pinnipedii. CT was used to determine whether foci of calcification in mediastinal lymph nodes, typically associated with pinniped tuberculosis, could be detected and whether CT was a useful diagnostic modality, in conjunction with other tests, for the diagnosis of tuberculosis in this species. Blood was collected from the caudal gluteal vein of each animal for serological testing using commercially available serological tests (ElephantTB STAT-PAK and DPP Vet; Chembio Diagnostic Systems) and a multiantigen print immunoassay (MAPIA), carried out at Chembio to verify the in-house kits. In four of nine animals that underwent CT scanning, lesions consistent with pinniped tuberculosis were apparent and these were confirmed at subsequent postmortem examination. The five remaining animals did not show any abnormalities on CT, with three being negative on serological tests, which were considered to be normal and potentially used as reference images for healthy sea lions. One animal could not be CT scanned due to its large size and weight (510 kg).

[Increased transparency in thin-slice computed tomography of lung parenchyma]. / Vermehrte Transparenz in der Dünnschicht-CT des Lungenparenchyms.

Areas with reduced density are a common finding with thin-slice computed tomography (CT) examinations of lung parenchyma. Diffuse and localized patterns can be differentiated. The most frequent cause for a diffuse decrease in density is pulmonary emphysema which can be classified into centrilobular, panlobular and paraseptal emphysema based on the distinct anatomical features. A mosaic pattern (due to mosaic perfusion or air trapping) is also characterized by a diffuse decrease in density. Focal cystic changes can be differentiated from emphysematous changes and mosaic patterns which do not reveal wall structures. Langerhans cell histiocytosis and lymphangioleiomyomatosis are the most frequent cystic diseases of the lung parenchyma. Based on the geometry and anatomical distribution of the cysts a differentiation is mostly possible. Rare diseases leading to a reduced lung density include the Birt-Hogg-Dubé syndrome, lymphoid interstitial pneumonia as well as congenital diseases or diseases which are acquired during early childhood, such as cystic adenomatoid malformation or Swyer-James syndrome.

MRI in lung cancer: a pictorial essay.

Imaging studies play a critical role in the diagnosis and staging of lung cancer. CT and 18-fluorodeoxyglucose positron emission tomography CT (PET/CT) are widely and routinely used for staging and assessment of treatment response. Many radiologists still use MRI only for the assessment of superior sulcus tumours, and in cases where invasion of the spinal cord canal is suspected. MRI can detect and stage lung cancer, and this method could be an excellent alternative to CT or PET/CT in the investigation of lung malignancies and other diseases. This pictorial essay discusses the use of MRI in the investigation of lung cancer.

Magnetic resonance imaging findings in acute pulmonary embolism.

Pulmonary embolism (PE) is the third most common acute cardiovascular disease after myocardial infarction and stroke, and results in thousands of deaths each year. Improvements in MRI accuracy are ongoing with the use of parallel imaging for angiography techniques and pulmonary perfusion. This, associated with other potential advantages of MRI (e.g. a radiation free method and better safety profile of MR contrast media), reinforces its use. The aim of this paper is to perform a pictorial review of the principal findings of MRI in acute PE. Acute PE can manifest itself as complete arterial occlusion and the affected artery may be enlarged. We report the main vascular and parenchymal signs, and an overview of current literature regarding accuracy, limitations and technical aspects is provided.

[Clinical trial endpoints in alpha-1-antitrypsin deficiency: interdisciplinary aspects]. / Studienendpunkte beim Alpha-1-Antitrypsin-Mangel:Interdisziplinäre Aspekte.

Patients with alpha-1-antitrypsin deficiency (AAD) represent a small subgroup of subjects with chronic obstructive pulmonary disease (COPD). Only about 800 patients are registered in the German AAD registry, so that about 90 % of the estimated 8000 afflicted individuals have not yet been diagnosed. Clinical trials to demonstrate the efficacy of alpha-1-antitrypsin augmentation therapy are difficult not only due to the small number of potential participants. As in recent COPD trials, FEV (1) and other standard respiratory function parameters have failed to demonstrate statistically significant differences between treatment groups. The present article reviews and discusses both established and potentially new study endpoints. Novel parameters emerge within the field of diagnostic imaging. IT-supported analysis of lung density allows to quantify the extent of emphysema. The EXACTLE trial has shown that CT densitometry is able to document the progression of emphysema over 2 to 3 years. Magnetic resonance imaging (MRI) can serve as an adjunct to assess lung perfusion, ventilation, and breathing dynamics. In the future, prospective multi-centre studies will rather use imaging endpoints than classical respiratory function measurements such as FEV (1). In addition, diffusion capacity and combined endpoints such as the BODE index, which correlates with mortality in COPD, should be considered.

Neuroblastoma imaging.

Neuroblastoma is an embryonic tumor of the sympathetic nervous system which represents one of the most common malignancies in early childhood. Its clinical and biological behavior show a remarkable heterogeneity, ranging from spontaneous regression to inexorable progression with a fatal outcome. This review summarizes the clinical risk stratification and treatment options. An extensive overview of the role of imaging during the course of the disease and typical imaging findings in all imaging modalities are demonstrated.

Four-dimensional visualization of thoracic blood flow by magnetic resonance imaging in a patient following correction of transposition of the great arteries (d-TGA) and uncorrected aortic coarctation.

Recent advances in flow-sensitive magnetic resonance imaging (MRI) and data analysis allow for comprehensive noninvasive three-dimensional (3D) visualization of complex blood flow. Electrocardiogram (ECG)-gated three-directional (3dir) flow measurements were employed to assess and visualize time-resolved 3D blood flow in the pulmonary arteries (PA) and thoracic aorta. We present findings in a juvenile patient with surgically corrected transposition of the great arteries (d-TGA) and aortic coarctation. For the first time, the complex flow patterns in the PA following d-TGA were visualized. Morphologically, a slight asymmetry of the PA was found, with considerable impact on vascular hemodynamics, resulting in diastolic retrograde flow in the larger vessel and diastolic filling of the smaller PA. Additionally, increased flow to the supraaortic vessels was found due to aortic coarctation.

Clinical application of MRI in children for the assessment of pulmonary diseases.

The standard examination technique for the chest in children is an X-ray examination - it is fast, cheap and provides a good overview of anatomy and pathology. In cases with an unclear pathology or if more details are needed (i. e. pre-therapeutically), computed tomography is most often performed with the well known drawbacks of limited soft tissue contrast and radiation. Radiation should be avoided in children, especially if follow-up examinations are needed. Recent magnetic resonance (MR) techniques allow for fast and reliable assessment of pulmonary diseases in children. Due to the inherent soft tissue contrast, diagnosis can be frequently performed without contrast media application. This review provides an exemplary MR examination protocol for routine application in pediatric patients. The diagnostic value of MRI is shown in patients with infectious diseases, patients with immunodeficiency, anatomic abnormalities, acquired chronic diseases, and pulmonary tumors. Since MRI is especially suitable for functional imaging, an MR protocol is provided for the examination of thoracic deformities. This review summarizes the use of thoracic MRI in the clinical pediatric setting with special focus on the clinical indications as a radiation-free method.

Intraindividual assessment of the thoracic aorta using contrast and non-contrast-enhanced MR angiography.

PURPOSE: To avoid intravenous contrast media application, new MRA sequences using inherent blood contrast are available. The clinical use of these non-contrast-enhanced MRA (non-CE-MRA) sequences is still limited for the aorta. Thus, the goal was to compare a standard CE-MRA with a non-CE-MRA for the thoracic aorta. MATERIALS AND METHODS: Ethics committee approval and informed consent were obtained. CE-MRA and non-CE-MRA (1.5 T) were performed in the same 50 healthy volunteers (mean age: 48). CE-MRA: GRE-Turbo-Flash-3D (1.2 x 1.2 x 1.6 mm (3)), 0.15 mmol Gd/kg, TA 22 +/- 2 sec. Non-CE-MRA: Respiratory-and cardiac-gated, T 2-prepared 3D-trueFISP (1.2 x 1.2 x 1.3 mm (3)), TA 14 +/- 5 min. Assessment included (3 readers, consensus): image quality (sharpness of vessel wall, signal homogeneity, artifacts) at the ascending aorta, arch, descending aorta and supra-aortic vessels. RESULTS: The image quality in the ascending aorta was rated 'excellent' in 78 %, 'moderate' in 22 %, 'poor' in 0 % for non-CE-MRA versus 22 %, 50 %, and 28 % for CE-MRA (Cohen's kappa = 29 %, McNemar p < 0.001). In a comparison of non-CE-MRA versus CE-MRA, the aortic arch and descending aorta showed no significant difference (kappa = 58 %/p = 0.250 and kappa = 100 %/p = 1.000, respectively). Supra-aortic vessels were rated 'excellent' 45 %/ 49 %, 'moderate' 30 %/ 49 % and 'poor' 13 %/ 2 %, 12 % of supra-aortic vessels were visualized < 1 cm at non-CE-MRA. CONCLUSION: Diagnostic image quality of the thoracic aorta can be achieved without application of intravenous contrast media. Images of the aortic root using ECG-gated non-CE-MRA are superior to standard CE-MRA. This technique might be applicable in NSF patients.

Reproducible Simulation of Respiratory Motion in Porcine Lung Explants.

PURPOSE: To develop a model for exactly reproducible respiration motion simulations of animal lung explants inside an MR-compatible chest phantom. MATERIALS AND METHODS: The materials included a piston pump and a flexible silicone reconstruction of a porcine diaphragm and were used in combination with an established MR-compatible chest phantom for porcine heart-lung preparations. The rhythmic inflation and deflation of the diaphragm at the bottom of the artificial thorax with water (1 - 1.5 L) induced lung tissue displacement resembling diaphragmatic breathing. This system was tested on five porcine heart-lung preparations using 1.5T MRI with transverse and coronal 3D-GRE (TR/TE = 3.63/1.58, 256 x 256 matrix, 350 mm FOV, 4 mm slices) and half Fourier T2-FSE (TR/TE = 545/29, 256 x 192, 350 mm, 6 mm) as well as multiple row detector CT (16 x 1 mm collimation, pitch 1.5, FOV 400 mm, 120 mAs) acquired at five fixed inspiration levels. Dynamic CT scans and coronal MRI with dynamic 2D-GRE and 2D-SS-GRE sequences (image frequencies of 10/sec and 3/sec, respectively) were acquired during continuous "breathing" (7/minute). The position of the piston pump was visually correlated with the respiratory motion visible through the transparent wall of the phantom and with dynamic displays of CT and MR images. An elastic body splines analysis of the respiratory motion was performed using CT data. RESULTS: Visual evaluation of MRI and CT showed three-dimensional movement of the lung tissue throughout the respiration cycle. Local tissue displacement inside the lung explants was documented with motion maps calculated from CT. The maximum displacement at the top of the diaphragm (mean 26.26 [SD 1.9] mm on CT and 27.16 [SD 1.5] mm on MRI, respectively [p = 0.25; Wilcoxon test]) was in the range of tidal breathing in human patients. CONCLUSION: The chest phantom with a diaphragmatic pump is a promising platform for multi-modality imaging studies of the effects of respiratory lung motion.

Time-resolved echo-shared parallel MRA of the lung: observer preference study of image quality in comparison with non-echo-shared sequences.

The aim of this study was to evaluate the image quality of time-resolved echo-shared parallel MRA of the lung. The pulmonary vasculature of nine patients (seven females, two males; median age: 44 years) with pulmonary disease was examined using a time-resolved MRA sequence combining echo sharing with parallel imaging (time-resolved echo-shared angiography technique, or TREAT). The sharpness of the vessel borders, conspicuousness of peripheral lung vessels, artifact level, and overall image quality of TREAT was assessed independently by four readers in a side-by-side comparison with non-echo-shared time-resolved parallel MRA data (pMRA) previously acquired in the same patients. Furthermore, the SNR of pulmonary arteries (PA) and veins (PV) achieved with both pulse sequences was compared. The mean voxel size of TREAT MRA was decreased by 24% compared with the non-echo-shared MRA. Regarding the sharpness of the vessel borders, conspicuousness of peripheral lung vessels, and overall image quality the TREAT sequence was rated superior in 75-76% of all cases. If the TREAT images were preferred over the pMRA images, the advantage was rated as major in 61-71% of all cases. The level of artifacts was not increased with the TREAT sequence. The mean interobserver agreement for all categories ranged between fair (artifact level) and good (overall image quality). The maximum SNR of TREAT did not differ from non-echo-shared parallel MRA (PA: TREAT: 273+/-45; pMRA: 280+/-71; PV: TREAT: 273+/-33; pMRA: 258+/-62). TREAT achieves a higher spatial resolution than non-echo-shared parallel MRA which is also perceived as an improved image quality.

[Visual assessment of functional lungs parenchyma on HRCT and (3)He-MRI in patients after single lung transplantation: comparison with quantitative volumetric results]. / Visuelle Abschätzung der funktionellen Lungenanteile in der HRCT und (3)He-MRT bei Patienten nach Einzel-Lungentransplantation: Vergleich mit der absoluten Volumetrie.

PURPOSE: Visual assessment of the ventilation using HRCT and (3)He-MRI in patients after single lung transplantation (SLTX). Analysis of specific ventilation defects found with (3)He-MRI and morphological changes found with HRCT. MATERIALS AND METHODS: We evaluated 8male patients (54 +/- 6 years) suffering from emphysema and six patients (3males and 3 females, 58 +/- 9.5 years) suffering from idiopathic pulmonary fibrosis (IPF) after SLTX. The morphological changes at HRCT were classified and localized. In (3)He-MRI (2D FLASH), 10 to 14 slices (slice thickness 10 mm, gap 5 mm) were acquired in coronal orientation to cover the whole lung. Ventilation defects were localized and characterized. The visually estimated ventilation was recorded on a 5-point scoring system. A double threshold technique was applied to volumetric quantification in (3)He-MRI to serve as internal reference. RESULTS: We found no correlation between morphological changes in HRCT and ventilation defects in (3)He-MRI. The visual assessment of ventilation in (3)He-MRI was sufficient in patients with emphysema, but this was not confirmed in patients with IPF. The visual assessment in HRCT did not correlate with the volumetric evaluation in both conditions. CONCLUSION: The various ventilation defects were not linked to specific morphological changes. For the visually assessed ventilation in patients with emphysema, (3)He-MRI is superior to HRCT.