Pierluigi Ciet

Monitoring Cystic Fibrosis Lung Disease by Computed Tomography Radiation Risk in Perspective

Computed tomography (CT) is a sensitive technique to monitor structural changes related to cystic fibrosis (CF) lung disease. It detects structural pulmonary abnormalities such as bronchiectasis and trapped air, at an early stage, before they become apparent with other diagnostic tests. Clinical decisions may be influenced by knowledge of these abnormalities. CT imaging, however, comes with risk related to ionizing radiation exposure. The aim of this review is to discuss the risk of routine CT imaging in patients with CF, using current models of radiation-induced cancer, and to put this risk in perspective with other medical and nonmedical risks. The magnitude of the risk is a complex, controversial matter. Risk analyses have largely been based on a linear no-threshold model, and excess relative and excess absolute risk estimates have been derived mainly from atomic bomb survivors. The estimates have large confidence intervals. Our risk estimates are in concordance with previously reported estimates. A large proportion of radiation to which humans are exposed is from natural background sources and varies widely depending on geographical location. The risk differences due to variation in background radiation can be larger than the risks associated with CF lung disease monitoring by CT. We conclude that the risk related to routine usage of CT in clinical care is small. In addition, a life-limiting disease, such as CF, lowers the risk of radiation-induced cancer. Nonetheless, the use of CT should always be justified and the radiation dose should be kept as low as reasonably achievable.

Cystic Fibrosis: Detecting Changes in Airway Inflammation with FDG PET/CT

PURPOSE To determine if fluorine 18 fluorodeoxyglucose (FDG) positron emission tomographic (PET)/computed tomographic (CT) imaging can depict a treatment effect from intravenous antibiotics for pulmonary exacerbation in cystic fibrosis (CF). MATERIALS AND METHODS The study was approved by the institutional review board of the Hospital for Sick Children and by Health Canada. Consent was obtained from all subjects. Patients with CF who were between 6 and 18 years of age and were admitted for a pulmonary exacerbation were eligible for the study. FDG PET/CT examinations (with low-dose CT) were performed on days 1 and 14 of admission (±72 hours). PET activity was quantified by using standardized uptake values (SUVs) through assessment of background activity (mean SUV [SUV(mean)]) and superimposed focal uptake (maximum SUV [SUV(max)]) for each lung zone. CT studies were scored by using the CF-CT model. SUVs from pre- and posttherapy studies were compared by using paired t tests. Unpaired t tests were used to compare data in patients with CF and data in 10 control subjects. RESULTS Twenty patients with CF were enrolled. Antibiotic therapy resulted in a significant decrease in SUV(max) (mean difference, 2.3 ± 2.1 [standard deviation], P < .0001). Pretherapy SUV(max) and SUV(mean) and posttherapy SUV(max) were significantly different from those in control subjects. The change in SUV(max) and percentage predicted forced expiratory volume in 1 second was negatively correlated. (R = -0.72, P = .004). Overall CF-CT scores significantly correlated with SUV(max) (R = 0.40, P = .01). CONCLUSION FDG PET/CT is a useful tool for detecting inflammatory changes resulting from treatment for pulmonary exacerbations in pediatric patients with CF. Inflammatory changes detected by using FDG PET/CT correlated with lung function, sputum neutrophil counts, and CF-CT scores. Analyzing focal lung inflammation (with SUV(max)) may be a feasible way to measure airway inflammation in patients with CF.

Lung MRI and impairment of diaphragmatic function in Pompe disease

BackgroundPompe disease is a progressive metabolic myopathy. Involvement of respiratory muscles leads to progressive pulmonary dysfunction, particularly in supine position. Diaphragmatic weakness is considered to be the most important component. Standard spirometry is to some extent indicative but provides too little insight into diaphragmatic dynamics. We used lung MRI to study diaphragmatic and chest-wall movements in Pompe disease.MethodsIn ten adult Pompe patients and six volunteers, we acquired two static spirometer-controlled MRI scans during maximum inspiration and expiration. Images were manually segmented. After normalization for lung size, changes in lung dimensions between inspiration and expiration were used for analysis; normalization was based on the cranial-caudal length ratio (representing vertical diaphragmatic displacement), and the anterior-posterior and left-right length ratios (representing chest-wall movements due to thoracic muscles).ResultsWe observed striking dysfunction of the diaphragm in Pompe patients; in some patients the diaphragm did not show any displacement. Patients had smaller cranial-caudal length ratios than volunteers (p < 0.001), indicating diaphragmatic weakness. This variable strongly correlated with forced vital capacity in supine position (r = 0.88) and postural drop (r = 0.89). While anterior-posterior length ratios also differed between patients and volunteers (p = 0.04), left-right length ratios did not (p = 0.1).ConclusionsMRI is an innovative tool to visualize diaphragmatic dynamics in Pompe patients and to study chest-walland diaphragmatic movements in more detail. Our data indicate that diaphragmatic displacement may be severely disturbed in patients with Pompe disease.

Magnetic resonance imaging in children: common problems and possible solutions for lung and airways imaging

Pediatric chest MRI is challenging. High-resolution scans of the lungs and airways are compromised by long imaging times, low lung proton density and motion. Low signal is a problem of normal lung. Lung abnormalities commonly cause increased signal intenstities. Among the most important factors for a successful MRI is patient cooperation, so the long acquisition times make patient preparation crucial. Children usually have problems with long breath-holds and with the concept of quiet breathing. Young children are even more challenging because of higher cardiac and respiratory rates giving motion blurring. For these reasons, CT has often been preferred over MRI for chest pediatric imaging. Despite its drawbacks, MRI also has advantages over CT, which justifies its further development and clinical use. The most important advantage is the absence of ionizing radiation, which allows frequent scanning for short- and long-term follow-up studies of chronic diseases. Moreover, MRI allows assessment of functional aspects of the chest, such as lung perfusion and ventilation, or airways and diaphragm mechanics. In this review, we describe the most common MRI acquisition techniques on the verge of clinical translation, their problems and the possible solutions to make chest MRI feasible in children.

Magnetic Resonance Imaging of Pediatric Lung Parenchyma, Airways, Vasculature, Ventilation, and Perfusion: State of the Art

Magnetic resonance (MR) imaging is a noninvasive imaging modality, particularly attractive for pediatric patients given its lack of ionizing radiation. Despite many advantages, the physical properties of the lung (inherent low signal-to-noise ratio, magnetic susceptibility differences at lung-air interfaces, and respiratory and cardiac motion) have posed technical challenges that have limited the use of MR imaging in the evaluation of thoracic disease in the past. However, recent advances in MR imaging techniques have overcome many of these challenges. This article discusses these advances in MR imaging techniques and their potential role in the evaluation of thoracic disorders in pediatric patients.

Spirometer-controlled cine magnetic resonance imaging used to diagnose tracheobronchomalacia in paediatric patients

Tracheobronchomalacia (TBM) is defined as an excessive collapse of the intrathoracic trachea. Bronchoscopy is the gold standard for diagnosing TBM; however it has major disadvantages, such as general anaesthesia. Cine computed tomography (CT) is a noninvasive alternative used to diagnose TBM, but its use in children is restricted by ionising radiation. Our aim was to evaluate the feasibility of spirometer-controlled cine magnetic resonance imaging (MRI) as an alternative to cine-CT in a retrospective study. 12 children with a mean age (range) of 12 years (7–17 years), suspected of having TBM, underwent cine-MRI. Static scans were acquired at end-inspiration and expiration covering the thorax using a three-dimensional spoiled gradient echo sequence. Three-dimensional dynamic scans were performed covering only the central airways. TBM was defined as a decrease of the trachea or bronchi diameter >50% at end-expiration in the static and dynamic scans. The success rate of the cine-MRI protocol was 92%. Cine-MRI was compared with bronchoscopy or chest CT in seven subjects. TBM was diagnosed by cine-MRI in seven (58%) out of 12 children and was confirmed by bronchoscopy or CT. In four patients, cine-MRI demonstrated tracheal narrowing that was not present in the static scans. Spirometer controlled cine-MRI is a promising technique to assess TBM in children and has the potential to replace bronchoscopy. Spirometer controlled cine-MRI: a technique to assess TBM in children with the potential to replace bronchoscopy/cine-CT http://ow.ly/pDNAA

Respiratory tract exacerbations revisited: Ventilation, inflammation, perfusion, and structure (VIPS) monitoring to redefine treatment

For cystic fibrosis (CF) patients older than 6 years there are convincing data that suggest respiratory tract exacerbations (RTE) play an important role in the progressive loss of functional lung tissue. There is a poor understanding of the pathobiology of RTE and whether specific treatment of RTE reduces lung damage in the long term. In addition, there are limited tools available to measure the various components of CF lung disease and responses to therapy. Therefore, in order to better understand the impact of RTE on CF lung disease we need to develop sensitive measures to characterize RTE and responses to treatment; and improve our understanding of structure‐function changes during treatment of RTE. In this paper we review our current knowledge of the impact of RTE on the progression of lung disease and identify strategies to improve our understanding of the pathobiology of RTE. By improving our knowledge regarding RTE in CF we will be better positioned to develop approaches to treatment that are individualized and that can prevent permanent structural damage. We suggest the development of a ventilation, perfusion, inflammation and structure (VIPS)‐MRI suite that supplies the clinician with data on ventilation, inflammation, perfusion, and structure in one MRI session. VIPS‐MRI could be an important step to better understand the factors that contribute to and limit treatment efficacy of RTE. Pediatr Pulmonol. 2015; 50:S57–S65.

Quantification of diaphragm mechanics in Pompe disease using dynamic 3D MRI

Background Diaphragm weakness is the main reason for respiratory dysfunction in patients with Pompe disease, a progressive metabolic myopathy affecting respiratory and limb-girdle muscles. Since respiratory failure is the major cause of death among adult patients, early identification of respiratory muscle involvement is necessary to initiate treatment in time and possibly prevent irreversible damage. In this paper we investigate the suitability of dynamic MR imaging in combination with state-of-the-art image analysis methods to assess respiratory muscle weakness. Methods The proposed methodology relies on image registration and lung surface extraction to quantify lung kinematics during breathing. This allows for the extraction of geometry and motion features of the lung that characterize the independent contribution of the diaphragm and the thoracic muscles to the respiratory cycle. Results Results in 16 3D+t MRI scans (10 Pompe patients and 6 controls) of a slow expiratory maneuver show that kinematic analysis from dynamic 3D images reveals important additional information about diaphragm mechanics and respiratory muscle involvement when compared to conventional pulmonary function tests. Pompe patients with severely reduced pulmonary function showed severe diaphragm weakness presented by minimal motion of the diaphragm. In patients with moderately reduced pulmonary function, cranial displacement of posterior diaphragm parts was reduced and the diaphragm dome was oriented more horizontally at full inspiration compared to healthy controls. Conclusion Dynamic 3D MRI provides data for analyzing the contribution of both diaphragm and thoracic muscles independently. The proposed image analysis method has the potential to detect less severe diaphragm weakness and could thus be used to determine the optimal start of treatment in adult patients with Pompe disease in prospect of increased treatment response.

MR Safety Issues Particular to Women

Because of its lack of ionizing radiation, MR imaging is increasingly used for patients with cardiovascular disease, including young women. However, the risks related to the MR environment need to be acknowledged and prevented. For women, there are unique gender-related safety issues that are important to address in cardiovascular MR examinations. This article familiarizes radiologists with MR safety issues and current, evidence-based recommendations for specific situations such as pregnancy or lactation and imaging of women who have pelvic gynecologic devices such as intrauterine devices. Practical algorithms to minimize risk and increase MR safety for these women are suggested.

Pediatric Chest MR Imaging: Lung and Airways

Advances in technology coupled with optimized protocols now permit evaluation of the lungs with magnetic resonance (MR) imaging in the pediatric population. Although computed tomography remains the preferred imaging modality for this purpose, MR imaging provides a radiation-free alternative that can answer many important clinical questions and provide additional data. In addition, the use of newer techniques and equipment such as MR-imaging-compatible spirometers allows for functional assessment of the pediatric airways. This article reviews the up-to-date MR imaging techniques as well as imaging findings of selected clinically important disorders that affect the lungs and airways in the pediatric population.