Structural lung disease in preschool children with cystic fibrosis: An 18 month natural history study.

Background: This study was performed to describe the natural history of CF lung disease in young children over an 18 month period to assess the use of CT scanning as an outcome measure for intervention trials.

Methods: Chest CT scans were obtained at baseline and after 18 months in 42 two- to six-year-old children with CF. CT scans were scored by 2 experienced radiologists for the presence and severity of bronchiectasis, mucous plugging, and air trapping.

Mucus plugging, air trapping, and bronchiectasis are important outcome measures in assessing progressive childhood cystic fibrosis lung disease.

Objective: To determine which outcome measures could detect early progression of disease in school-age children with mild cystic fibrosis (CF) lung disease over a two-year time interval utilizing chest computed tomography (CT) scores, quantitative CT air trapping (QAT), and spirometric measurements.

Methods: Thirty-six school-age children with mild CF lung disease (median [interquartile range] age 12 [3.7] years; percent predicted forced expiratory volume in 1 second (ppFEV ) 99 [12.

Changes in Pulmonary Function and Controlled Ventilation-High Resolution CT of Chest After Antibiotic Therapy in Infants and Young Children with Cystic Fibrosis.

Background: Infants with cystic fibrosis (CF) develop early progressive lung disease which may be asymptomatic. Infant pulmonary function tests (IPFT) and controlled ventilation-high resolution computed tomography (CV-HRCT) of chest can detect early asymptomatic lung disease. It is not well established that these objective measures can detect changes in lung disease after clinical interventions.

Computed tomography correlates with improvement with ivacaftor in cystic fibrosis patients with G551D mutation.

Background: Ivacaftor corrects the cystic fibrosis transmembrane conductance regulator (CFTR) gating defect associated with G551D mutation and is quickly becoming an important treatment in patients with cystic fibrosis (CF) due to this genetic mutation.

Methods: A single-center study was performed in CF patients receiving ivacaftor to evaluate the usefulness of high resolution computed tomography (HRCT) of the chest as a way to gauge response to ivacaftor therapy.

Results: Ten patients with CF were enrolled for at least one year before and after starting ivacaftor.

Image analysis for cystic fibrosis: computer-assisted airway wall and vessel measurements from low-dose, limited scan lung CT images.

Cystic fibrosis (CF) is a life-limiting genetic disease that affects approximately 30,000 Americans. When compared to those of normal children, airways of infants and young children with CF have thicker walls and are more dilated in high-resolution computed tomographic (CT) imaging. In this study, we develop computer-assisted methods for assessment of airway and vessel dimensions from axial, limited scan CT lung images acquired at low pediatric radiation doses.

Volume-monitored chest CT: a simplified method for obtaining motion-free images near full inspiratory and end expiratory lung volumes.

Background: Lung inflation and respiratory motion during chest CT affect diagnostic accuracy and reproducibility.

Objective: To describe a simple volume-monitored (VM) method for performing reproducible, motion-free full inspiratory and end expiratory chest CT examinations in children.

Materials And Methods: Fifty-two children with cystic fibrosis (mean age 8.

An airway phantom to standardize CT acquisition in multicenter clinical trials.

Rationale And Objectives: The purpose of this study was to demonstrate the use of a phantom to standardize low-dose chest computed tomographic (CT) protocols in children with cystic fibrosis.

Materials And Methods: Spiral chest CT scans of a Plexiglas phantom simulating airway sizes (internal diameter, 1.1-16.

Usefulness of multidetector CT imaging to assess vascular stents in children with congenital heart disease: an in vivo and in vitro study.

Objective: To evaluate varying CT settings to visualize pediatric vascular stents in comparison to digital angiography (DA).

Background: There is a great clinical interest in substituting noninvasive methods to follow up children with congenital heart disease after interventional treatment.

Materials And Methods: CT studies in small children with transcatheter placed stents were reviewed, retrospectively.

CT of pediatric vascular stents used to treat congenital heart disease.

Objective: The purpose of our study was to assess the visibility of lumen narrowing of pediatric vascular stents using various CT dose parameters in an in vitro model.

Materials And Methods: Ten steel stents of varying designs and sizes commonly used in the treatment of congenital heart disease were implanted in polyvinyl chloride (PVC) tubes and three of the 10 stents were partially obstructed with wax by filling 25% (mild) to 60% (moderate) of the lumen with contrast material. On a 64-MDCT scanner, the stents were scanned at tube voltages (kVp) of 80, 100, and 120 and at tube currents (mA) of 40, 80, 120, and 160.

High-resolution computed tomography of the lung in children with cystic fibrosis: technical factors.

A standard technique that controls for respiratory motion and lung volumes during imaging is necessary if high-resolution computed tomography is to be used as an outcome measure in children with cystic fibrosis. End-inspiratory and expiratory imaging allows for the detection and differentiation of early lung disease. In children ages 0-5 years, a noninvasive controlled ventilation technique is ideal, and can be used in combination with raised-volume infant pulmonary function tests.

Computed tomography dose and variability of airway dimension measurements: how low can we go?

Background: Quantitative CT shows promise as an outcome measure for cystic fibrosis (CF) lung disease in infancy, but must be accomplished at a dose as low as reasonably achievable.

Objective: To determine the feasibility of ultra-low-dose CT for quantitative measurements of airway dimensions.

Materials And Methods: Two juvenile pigs were anesthetized and their lungs scanned at 25 cm H(2)O face-mask pressure in apnoea using beam currents of 5, 10, 20, 40 and 100 mAs.

Assessment of in-stent stenosis in small children with congenital heart disease using multi-detector computed tomography: a validation study.

Objectives: Our purpose was to investigate the diagnostic reliability of multi-detector computed tomography (MDCT) in assessing in-stent stenosis compared to digital angiography (DA) in small children.

Background: Little is known about the feasibility of using MDCT to assess stents placed to treat children with congenital heart disease (CHD).

Methods: Twenty-two children (median age [range], 2(3/4) [(1/2) to 12] years) with 42 transcatheter placed stents (median diameter: 7.

Multislice computed tomography as an adjunct to the management of an in-stent stenosis in an infant with congenital heart disease: imaging for the future.

The case report demonstrates the value of multslice computed tomography for stent imaging and detection of an in-stent stenosis in an infant with pulmonary atresia and ventricular septal defect after stent placement in the right pulmonary artery after biventricular repair.

Computed tomography in the evaluation of cystic fibrosis lung disease.

The first report of computed tomography (CT) scanning to monitor cystic fibrosis (CF)-related lung disease was published in 1986. Further publications followed, but in general there was little interest in this technique until recently. Two factors in particular have led to this increased interest.

Comparison of quiet breathing and controlled ventilation in the high-resolution CT assessment of airway disease in infants with cystic fibrosis.

Background: Respiratory motion and low lung volumes limit the quality of HRCT examinations in infants and young children.

Objective: To assess the effects of respiratory motion and lung inflation on the ability to diagnose airway abnormalities and air trapping (AT) using HRCT in infants with cystic fibrosis (CF).

Materials And Methods: HRCT images of the lungs were obtained at four anatomical levels in 16 sedated children (age 2.

Changes in airway dimensions on computed tomography scans of children with cystic fibrosis.

Rationale: In cystic fibrosis (CF), chronic bacterial infection and inflammation lead to progressive airway wall thickening and lumen dilatation.

Objectives: To quantify airway wall thickening and lumen dilatation in children with CF over a 2-year interval.

Methods: Children with CF (n = 23) who had two computed tomography (CT) scans (CT(cf1) and CT(cf2)) combined with pulmonary function tests (PFTs), with a 2-year interval between measurements, were compared with control subjects (n = 21) who had one CT (CT(controls)).

Inspiratory and expiratory CT lung density in infants and young children.

Background: There is a lack of information on normal inspiratory and expiratory CT lung density in infants.

Objective: To describe normal regional CT lung density at end inspiratory and end expiratory lung volumes in children ages 0--5 years.

Materials And Methods: Motionless HRCT images were obtained at 25 cm (inspiratory) and 0 cm (expiratory) water pressure at apical (top of arch) and basal (2 cm above diaphragm) levels in 16 sedated children (mean age 1.

Imaging evolution of airway disorders in children.

This article discusses and illustrates the insights gained from CT imaging of the lungs in infants and young children who do not have respiratory motion and at end-inspiratory and resting end-expiratory lung volumes using noninvasive controlled ventilation CT technique. The potential role of noninvasive controlled ventilation CT in developing quantitative measures of airway disease is highlighted and clinical examples demonstrating the use of the technique are shown.

Structural airway abnormalities in infants and young children with cystic fibrosis.

Objectives: To determine whether the airway structure of infants and young children with cystic fibrosis (CF) differs from that of normal children by using high-resolution computed tomography (HRCT) imaging. Study design Full-inflation, controlled ventilation HRCT images of the lungs were obtained at four anatomic levels in 34 infants with CF (age, 2.4+/-1.

Lateral thoracic expansion for Jeune syndrome: evidence of rib healing and new bone formation.

Background: Lateral thoracic expansion is a procedure that has been described to enlarge the thoracic cage in patients with Jeune's asphyxiating thoracic dystrophy. The procedure involves separating ribs from their periosteum and plating them together in an expanded fashion with titanium struts. We have speculated that the ribs heal in this situation, despite the absence of surrounding periosteum, and that new rib formation occurs in the liberated periosteum.