Abstract
Despite our current treatment, many cystic fibrosis (CF) patients still show progressive bronchiectasis and small airways disease. Adequate detection and monitoring of progression of these structural abnormalities is needed to personalize treatment to the severity of CF lung disease of the patient. Chest computed tomography (CT) is the gold standard to diagnose and monitor bronchiectasis. Many studies have been done to validate the role of chest CT in CF and to improve the protocols. From these studies it became clear that for correct interpretation of the severity of bronchiectasis and small airways disease standardization of lung volume for the inspiratory and expiratory CT scan acquisition is needed. The risk related to the radiation exposure of a chest CT scan every second year is considered low. Automated and quantitative image analysis systems are developed to improve the reliability and sensitivity of assessments of structural lung changes in CF, particularly in early life. In this paper an overview is given of the lessons learned from two decades of monitoring CF lung disease using chest CT.
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References
Armstrong DS, Grimwood K, Carlin JB et al (1997) Lower airway inflammation in infants and young children with cystic fibrosis. Am J Respir Crit Care Med 156:1197–1204
Nixon GM, Armstrong DS, Carzino R et al (2002) Early airway infection, inflammation, and lung function in cystic fibrosis. Arch Dis Child 87:306–311
Sly PD, Brennan S, Gangell C et al (2009) Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening. Am J Respir Crit Care Med 180:146–152
Sly PD, Gangell CL, Chen L et al (2013) Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med 368:1963–1970
Bennett TI (1945) Discussion on the stethoscope versus X-rays. Proc R Soc Med 355:7–9
Young K, Aspestrand F, Kolbenstvedt A (1991) High resolution CT and bronchography in the asssessment of bronchiectasis. Acta Radiol 32:439–441
Hansell DM (1998) Bronchiectasis. Radiol Clin North Am 36:107–128
Munro NC, Cooke JC, Currie DC et al (1990) Comparison of thin section computed tomography with bronchography for identifying bronchiectatic segments in patients with chronic sputum production. Thorax 45:135–139
Webb R, Muller NL, Naidich DP (eds) (2008) High-resolution CT of the lung. Lippincott Williams & Wilkins, Philadelphia
Loeve M, van Hal PT, Robinson P et al (2009) The spectrum of structural abnormalities on CT scans from patients with CF with severe advanced lung disease. Thorax 64:876–882
Loeve M, Hop WC, de Bruijne M et al (2012) Chest computed tomography scores are predictive of survival in patients with cystic fibrosis awaiting lung transplantation. Am J Respir Crit Care Med 185:1096–1103
Davis S, Fordham LA, Brody AS et al (2007) Computed tomography reflects lower airway inflammation and tracks changes in early cystic fibrosis. Am J Respir Crit Care Med 175:943–950
de Jong PA, Nakano Y, Lequin MH et al (2004) Progressive damage on high-resolution computed tomography despite stable lung function in CF. Eur Respir J 23:93–97
Loeve M, Gerbrands K, Hop WC et al (2010) Bronchiectasis and pulmonary exacerbations in children and young adults with cystic fibrosis. Chest 140:178–185
Tepper LA, Utens E, Caudri D et al (2013) Impact of bronchiectasis and trapped air on quality of life and exacerbations in CF. Eur Respir J 42:371–379
Goris ML, Zhu HJ, Blankenberg F et al (2003) An automated approach to quantitative air trapping measurements in mild cystic fibrosis. Chest 123:1655–1663
Robinson TE, Leung AN, Northway WH et al (2001) Spirometer-triggered high-resolution computed tomography and pulmonary function measurements during an acute exacerbation in patients with cystic fibrosis. J Pediatr 138:553–559
Robinson TE, Leung AN, Moss RB et al (1999) Standardized high-resolution CT of the lung using a spirometer-triggered electron beam CT scanner. AJR Am J Roentgenol 172:1636–1638
Long FR, Williams RS, Castile RG (2004) Structural airway abnormalities in infants and young children with cystic fibrosis. J Pediatr 144:154–161
Tiddens HA, Koopman LP, Lambert RK et al (2000) Cartilaginous airway wall dimensions and airway resistance in cystic fibrosis lungs. Eur Respir J 15:735–742
de Jong PA, Ottink MD, Robben SGF et al (2004) Pulmonary disease assessment in cystic fibrosis: Comparison of CT scoring systems and value of bronchial and arterial dimension measurements. Radiology 231:434–439
Tiddens HA, Donaldson SH, Rosenfeld M et al (2010) Cystic fibrosis lung disease starts in the small airways: Can we treat it more effectively? Pediatr Pulmonol 45:107–117
McDermott S, Barry SC, Judge EE et al (2009) Tracheomalacia in adults with cystic fibrosis: determination of prevalence and severity with dynamic cine CT. Radiology 252:577–586
Stick SM, Brennan S, Murray C et al (2009) Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening. J Pediatr 155:623–628
Mott LS, Gangell CL, Murray CP et al (2009) Bronchiectasis in an asymptomatic infant with cystic fibrosis diagnosed following newborn screening. J Cyst Fibros 8:285–287
Mott LS, Park J, Murray CP et al (2012) Progression of early structural lung disease in young children with cystic fibrosis assessed using CT. Thorax 67:509–516
Wainwright CE, Vidmar S, Armstrong DS et al (2011) Effect of bronchoalveolar lavage-directed therapy on Pseudomonas aeruginosa infection and structural lung injury in children with cystic fibrosis: a randomized trial. JAMA 306:163–171
Tiddens HA (2002) Detecting early structural lung damage in cystic fibrosis. Pediatr Pulmonol 34:228–231
Owens CM, Aurora P, Stanojevic S et al (2011) Lung Clearance Index and HRCT are complementary markers of lung abnormalities in young children with CF. Thorax 66:481–488
de Jong PA, Lindblad A, Rubin L et al (2006) Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax 61:80–85
Tiddens HA, Stick SM, Davis S (2014) Multi-modality monitoring of cystic fibrosis lung disease: the role of chest computed tomography. Paediatr Respir Rev 15:92–97
de Jong PA, Nakano Y, Lequin MH et al (2005) Dose reduction for CT in children with cystic fibrosis: is it feasible to reduce the number of images per scan. Pediatr Radiol 36:50–53
Loeve M, de Bruijne M, Hartmann IC et al (2012) Three-section expiratory CT: insufficient for trapped air assessment in patients with cystic fibrosis? Radiology 262:969–976
Willemink MJ, de Jong PA (2013) Pediatric chest computed tomography at a radiation dose approaching a chest radiograph. Am J Respir Crit Care Med 188:626–627
de Jong PA, Mayo JR, Golmohammadi K et al (2005) Estimation of cancer mortality associated with repetitive computed tomography scanning (CT) scanning in cystic fibrosis. Am J Respir Crit Care Med 173:199–203
Long FR, Castile RG (2001) Technique and clinical applications of full-inflation and end-exhalation controlled-ventilation chest CT in infants and young children. Pediatr Radiol 31:413–422
Long FR, Castile RG, Brody AS et al (1999) Lungs in infants and young children: improved thin-section CT with a noninvasive controlled-ventilation technique–initial experience. Radiology 212:588–593
Long FR, Williams RS, Adler BH et al (2005) Comparison of quiet breathing and controlled ventilation in the high-resolution CT assessment of airway disease in infants with cystic fibrosis. Pediatr Radiol 35:1075–1080
Long FR, Williams RS, Castile RG (2005) Inspiratory and expiratory CT lung density in infants and young children. Pediatr Radiol 35:677–683
de Jong PA, Nakano Y, Hop WC et al (2005) Changes in airway dimensions on computed tomography scans of children with cystic fibrosis. Am J Respir Crit Care Med 172:218–224
Lever S, van der Wiel EC, Koch A et al (2009) Feasibility of spirometry controlled chest CT in children. Eur Respir J 34:A344
Bonnel AS, Song SM, Kesavarju K et al (2004) Quantitative air-trapping analysis in children with mild cystic fibrosis lung disease. Pediatr Pulmonol 38:396–405
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Tiddens, H.A.W.M., Rosenow, T. What did we learn from two decades of chest computed tomography in cystic fibrosis?. Pediatr Radiol 44, 1490–1495 (2014). https://doi.org/10.1007/s00247-014-2964-6
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DOI: https://doi.org/10.1007/s00247-014-2964-6