Skip to main content
Log in

Structural and perfusion magnetic resonance imaging of the lung in cystic fibrosis

  • Original Article
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

Background

Because of its absence of ionising radiation and possibility for obtaining functional information, MRI is promising for assessing lung disease in children who require repetitive imaging for long-term follow-up.

Objective

To describe MRI findings in children with cystic fibrosis and evaluate semi-quantitative dynamic contrast-enhanced lung perfusion.

Materials and methods

We retrospectively compared lung MRI in 25 children and young adults with cystic fibrosis (median age 3.7 years) to 12 children (median age 2 years) imaged for other pathologies. MRI at 1.5 T included respiratory-gated sequences and contrast-enhanced lung perfusion imaging. We described and graded any morphologic change. Signal enhancement and time to peak values of perfusion abnormalities were compared to those of normally enhancing lung parenchyma.

Results

Frequent findings in patients with cystic fibrosis were bronchial wall thickening (24/25, 96%), areas of consolidation (22/25, 88%), enlarged lymph nodes (20/25, 80%), bronchiectasis (5/25, 20%) and mucus plugging (3/25, 12%). Compared to normally enhancing lung, perfusion defects (21/25, 84%), characterised by decreased enhancement, showed prolonged time to peak. Areas of consolidation showed increased enhancement. While time to peak of procedure-related atelectasis was not significantly different from that of normal lung, disease-related consolidation showed prolonged time to peak (P=0.01).

Conclusion

Lung MRI demonstrates structural and perfusion abnormalities in children and young people with cystic fibrosis. Semi-quantitative assessment of dynamic contrast-enhanced perfusion imaging might allow differentiation between procedure-related atelectasis and disease-related consolidation.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8

Similar content being viewed by others

References

  1. Gibson RL, Burns JL, Ramsey BW (2003) Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med 168:918–951

    Article  PubMed  Google Scholar 

  2. Moskowitz SM, Gibson RL, Effmann EL (2005) Cystic fibrosis lung disease: genetic influences, microbial interactions, and radiological assessment. Pediatr Radiol 35:739–757

    Article  PubMed  Google Scholar 

  3. Stoltz DA, Meyerholz DK, Welsh MJ (2015) Origins of cystic fibrosis lung disease. N Engl J Med 372:351–362

    Article  PubMed  PubMed Central  Google Scholar 

  4. Wielpütz MO, Eichinger M, Biederer J et al (2016) Imaging of cystic fibrosis lung disease and clinical interpretation. Rofo 188:834–845

    Article  PubMed  Google Scholar 

  5. Kerem E, Conway S, Elborn S et al (2005) Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros 4:7–26

    Article  PubMed  Google Scholar 

  6. Henzler T, Schmid-Bindert G, Schoenberg SO et al (2010) Diffusion and perfusion MRI of the lung and mediastinum. Eur J Radiol 76:329–336

    Article  PubMed  Google Scholar 

  7. Puderbach M, Eichinger M (2010) The role of advanced imaging techniques in cystic fibrosis follow-up: is there a place for MRI? Pediatr Radiol 40:844–849

    Article  PubMed  Google Scholar 

  8. Biederer J, Mirsadraee S, Beer M et al (2012) MRI of the lung (3/3)-current applications and future perspectives. Insights Imaging 3:373–386

    Article  PubMed  PubMed Central  Google Scholar 

  9. Wielpütz M, Kauczor HU (2012) MRI of the lung: state of the art. Diagn Interv Radiol 18:344–353

    PubMed  Google Scholar 

  10. Eichinger M, Tetzlaff R, Puderbach M et al (2007) Proton magnetic resonance imaging for assessment of lung function and respiratory dynamics. Eur J Radiol 64:329–334

    Article  PubMed  Google Scholar 

  11. Wielpütz MO, Puderbach M, Kopp-Schneider A et al (2014) Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease. Am J Respir Crit Care Med 189:956–965

    Article  PubMed  Google Scholar 

  12. Biederer J, Heussel CP, Puderbach M et al (2014) Functional magnetic resonance imaging of the lung. Semin Respir Crit Care Med 35:74–82

    Article  CAS  PubMed  Google Scholar 

  13. Eichinger M, Puderbach M, Fink C et al (2006) Contrast-enhanced 3D MRI of lung perfusion in children with cystic fibrosis - initial results. Eur Radiol 16:2147–2152

    Article  PubMed  Google Scholar 

  14. Wild JM, Marshall H, Bock M et al (2012) MRI of the lung (1/3): methods. Insights Imaging 3:345–353

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Gnannt R, Amaxopoulou CE, Goetti R et al (2014) Qualitative assessment of MRI sequences for morphological imaging of the lungs in children with cystic fibrosis. Pediatr Radiol 44:308

    Google Scholar 

  16. Eichinger M, Optazaite DE, Kopp-Schneider A et al (2012) Morphologic and functional scoring of cystic fibrosis lung disease using MRI. Eur J Radiol 81:1321–1329

    Article  PubMed  Google Scholar 

  17. de Jong PA, Nievelstein RJ (2012) Normal mediastinal and hilar lymph nodes in children on multi-detector row chest computed tomography. Eur Radiol 22:318–321

    Article  PubMed  Google Scholar 

  18. Rossi UG, Owens CM (2005) The radiology of chronic lung disease in children. Arch Dis Child 90:601–607

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. 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

    Article  PubMed  Google Scholar 

  20. 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

    Article  CAS  PubMed  Google Scholar 

  21. Puderbach M, Eichinger M, Haeselbarth J et al (2007) Assessment of morphological MRI for pulmonary changes in cystic fibrosis (CF) patients: comparison to thin-section CT and chest x-ray. Investig Radiol 42:715–725

    Article  Google Scholar 

  22. Swift AJ, Telfer A, Rajaram S et al (2014) Dynamic contrast-enhanced magnetic resonance imaging in patients with pulmonary arterial hypertension. Pulm Circ 4:61–70

    Article  PubMed  PubMed Central  Google Scholar 

  23. Mirsadraee S, van Beek EJ (2015) Functional imaging: computed tomography and MRI. Clin Chest Med 36:349–363

    Article  PubMed  Google Scholar 

  24. Altes TA, Eichinger M, Puderbach M (2007) Magnetic resonance imaging of the lung in cystic fibrosis. Proc Am Thorac Soc 4:321–327

    Article  PubMed  Google Scholar 

  25. Rajaram S, Swift AJ, Telfer A et al (2013) 3D contrast-enhanced lung perfusion MRI is an effective screening tool for chronic thromboembolic pulmonary hypertension: results from the ASPIRE registry. Thorax 68:677–678

    Article  PubMed  Google Scholar 

  26. Newman B, Krane EJ, Gawande R et al (2014) Chest CT in children: anesthesia and atelectasis. Pediatr Radiol 44:164–172

    Article  PubMed  Google Scholar 

  27. Macgowan CK, Al-Kwifi O, Varodayan F et al (2005) Optimization of 3D contrast-enhanced pulmonary magnetic resonance angiography in pediatric patients with congenital heart disease. Magn Reson Med 54:207–212

    Article  PubMed  Google Scholar 

  28. Berthezene Y, Vexler V, Clement O et al (1992) Contrast-enhanced MR imaging of the lung: assessments of ventilation and perfusion. Radiology 183:667–672

    Article  CAS  PubMed  Google Scholar 

  29. Guan Y, Xia Y, Fan L et al (2015) Quantitative assessment of pulmonary perfusion using dynamic contrast-enhanced CT in patients with chronic obstructive pulmonary disease: correlations with pulmonary function test and CT volumetric parameters. Acta Radiol 56:573–580

    Article  PubMed  Google Scholar 

  30. Calder AD, Bush A, Brody AS et al (2014) Scoring of chest CT in children with cystic fibrosis: state of the art. Pediatr Radiol 44:1496–1506

    Article  PubMed  Google Scholar 

  31. Scholz O, Denecke T, Bottcher J et al (2017) MRI of cystic fibrosis lung manifestations: sequence evaluation and clinical outcome analysis. Clin Radiol 72:754–763

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Christina Amaxopoulou.

Ethics declarations

Conflicts of interest

None

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Amaxopoulou, C., Gnannt, R., Higashigaito, K. et al. Structural and perfusion magnetic resonance imaging of the lung in cystic fibrosis. Pediatr Radiol 48, 165–175 (2018). https://doi.org/10.1007/s00247-017-4021-8

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-017-4021-8

Keywords

Navigation