La radiologia medica

, Volume 123, Issue 8, pp 577–585 | Cite as

CT features of diffuse lung disease in infancy

  • Paolo Toma
  • Aurelio Secinaro
  • Oliviero Sacco
  • Davide Curione
  • Renato Cutrera
  • Nicola Ullmann
  • Claudio Granata


Diffuse lung disease in infancy includes a wide range of very rare and peculiar pulmonary conditions usually not seen in older children, in whom diffuse lung disease has much greater overlap with adult disorders. The acronym chILD (childhood Interstitial Lung Disease) commonly defines these disorders, although air spaces, airways, alveolar epithelium, vasculature, pleura, and pleural spaces can also be involved, besides the pulmonary interstitium. chILD can be caused by diffuse developmental disorders, alveolar growth abnormalities, surfactant dysfunction disorders, and other specific conditions of poorly understood etiology. Chest CT imaging studies play a pivotal role in the evaluation of chILD. In some conditions CT findings can be specific, and thus make it possible avoiding further testing. In other disorders, findings are nonspecific, although they may suggest a diagnostic pattern and guide further testing. Nevertheless, chILD disorders often remain unrecognized on imaging studies, as they are very rare. The aim of this article is to review the CT patterns of lung involvement in a series of infants with chILD.


Interstitial lung disease Diffuse lung disease Computed tomography Infant 


Compliance with ethical standards

Conflict of interest

The authors do not have conflict of interest or financial relationships to declare.

Ethical standards

Informed consent was obtained from all individual participants included in the study.


  1. 1.
    Griese M, Haug M, Brasch F, Freihorst A, Lohse P, von Kries R, Zimmermann T, Hartl D (2009) Incidence and classification of pediatric diffuse parenchymal lung diseases in Germany. Orphanet J Rare Dis 4:26. CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Kornum JB, Christensen S, Grijota M, Pedersen L, Wogelius P, Beiderbeck A, Sorensen HT (2008) The incidence of interstitial lung disease 1995–2005: a Danish nationwide population-based study. BMC Pulm Med 8:24. CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Semple T, Owens CM (2016) The radiology of diffuse interstitial pulmonary disease in children: pearls, pitfalls and new kids on the block in 2015. Radiol Med (Torino) 121(5):352–361. CrossRefGoogle Scholar
  4. 4.
    Kurland G, Deterding RR, Hagood JS, Young LR, Brody AS, Castile RG, Dell S, Fan LL, Hamvas A, Hilman BC, Langston C, Nogee LM, Redding GJ, American Thoracic Society Committee on Childhood Interstitial Lung Disease (chILD) and the chILD Research Network (2013) An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy. Am J Respir Crit Care Med 188(3):376–394. CrossRefGoogle Scholar
  5. 5.
    Clement A, Eber E (2008) Interstitial lung diseases in infants and children. Eur Respir J 31(3):658–666. CrossRefPubMedGoogle Scholar
  6. 6.
    Bush A, Cunningham S, de Blic J, Barbato A, Clement A, Epaud R, Hengst M, Kiper N, Nicholson AG, Wetzke M, Snijders D, Schwerk N, Griese M; chILD-EU Collaboration (2015) European protocols for the diagnosis and initial treatment of interstitial lung disease in children. Thorax 70(11):1078–1084. CrossRefPubMedGoogle Scholar
  7. 7.
    Vece TJ, Young LR (2016) Update on diffuse lung disease in children. Chest 149(3):836–845. CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Sverzellati N, Lynch DA, Hansell DM, Johkoh T, King TE Jr, Travis WD (2015) American thoracic society-european respiratory society classification of the idiopathic interstitial pneumonias: advances in knowledge since 2002. Radiographics 35(7):1849–1871. CrossRefPubMedGoogle Scholar
  9. 9.
    Deutsch GH, Young LR, Deterding RR, Fan LL, Dell SD, Bean JA, Brody AS, Nogee LM, Trapnell BC, Langston C; Pathology Cooperative Group, Albright EA, Askin FB, Baker P, Chou PM, Cool CM, Coventry SC, Cutz E, Davis MM, Dishop MK, Galambos C, Patterson K, Travis WD, Wert SE, White FV; ChILD Research Co-operative (2007) Diffuse lung disease in young children: application of a novel classification scheme. Am J Respir Crit Care Med 176(11):1120–1128. CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Lee EY (2013) Interstitial lung disease in infants: new classification system, imaging technique, clinical presentation and imaging findings. Pediatr Radiol 43(1):3–13. CrossRefPubMedGoogle Scholar
  11. 11.
    Thacker PG, Vargas SO, Fishman MP, Casey AM, Lee EY (2016) Current update on interstitial lung disease of infancy: new classification system, diagnostic evaluation, imaging algorithms, imaging findings, and prognosis. Radiol Clin N Am 54(6):1065–1076. CrossRefPubMedGoogle Scholar
  12. 12.
    (2015) European Guidelines on DRLs for paediatric imaging final complete draft for PiDRL Workshop 9. Accessed 19 Mar 2018
  13. 13.
    Lee EY, Cleveland RH, Langston C (2012) Interstitial lung disease in infants and children: new classification system with emphasis on clinical, imaging, and pathological correlation. In: Cleveland RH (ed) Imaging in pediatric pulmonology. Springer, New York, pp 99–154CrossRefGoogle Scholar
  14. 14.
    Dishop MK (2011) Paediatric interstitial lung disease: classification and definitions. Paediatr Respir Rev 12(4):230–237. CrossRefPubMedGoogle Scholar
  15. 15.
    Nogee LM (2017) Interstitial lung disease in newborns. Semin Fetal Neonatal Med. PubMedGoogle Scholar
  16. 16.
    Stankiewicz P, Sen P, Bhatt SS, Storer M, Xia Z, Bejjani BA, Ou Z, Wiszniewska J, Driscoll DJ, Maisenbacher MK, Bolivar J, Bauer M, Zackai EH, McDonald-McGinn D, Nowaczyk MM, Murray M, Hustead V, Mascotti K, Schultz R, Hallam L, McRae D, Nicholson AG, Newbury R, Durham-O’Donnell J, Knight G, Kini U, Shaikh TH, Martin V, Tyreman M, Simonic I, Willatt L, Paterson J, Mehta S, Rajan D, Fitzgerald T, Gribble S, Prigmore E, Patel A, Shaffer LG, Carter NP, Cheung SW, Langston C, Shaw-Smith C (2009) Genomic and genic deletions of the FOX gene cluster on 16q24.1 and inactivating mutations of FOXF1 cause alveolar capillary dysplasia and other malformations. Am J Hum Genet 84(6):780–791. CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Biyyam DR, Chapman T, Ferguson MR, Deutsch G, Dighe MK (2010) Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic–pathologic correlation. Radiographics 30(6):1721–1738. CrossRefPubMedGoogle Scholar
  18. 18.
    Cherukupalli K, Larson JE, Rotschild A, Thurlbeck WM (1996) Biochemical, clinical, and morphologic studies on lungs of infants with bronchopulmonary dysplasia. Pediatr Pulmonol 22(4):215–229.<215:AID-PPUL1>3.0.CO;2-LGoogle Scholar
  19. 19.
    Biko DM, Schwartz M, Anupindi SA, Altes TA (2008) Subpleural lung cysts in down syndrome: prevalence and association with coexisting diagnoses. Pediatr Radiol 38(3):280–284. CrossRefPubMedGoogle Scholar
  20. 20.
    Momma K, Matsuoka R, Takao A (1999) Aortic arch anomalies associated with chromosome 22q11 deletion (CATCH 22). Pediatr Cardiol 20(2):97–102. CrossRefPubMedGoogle Scholar
  21. 21.
    Robertson SP (2005) Filamin A: phenotypic diversity. Curr Opin Genet Dev 15(3):301–307. CrossRefPubMedGoogle Scholar
  22. 22.
    Guillerman RP (2010) Imaging of childhood interstitial lung disease. Pediatr Allergy Immunol Pulmonol 23(1):43–68. CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Hime NJ, Zurynski Y, Fitzgerald D, Selvadurai H, Phu A, Deverell M, Elliott EJ, Jaffe A (2015) Childhood interstitial lung disease: a systematic review. Pediatr Pulmonol 50(12):1383–1392. CrossRefPubMedGoogle Scholar
  24. 24.
    Deterding RR (2015) Children’s interstitial and diffuse lung disease. Progress and future horizons. Ann Am Thorac Soc 12(10):1451–1457. CrossRefPubMedGoogle Scholar
  25. 25.
    Doan ML, Guillerman RP, Dishop MK, Nogee LM, Langston C, Mallory GB, Sockrider MM, Fan LL (2008) Clinical, radiological and pathological features of ABCA3 mutations in children. Thorax 63(4):366–373. CrossRefPubMedGoogle Scholar
  26. 26.
    Cameron HS, Somaschini M, Carrera P, Hamvas A, Whitsett JA, Wert SE, Deutsch G, Nogee LM (2005) A common mutation in the surfactant protein C gene associated with lung disease. J Pediatr 146(3):370–375. CrossRefPubMedGoogle Scholar
  27. 27.
    Thouvenin G, Abou Taam R, Flamein F, Guillot L, Le Bourgeois M, Reix P, Fayon M, Counil F, Depontbriand U, Feldmann D, Pointe HD, de Blic J, Clement A, Epaud R (2010) Characteristics of disorders associated with genetic mutations of surfactant protein C. Arch Dis Child 95(6):449–454. CrossRefPubMedGoogle Scholar
  28. 28.
    Stevens PA, Pettenazzo A, Brasch F, Mulugeta S, Baritussio A, Ochs M, Morrison L, Russo SJ, Beers MF (2005) Nonspecific interstitial pneumonia, alveolar proteinosis, and abnormal proprotein trafficking resulting from a spontaneous mutation in the surfactant protein C gene. Pediatr Res 57(1):89–98. CrossRefPubMedGoogle Scholar
  29. 29.
    Whitsett JA, Wert SE, Weaver TE (2015) Diseases of pulmonary surfactant homeostasis. Annu Rev Pathol 10:371–393. CrossRefPubMedPubMedCentralGoogle Scholar
  30. 30.
    Salerno T, Peca D, Menchini L, Schiavino A, Petreschi F, Occasi F, Cogo P, Danhaive O, Cutrera R (2014) Respiratory insufficiency in a newborn with congenital hypothyroidism due to a new mutation of TTF-1/NKX2.1 gene. Pediatr Pulmonol 49(3):E42–E44. CrossRefPubMedGoogle Scholar
  31. 31.
    Valimahamed-Mitha S, Berteloot L, Ducoin H, Ottolenghi C, de Lonlay P, de Blic J (2015) Lung involvement in children with lysinuric protein intolerance. J Inherit Metab Dis 38(2):257–263. CrossRefPubMedGoogle Scholar
  32. 32.
    Popler J, Gower WA, Mogayzel PJ Jr, Nogee LM, Langston C, Wilson AC, Hay TC, Deterding RR (2010) Familial neuroendocrine cell hyperplasia of infancy. Pediatr Pulmonol 45(8):749–755. CrossRefPubMedGoogle Scholar
  33. 33.
    Davies SJ, Gosney JR, Hansell DM, Wells AU, du Bois RM, Burke MM, Sheppard MN, Nicholson AG (2007) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax 62(3):248–252. CrossRefPubMedGoogle Scholar
  34. 34.
    Cutz E, Yeger H, Wong V, Bienkowski E, Chan W (1985) In vitro characteristics of pulmonary neuroendocrine cells isolated from rabbit fetal lung. I. Effects of culture media and nerve growth factor. Lab Investig 53(6):672–683PubMedGoogle Scholar
  35. 35.
    Carr LL, Kern JA, Deutsch GH (2016) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and neuroendocrine hyperplasia of infancy. Clin Chest Med 37(3):579–587. CrossRefPubMedGoogle Scholar
  36. 36.
    Brody AS, Guillerman RP, Hay TC, Wagner BD, Young LR, Deutsch GH, Fan LL, Deterding RR (2010) Neuroendocrine cell hyperplasia of infancy: diagnosis with high-resolution CT. AJR Am J Roentgenol 194(1):238–244.
  37. 37.
    Guillerman RP, Brody AS (2011) Contemporary perspectives on pediatric diffuse lung disease. Radiol Clin N Am 49(5):847–868. CrossRefPubMedGoogle Scholar

Copyright information

© Italian Society of Medical Radiology 2018

Authors and Affiliations

  1. 1.Department of ImagingIRCCS Bambino Gesù Children’s HospitalRomeItaly
  2. 2.Pulmonary and Allergy Disease Unit and Cystic Fibrosis Center, Department of PediatricsIRCCS Istituto Giannina GasliniGenoaItaly
  3. 3.Paediatric Pulmonology and Sleep and Long Term Ventilation UnitIRCCS Bambino Gesù Children’s HospitalRomeItaly
  4. 4.Department of Pediatric RadiologyIRCCS Istituto Giannina GasliniGenoaItaly

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