Skip to main content
Log in

Interstitial lung disease in infants: new classification system, imaging technique, clinical presentation and imaging findings

  • Review
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

Interstitial lung disease (ILD) is defined as a rare, heterogeneous group of parenchymal lung conditions that develop primarily because of underlying developmental or genetic disorders. Affected infants typically present with clinical syndromes characterized by dyspnea, tachypnea, crackles and hypoxemia. Until recently, the understanding of ILD in infants has been limited largely owing to a lack of evidence-based information of underlying pathogenesis, natural history, imaging findings and histopathological features. However, ILD in infants is now better understood and managed because of (1) advances in imaging methods that result in rapid and accurate detection, (2) improved thoracoscopic techniques for lung biopsy, (3) a consensus regarding the pathological criteria for these particular lung conditions and (4) a new classification system based on the underlying etiology of ILD. This article reviews the new classification system, imaging technique, clinical presentation and imaging findings of ILD in infants. Specialized knowledge of this new classification system in conjunction with recognition of characteristic imaging findings of ILD in infants has great potential for early and accurate diagnosis, which in turn can lead to optimal patient management.

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.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10
Fig. 11
Fig. 12

Similar content being viewed by others

References

  1. 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 Science + Business Media, Dordrecht, The Netherlands, pp 156–183

    Google Scholar 

  2. Deterding RR (2010) Infants and young children with children’s interstitial lung disease. Pediatr Allergy Immunol Pulmonol 23:25–31

    Article  PubMed  Google Scholar 

  3. Vece TJ, Fan LL (2011) Diagnosis and management of diffuse lung disease in children. Paediatr Respir Rev 12:238–242

    Article  PubMed  Google Scholar 

  4. Das S, Langston C, Fan LL (2011) Interstitial lung disease in children. Curr Opin Pediatr 23:325–331

    Article  PubMed  Google Scholar 

  5. Deterding RR (2010) Expanding our understanding of children’s interstitial lung disease. Pediatr Allergy Immunol Pulmonol 23:3–4

    Article  PubMed  Google Scholar 

  6. Prestridge A, Deterding RR (2010) Diffuse lung disease in children. Pediatr Ann 39:777–783

    Article  PubMed  Google Scholar 

  7. Guillerman RP, Brody AS (2011) Contemporary perspective on pediatric diffuse lung disease. Radiol Clin North Am 49:847–868

    Article  PubMed  Google Scholar 

  8. Deterding R (2007) Evaluating infants and children with interstitial lung disease. Semin Respir Crit Care Med 28:333–341

    Article  PubMed  Google Scholar 

  9. Deutsch GH, Young LR, Deterding RR et al (2007) Diffuse lung disease in young children: application of a novel classification scheme. Am J Respir Crit Care Med 176:1120–1128

    Article  PubMed  Google Scholar 

  10. Ponsky TA, Rothenberg SS, Tsao K et al (2009) Thoracoscopy in children: is a chest tube necessary? J Laparoendosc Adv Surg Tech A 19(Suppl 1):S23–S25

    Article  PubMed  Google Scholar 

  11. Ponsky TA, Rothenberg SS (2008) Thoracoscopic lung biopsy in infants and children with endoloops allows smaller trocar sites and discreet biopsies. J Laparoendosc Adv Surg Tech A 18:120–122

    Article  PubMed  Google Scholar 

  12. Fan LL, Langston C (2002) Pediatric interstitial lung disease: children are not small adults. Am J Respir Crit Care Med 165:1466–1467

    Article  PubMed  Google Scholar 

  13. Langston C, Dishop MK (2004) Infant lung biopsy: clarifying the pathologic spectrum. Pathol Int 54:S419–S421

    Google Scholar 

  14. Guillerman RP (2010) Imaging of childhood interstitial lung disease. Pediatr Allergy Immunol Pulmonol 23:43–68

    Article  PubMed  Google Scholar 

  15. Clement A, Task Force ERS (2004) Task force on chronic interstitial lung disease in immunocompetent children. Eur Respir J 24:686–697

    Article  PubMed  CAS  Google Scholar 

  16. Furlow B (2011) Radiation protection in pediatric imaging. Radiol Technol 82:421–439

    PubMed  Google Scholar 

  17. Seely JM, Effmann EL, Muller NL (1997) High-resolution CT of pediatric lung disease: imaging findings. AJR 168:1269–1275

    PubMed  CAS  Google Scholar 

  18. Garcia-Pena P, Lucaya J (2004) HRCT in children: technique and indications. Eur Radiol 14(Suppl 4):L13–L30

    PubMed  Google Scholar 

  19. Garcia-Pena P, Boixadera H, Barber I et al (2011) Thoracic findings of systemic diseases at high-resolution CT in children. Radiographics 31:465–482

    Article  PubMed  Google Scholar 

  20. Kazerooni EA (2001) High-resolution CT of the lungs. AJR 177:501–519

    PubMed  CAS  Google Scholar 

  21. Bastos M, Lee EY, Strauss KJ et al (2009) Motion artifact on high-resolution CT images of pediatric patients: comparison of volumetric and axial CT methods. AJR 193:1414–1418

    Article  PubMed  Google Scholar 

  22. Sumikawa H, Johkoh T, Koyama M et al (2005) Image quality of high-resolution CT with 16-channel multidetector-row CT: comparison between helical scan and conventional step-shoot scan. Radiat Med 23:539–544

    PubMed  Google Scholar 

  23. Mayo JR (2009) CT evaluation of diffuse infiltrative lung disease: dose considerations and optimal technique. J Thorac Imag 24:252–259

    Article  Google Scholar 

  24. Lucaya J, Garcia-Pena P, Herrera L et al (2000) Expiratory chest CT in children. AJR 174:235–241

    PubMed  CAS  Google Scholar 

  25. Long FR, Castile RG, Brody AS (1999) Lungs in infants and young children: improved thin-section CT with a noninvasive controlled-ventilation technique—initial experience. Radiology 212:588–593

    PubMed  CAS  Google Scholar 

  26. 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

    Article  PubMed  CAS  Google Scholar 

  27. Lee EY, Mason KP, Zurakowski D et al (2008) MDCT assessment of tracheomalacia in symptomatic infants with mediastinal aortic vascular anomalies: preliminary technical experience. Pediatr Radiol 38:82–88

    Article  PubMed  CAS  Google Scholar 

  28. Lee EY, Boiselle PM (2009) Tracheobronchomalacia in infants and children: multidetector CT evaluation. Radiology 252:7–22

    Article  PubMed  Google Scholar 

  29. Lee EY, Strauss KJ, Tracy DA et al (2010) Comparison of standard-dose and reduced-dose expiratory MDCT techniques for assessment of tracheomalacia in children. Acad Radiol 17:504–510

    Article  PubMed  Google Scholar 

  30. Rutledge JC, Jensen P (1986) Acinar dysplasia: a new form of pulmonary maldevelopment. Hum Pathol 17:1290–1293

    Article  PubMed  CAS  Google Scholar 

  31. Gillespie LM, Fenton AC, Wright C (2004) Acinar dysplasia: a rare cause of neonatal respiratory failure. Acta Paediatr 93:712–713

    Article  PubMed  CAS  Google Scholar 

  32. Hugosson CO, Salama HM, Al-Dayel F et al (2005) Primary alveolar capillary dysplasia (acinar dysplasia) and surfactant protein B deficiency: a clinical, radiological and pathological study. Pediatr Radiol 35:311–316

    Article  PubMed  Google Scholar 

  33. Michalsky MP, Arca MJ, Groenman F et al (2005) Alveolar capillary dysplasia: a logical approach to a fatal disease. J Pediatr Surg 40:1100–1105

    Article  PubMed  Google Scholar 

  34. Haraida S, Lochbuhler H, Heger A et al (1997) Congenital alveolar capillary dysplasia: rare cause of persistent pulmonary hypertension. Pediatr Pathol Lab Med 17:959–975

    Article  PubMed  CAS  Google Scholar 

  35. Eulmesekian P, Cutz E, Parvez B et al (2005) Alveolar capillary dysplasia: a six-year single center experience. J Perinat Med 33:347–352

    Article  PubMed  Google Scholar 

  36. Bishop NB, Stankiewicz P, Steinhorn RH (2011) Alveolar capillary dysplasia. Am J Respir Crit Care Med 184:172–179

    Article  PubMed  Google Scholar 

  37. Hung SP, Hung SH, Wu CH et al (2011) Misalignment of lung vessels and alveolar capillary dysplasia: a case report with autopsy. Pediatri Neonatal 52:232–236

    Article  Google Scholar 

  38. Sen P, Choudhury T, Smith EO et al (2010) Expression of angiogenic and vasculogenic proteins in the lung in alveolar capillary dysplasia/misalignment of pulmonary veins: an immunohistochemical study. Pediatr Dev Pathol 13:354–361

    Article  PubMed  Google Scholar 

  39. Sihoe AD, Lee AT, To KF et al (2005) Alveolar capillary dysplasia with congenital misalignment of pulmonary vessels. Asian Cardiovasc Thorac Ann 13:82–84

    PubMed  Google Scholar 

  40. Nogee LM (2010) Genetic basis of children’s interstitial lung disease. Pediatr Allergy Immunol Pulmonol 23:15–24

    Article  PubMed  Google Scholar 

  41. Stankiewicz P, Sen P, Bhatt SS et al (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 84:780–791

    Article  PubMed  CAS  Google Scholar 

  42. Gamillscheg A, Zobel G, Spuller E et al (2008) Aortic coarctation associated with alveolar capillary dysplasia and misalignment of the pulmonary veins. Pediatr Cardiol 29:191–194

    Article  PubMed  Google Scholar 

  43. Taborosi B, Todt-Pingel I, Kayser G et al (2007) A rare case of aortic coarctation and ventricular septal defect combined with alveolar capillary dysplasia. Pediatr Cardiol 28:319–323

    Article  PubMed  CAS  Google Scholar 

  44. Roth W, Bucsenez D, Blaker H et al (2006) Misalignment of pulmonary vessels with alveolar capillary dysplasia: association with atrioventricular septal defect and quadricuspid pulmonary valve. Virchows Arch 448:375–378

    Article  PubMed  Google Scholar 

  45. Ahmed S, Ackerman V, Faught P et al (2008) Profound hypoxemia and pulmonary hypertension in a 7-month-old infant: late presentation of alveolar capillary dysplasia. Pediatr Crit Care Med 9:e43–e46

    Article  PubMed  Google Scholar 

  46. Roy PG, Patel P, Vayalakkad A et al (2007) Alveolar capillary dysplasia presenting as pneumothorax: a case report and review of literature. Pediatr Surg Int 23:915–917

    Article  PubMed  Google Scholar 

  47. Mahut B, De Blic J, Emond S et al (2007) Chest computed tomography findings in bronchopulmonary dysplasia and correlation with lung function. Arch Dis Child Fetal Neonatal Ed 92:F459–F464

    Article  PubMed  Google Scholar 

  48. Aukland SM, Halvorsen T, Fosse KR et al (2006) High-resolution CT of the chest in children and young adults who were born prematurely: findings in a population-based study. AJR 187:1012–1018

    Article  PubMed  Google Scholar 

  49. Biko DM, Schwartz M, Anupindi SA et al (2008) Subpleural lung cysts in Down syndrome: prevalence and association with coexisting diagnoses. Pediatr Radiol 38:280–284

    Article  PubMed  Google Scholar 

  50. Taylor PA, Dishop MK, Lotze TE et al (2009) Congenital multilobar emphysema: a characteristic lung growth disorder attributable to filamin A gene mutations. Pediatr Radiol 39(Suppl 3):S516

    Google Scholar 

  51. De Wit MC, Tiddens HA, de Coo IF et al (2011) Lung disease in FLAN mutation: confirmatory report. Eur J Med Genet 54:299–300

    Article  PubMed  Google Scholar 

  52. Masurel-Paulet A, Haan E, Thompson EM et al (2011) Lung disease associated with periventricular nodular heterotopia and an FLNA mutation. Eur J Med Genet 54:25–28

    Article  PubMed  Google Scholar 

  53. Epaud R, Jonard L, Ducou-le-Pointe H et al (2012) Genetic disorders of surfactant. Arch Pediatr 19:212–219

    Article  PubMed  CAS  Google Scholar 

  54. Wert SE, Whitsett JA, Nogee LM (2009) Genetic disorders of surfactant dysfunction. Pediatr Dev Pathol 12:253–274

    Article  PubMed  CAS  Google Scholar 

  55. Somaschini M, Nogee LM, Sassi I et al (2007) Unexplained neonatal respiratory distress due to congenital surfactant deficiency. J Pediatr 150:649–653

    Article  PubMed  Google Scholar 

  56. Wilder MA (2004) Surfactant protein B deficiency in infants with respiratory failure. J Perinat Neonatal Nurs 18:61–67

    PubMed  Google Scholar 

  57. Garmany TH, Moxley MA, White FV et al (2006) Surfactant composition and function in patients with ABCA3 mutations. Pediatr Res 59:801–805

    Article  PubMed  CAS  Google Scholar 

  58. Somaschini M, Wert S, Mangili G et al (2000) Hereditary surfactant protein B deficiency resulting from a novel mutation. Intensive Care Med 26:97–100

    Article  PubMed  CAS  Google Scholar 

  59. Hamvas A (2006) Inherited surfactant protein-B deficiency and surfactant protein-C associated disease: clinical features and evaluation. Semin Perinatol 30:316–326

    Article  PubMed  Google Scholar 

  60. Matsumura Y, Ban N, Ueda K et al (2006) Characterization and classification of ATP-binding cassette transporter ABCA3 mutants in fatal surfactant deficiency. J Bio Chem 281:34503–34514

    Article  CAS  Google Scholar 

  61. Doan ML, Guillerman RP, Dishop MK et al (2008) Clinical, radiological and pathological features of ABCA3 mutations in children. Thorax 63:366–373

    Article  PubMed  CAS  Google Scholar 

  62. Newman B, Kuhn JP, Kramer SS et al (2001) Congenital surfactant protein B deficiency—emphasis on imaging. Pediatr Radiol 31:327–331

    Article  PubMed  CAS  Google Scholar 

  63. Galambos C, Levy H, Cannon CL et al (2010) Pulmonary pathology in thyroid transcription factor-1 deficiency syndrome. Am J Respir Crit Care Med 182:549–554

    Article  PubMed  Google Scholar 

  64. Parto K, Svedstrom E, Majurin ML et al (1993) Pulmonary manifestations in lysinuric protein intolerance. Chest 104:1176–1182

    Article  PubMed  CAS  Google Scholar 

  65. Santamaria F, Parenti G, Guidi G et al (1996) Early detection of lung involvement in lysinuric protein intolerance: role of high-resolution computed tomography and radioisotopic methods. Am J Respir Crit Care Med 153:731–735

    PubMed  CAS  Google Scholar 

  66. Rossi SE, Erasmus JJ, Volpacchio M et al (2003) ‘Crazy-paving’ pattern at thin-section CT of the lungs: radiologic-pathologic overview. Radiographics 23:1509–1519

    Article  PubMed  Google Scholar 

  67. Zontsich T, Helbich TH, Wojnarovsky C et al (1998) Pulmonary alveolar proteinosis in a child: HRCT findings before and after bronchoalveolar lavage. Eur Radiol 8:1680–1682

    Article  PubMed  CAS  Google Scholar 

  68. Cetin E, Lee EY (2007) Pneumocystis carinii pneumonia in an infant with hypogammaglobulinemia. Pediatr Radiol 37:329

    Article  PubMed  Google Scholar 

  69. Gower WA, Nogee LM (2011) Surfactant dysfunction. Paediatr Respir Rev 12:223–229

    Article  PubMed  Google Scholar 

  70. Deterding RR, Pye C, Fan LL et al (2005) Persistent tachypnea of infancy is associated with neuroendocrine cell hyperplasia. Pediatr Pulmonol 40:157–165

    Article  PubMed  Google Scholar 

  71. Cutz E, Yeger H, Pan J (2007) Pulmonary neuroendocrine cell system in pediatric lung disease—recent advances. Pediatr Dev Pathol 10:419–435

    Article  PubMed  CAS  Google Scholar 

  72. Popler J, Gower WA, Mogayzel PJ Jr et al (2010) Familial neuroendocrine cell hyperplasia of infancy. Pediatr Pulmonol 45:749–755

    Article  PubMed  CAS  Google Scholar 

  73. Brody AS, Guillerman RP, Hay TC et al (2010) Neuroendocrine cell hyperplasia of infancy: diagnosis with high-resolution CT. AJR 194:238–244

    Article  PubMed  Google Scholar 

  74. Young LR, Brody AS, Inge TH et al (2011) Neuroendocrine cell distribution and frequency distinguish neuroendocrine cell hyperplasia of infancy from other pulmonary disorders. Chest 139:1060–1071

    Article  PubMed  Google Scholar 

  75. Canakis AM, Cutz E, Monson D et al (2002) Pulmonary interstitial glycogenosis: a new variant of neonatal interstitial lung disease. Am J Respir Crit Care Med 165:1557–1565

    Article  PubMed  Google Scholar 

  76. Smets K, Dhaene K, Schelstraete P et al (2004) Neonatal pulmonary interstitial glycogen accumulation disorder. Eur J Pediatr 163:408–409

    Article  PubMed  Google Scholar 

  77. Deutsch GH, Young LR (2009) Histologic resolution of pulmonary interstitial glycogenosis. Pediatr Dev Pathol 12:475–480

    Article  PubMed  Google Scholar 

  78. Lanfranchi M, Allbery SM, Wheelock L et al (2010) Pulmonary interstitial glycogenosis. Pediatr Radiol 40:361–365

    Article  PubMed  Google Scholar 

  79. Castillo M, Vade A, Lim-Dunham JE et al (2010) Pulmonary interstitial glycogenosis in the setting of lung growth abnormality: radiographic and pathologic correlation. Pediatr Radiol 40:1562–1565

    Article  PubMed  Google Scholar 

Download references

Disclosure

The author has indicated he has no relevant financial relationships or potential conflicts of interest related to the material.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Edward Y. Lee.

Additional information

This article has been selected as the CME activity for the current month. Please visit the SPR Web site at www.pedrad.org on the Education page and follow the instructions to complete this CME activity.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Lee, E.Y. Interstitial lung disease in infants: new classification system, imaging technique, clinical presentation and imaging findings. Pediatr Radiol 43, 3–13 (2013). https://doi.org/10.1007/s00247-012-2524-x

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-012-2524-x

Keywords

Navigation