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Congenital lung lesions: a radiographic pattern approach

  • Neonatal imaging
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Abstract

Congenital lung malformations represent a spectrum of abnormalities that can overlap in imaging appearance and frequently coexist in the same child. Imaging diagnosis in the neonatal period can be challenging; however, the recognition of several archetypal radiographic patterns can aid in narrowing the differential diagnosis. Major radiographic archetypes include (1) hyperlucent lung, (2) pulmonary cysts, (3) focal opacity and (4) normal radiograph. Here we review the multimodality imaging appearances of the most commonly seen congenital lung malformations, categorized by their primary imaging archetypes. Along with the congenital lung malformations, we present several important imaging mimickers.

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References

  1. Langston C (2003) New concepts in the pathology of congenital lung malformations. Semin Pediatr Surg 12:17–37

    Article  PubMed  Google Scholar 

  2. Riedlinger WFJ, Vargas SO, Jennings RW et al (2006) Bronchial atresia is common to extralobar sequestration, intralobar sequestration, congenital cystic adenomatoid malformation, and lobar emphysema. Pediatr Dev Pathol 9:361–373

    Article  PubMed  Google Scholar 

  3. Criss CN, Musili N, Matusko N et al (2018) Asymptomatic congenital lung malformations: is nonoperative management a viable alternative? J Pediatr Surg 53:1092–1097

    Article  PubMed  Google Scholar 

  4. Laberge JM, Flageole H, Pugash D et al (2001) Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther 16:178–186

    Article  CAS  PubMed  Google Scholar 

  5. Newman B (2006) Congenital bronchopulmonary foregut malformations: concepts and controversies. Pediatr Radiol 36:773–791

    Article  PubMed  Google Scholar 

  6. Cleveland RH, Weber B (1993) Retained fetal lung liquid in congenital lobar emphysema: a possible predictor of polyalveolar lobe. Pediatr Radiol 23:291–295

    Article  CAS  PubMed  Google Scholar 

  7. Biyyam DR, Chapman T, Ferguson MR et al (2010) Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic pathologic correlation. Radiographics 30:1721–1738

    Article  PubMed  Google Scholar 

  8. Style CC, Mehollin-Ray AR, Verla MA et al (2020) Accuracy of prenatal and postnatal imaging for management of congenital lung malformations. J Pediatr Surg 55:844–847

    Article  PubMed  Google Scholar 

  9. Lee EY (2012) Congenital lung masses. In: Imaging in pediatric pulmonology. Springer, New York, pp 31–38

    Chapter  Google Scholar 

  10. Berrocal T, Madrid C, Novo S et al (2004) Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics 24:e17

    Article  PubMed  Google Scholar 

  11. Oliver ER, DeBari SE, Horii SC et al (2019) Congenital lobar overinflation: a rare enigmatic lung lesion on prenatal ultrasound and magnetic resonance imaging. J Ultrasound Med 38:1229–1239

    Article  PubMed  Google Scholar 

  12. Kunisaki SM, Saito JM, Fallat ME et al (2019) Current operative management of congenital lobar emphysema in children: a report from the Midwest pediatric surgery consortium. J Pediatr Surg 54:1138–1142

    Article  PubMed  Google Scholar 

  13. Alamo L, Vial Y, Gengler C, Meuli R (2016) Imaging findings of bronchial atresia in fetuses, neonates and infants. Pediatr Radiol 46:383–390

    Article  PubMed  Google Scholar 

  14. Berdon WE (2000) Rings, slings, and other things: vascular compression of the infant trachea updated from the midcentury to the millennium — the legacy of Robert E. Gross, MD, and Edward B. D. Neuhauser, MD. Radiology 216:624–632

    Article  CAS  PubMed  Google Scholar 

  15. Rosenberg BF, Tantiwongse T, Wittenborg MH (1956) Anomalous course of left pulmonary artery with respiratory obstruction. Radiology 67:339–345

    Article  CAS  PubMed  Google Scholar 

  16. Ramaswamy M, Rudrappa S, Beeman A et al (2021) Lung hypoplasia associated with ring-sling complex is usually right-sided. Ann Thorac Surg. https://doi.org/10.1016/j.athoracsur.2021.01.059

  17. Yong MS, D’Udekem Y, Brizard CP et al (2013) Surgical management of pulmonary artery sling in children. J Thorac Cardiovasc Surg 145:1033–1039

    Article  PubMed  Google Scholar 

  18. Riggs KW, Zafar F, Jacobs ML et al (2021) Tracheal surgery for airway anomalies associated with increased mortality in pediatric patients undergoing heart surgery: Society of Thoracic Surgeons database analysis. J Thorac Cardiovasc Surg 161:1112–1121.e7

    Article  PubMed  Google Scholar 

  19. Brunner S, Nissen E (1963) Agenesis of the lung. Am Rev Respir Dis 87:103–106

    CAS  PubMed  Google Scholar 

  20. Osborne J, Masel J, Mccredie J (1989) A spectrum of skeletal anomalies associated with pulmonary agenesis: possible neural crest injuries. Pediatr Radiol 19:425–432

    Article  CAS  PubMed  Google Scholar 

  21. Wang H, Kalfa D, Rosenbaum MS et al (2018) Scimitar syndrome in children and adults: natural history, outcomes, and risk analysis. Ann Thorac Surg 105:592–598

    Article  PubMed  Google Scholar 

  22. Lee EY, Greenberg SB, Boiselle PM (2011) Multidetector computed tomography of pediatric large airway diseases: state-of-the-art. Radiol Clin N Am 49:869–893

    Article  PubMed  Google Scholar 

  23. Mata JM, Castellote A (2008) Pulmonary malformations beyond the neonatal period. Springer, Berlin, pp 123–146

    Google Scholar 

  24. Jones J, Almond C, Snyder H et al (1965) Lobar emphysema and congenital heart disease in infancy. J Thorac Cardiovasc Surg 49:1–10

    Article  CAS  PubMed  Google Scholar 

  25. Hishitani T, Ogawa K, Hoshino K et al (2003) Lobar emphysema due to ductus arteriosus compressing right upper bronchus in an infant with congenital heart disease. Ann Thorac Surg 75:1308–1310

    Article  PubMed  Google Scholar 

  26. Doğan ÖF, Duman Ü, Yorgancıoğlu C, Böke E (2006) Spontaneous regression of left upper lobe emphysema after division of large ductus arteriosus in an infant. Turk J Pediatr 48:269–271

    PubMed  Google Scholar 

  27. DiGeorge NW, El-Ali AM, White AM et al (2020) Pediatric cardiac CT and MRI: considerations for the general radiologist. AJR Am J Roentgenol 215:1464–1473

    Article  PubMed  Google Scholar 

  28. Shelmerdine SC, Semple T, Wallis C et al (2017) Filamin A (FLNA) mutation — a newcomer to the childhood interstitial lung disease (ChILD) classification. Pediatr Pulmonol 52:1306–1315

    Article  PubMed  Google Scholar 

  29. Burrage LC, Guillerman RP, Das S et al (2017) Lung transplantation for FLNA-associated progressive lung disease. J Pediatr 186:118–123.e6

    Article  PubMed  PubMed Central  Google Scholar 

  30. Loo DT, Kanner SB, Aruffo A (1998) Filamin binds to the cytoplasmic domain of the β1-integrin: identification of amino acids responsible for this interaction. J Biol Chem 273:23304–23312

    Article  CAS  PubMed  Google Scholar 

  31. Playford MP, Nurminen E, Pentikäinen OT et al (2010) Cystic fibrosis transmembrane conductance regulator interacts with multiple immunoglobulin domains of filamin A. J Biol Chem 285:17156–17165

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Pelizzo G, Collura M, Puglisi A et al (2019) Congenital emphysematous lung disease associated with a novel filamin A mutation. Case report and literature review. BMC Pediatr 19:1–10

    Article  Google Scholar 

  33. Shah AS, Black ED, Simon DM et al (2021) Heterogeneous pulmonary phenotypes in filamin A mutation-related lung disease. Pediatr Allergy Immunol Pulmonol 34:7–14

    Article  PubMed  PubMed Central  Google Scholar 

  34. Lange M, Kasper B, Bohring A et al (2015) 47 patients with FLNA associated periventricular nodular heterotopia. Orphanet J Rare Dis 10:1–11

    Article  Google Scholar 

  35. Woodward PJ, Kennedy A, Sohaey R et al (2005) Diagnostic imaging: obstetrics, 1st edn. Amirsys, Salt Lake City

    Google Scholar 

  36. Lee EY, Dorkin H, Vargas SO (2011) Congenital pulmonary malformations in pediatric patients: review and update on etiology, classification, and imaging findings. Radiol Clin N Am 49:921–948

    Article  PubMed  Google Scholar 

  37. Stocker JT (2009) Cystic lung disease in infants and children. Fetal Pediatr Pathol 28:155–184

    Article  PubMed  Google Scholar 

  38. Stocker JT (2002) Congenital pulmonary airway malformation: a new name for and an expanded classification of congenital cystic adenomatoid malformation of the lung. Histopathology 41:424–430

    Google Scholar 

  39. MacSweeney F, Papagiannopoulos K, Goldstraw P et al (2003) An assessment of the expanded classification of congenital cystic adenomatoid malformations and their relationship to malignant transformation. Am J Surg Pathol 27:1139–1146

    Article  PubMed  Google Scholar 

  40. Priest JR, Williams GM, Hill DA et al (2009) Pulmonary cysts in early childhood and the risk of malignancy. Pediatr Pulmonol 44:14–30

    Article  PubMed  Google Scholar 

  41. Cass DL, Quinn TM, Yang EY et al (1998) Increased cell proliferation and decreased apoptosis characterize congenital cystic adenomatoid malformation of the lung. J Pediatr Surg 33:1043–1046

    Article  CAS  PubMed  Google Scholar 

  42. Vu L, Tsao KJ, Lee H et al (2007) Characteristics of congenital cystic adenomatoid malformations associated with nonimmune hydrops and outcome. J Pediatr Surg 42:1351–1356

    Article  PubMed  Google Scholar 

  43. Epelman M, Kreiger PA, Servaes S et al (2010) Current imaging of prenatally diagnosed congenital lung lesions. Semin Ultrasound CT MRI 31:141–157

    Article  Google Scholar 

  44. Ng C, Stanwell J, Burge DM, Stanton MP (2014) Conservative management of antenatally diagnosed cystic lung malformations. Arch Dis Child 99:432–437

    Article  PubMed  Google Scholar 

  45. Wong A, Vieten D, Singh S et al (2009) Long-term outcome of asymptomatic patients with congenital cystic adenomatoid malformation. Pediatr Surg Int 25:479–485

    Article  CAS  PubMed  Google Scholar 

  46. Singh R, Davenport M (2015) The argument for operative approach to asymptomatic lung lesions. Semin Pediatr Surg 24:187–195

    Article  PubMed  Google Scholar 

  47. Hill DA, Jarzembowski JA, Priest JR et al (2008) Type I pleuropulmonary blastoma: pathology and biology study of 51 cases from the International Pleuropulmonary Blastoma Registry. Am J Surg Pathol 32:282–295

    Article  PubMed  Google Scholar 

  48. Schultz KAP, Rednam SP, Kamihara J et al (2017) PTEN, DICER1, FH, and their associated tumor susceptibility syndromes: clinical features, genetics, and surveillance recommendations in childhood. Clin Cancer Res 23:e76–e82

    Article  CAS  PubMed  Google Scholar 

  49. Stewart D, Best A, Williams GM et al (2019) Neoplasm risk among individuals with a pathogenic germline variant in DICER1. J Clin Oncol 37:668–676

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  50. Schultz KAP, Williams GM, Kamihara J et al (2018) DICER1 and associated conditions: identification of at-risk individuals and recommended surveillance strategies. Clin Cancer Res 24:2251–2261

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  51. Messinger YH, Stewart DR, Priest JR et al (2015) Pleuropulmonary blastoma: a report on 350 central pathology-confirmed pleuropulmonary blastoma cases by the International Pleuropulmonary Blastoma Registry. Cancer 121:276–285

    Article  PubMed  Google Scholar 

  52. Guillerman RP, Foulkes WD, Priest JR (2019) Imaging of DICER1 syndrome. Pediatr Radiol 49:1488–1505

    Article  PubMed  Google Scholar 

  53. Lima JS, Camargos PAM, Aguiar RALP et al (2014) Pre and perinatal aspects of congenital cystic adenomatoid malformation of the lung. J Matern Fetal Neonatal Med 27:228–232

    Article  PubMed  Google Scholar 

  54. Feinberg A, Hall NJ, Williams GM et al (2016) Can congenital pulmonary airway malformation be distinguished from type I pleuropulmonary blastoma based on clinical and radiological features? J Pediatr Surg 51:33–37

    Article  PubMed  Google Scholar 

  55. Waelti SL, Garel L, Soglio DD et al (2017) Neonatal congenital lung tumors — the importance of mid-second-trimester ultrasound as a diagnostic clue. Pediatr Radiol 47:1766–1775

    Article  PubMed  Google Scholar 

  56. Macklin MT, Macklin CC (1944) Malignant interstitial emphysema of the lungs and mediastinum as an important occult complication in many respiratory diseases and other conditions: an interpretation of the clinical literature in the light of laboratory experiment. Medicine 23:281–358

    Article  Google Scholar 

  57. Carey B (1999) Neonatal air leaks: pneumothorax, pneumomediastinum, pulmonary interstitial emphysema, pneumopericardium. Neonatal Netw 18:81–84

    Article  CAS  PubMed  Google Scholar 

  58. Greenough A, Dixon AK, Roberton NRC (1984) Pulmonary interstitial emphysema. Arch Dis Child 59:1046–1051

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  59. Li S, Still GG, Abdelbaki A et al (2018) Diffuse pulmonary interstitial emphysema in a late preterm neonate without mechanical ventilation. Case Reports Perinat Med. https://doi.org/10.1515/crpm-2017-0066

  60. Gürakan B, Tarcan A, Arda IS, Coşkun M (2002) Persistent pulmonary interstitial emphysema in an unventilated neonate. Pediatr Pulmonol 34:409–411

    Article  PubMed  Google Scholar 

  61. Gronbach J, Ehrhardt H, Zimmer K-P, Waitz M (2018) Early pulmonary interstitial emphysema in preterm neonates — respiratory management and case report in nonventilated very low birth weight twins. Am J Perinatol Reports 08:e99–e105

    Article  Google Scholar 

  62. Agrons GA, Courtney SE, Stocker JT, Markowitz RI (2005) Lung disease in premature neonates: radiologic-pathologic correlation. Radiographics 25:1047–1073

    Article  PubMed  Google Scholar 

  63. Donnelly LF, Frush DP (1999) Localized radiolucent chest lesions in neonates: causes and differentiation. AJR Am J Roentgenol 172:1651–1658

    Article  CAS  PubMed  Google Scholar 

  64. Jabra AA, Fishman EK, Shehata BM, Perlman EJ (1997) Case report. Localized persistent pulmonary interstitial emphysema: CT findings with radiographic-pathologic correlation. AJR Am J Roentgenol 169:1381–1384

    Article  CAS  PubMed  Google Scholar 

  65. Hansell DM, Bankier AA, MacMahon H et al (2008) Fleischner Society: glossary of terms for thoracic imaging. Radiology 246:697–722

    Article  PubMed  Google Scholar 

  66. Quigley MJ, Fraser RS (1988) Pulmonary pneumatocele: pathology and pathogenesis. AJR Am J Roentgenol 150:1275–1277

    Article  CAS  PubMed  Google Scholar 

  67. Armstrong LB, Mooney DP (2018) Pneumatoceles in pediatric blunt trauma: common and benign. J Pediatr Surg 53:1310–1312

    Article  PubMed  Google Scholar 

  68. Gerdung CA, Ross BC, Dicken BJ, Bjornson CL (2019) Pneumonectomy in a child with multilobar pneumatocele secondary to necrotizing pneumonia: case report and review of the literature. Case Rep Pediatr 2019:1–5

    Article  Google Scholar 

  69. Harris VJ, Brown R (1975) Pneumatoceles as a complication of chemical pneumonia after hydrocarbon ingestion. AJR Am J Roentgenol 125:531–537

    Article  CAS  Google Scholar 

  70. Clarke TA, Edwards DK (1979) Pulmonary pseudocysts in newborn infants with respiratory distress syndrome. AJR Am J Roentgenol 133:417–421

    Article  CAS  PubMed  Google Scholar 

  71. Victoria MS, Steiner P, Rao M (1981) Persistent postpneumonic pneumatoceles in children. Chest 79:359–361

    Article  CAS  PubMed  Google Scholar 

  72. Amitai I, Mogle P, Godfrey S, Aviad I (1983) Pneumatocele in infants and children: report of 12 cases. Clin Pediatr 22:420–422

    Article  CAS  Google Scholar 

  73. Chow JS, Chung EM, Colin AA, Cleveland RH (2012) Focal lung disease. In: Imaging in pediatric pulmonology. Springer, New York, pp 193–248

    Chapter  Google Scholar 

  74. Imamoǧlu M, Çay A, Koşucu P et al (2005) Pneumatoceles in postpneumonic empyema: an algorithmic approach. J Pediatr Surg 40:1111–1117

    Article  PubMed  Google Scholar 

  75. Puligandla PS, Laberge JM (2012) Infections and diseases of the lungs, pleura, and mediastinum. In: Pediatric surgery. Elsevier, Amsterdam, pp 855–880

    Chapter  Google Scholar 

  76. Burgos CM, Frenckner B, Luco M et al (2019) Prenatally versus postnatally diagnosed congenital diaphragmatic hernia — side, stage, and outcome. J Pediatr Surg 54:651–655

    Article  PubMed  Google Scholar 

  77. Coren ME, Rosenthal M, Bush A (1997) Congenital diaphragmatic hernia misdiagnosed as tension pneumothorax. Pediatr Pulmonol 24:119–121

    Article  CAS  PubMed  Google Scholar 

  78. Tsui KP, Chan KW, Lee KH (2010) Acute complications of late-presenting congenital diaphragmatic hernia in children: report of 2 cases and review of literature. Hong Kong J Paediatr 15:157–160

    Google Scholar 

  79. Juwarkar CS, Kamble DS, Sawant V (2010) A late presenting congenital diaphragmatic hernia misdiagnosed as spontaneous pneumothorax. Indian J Anaesth 54:464–466

    Article  PubMed  PubMed Central  Google Scholar 

  80. Stolar CJH, Dillon PW (2012) Congenital diaphragmatic hernia and eventration. In: Pediatric surgery. Elsevier, Amsterdam, pp 809–824

    Chapter  Google Scholar 

  81. Baerg J, Kanthimathinathan V, Gollin G (2012) Late-presenting congenital diaphragmatic hernia: diagnostic pitfalls and outcome. Hernia 16:461–466

    Article  CAS  PubMed  Google Scholar 

  82. Taber P, Gyepes MT, Lackey DA (1973) Malposition of umbilical vascular catheters in the diagnosis of congenital diaphragmatic hernia. Radiology 109:413–415

    Article  CAS  PubMed  Google Scholar 

  83. Sakural M, Donnelly LF, Klosterman LA, Strîfe JL (2000) Congenital diaphragmatic hernia in neonates: variations in umbilical catheter and enteric tube position. Radiology 216:112–116

    Article  Google Scholar 

  84. Winters WD, Effmann EL (2001) Congenital masses of the lung: prenatal and postnatal imaging evaluation. J Thorac Imaging 16:196–206

    Article  CAS  PubMed  Google Scholar 

  85. Zhang H, Tian J, Chen Z et al (2014) Retrospective study of prenatal diagnosed pulmonary sequestration. Pediatr Surg Int 30:47–53

    Article  PubMed  Google Scholar 

  86. Lager DJ, Kuper KA, Haake GK (1991) Subdiaphragmatic extralobar pulmonary sequestration. Arch Pathol Lab Med 115:536–538

    CAS  PubMed  Google Scholar 

  87. Adzick NS, Harrison MR, Crombleholme TM et al (1998) Fetal lung lesions: management and outcome. Am J Obstet Gynecol 179:884–889

    Article  CAS  PubMed  Google Scholar 

  88. Riley JS, Urwin JW, Oliver ER et al (2018) Prenatal growth characteristics and pre/postnatal management of bronchopulmonary sequestrations. J Pediatr Surg 53:265–269

    Article  PubMed  Google Scholar 

  89. Brown SC, De Laat M, Proesmans M et al (2012) Treatment strategies for pulmonary sequestration in childhood: resection, embolization, observation? Acta Cardiol 67:629–634

    Article  PubMed  Google Scholar 

  90. Fagan CJ, Swischuk LE (1972) The opaque lung in lobar emphysema. AJR Am J Roentgenol Radium Ther Nucl Med 114:300–304

    Article  CAS  Google Scholar 

  91. Epelman M, Lee EY (2013) Congenital lung anomalies. In: Coley B (ed) Caffey’s pediatric diagnostic imaging, 12th edn. Saunders, Philadelphia, p 551

    Google Scholar 

  92. Bellini C, Ergaz Z, Radicioni M et al (2012) Congenital fetal and neonatal visceral chylous effusions: neonatal chylothorax and chylous ascites revisited. A multicenter retrospective study. Lymphology 45:91–102

    CAS  PubMed  Google Scholar 

  93. Groom KR, Murphey MD, Howard LM et al (2002) Mesenchymal hamartoma of the chest wall: radiologic manifestations with emphasis on cross-sectional imaging and histopathologic comparison. Radiology 222:205–211

    Article  PubMed  Google Scholar 

  94. Dishop MK, McKay EM, Kreiger PA et al (2010) Fetal lung interstitial tumor (FLIT): a proposed newly recognized lung tumor of infancy to be differentiated from cystic pleuropulmonary blastoma and other developmental pulmonary lesions. Am J Surg Pathol 34:1762–1772

    Article  PubMed  Google Scholar 

  95. Orman G, Masand P, Hicks J et al (2020) Pediatric thoracic mass lesions: beyond the common. Eur J Radiol Open 7:100240

    Article  PubMed  PubMed Central  Google Scholar 

  96. Guillerman PR, Vogelius E, Pinto-Rojas A, Parham DM (2015) Malignancies of the pediatric lower respiratory tract. In: Pediatric malignancies: pathology and imaging. Springer, New York, pp 227–243

    Chapter  Google Scholar 

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El-Ali, A.M., Strubel, N.A. & Lala, S.V. Congenital lung lesions: a radiographic pattern approach. Pediatr Radiol 52, 622–636 (2022). https://doi.org/10.1007/s00247-021-05210-9

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