Skip to main content
Log in

Spectrum of pulmonary sequestration: Association with anomalous pulmonary venous drainage in infants

  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Summary

Pulmonary sequestration is a spectrum of related lesions, each of which may be absent or present: (1) bronchial sequestration of pulmonary parenchyma; (2) arterial supply from systemic circulation; (3) anomalous pulmonary venous drainage to the right atrium; (4) communications between bronchus and esophagus; (5) defects of diaphragm; (6) gross lung anomalies, such as horseshoe lungs or hypoplasia. Any combination of these primary lesions can occur in an individual patient. Diagnosis should be directed towards each component of the spectrum. Of special importance is the venous connection, as anomalous pulmonary venous drainage can involve not only the sequestered segment but the entire ipsilateral lung, making surgical therapy far more complex. Treatment of choice is surgical resection, associated, if needed, with rerouting of the pulmonary venous return.

Classification of sequestration of the lung as intra- and extralobar is of secondary importance: these 2 groups do not represent lesions of different embryological significance.

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.

Similar content being viewed by others

References

  1. Abbey Smith R (1956) A theory of the origin of intralobar sequestration of lung. Thorax11:10–24

    PubMed  Google Scholar 

  2. Blesovsky A (1967) Pulmonary sequestration. Thorax22:351–357

    PubMed  Google Scholar 

  3. Bolck F (1950) Zur Frage der Entstehung von Nebenlungen. Virchows Archiv319:20–43

    PubMed  Google Scholar 

  4. Boyd G (1953) Intralobar pulmonary sequestration. Dis Chest24:162–172

    PubMed  Google Scholar 

  5. Boyden EA (1958) Bronchogenic cysts and the theory of intralobar sequestration: new embryologic data. J Thorac Surg35:604–616

    PubMed  Google Scholar 

  6. Choplin RH, Siegel MJ (1980) Pulmonary sequestration. Am J Roentg134:695–700

    Google Scholar 

  7. Cipriano P, Sweeney LJ, Hutchins GM, Rosenquist GC (1975) Horseshoe lung in an infant with recurrent pulmonary infections. Am J Dis Child129:1343–1345

    PubMed  Google Scholar 

  8. Cole FH, Alley FH, Jones RS (1951) Aberrant systemic arteries to the lower lung. Surg Gynecol Obstet93:589–596

    PubMed  Google Scholar 

  9. Currarino G, Willis K, Miller W (1975) Congenital fistula between an aberrant systemic artery and a pulmonary vein without sequestration. J Pediatr87:554–557

    PubMed  Google Scholar 

  10. Derksen OS (1977) Scimitar syndrome and pulmonary sequestration. Radiol Clin Biol46:81–93

    Google Scholar 

  11. Dische MR, Teixeira ML, Winchester PH, Engle MA (1974) Horseshoe lung associated with a variant of the “scimitar” syndrome. Br Heart J36:617–620

    PubMed  Google Scholar 

  12. Flye MW, Conley M, Silver D (1976) Spectrum of pulmonary sequestration. Ann Thorac Surg22:478–482

    PubMed  Google Scholar 

  13. Fusonie D, Molnar W (1966) Anomalous pulmonary venous return, pulmonary sequestration, bronchial atresia, aplastic right upper lobe, pericardial defect and intrathoracic kidney. Am J Roentg97:350–354

    PubMed  Google Scholar 

  14. Gans SL, Potts WJ (1951) Anomalous lobe of lung arising from the esophagus. J Thorac Surg21:313–318

    PubMed  Google Scholar 

  15. Gerle RD, Jaretzki A III, Ashley CA, Berne AS (1968) Congenital bronchopulmonary-foregut malformation. N Engl J Med278:1413–1419

    PubMed  Google Scholar 

  16. Halasz NA, Halloran KH, Liebow AA (1956) Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava. Circulation14:826–846

    PubMed  Google Scholar 

  17. Heithoff KB, Sane SM, Williams HJ, Jarvic CJ, Carter J, Kane P, Brennom W (1976) Am J Roentg126:46–55

    Google Scholar 

  18. Ishihara Y, Fukuda R, Awaya Y, et al (1979) Anomalous systemic arterial supply to the basal segments of the lung presenting with a murmur. Eur J Pediat131:125–131

    Google Scholar 

  19. Jona JZ, Raffensperger JG (1975) Total sequestration of the lung. J Thorac Cardiovasc Surg69:361–364

    PubMed  Google Scholar 

  20. Kafka V, Beco V (1960) Simultaneous intra- and extrapulmonary sequestration. Arch Dis Child35:51–56

    PubMed  Google Scholar 

  21. Kiely B, Filler J, Stone S, Doyle EF (1967) Syndrome of anomalous venous drainage of the right lung to the inferior vena cava. Am J Cardiol20:102–116

    Google Scholar 

  22. Kirks DR, Kane PE, Free EA, Taybi H (1976) Systemic arterial supply to normal basilar segments of the left lower lobe. Am J Roentg126:817–821

    Google Scholar 

  23. Kyllönen KE (1964) Intralobar pulmonary sequestration and a theory as to its aetiology. Acta Chir Scand127:307–310

    PubMed  Google Scholar 

  24. Lacina S, Townley R, Radecki L, Stockinger F, Wyngaarden M (1981) Esophageal lung with cardiac abnormalities. Chest79:468–470

    PubMed  Google Scholar 

  25. Litwin SB, Plauth WH, Nadas AS (1970) Anomalous systemic arterial supply to the lung causing pulmonary-artery hypertension. N Engl J Med283:1098–1099

    PubMed  Google Scholar 

  26. Mathey J, Galey JJ, Logeais Y, Santoro E, Vanetti A, Maurel A, Wuerflein R (1968) Anomalous pulmonary venous return into inferior vena cava and associated bronchovascular anomalies (the scimitar syndrome). Thorax23:398–407

    PubMed  Google Scholar 

  27. Neill CA, Ferencz C, Sabiston DC, Sheldon H (1960) The familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage, “scimitar syndrome.” J Hopkins Hosp Bull107:1–21

    Google Scholar 

  28. Pendse P, Alexander J, Khademi M, Groff DB (1972) Pulmonary sequestration. J Thorac Cardiovasc Surg64:127–131

    PubMed  Google Scholar 

  29. Potter EL (1952) Pathology of the fetus and the newborn. The Yearbook Publishers, Chicago, pp 261–263

    Google Scholar 

  30. Pryce DM (1946) Lower accessory pulmonary artery with intralobar sequestration of lung: a report of seven cases. J Path Bact58:457–467

    Google Scholar 

  31. Ransom JM, Norton JB, Williams GD (1978) Pulmonary sequestration presenting as congestive heart failure. J Thorac Cardiovasc Surg76:378–380

    PubMed  Google Scholar 

  32. Roe JP, Mack JW, Shirley JH (1980) Bilateral pulmonary sequestrations. J Thorac Cardiovasc Surg80:8–10

    PubMed  Google Scholar 

  33. Sade RM, Clouse M, Ellis FH Jr (1974) The spectrum of pulmonary sequestration. Ann Thorac Surg18:644–658

    PubMed  Google Scholar 

  34. Saegesser F, Besson A (1973) Extralobar and intralobar pulmonary sequestrations of the upper and lower lobes. Chest63:69–73

    PubMed  Google Scholar 

  35. Savić B, Birtel FJ, Knoche R, Tholen W, Schild H (1979) Pulmonary sequestration. Ergeb Kinderheilk43:57–92

    Google Scholar 

  36. Savic (or Savich) B, Birtel FJ, Knoche R, Tholen W, Schild H (1979) Pulmonary sequestration (abbreviated). Thorax34:96–101

    PubMed  Google Scholar 

  37. St Raymond AH Jr, Hardy JD, Robbins SG (1956) Lower accessory lung communicating with the esophagus and associated with congenital diaphragmatic hernia. J Thorac Cardiovasc Surg31:354–358

    Google Scholar 

  38. Talalak P (1960) Pulmonary sequestration. Arch Dis Child35:57–60

    PubMed  Google Scholar 

  39. Takahashi M, Ohno M, Mihara K, Matsuura K, Sumiyoshi A (1975) Intralobar pulmonary sequestration. Radiol114:543–549

    Google Scholar 

  40. Telander RL, Lennox C, Sieber W (1976) Sequestration of the lung in children. Mayo Clin Proc51:578–584

    PubMed  Google Scholar 

  41. Weisel W, Docksey JW, Glicklich M (1955) Vascular anomalies associated with intrapulmonary bronchial cysts. Am Rev Tuberc71:573–583

    PubMed  Google Scholar 

  42. Werthammer JW, Hatten HP Jr, Blake WB Jr (1980) Upper thoracic extralobar pulmonary sequestration, presenting with respiratory distress in a newborn. Pediatr Radiol9:11–16

    PubMed  Google Scholar 

  43. Wolf G, Hayek HW (1971) Zur Problematik eines Lungensequesters im Säuglingsalter. Radiol Clin Biol40:325–333A0946014 00004 CS-SPJRNPDF [HEADSUP]

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Thilenius, O.G., Ruschhaupt, D.G., Replogle, R.L. et al. Spectrum of pulmonary sequestration: Association with anomalous pulmonary venous drainage in infants. Pediatr Cardiol 4, 97–103 (1983). https://doi.org/10.1007/BF02076333

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02076333

Key words

Navigation