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. 1987 Jun;42(6):409–416. doi: 10.1136/thx.42.6.409

Congenital bronchopulmonary vascular malformations: clinical application of a simple anatomical approach in 25 cases.

B S Clements 1, J O Warner 1, E A Shinebourne 1
PMCID: PMC460771  PMID: 3660298

Abstract

Congenital malformations of the bronchopulmonary airway and related arterial blood supply are a complex group of lesions in which abnormalities of venous drainage and lung parenchyma may coexist. Twenty five cases have been analysed, by a method whereby each anatomical component is separately considered. All 25 patients had abnormalities of the tracheobronchial tree, with no connection to the abnormal segment in nine cases. The aberrant arterial blood supply was single in 16 cases and multiple in nine cases, one patient from the latter group having a mixed pulmonary and systemic arterial supply to a part of the abnormal segment. Seventeen patients had anomalous venous drainage. In nine of these the vein or veins (they were multiple in four cases) drained the major part or the whole of the lung, whereas the aberrant arterial supply was limited to the right lower zone--that is, mismatched anomalous venous drainage. Abnormalities of lung parenchyma included changes within the lesion (for example, cysts, foregut inclusions) and associated abnormalities of surrounding lung (for example, hypoplasia, abnormal lobation). This information, together with the clinical features and haemodynamic data, was found to be essential for decisions on appropriate management. Patients presenting in infancy with haemodynamic disturbance continue to present major management problems (50% mortality), particularly if there are associated congenital heart defects. The role of aberrant systemic artery occlusion or ligation as a first stage procedure is well established in patients with haemodynamic abnormalities. There may be a place for this procedure in selected patients who have no haemodynamic disturbance at presentation.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Alivizatos P., Cheatle T., de Leval M., Stark J. Pulmonary sequestration complicated by anomalies of pulmonary venous return. J Pediatr Surg. 1985 Feb;20(1):76–79. doi: 10.1016/s0022-3468(85)80398-5. [DOI] [PubMed] [Google Scholar]
  2. Briccoli A., Mastrorilli M., Spangaro M., Vecchi R., Mittiga R., Canini R., De Florio L., Grigioni W., Saviano M. S. La sequestrazione polmonare. Contributo casistico. Minerva Chir. 1982 Mar 31;37(6):523–540. [PubMed] [Google Scholar]
  3. Clements B. S., Warner J. O. The crossover lung segment: congenital malformation associated with a variant of scimitar syndrome. Thorax. 1987 Jun;42(6):417–419. doi: 10.1136/thx.42.6.417. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Derksen O. S. Scimitar syndrome and pulmonary sequestration. Pulmonary anomalies with persistent embryonic communications between the pulmonary and the systemic circulation. Radiol Clin (Basel) 1977;46(2):81–93. [PubMed] [Google Scholar]
  5. Dickinson D. F., Galloway R. W., Massey R., Sankey R., Arnold R. Scimitar syndrome in infancy. Role of embolisation of systemic arterial supply to right lung. Br Heart J. 1982 May;47(5):468–472. doi: 10.1136/hrt.47.5.468. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Flisak M. E., Chandrasekar A. J., Marsan R. E., Ali M. M. Systemic arterialization of lung without sequestration. AJR Am J Roentgenol. 1982 Apr;138(4):751–753. doi: 10.2214/ajr.138.4.751. [DOI] [PubMed] [Google Scholar]
  7. Flye M. W., Conley M., Silver D. Spectrum of pulmonary sequestration. Ann Thorac Surg. 1976 Nov;22(5):478–482. doi: 10.1016/s0003-4975(10)64457-8. [DOI] [PubMed] [Google Scholar]
  8. Groot H. Proceedings: Lung sequestration. Radiol Clin (Basel) 1976;45(1):49–50. [PubMed] [Google Scholar]
  9. Haworth S. G., Sauer U., Bühlmeyer K. Pulmonary hypertension in scimitar syndrome in infancy. Br Heart J. 1983 Aug;50(2):182–189. doi: 10.1136/hrt.50.2.182. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Honey M. Anomalous pulmonary venous drainage of right lung to inferior vena cava ("scimitar syndrome"): clinical spectrum in older patients and role of surgery. Q J Med. 1977 Oct;46(184):463–483. [PubMed] [Google Scholar]
  11. Jue K. L., Amplatz K., Adams P., Jr, Anderson R. C. Anomalies of great vessels associated with lung hypoplasia. The scimitar syndrome. Am J Dis Child. 1966 Jan;111(1):35–44. doi: 10.1001/archpedi.1966.02090040071006. [DOI] [PubMed] [Google Scholar]
  12. KERGIN F. G. Congenital cystic disease of lung associated with anomalous arteries. J Thorac Surg. 1952 Jan;23(1):55–65. [PubMed] [Google Scholar]
  13. Lau Y. L., Radhi J. M., Blesovsky A., Hunter A. S. A case of middle lobe pulmonary sequestration. Thorax. 1986 Oct;41(10):810–811. doi: 10.1136/thx.41.10.810. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Levine M. M., Nudel D. B., Gootman N., Wolpowitz A., Wisoff B. G. Pulmonary sequestration causing congestive heart failure in infancy: a report of two cases and review of the literature. Ann Thorac Surg. 1982 Nov;34(5):581–585. doi: 10.1016/s0003-4975(10)63007-x. [DOI] [PubMed] [Google Scholar]
  15. Litwin S. B., Plauth W. H., Jr, Nadas A. S. Anomalous systemic arterial supply to the lung causing pulmonary-artery hypertension. N Engl J Med. 1970 Nov 12;283(20):1098–1099. doi: 10.1056/NEJM197011122832009. [DOI] [PubMed] [Google Scholar]
  16. Macpherson R. I., Whytehead L. Pseudosequestration. J Can Assoc Radiol. 1977 Mar;28(1):17–25. [PubMed] [Google Scholar]
  17. Masuda H., Ogata T., Tanaka S., Kikuchi K., Yoshizu H., Takagi K. [Blood flow in the abnormal artery of an intralobar sequestration]. Nihon Kyobu Geka Gakkai Zasshi. 1984 Dec;32(12):2119–2122. [PubMed] [Google Scholar]
  18. Mathey J., Galey J. J., Logeais Y., Santoro E., Vanetti A., Maurel A., Wuerflein R. Anomalous pulmonary venous return into inferio vena cava and associated bronchovascular anomalies (the scimitar syndrome). Thorax. 1968 Jul;23(4):398–407. doi: 10.1136/thx.23.4.398. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Moritz E., Keiler A., Schlick W. Intralobäre Lungensequester. Zentralbl Chir. 1982;107(16):1022–1027. [PubMed] [Google Scholar]
  20. Mortensson W., Lundström N. R. Broncho-pulmonary vascular malformation syndrome causing left heart failure during infancy. Acta Radiol Diagn (Stockh) 1971 Sep;11(5):449–458. doi: 10.1177/028418517101100501. [DOI] [PubMed] [Google Scholar]
  21. Mäkinen E. O., Merikanto J., Rikalainen H., Satokari K. Intralobar pulmonary sequestration occurring without alteration of pulmonary parenchyma. Pediatr Radiol. 1981;10(4):237–240. doi: 10.1007/BF01001590. [DOI] [PubMed] [Google Scholar]
  22. NEILL C. A., FERENCZ C., SABISTON D. C., SHELDON H. The familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage "scimitar syndrome". Bull Johns Hopkins Hosp. 1960 Jul;107:1–21. [PubMed] [Google Scholar]
  23. O'Mara C. S., Baker R. R., Jeyasingham K. Pulmonary sequestration. Surg Gynecol Obstet. 1978 Oct;147(4):609–616. [PubMed] [Google Scholar]
  24. Ransom J. M., Norton J. B., Williams G. D. Pulmonary sequestration presenting as congestive heart failure. J Thorac Cardiovasc Surg. 1978 Sep;76(3):378–380. [PubMed] [Google Scholar]
  25. SMITH R. A. A theory of the origin of intralobar sequestration of lung. Thorax. 1956 Mar;11(1):10–24. doi: 10.1136/thx.11.1.10. [DOI] [PMC free article] [PubMed] [Google Scholar]
  26. SOLIT R. W., FRAIMOW W., WALLACE S., COHN H. E. THE EFFECT OF INTRALOBAR PULMONARY SEQUESTRATION ON CARDIAC OUTPUT. J Thorac Cardiovasc Surg. 1965 May;49:844–853. [PubMed] [Google Scholar]
  27. Sade R. M., Clouse M., Ellis F. H., Jr The spectrum of pulmonary sequestration. Ann Thorac Surg. 1974 Dec;18(6):644–658. doi: 10.1016/s0003-4975(10)64417-7. [DOI] [PubMed] [Google Scholar]
  28. TURK L. N., 3rd, LINDSKOG G. E. The importance of angiographic diagnosis in intralobar pulmonary sequestration. J Thorac Cardiovasc Surg. 1961 Mar;41:299–305. [PubMed] [Google Scholar]
  29. Takahashi M., Ohno M., Milhara K., Matsuura K., Sumiyoshi A. Intralobar pulmonary sequestration; with special emphasis on bronchial communication. Radiology. 1975 Mar;114(3):543–549. doi: 10.1148/114.3.543. [DOI] [PubMed] [Google Scholar]
  30. Telander R. L., Lennox C., Sieber W. Sequestration of the lung in children. Mayo Clin Proc. 1976 Sep;51(9):578–584. [PubMed] [Google Scholar]
  31. Thilenius O. G., Ruschhaupt D. G., Replogle R. L., Bharati S., Herman T., Arcilla R. A. Spectrum of pulmonary sequestration: association with anomalous pulmonary venous drainage in infants. Pediatr Cardiol. 1983 Apr-Jun;4(2):97–103. doi: 10.1007/BF02076333. [DOI] [PubMed] [Google Scholar]
  32. Woody J. N., Graham T. P., Jr, Bradford W. D., Sabiston D. C., Jr, Canent R. V., Jr Hypoplastic right lung with systemic blood supply and anomalous pulmonary venous drainage: reversal of pulmonary hypertension with surgical management in infancy. Am Heart J. 1972 Jan;83(1):82–88. doi: 10.1016/0002-8703(72)90109-3. [DOI] [PubMed] [Google Scholar]
  33. Yonehiro E. G., Hallman G. L., Cooley D. A. Anomalous pulmonary venous return from a hypoplastic right lung to the inferior vena cava (scimitar syndrome): report of successful correction and review of surgical treatment. Cardiovasc Res Cent Bull. 1966 Apr-Jun;4(4):106–117. [PubMed] [Google Scholar]

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