Pulmonary Sequestration and Related Congenital Bronchopulmonary-vascular Malformations: Nomenclature and Classification Based on Anatomical and Embryological Considerations

Overview

Journal Thorax

Date 1987 Jun 1

PMID 3660297

Citations 47

Authors

B S Clements

J O Warner

Affiliations

Soon will be listed here.

Abstract

The pulmonary sequestration spectrum and related congenital lung anomalies present an extremely complex and varied group of bronchopulmonary-vascular malformations. Previous attempts at nomenclature and classification have proved inadequate. In this article we present a classification of the newly named pulmonary malinosculation spectrum, which includes all congenital lung anomalies where there is abnormal connection (that is, malinosculation) of one or more of the four major components of lung tissue--namely, tracheobronchial airway, lung parenchyma, arterial supply, and venous drainage, which in various combinations make up these lesions. We feel that this simple descriptive approach will improve our understanding and management of these complicated lesions and this is supported by the clinical experience we report in the next article.

Citing Articles

Incidental diagnosis of intra-lobar pulmonary sequestration in an asymptomatic young male: A case report.

Duwadee P, Sharma S, Bam P, Bhusal A, Yogi T, Khatri T Radiol Case Rep. 2025; 20(3):1703-1707.

PMID: 39868065 PMC: 11760790. DOI: 10.1016/j.radcr.2024.12.004.

Coil embolization of anomalous systemic artery to the left lower lobe in an asymptomatic adult: a case report.

Sun M, Le Fang , Wang F, Wang T J Cardiothorac Surg. 2025; 20(1):5.

PMID: 39755663 PMC: 11699713. DOI: 10.1186/s13019-024-03266-w.

Symptomatic right side extralobular pulmonary sequestration in a young woman: A rare case report.

Alemu M, Getachew Z, Wondemagegn Z, Worku A, Beyene H, Tareke D Clin Case Rep. 2024; 12(9):e9393.

PMID: 39210930 PMC: 11358205. DOI: 10.1002/ccr3.9393.

Neonatal pleural effusion associated with pulmonary sequestration: A case report.

Twanabasu S, Chalise S Clin Case Rep. 2024; 12(5):e8956.

PMID: 38765611 PMC: 11099781. DOI: 10.1002/ccr3.8956.

A surgical case of pulmonary adenocarcinoma in the right upper lobe associated with a systemic artery-to-pulmonary artery fistula.

Ishida Y, Yukawa T, Nagasaki Y, Minami D, Fujiwara H, Monobe Y Thorac Cancer. 2023; 14(21):2085-2089.

PMID: 37308179 PMC: 10363778. DOI: 10.1111/1759-7714.14985.

References

Carter R . Pulmonary sequestration. Ann Thorac Surg. 1969; 7(1):68-88. DOI: 10.1016/s0003-4975(10)66147-4. View

Kirks D, Kane P, FREE E, TAYBI H . Systemic arterial supply to normal basilar segments of the left lower lobe. AJR Am J Roentgenol. 1976; 126(4):817-21. DOI: 10.2214/ajr.126.4.817. View

Smith R . A theory of the origin of intralobar sequestration of lung. Thorax. 1956; 11(1):10-24. PMC: 1019399. DOI: 10.1136/thx.11.1.10. View

NEILL C, Ferencz C, SABISTON D, Sheldon H . The familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage "scimitar syndrome". Bull Johns Hopkins Hosp. 1960; 107:1-21. View

NEILL C . Development of the pulmonary veins; with reference to the embryology of anomalies of pulmonary venous return. Pediatrics. 1956; 18(6):880-7. View

DEMOS N, Teresi A . Congenital lung malformations: a unified concept and a case report. J Thorac Cardiovasc Surg. 1975; 70(2):260-4. View

HALLER Jr J, Golladay E, Pickard L, Tepas 3rd J, Shorter N, Shermeta D . Surgical management of lung bud anomalies: lobar emphysema, bronchogenic cyst, cystic adenomatoid malformation, and intralobar pulmonary sequestration. Ann Thorac Surg. 1979; 28(1):33-43. DOI: 10.1016/s0003-4975(10)63389-9. View

Buntain W, Isaacs Jr H, Payne Jr V, LINDESMITH G, ROSENKRANTZ J . Lobar emphysema, cystic adenomaloid malformation, pulmonary sequestration, and bronchogenic cyst in infancy and childhood: A clinical group. J Pediatr Surg. 1974; 9(1):85-93. DOI: 10.1016/0022-3468(74)90013-x. View

Edwards J . Pathologic and developmental considerations in anomalous pulmonary venous connection. Proc Staff Meet Mayo Clin. 1953; 28(17):441-52. View

10.

Folger G . The scimitar syndrome. Anatomic, physiologic, developmental and therapeutic considerations. Angiology. 1976; 27(6):373-407. DOI: 10.1177/000331977602700605. View

11.

Takahashi M, Ohno M, Milhara K, Matsuura K, Sumiyoshi A . Intralobar pulmonary sequestration; with special emphasis on bronchial communication. Radiology. 1975; 114(3):543-9. DOI: 10.1148/114.3.543. View

12.

Sade R, CLOUSE M, ELLIS Jr F . The spectrum of pulmonary sequestration. Ann Thorac Surg. 1974; 18(6):644-58. DOI: 10.1016/s0003-4975(10)64417-7. View

13.

GOLDING M, Kwon K, Chiu C, NICASTRI A . Pulmonary sequestration. A report of an unusual case. J Thorac Cardiovasc Surg. 1967; 54(1):121-5. View

14.

Chiu R, Herba M, Viloria J, Mulder D . Thoracopulmonary hypogenesis with systemic artery-pulmonary vessel fistulae: report of a case. Ann Thorac Surg. 1981; 31(4):360-3. DOI: 10.1016/s0003-4975(10)60968-x. View

15.

Derksen O . 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. View

16.

Alivizatos P, Cheatle T, de Leval M, Stark J . Pulmonary sequestration complicated by anomalies of pulmonary venous return. J Pediatr Surg. 1985; 20(1):76-9. DOI: 10.1016/s0022-3468(85)80398-5. View

17.

BOYDEN E . Bronchogenic cysts and the theory of intralobar sequestration: new embryologic data. J Thorac Surg. 1958; 35(5):604-16. View

18.

Moulaert A . The scimitar syndrome in infancy and childhood. Eur J Cardiol. 1973; 1(1):55-61. View

19.

Phelan P . Does adult chronic obstructive lung disease really begin in childhood?. Br J Dis Chest. 1984; 78(1):1-9. View

20.

THILENIUS O, Ruschhaupt D, Replogle R, Bharati S, Herman T, ARCILLA R . Spectrum of pulmonary sequestration: association with anomalous pulmonary venous drainage in infants. Pediatr Cardiol. 1983; 4(2):97-103. DOI: 10.1007/BF02076333. View