Extralobar sequestration — not obvious radiologic finding. Illustrated overview. Case report

INTRODUCTION: Bronchopulmonary sequestration is a rare congenital pulmonary malformation. A sequestration is generally defined as non-functioning lung tissue that is not in normal continuity with the tracheobronchial tree and derives its blood supply from systemic vessels. 

OBJECTIVE: To define the main clinical and radiologic features of intra- and extralobar sequestration of the lung, to study vessel variability and to analyse case of not obvious CT images of intralobar sequestration that was diagnosed intraoperatively. MATERIALS AND METHODS: We analysed literature found in PubMed and ScienceDirect regarding lung sequestration since 1979. The case report of a patient with extralobar sequestration diagnosed intraoperatively.

RESULTS: The main clinical and radiologic features of lung sequestration, challenges of differential diagnosis were summarised, including specificity of case report.

DISCUSSION: Presence of aberrant vessel requires verification of pathology before operation due to high risk of haemorrhage. The leading role in the verification belongs to radiologic imaging, however in rare cases aberrant vessel may not be visualised due to small diameter or occlusion.

CONCLUSION: The anatomy of aberrant vessels in sequestrations is extremely variable. In case report sequestration was seen in an adult. The pathology was not diagnosed prior to the operation due to failure of the vessel visualisation. Knowledge of possible diagnostic challenges decreases the risk of intraoperative complications.

1. Gottrup F., Lund C. Intralobar pulmonary sequestration. A report of 12 cases // Scand. J. Respir. Dis. 1978. Vol. 59. Р. 21–9. PMID 653324.

2. Corbett H.J., Humphrey G.M.E. Pulmonary sequestration // Paediatr. Respir. Rev. 2004. Vol. 5. Р. 59–68. https://doi.org/10.1016/j.prrv.2003.09.009.

3. Frazier A.A., Rosado de Christenson M.L., Stocker J.T., Templeton P.A. Intralobar sequestration: radiologic-pathologic correlation // Radiographics. 1997. Vol. 17. Р. 725–745. https://doi.org/10.1148/radiographics.17.3.9153708.

4. Miller E.J., Singh S.P., Cerfolio R.J., et al. Pryce’s type I pulmonary intralobar sequestration presenting with massive hemoptysis // Ann. Diagn. Pathol. 2001. Vol. 5, No. 2. Р. 91–95. doi: 10.1053/adpa.2001.23026.

5. Walford N., Htun K., Chen J., et al. Intralobar sequestration of the lung is a congenital anomaly: anatomopathological analysis of four cases diagnosed in fetal life // Pediatr. Dev. Pathol. 2003. Vol. 6, No. 4. Р. 314–321. doi: 10.1007/s10024-001-0194-z.

6. Savic B., Bertel F.J., Tholen W. et al. Lung sequestration: report of seven cases and review of 540 published cases // Thorax. 1979. Vol. 34. Р. 96–101. doi: 10.1136/thx.34.1.96.

7. Pikwer A., Gyllstedt E., Lillo-Gil R. et al. Pulmonary sequestration — a review of 8 cases treated with lobectomy // Scand. J. Surg. 2006. Vol. 95. Р. 190–4. https://doi.org/10.1177/145749690609500312.

8. Nicolette L.A., Kosloske A.M., Bartow S.A., Murphy S. Intralobar pulmonary sequestration: A clinical and pathological spectrum // J. Pediatr. Surg. 1993. Vol. 28. Р. 802–805. doi: 10.1016/0022-3468(93)90331-e.

9. Zangwill В.С., Stocker J.T. Congenital cystic adenomatoid malformation within an extralobar pulmonary sequestration // Pediatr. Pathol. 1993. Vol. 13. Р. 309–315. doi: 10.3109/15513819309048218.

10. Evers W.B., Vissers R., van Noord J.A. Pulmonary sequestration with congenital broncho-oesophageal fistula // Eur. Respir. J. 1990. Vol. 3. Р. 1067–1069. PMID 2289555.

11. Stocker J.T., Kagan-Hallet K. Extralobar pulmonary sequestration: analysis of 15 cases // Am. J. Clin. Pathol. 1979. Vol. 72. Р. 917–925. doi: 10.1093/ajcp/72.6.917.

12. Halkic N., Cuénoud P.F., Corthésy M.E., et al. Pulmonary sequestration: a review of 26 cases // Eur. J. Cardiothorac. Surg. 1998. Vol. 14, No. 2. Р. 127–133. doi: 10.1016/s1010-7940(98)00154-7.

13. Rosado-de-Christenson M.L., Frazier A.A., Stocker J.T., Templeton P.A. From the archives of the AFIP. Extralobar sequestration: radiologic-pathologic correlation // Radiographics. 1993. Vol. 13, No. 2. Р. 425–441. doi: 10.1148/radiographics.13.2.8460228.

14. Abbey P., Das C.J., Pangtey G.S. et al. Imaging in bronchopulmonary sequestration // J. Med. Imaging Radiat. Oncol. 2009. Vol. 53, No. 1, pp. 22–31. doi: 10.1111/j.1754–9485.2009.02033.x.

15. Uppal M.S., Kohman L.J., Katzenstein A.L. Mycetoma within an intralobar sequestration: Evidence supporting acquired origin for this pulmonary anomaly // Chest. 1993. Vol. 103. Р. 1627–1628. doi: 10.1378/chest.103.5.1627.

16. Paksoy N., Demircan A., Altiner M., Artvinh M. Localized fibrous mesothelioma arising in an intralobar pulmonary sequestration // Thorax. 1992. Vol. 47. Р. 837– 838. doi: 10.1136/thx.47.10.837.

17. Polaczek M., Baranska I., Szolkowska M., et al. Clinical presentation and characteristics of 25 adult cases of pulmonary sequestration // J. Thorac. Dis. 2017. Vol. 9, No. 3. Р. 762–767. doi: 10.21037/jtd.2017.03.107.

18. Avishai V., Dolev E., Weissberg D. et al. Extralobar sequestration presenting a massive hemothorax // Chest. 1996. Vol. 109, No. 3. Р. 843–845. doi: 10.1378/chest.109.3.843.

19. Adzick N.S., Harrison M.R., Crombleholme T.M. et al. Fetal lung lesions: management and outcome // Am.J.Obstet. Gynecol. 1998. Vol. 179. Р. 884–889. https://doi.org/10.1016/S0002-9378(98)70183–8.

20. Dolkart L.A., Reimers F.T., Helmuth W.V. el al. Antenatal diagnosis of pulmonary sequestration: A review // Obstet. Gynecol. Surv. 1992. Vol. 47. Р. 515–520. doi: 10.1097/00006254-199208000-00001.

21. Sugio K., Kaneko S., Yokoyama H. et al. Pulmonary sequestration in older children and in adults // Int. Surg. 1992. Vol. 77. Р. 102–107. PMID 1644534.

22. Zylak C.J., Eyler W.R., Spizarny D.L., Stone C.H. Developmental lung anomalies in the adult: radiologic-pathologic correlation // Radiographics. 2002. Vol. 22. Р. 25–43. doi: 10.1148/radiographics.22.suppl_1.g02oc26s25.

23. Ko S.F., Ng S.H., Lee T.Y., et al. Noninvasive imaging of bronchopulmonary sequestration // AJR Am.J. Roentgenol. 2000. Vol. 175. Р. 1005–12. https://doi.org/10.2214/ajr.175.4.1751005.

24. Dhingsa R., Coakley F.V., Albanese C.T. et al. Prenatal sonography and MR imaging of pulmonary sequestration // AJR Am.J.Roentgenol. 2003. Vol. 180. Р. 433–437. https://doi.org/10.2214/ajr.180.2.1800433.