Skip to main content
SpringerLink
Log in

Presentation and management of pulmonary sequestration with total visceral inflow and outflow

  • Case Report
  • Published:
Pediatric Surgery International Aims and scope Submit manuscript

Abstract

Two of the most common types of congenital thoracic malformations are congenital pulmonary airway malformations (CPAMs) and bronchopulmonary sequestrations (BPS). Here we present the first known case of a hybrid lesion, with coexisting features of an extralobar sequestration (ELS) and CPAM type 2, with arterial inflow from the celiac trunk and venous outflow to the portal vein. The clinical presentation, investigative imaging and timing of surgery are discussed.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

References

  1. Bush A (2001) Congenital lung disease: a plea for clear thinking and clear nomenclature. Pediatr Pulmonol 32(4):328–337

    Article  CAS  PubMed  Google Scholar 

  2. Eber E (2007) Antenatal diagnosis of congenital thoracic malformations: early surgery, late surgery, or no surgery? Semin Respir Crit Care Med 28(3):355–366

    Article  PubMed  Google Scholar 

  3. Conran RM, Stocker JT (1999) Extralobar sequestration with frequently associated congenital cystic adenomatoid malformation, type 2: report of 50 cases. Pediatr Dev Pathol 2(5):454–463

    Article  CAS  PubMed  Google Scholar 

  4. Cass DL, Crombleholme TM, Howell LJ, Stafford PW, Ruchelli ED, Adzick NS (1997) Cystic lung lesions with systemic arterial blood supply: a hybrid of congenital cystic adenomatoid malformation and bronchopulmonary sequestration. J Pediatr Surg 32(7):986–990

    Article  CAS  PubMed  Google Scholar 

  5. Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ (1998) Fetal lung lesions: management and outcome. Am J Obstet Gynecol 179(4):884–889

    Article  CAS  PubMed  Google Scholar 

  6. Stocker JT, Madewell J, Drake R (1977) Congenital cystic adenomatoid malformation of the lung: classification and morphologic spectrum. J Hum Pathol 8:155–171

    Article  CAS  Google Scholar 

  7. Priest JR, Williams GM, Hill DA, Dehner LP, Jaffé A (2009) Pulmonary cysts in early childhood and the risk of malignancy. Pediatr Pulmonol 44(1):14–30

    Article  PubMed  Google Scholar 

  8. Stocker JT (2002) Congenital pulmonary airway malformation: a new name for and an expanded classification of congenital cystic adenomatoid malformation of the lung. Histopathology 41:424–431

    Article  Google Scholar 

  9. Pryce DM (1946) Lower accessory pulmonary artery with intralobar sequestration of lung; a report of seven cases. J Pathol Bacteriol. 58(3):457–467

    Article  CAS  PubMed  Google Scholar 

  10. Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R (1979) Lung sequestration: report of seven cases and review of 540 published cases. Thorax 34(1):96–101

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Corbett HJ, Humphrey GME (2004) Pulmonary sequestration. Paediatr Respir Rev 5(1):59–68

    Article  PubMed  Google Scholar 

  12. Choplin R, Sigel M (1980) Six unusual sequestration: presentations. Am J Roentgenol 134:695–700

    Article  CAS  Google Scholar 

  13. Hofman FN, Pasker HG, Speekenbrink RGH (2005) Hemoptysis and massive hemothorax as presentation of intralobar sequestration. Ann Thorac Surg 80(6):2343–2344

    Article  PubMed  Google Scholar 

  14. Rubin EM, Garcia H, Horowitz MD, Guerra JJ (1994) Fatal massive hemoptysis secondary to intralobar sequestration. Chest 106(3):954–955

    Article  CAS  PubMed  Google Scholar 

  15. Ko SF, Ng SH, Lee TY, Wan YL, Liang CD, Lin JW, Chen WJ, Hsieh MJ (2000) Noninvasive imaging of bronchopulmonary sequestration. Am J Roentgenol 175(4):1005–1012

    Article  CAS  Google Scholar 

  16. Bratu I, Flageole H, Chen MF, Di Lorenzo M, Yazbeck S, Laberge JM (2001) The multiple facets of pulmonary sequestration. J Pediatr Surg 36(5):784–790

    Article  CAS  PubMed  Google Scholar 

  17. Srikanth MS, Ford EG, Stanley P, Mahour GH (1992) Communicating bronchopulmonary foregut malformations: classification and embryogenesis. J Pediatr Surg 27(6):732–736

    Article  CAS  PubMed  Google Scholar 

  18. Aziz D, Langer JC, Tuuha SE, Ryan G, Ein SH, Kim PCW, Adzick NS, Bagolan P, Lund D, Flageole H, Jona J (2004) Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not? J Pediatr Surg 39(3):329–334

    Article  PubMed  Google Scholar 

  19. Papagiannopoulos K, Hughes S, Nicholson AG, Goldstraw P (2002) Cystic lung lesions in the pediatric and adult population: surgical experience at the Brompton hospital. Ann Thorac Surg 73(5):1594–1598

    Article  PubMed  Google Scholar 

  20. John P, Beasley S, Mayne V (1989) Pulmonary sequestration and related congenital disorders. Pediatr Radiol 20:4–9

    Article  CAS  PubMed  Google Scholar 

  21. Wei Y, Li F (2011) Pulmonary sequestration: a retrospective analysis of 2625 cases in China. Eur J Cardio Thorac Surg. 40(1):40–43

    Article  CAS  Google Scholar 

  22. Theodoropoulos I, Schwartz MZ (2012) Intralobar pulmonary sequestration: an uncommon case with triple arterial supply and systemic venous drainage. Pediatr Surg Int 28(7):741–744

    Article  PubMed  Google Scholar 

  23. Ren J-Z, Zhang K, Huang G-H, Zhang M-F, Zhou P-L, Han X-W, Duan X-H, Li Z (2014) Assessment of 64-row computed tomographic angiography for diagnosis and pretreatment planning in pulmonary sequestration. Radiol Med 119(1):27–32

    Article  PubMed  Google Scholar 

  24. McLean SE, Pfeifer JD, Siegel MJ, Jensen ER, Schuler PM, Hirsch R, Mychaliska GB (2004) Congenital cystic adenomatoid malformation connected to an extralobar pulmonary sequestration in the contralateral chest: common origin? J Pediatr Surg 39(8):13–17

    Article  Google Scholar 

  25. Khosa JK, Leong SL, Borzi PA (2004) Congenital cystic adenomatoid malformation of the lung: indications and timing of surgery. Pediatr Surg Int. 20(7):505–508

    Article  CAS  PubMed  Google Scholar 

  26. Laberge J-M, Bratu I, Flageole H (2004) The management of asymptomatic congenital lung malformations. Paediatr Respir Rev 5(Suppl A):S305–S312

    Article  PubMed  Google Scholar 

  27. Eber E (2006) Adult outcome of congenital lower respiratory tract malformations. Swiss Med Wkly 136(15–16):233–240

    PubMed  Google Scholar 

  28. Jariwala P, Ramesh G, Sarat Chandra K (2013) Congenital anomalous/aberrant systemic artery to pulmonary venous fistula: closure with vascular plugs and coil embolization. Indian Heart J 66(1):95–103

    Article  Google Scholar 

  29. Abdelrahman H, Ajaj A, Atique S, El-Menyar A, Al-Thani H (2013) Conservative management of major liver necrosis after angioembolization a patient with blunt trauma. Case Rep Surg. doi:10.1155/2013/954050

    PubMed  PubMed Central  Google Scholar 

  30. Kamata S, Usui N, Kamiyama M, Nose K, Sawai T, Fukuzawa M (2006) Long-term outcome in patients with prenatally diagnosed cystic lung disease: special reference to ventilation and perfusion scan in the affected lung. J Pediatr Surg 41(12):2023–2027

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

The authors would like to acknowledge the financial support provided by the Emory/Children’s Pediatric Research Trust and the Department of Surgery at Emory University.

Author information

Authors and Affiliations

  1. Division of Pediatric Surgery, Department of Surgery, Emory University School of Medicine, Children’s Healthcare of Atlanta, 1405 Clifton Road NE, 3rd Floor Surgical Suite, Atlanta, GA, 30322, USA

    Christopher L. Sudduth, Sarah J. Hill & Mehul V. Raval

Authors
  1. Christopher L. Sudduth
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sarah J. Hill
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Mehul V. Raval
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mehul V. Raval.

Ethics declarations

Conflict of interest

The authors do not have any conflicts of interest to report.

Informed consent

Informed consent was obtained from all individual participants included in the case report.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Sudduth, C.L., Hill, S.J. & Raval, M.V. Presentation and management of pulmonary sequestration with total visceral inflow and outflow. Pediatr Surg Int 32, 709–712 (2016). https://doi.org/10.1007/s00383-016-3882-5

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00383-016-3882-5

Keywords

Search

Navigation