European Radiology

, Volume 27, Issue 6, pp 2489–2496 | Cite as

Extralobar pulmonary sequestration in neonates: The natural course and predictive factors associated with spontaneous regression

  • Hee Mang Yoon
  • Ellen Ai-Rhan Kim
  • Sung-Hoon Chung
  • Seon-Ok Kim
  • Ah Young Jung
  • Young Ah Cho
  • Chong Hyun Yoon
  • Jin Seong LeeEmail author



To describe the natural course of extralobar pulmonary sequestration (EPS) and identify factors associated with spontaneous regression of EPS.

Materials and methods

We retrospectively searched for patients diagnosed with EPS on initial contrast CT scan within 1 month after birth and had a follow-up CT scan without treatment. Spontaneous regression of EPS was assessed by percentage decrease in volume (PDV) and percentage decrease in sum of the diameter of systemic feeding arteries (PDD) by comparing initial and follow-up CT scans. Clinical and CT features were analysed to determine factors associated with PDV and PDD rates.


Fifty-one neonates were included. The cumulative proportions of patients reaching PDV > 50 % and PDD > 50 % were 93.0 % and 73.3 % at 4 years, respectively. Tissue attenuation was significantly associated with PDV rate (B = −21.78, P < .001). The tissue attenuation (B = −22.62, P = .001) and diameter of the largest systemic feeding arteries (B = −48.31, P = .011) were significant factors associated with PDD rate.


The volume and diameter of systemic feeding arteries of EPS spontaneously decreased within 4 years without treatment. EPSs showing a low tissue attenuation and small diameter of the largest systemic feeding arteries on initial contrast-enhanced CT scans were likely to regress spontaneously.

Key Points

Extralobar pulmonary sequestration (EPS) could show spontaneous regression.

Initial CT features may predict spontaneous regression of EPS.

The tissue attenuation and diameter of systemic feeding artery are important factors.


Extralobar pulmonary sequestration Natural course Spontaneous regression Neonate Computed tomography 



Confidence interval


Computed tomography


Extralobar pulmonary sequestration


Percentage decrease in the sum of the diameters of all of the systemic feeding arteries


Percentage decrease in volume


Region of interest


Standard deviation


Systemic feeding arteries



The scientific guarantor of this publication is Jin Seong Lee. The authors of this manuscript declare no relationships with any companies whose products or services may be related to the subject matter of the article. The authors state that this work has not received any funding. One of the authors (Seon Ok Kim) has significant statistical expertise. Institutional Review Board approval was obtained. Written informed consent was waived by the Institutional Review Board. Methodology: retrospective, observational, performed at one institution.

Supplementary material

330_2016_4594_MOESM1_ESM.docx (24 kb)
Table A1 (DOCX 24 kb)
330_2016_4594_MOESM2_ESM.docx (25 kb)
Table A2 (DOCX 25 kb)
330_2016_4594_MOESM3_ESM.docx (24 kb)
Table A3 (DOCX 24 kb)


  1. 1.
    Landing BH, Dixon LG (1979) Congenital malformations and genetic disorders of the respiratory tract (larynx, trachea, bronchi, and lungs). Am Rev Respir Dis 120:151–185PubMedGoogle Scholar
  2. 2.
    Corbett HJ, Humphrey GM (2004) Pulmonary sequestration. Paediatr Respir Rev 5:59–68CrossRefPubMedGoogle Scholar
  3. 3.
    Samuel M, Burge DM (1999) Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation. Thorax 54:701–706CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Van Raemdonck D, De Boeck K, Devlieger H et al (2001) Pulmonary sequestration: a comparison between pediatric and adult patients. Eur J Cardiothorac Surg 19:388–395CrossRefPubMedGoogle Scholar
  5. 5.
    Hekelaar N, van Uffelen R, van Vliet AC, Varin OC, Westenend PJ (2000) Primary lymphoepithelioma-like carcinoma within an intralobular pulmonary sequestration. Eur Respir J 16:1025–1027PubMedGoogle Scholar
  6. 6.
    Cho MJ, Kim DY, Kim SC, Kim KS, Kim EA, Lee BS (2012) Embolization versus surgical resection of pulmonary sequestration: clinical experiences with a thoracoscopic approach. J Pediatr Surg 47:2228–2233CrossRefPubMedGoogle Scholar
  7. 7.
    Gezer S, Tastepe I, Sirmali M et al (2007) Pulmonary sequestration: a single-institutional series composed of 27 cases. J Thorac Cardiovasc Surg 133:955–959CrossRefPubMedGoogle Scholar
  8. 8.
    Lee BS, Kim JT, Kim EA et al (2008) Neonatal pulmonary sequestration: clinical experience with transumbilical arterial embolization. Pediatr Pulmonol 43:404–413CrossRefPubMedGoogle Scholar
  9. 9.
    Lee KH, Sung KB, Yoon HK et al (2003) Transcatheter arterial embolization of pulmonary sequestration in neonates: long-term follow-up results. J Vasc Interv Radiol 14:363–367CrossRefPubMedGoogle Scholar
  10. 10.
    King SJ, Pilling DW, Walkinshaw S (1995) Fetal echogenic lung lesions: prenatal ultrasound diagnosis and outcome. Pediatr Radiol 25:208–210CrossRefPubMedGoogle Scholar
  11. 11.
    Curtis MR, Mooney DP, Vaccaro TJ et al (1997) Prenatal ultrasound characterization of the suprarenal mass: distinction between neuroblastoma and subdiaphragmatic extralobar pulmonary sequestration. J Ultrasound Med 16:75–83CrossRefPubMedGoogle Scholar
  12. 12.
    Garcia-Pena P, Lucaya J, Hendry GM, McAndrew PT, Duran C (1998) Spontaneous involution of pulmonary sequestration in children: a report of two cases and review of the literature. Pediatr Radiol 28:266–270CrossRefPubMedGoogle Scholar
  13. 13.
    Laberge JM, Puligandla P, Flageole H (2005) Asymptomatic congenital lung malformations. Semin Pediatr Surg 14:16–33CrossRefPubMedGoogle Scholar
  14. 14.
    Laberge JM, Bratu I, Flageole H (2004) The management of asymptomatic congenital lung malformations. Paediatr Respir Rev 5:S305-312Google Scholar
  15. 15.
    Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ (1998) Fetal lung lesions: management and outcome. Am J Obstet Gynecol 179:884–889CrossRefPubMedGoogle Scholar
  16. 16.
    Chowdhury M, Samuel M, Ramsay A, Constantinou J, McHugh K, Pierro A (2004) Spontaneous postnatal involution of intraabdominal pulmonary sequestration. J Pediatr Surg 39:1273–1275CrossRefPubMedGoogle Scholar
  17. 17.
    Sintzoff SA Jr, Avni EF, Rocmans P, Pardou A, Struyven J (1991) Pulmonary sequestration-like anomaly presenting as a spontaneously resolving mass. Pediatr Radiol 21:143–144CrossRefPubMedGoogle Scholar
  18. 18.
    Daneman A, Baunin C, Lobo E et al (1997) Disappearing suprarenal masses in fetuses and infants. Pediatr Radiol 27:675–681CrossRefPubMedGoogle Scholar
  19. 19.
    Lin CH, Chuang CY, Hsia JY et al (2013) Pulmonary sequestration-differences in diagnosis and treatment in a single institution. J Chin Med Assoc 76:385–389CrossRefPubMedGoogle Scholar
  20. 20.
    Rosado-de-Christenson ML, Frazier AA, Stocker JT, Templeton PA (1993) From the archives of the AFIP. Extralobar sequestration: radiologic-pathologic correlation. Radiographics 13:425–441CrossRefPubMedGoogle Scholar
  21. 21.
    Lager DJ, Kuper KA, Haake GK (1991) Subdiaphragmatic extralobar pulmonary sequestration. Arch Pathol Lab Med 115:536–538PubMedGoogle Scholar
  22. 22.
    Haddon MJ, Bowen A (1991) Bronchopulmonary and neurenteric forms of foregut anomalies. Imaging for diagnosis and management. Radiol Clin North Am 29:241–254PubMedGoogle Scholar
  23. 23.
    Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R (1979) Lung sequestration: report of seven cases and review of 540 published cases. Thorax 34:96–101CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Chatelain S, Comp RA, Grace RR, Sabbath AM (2015) Cardiomyopathy induced by pulmonary sequestration in a 50-year-old man. Tex Heart Inst J 42:63–65CrossRefPubMedPubMedCentralGoogle Scholar
  25. 25.
    Kimbrell B, Degner T, Glatleider P, Applebaum H (1998) Pulmonary sequestration presenting as mitral valve insufficiency. J Pediatr Surg 33:1648–1650CrossRefPubMedGoogle Scholar
  26. 26.
    Munson I, Young O, Huebsch I (2009) Fundamentals of fluid mechanics. John Wiley &Sons, Inc, New JerseyGoogle Scholar
  27. 27.
    Shanti CM, Klein MD (2008) Cystic lung disease. Semin Pediatr Surg 17:2–8CrossRefPubMedGoogle Scholar
  28. 28.
    Avishai V, Dolev E, Weissberg D, Zajdel L, Priel IE (1996) Extralobar sequestration presenting as massive hemothorax. Chest 109:843–845CrossRefPubMedGoogle Scholar
  29. 29.
    Choe J, Goo HW (2015) Extralobar pulmonary sequestration with hemorrhagic infarction in a child: preoperative imaging diagnosis and pathological correlation. Korean J Radiol 16:662–667CrossRefPubMedPubMedCentralGoogle Scholar
  30. 30.
    Chen W, Wagner L, Boyd T, Nagarajan R, Dasgupta R (2011) Extralobar pulmonary sequestration presenting with torsion: a case report and review of literature. J Pediatr Surg 46:2025–2028CrossRefPubMedGoogle Scholar
  31. 31.
    Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP (1997) Pleuropulmonary blastoma: a clinicopathologic study of 50 cases. Cancer 80:147–161CrossRefPubMedGoogle Scholar
  32. 32.
    MacGillivray TE, Harrison MR, Goldstein RB, Adzick NS (1993) Disappearing fetal lung lesions. J Pediatr Surg 28:1321–1324, discussion 1324-1325CrossRefPubMedGoogle Scholar
  33. 33.
    Burton AC, Patel DJ (1958) Effect on pulmonary vascular resistance of inflation of the rabbit lungs. J Appl Physiol 12:239–246PubMedGoogle Scholar
  34. 34.
    Duggan M, Kavanagh BP (2005) Pulmonary atelectasis: a pathogenic perioperative entity. Anesthesiology 102:838–854CrossRefPubMedGoogle Scholar
  35. 35.
    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 32:986–990CrossRefPubMedGoogle Scholar
  36. 36.
    Zeidan S, Gorincour G, Potier A et al (2009) Congenital lung malformation: evaluation of prenatal and postnatal radiological findings. Respirology 14:1005–1011CrossRefPubMedGoogle Scholar
  37. 37.
    Zeidan S, Hery G, Lacroix F et al (2009) Intralobar sequestration associated with cystic adenomatoid malformation: diagnostic and thoracoscopic pitfalls. Surg Endosc 23:1750–1753CrossRefPubMedGoogle Scholar
  38. 38.
    Carsin A, Mely L, Chrestian MA et al (2010) Association of three different congenital malformations in a same pulmonary lobe in a 5-year-old girl. Pediatr Pulmonol 45:832–835CrossRefPubMedGoogle Scholar

Copyright information

© European Society of Radiology 2016

Authors and Affiliations

  • Hee Mang Yoon
    • 1
  • Ellen Ai-Rhan Kim
    • 2
  • Sung-Hoon Chung
    • 3
  • Seon-Ok Kim
    • 4
  • Ah Young Jung
    • 1
  • Young Ah Cho
    • 1
  • Chong Hyun Yoon
    • 1
  • Jin Seong Lee
    • 1
    Email author
  1. 1.Department of Radiology and Research Institute of RadiologyAsan Medical Center Children’s Hospital, University of Ulsan College of MedicineSongpa-guKorea
  2. 2.Division of Neonatology, Asan Medical Center Children’s HospitalUniversity of Ulsan College of MedicineSeoulKorea
  3. 3.Department of PediatricsKyung Hee University School of MedicineSeoulKorea
  4. 4.Department of Clinical Epidemiology & BiostatisticsAsan Medical CenterSeoulKorea

Personalised recommendations