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Bronchopulmonary sequestration

MedGen UID:
669
•Concept ID:
C0006288
•
Congenital Abnormality
Synonyms: Bronchopulmonary Sequestration; Bronchopulmonary Sequestrations; Pulmonary Sequestration; Pulmonary Sequestrations; Sequestration, Bronchopulmonary; Sequestration, Pulmonary; Sequestrations, Bronchopulmonary; Sequestrations, Pulmonary
SNOMED CT: Sequestration of lung (18620009); Pulmonary sequestration (18620009); Congenital sequestration of lung (18620009)

Definition

A rare congenital abnormality of the lungs. It consists of a mass of lung parenchyma that does not communicate with the bronchial tree and receives its blood supply from the systemic circulation instead of the pulmonary circulation. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBronchopulmonary sequestration

Professional guidelines

PubMed

A clinical guideline for structured assessment of CT-imaging in congenital lung abnormalities.
Hermelijn SM, Elders BBLJ, Ciet P, Wijnen RMH, Tiddens HAWM, Schnater JM
Paediatr Respir Rev 2021 Mar;37:80-88. Epub 2020 Feb 20 doi: 10.1016/j.prrv.2019.12.004. PMID: 32178987
Prenatal diagnosis of congenital lobar fluid overload.
Tsai PS, Chen CP, Lin DC, Liu YP
Taiwan J Obstet Gynecol 2017 Aug;56(4):425-431. doi: 10.1016/j.tjog.2017.05.001. PMID: 28805595
Management of perinatal lung malformations.
Macardle CA, Kunisaki SM
Minerva Ginecol 2015 Feb;67(1):81-94. Epub 2014 Oct 13 PMID: 25310108

Recent clinical studies

Etiology

Congenital lung malformations.
Pederiva F, Rothenberg SS, Hall N, Ijsselstijn H, Wong KKY, von der Thüsen J, Ciet P, Achiron R, Pio d'Adamo A, Schnater JM
Nat Rev Dis Primers 2023 Nov 2;9(1):60. doi: 10.1038/s41572-023-00470-1. PMID: 37919294
Fetal bronchopulmonary malformations.
Nunes C, Pereira I, Araújo C, Santo SF, Carvalho RM, Melo A, Graça LM
J Matern Fetal Neonatal Med 2015 Nov;28(16):1996-2000. Epub 2014 Dec 4 doi: 10.3109/14767058.2014.984603. PMID: 25394612
Congenital lung lesions.
Puligandla PS, Laberge JM
Clin Perinatol 2012 Jun;39(2):331-47. doi: 10.1016/j.clp.2012.04.009. PMID: 22682383
Congenital lung malformations.
Andrade CF, Ferreira HP, Fischer GB
J Bras Pneumol 2011 Mar-Apr;37(2):259-71. doi: 10.1590/s1806-37132011000200017. PMID: 21537663
Pulmonary sequestration.
Corbett HJ, Humphrey GM
Paediatr Respir Rev 2004 Mar;5(1):59-68. doi: 10.1016/j.prrv.2003.09.009. PMID: 15222956

Diagnosis

Fetal bronchopulmonary malformations.
Nunes C, Pereira I, Araújo C, Santo SF, Carvalho RM, Melo A, Graça LM
J Matern Fetal Neonatal Med 2015 Nov;28(16):1996-2000. Epub 2014 Dec 4 doi: 10.3109/14767058.2014.984603. PMID: 25394612
Imaging in bronchopulmonary sequestration.
Abbey P, Das CJ, Pangtey GS, Seith A, Dutta R, Kumar A
J Med Imaging Radiat Oncol 2009 Feb;53(1):22-31. doi: 10.1111/j.1754-9485.2009.02033.x. PMID: 19453525
Intraabdominal pulmonary sequestration.
Franko J, Bell K, Pezzi CM
Curr Surg 2006 Jan-Feb;63(1):35-8. doi: 10.1016/j.cursur.2005.04.004. PMID: 16373158
Pulmonary sequestration.
Corbett HJ, Humphrey GM
Paediatr Respir Rev 2004 Mar;5(1):59-68. doi: 10.1016/j.prrv.2003.09.009. PMID: 15222956
Pulmonary sequestration.
O'Mara CS, Baker RR, Jeyasingham K
Surg Gynecol Obstet 1978 Oct;147(4):609-16. PMID: 360452

Therapy

Intrauterine Ultrasound-Guided Laser Coagulation as a First Step for Treatment of Prenatally Complicated Bronchopulmonary Sequestration: Our Experience and Literature Review.
Zanini A, Macchini F, Boito S, Morandi A, Ferrara G, Persico N, Leva E
Eur J Pediatr Surg 2022 Dec;32(6):536-542. Epub 2022 Mar 14 doi: 10.1055/s-0042-1744149. PMID: 35288883
Management of prenatally diagnosed lung lesions.
Khalek N, Johnson MP
Semin Pediatr Surg 2013 Feb;22(1):24-9. doi: 10.1053/j.sempedsurg.2012.10.005. PMID: 23395142
Congenital lung lesions.
Puligandla PS, Laberge JM
Clin Perinatol 2012 Jun;39(2):331-47. doi: 10.1016/j.clp.2012.04.009. PMID: 22682383
Prenatal interventions for fetal lung lesions.
Witlox RS, Lopriore E, Oepkes D
Prenat Diagn 2011 Jul;31(7):628-36. Epub 2011 May 25 doi: 10.1002/pd.2778. PMID: 21618254
Recent advances in fetal surgery.
Cortes RA, Farmer DL
Semin Perinatol 2004 Jun;28(3):199-211. doi: 10.1053/j.semperi.2004.03.006. PMID: 15283099

Prognosis

Imaging and pathological characteristics, treatment, and prognosis of pulmonary sequestration-A retrospective study of 13 cases.
Liu X, Wu R, Zhu S, Gu L, Tang Z
Clin Respir J 2023 Sep;17(9):865-873. Epub 2023 Aug 2 doi: 10.1111/crj.13672. PMID: 37533295Free PMC Article
Congenital lung lesions.
Zobel M, Gologorsky R, Lee H, Vu L
Semin Pediatr Surg 2019 Aug;28(4):150821. Epub 2019 Jul 18 doi: 10.1053/j.sempedsurg.2019.07.004. PMID: 31451177
Fetal bronchopulmonary malformations.
Nunes C, Pereira I, Araújo C, Santo SF, Carvalho RM, Melo A, Graça LM
J Matern Fetal Neonatal Med 2015 Nov;28(16):1996-2000. Epub 2014 Dec 4 doi: 10.3109/14767058.2014.984603. PMID: 25394612
Fetal pleural effusions.
Yinon Y, Kelly E, Ryan G
Best Pract Res Clin Obstet Gynaecol 2008 Feb;22(1):77-96. doi: 10.1016/j.bpobgyn.2007.09.004. PMID: 18280793
Pulmonary sequestration.
Corbett HJ, Humphrey GM
Paediatr Respir Rev 2004 Mar;5(1):59-68. doi: 10.1016/j.prrv.2003.09.009. PMID: 15222956

Clinical prediction guides

Congenital Pulmonary Airway Malformations With a Reconsideration and Current Perspective on the Stocker Classification.
Dehner LP, Schultz KAP, Hill DA
Pediatr Dev Pathol 2023 May-Jun;26(3):241-249. Epub 2023 Feb 21 doi: 10.1177/10935266221146823. PMID: 37334833
Learning curve for total thoracoscopic segmentectomy in treating pediatric patients with congenital lung malformation.
He T, Sun X, Liu C, Yuan M, Yang G, Cheng K, Dai S, Xu C
Surg Endosc 2023 Jul;37(7):5129-5136. Epub 2023 Mar 20 doi: 10.1007/s00464-023-09987-8. PMID: 36941411
Elevated tumor markers in a benign lung disease.
Chen Q, Lu Q, Fei X, Li C, Li B
J Cardiothorac Surg 2021 Oct 18;16(1):308. doi: 10.1186/s13019-021-01688-4. PMID: 34663400Free PMC Article
Long-term outcomes of congenital lung malformations.
Hall NJ, Stanton MP
Semin Pediatr Surg 2017 Oct;26(5):311-316. Epub 2017 Sep 9 doi: 10.1053/j.sempedsurg.2017.09.001. PMID: 29110827
Asymptomatic congenital lung malformations.
Laberge JM, Puligandla P, Flageole H
Semin Pediatr Surg 2005 Feb;14(1):16-33. doi: 10.1053/j.sempedsurg.2004.10.022. PMID: 15770585

Recent systematic reviews

The Utility of the Congenital Pulmonary Airway Malformation-Volume Ratio in the Assessment of Fetal Echogenic Lung Lesions: A Systematic Review.
Kane SC, Ancona E, Reidy KL, Palma-Dias R
Fetal Diagn Ther 2020;47(3):171-181. Epub 2019 Oct 8 doi: 10.1159/000502841. PMID: 31593968
Systematic review and meta-analysis of the postnatal management of congenital cystic lung lesions.
Stanton M, Njere I, Ade-Ajayi N, Patel S, Davenport M
J Pediatr Surg 2009 May;44(5):1027-33. doi: 10.1016/j.jpedsurg.2008.10.118. PMID: 19433193

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