U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Congenital pulmonary airway malformation type 4

MedGen UID:
1777484
•Concept ID:
C5437760
•
Congenital Abnormality
Synonyms: Congenital cystic adenomatoid malformation of the lung type 4; congenital cystic adenomatoid malformation of the lung type 4; congenital cystic adenomatous malformation of the lung type 4; Congenital cystic adenomatous malformation of the lung type 4; congenital pulmonary airway malformation type 4; CPAM type 4
SNOMED CT: Congenital pulmonary airway malformation type 4 (890388006)
 
Monarch Initiative: MONDO:0017252
Orphanet: ORPHA280854

Definition

Congenital pulmonary airway malformation type 4 originates in the acinar structures of the lung. It consists of peripheral thin-walled cysts, typically affecting a single lobe. There is a strong association with type 1 pleuropulmonary blastoma. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital pulmonary airway malformation type 4

Professional guidelines

PubMed

Congenital cystic adenomatoid malformations of the lung: a retrospective study of diagnosis, treatment strategy and postoperative morbidity in surgically treated patients.
Verhalleman Q, Richter J, Proesmans M, Decaluwé H, Debeer A, Van Raemdonck D
Eur J Cardiothorac Surg 2022 Sep 2;62(4) doi: 10.1093/ejcts/ezac464. PMID: 36130242
Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases.
Giubergia V, Barrenechea M, Siminovich M, Pena HG, Murtagh P
J Pediatr (Rio J) 2012 Mar-Apr;88(2):143-8. doi: 10.2223/JPED.2177. PMID: 22543527
Percutaneous thoracic drainage in neonates: catheter drainage versus treatment with aspiration alone.
Margau R, Amaral JG, Chait PG, Cohen J
Radiology 2006 Oct;241(1):223-7. Epub 2006 Aug 23 doi: 10.1148/radiol.2411050966. PMID: 16928976

Recent clinical studies

Etiology

Neonatal congenital pulmonary airway malformation associated with mucinous adenocarcinoma and KRAS mutations.
Muntean A, Banias LE, Ade-Ajayi N, Patel SB, McKinney O, Davenport M
J Pediatr Surg 2022 Nov;57(11):520-526. Epub 2021 Dec 25 doi: 10.1016/j.jpedsurg.2021.12.018. PMID: 34980466
Congenital Cystic Adenomatoid Malformation Volume Ratio in Prenatal Assessment of Prognosis of Fetal Pulmonary Sequestrations.
An P, Wang Y, Feng W, Zhang JQ, Ning YX, Yin JB, Ye HB, Song J, Chen XN, Xu JZ, He QY, Zeng H, Li Y, Yuan W, He ZZ
Curr Med Sci 2019 Aug;39(4):658-662. Epub 2019 Jul 25 doi: 10.1007/s11596-019-2088-1. PMID: 31347005
Congenital pulmonary airway malformations: state-of-the-art review for pediatrician's use.
Leblanc C, Baron M, Desselas E, Phan MH, Rybak A, Thouvenin G, Lauby C, Irtan S
Eur J Pediatr 2017 Dec;176(12):1559-1571. Epub 2017 Oct 19 doi: 10.1007/s00431-017-3032-7. PMID: 29046943
Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features?
Feinberg A, Hall NJ, Williams GM, Schultz KA, Miniati D, Hill DA, Dehner LP, Messinger YH, Langer JC
J Pediatr Surg 2016 Jan;51(1):33-7. Epub 2015 Oct 23 doi: 10.1016/j.jpedsurg.2015.10.019. PMID: 26561249Free PMC Article
Surgical management of bronchopulmonary malformations.
Wagenaar AE, Tashiro J, Hirzel A, Rodriguez LI, Perez EA, Hogan AR, Neville HL, Sola JE
J Surg Res 2015 Oct;198(2):406-12. Epub 2015 Apr 1 doi: 10.1016/j.jss.2015.03.080. PMID: 25918001

Diagnosis

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
Neonatal congenital pulmonary airway malformation associated with mucinous adenocarcinoma and KRAS mutations.
Muntean A, Banias LE, Ade-Ajayi N, Patel SB, McKinney O, Davenport M
J Pediatr Surg 2022 Nov;57(11):520-526. Epub 2021 Dec 25 doi: 10.1016/j.jpedsurg.2021.12.018. PMID: 34980466
Congenital pulmonary airway malformations: state-of-the-art review for pediatrician's use.
Leblanc C, Baron M, Desselas E, Phan MH, Rybak A, Thouvenin G, Lauby C, Irtan S
Eur J Pediatr 2017 Dec;176(12):1559-1571. Epub 2017 Oct 19 doi: 10.1007/s00431-017-3032-7. PMID: 29046943
Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features?
Feinberg A, Hall NJ, Williams GM, Schultz KA, Miniati D, Hill DA, Dehner LP, Messinger YH, Langer JC
J Pediatr Surg 2016 Jan;51(1):33-7. Epub 2015 Oct 23 doi: 10.1016/j.jpedsurg.2015.10.019. PMID: 26561249Free PMC Article
Surgical management of bronchopulmonary malformations.
Wagenaar AE, Tashiro J, Hirzel A, Rodriguez LI, Perez EA, Hogan AR, Neville HL, Sola JE
J Surg Res 2015 Oct;198(2):406-12. Epub 2015 Apr 1 doi: 10.1016/j.jss.2015.03.080. PMID: 25918001

Therapy

Early vs late resection of asymptomatic congenital lung malformations.
Style CC, Cass DL, Verla MA, Cruz SM, Lau PE, Lee TC, Fernandes CJ, Keswani SG, Olutoye OO
J Pediatr Surg 2019 Jan;54(1):70-74. Epub 2018 Oct 5 doi: 10.1016/j.jpedsurg.2018.10.035. PMID: 30366720
Outcome of infants operated on for congenital pulmonary malformations.
Calzolari F, Braguglia A, Valfrè L, Dotta A, Bagolan P, Morini F
Pediatr Pulmonol 2016 Dec;51(12):1367-1372. Epub 2016 May 27 doi: 10.1002/ppul.23472. PMID: 27232731
Treatment of congenital pulmonary airway malformation induced hydrops fetalis via percutaneous sclerotherapy.
Lee FL, Said N, Grikscheit TC, Shin CE, Llanes A, Chmait RH
Fetal Diagn Ther 2012;31(4):264-8. Epub 2012 Feb 18 doi: 10.1159/000336226. PMID: 22354268
Congenital pulmonary airway malformation: CT-pathologic correlation.
Shimohira M, Hara M, Kitase M, Takeuchi M, Shibamoto Y, Kurono K, Shimizu S
J Thorac Imaging 2007 May;22(2):149-53. doi: 10.1097/01.rti.0000213586.06602.d3. PMID: 17527118
Successful treatment of juvenile laryngeal papillomatosis-related multicystic lung disease with cidofovir: case report and review of the literature.
Dancey DR, Chamberlain DW, Krajden M, Palefsky J, Alberti PW, Downey GP
Chest 2000 Oct;118(4):1210-4. doi: 10.1378/chest.118.4.1210. PMID: 11035700

Prognosis

Mucinous adenocarcinoma arising in congenital pulmonary airway malformation: clinicopathological analysis of 37 cases.
Chang WC, Zhang YZ, Wolf JL, Hermelijn SM, Schnater JM, von der Thüsen JH, Rice A, Lantuejoul S, Mastroianni B, Farver C, Black F, Popat S, Nicholson AG
Histopathology 2021 Feb;78(3):434-444. Epub 2020 Oct 23 doi: 10.1111/his.14239. PMID: 32810914
Congenital Cystic Adenomatoid Malformation Volume Ratio in Prenatal Assessment of Prognosis of Fetal Pulmonary Sequestrations.
An P, Wang Y, Feng W, Zhang JQ, Ning YX, Yin JB, Ye HB, Song J, Chen XN, Xu JZ, He QY, Zeng H, Li Y, Yuan W, He ZZ
Curr Med Sci 2019 Aug;39(4):658-662. Epub 2019 Jul 25 doi: 10.1007/s11596-019-2088-1. PMID: 31347005
An investigation on clinical differences between congenital pulmonary airway malformation and bronchial atresia.
Watanabe T, Ohno M, Tahara K, Tomonaga K, Fuchimoto Y, Fujino A, Hishiki T, Tsukamoto K, Ito Y, Sugibayashi R, Wada S, Sago H, Higuchi M, Kawasaki K, Yoshioka T, Kanamori Y
J Pediatr Surg 2018 Dec;53(12):2390-2393. Epub 2018 Sep 1 doi: 10.1016/j.jpedsurg.2018.08.031. PMID: 30227995
Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features?
Feinberg A, Hall NJ, Williams GM, Schultz KA, Miniati D, Hill DA, Dehner LP, Messinger YH, Langer JC
J Pediatr Surg 2016 Jan;51(1):33-7. Epub 2015 Oct 23 doi: 10.1016/j.jpedsurg.2015.10.019. PMID: 26561249Free PMC Article
Congenital pulmonary airway malformation: CT-pathologic correlation.
Shimohira M, Hara M, Kitase M, Takeuchi M, Shibamoto Y, Kurono K, Shimizu S
J Thorac Imaging 2007 May;22(2):149-53. doi: 10.1097/01.rti.0000213586.06602.d3. PMID: 17527118

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
Value of routine ultrasound examination at 35-37 weeks' gestation in diagnosis of fetal abnormalities.
Ficara A, Syngelaki A, Hammami A, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 Jan;55(1):75-80. Epub 2019 Oct 8 doi: 10.1002/uog.20857. PMID: 31595569
Congenital Cystic Adenomatoid Malformation Volume Ratio in Prenatal Assessment of Prognosis of Fetal Pulmonary Sequestrations.
An P, Wang Y, Feng W, Zhang JQ, Ning YX, Yin JB, Ye HB, Song J, Chen XN, Xu JZ, He QY, Zeng H, Li Y, Yuan W, He ZZ
Curr Med Sci 2019 Aug;39(4):658-662. Epub 2019 Jul 25 doi: 10.1007/s11596-019-2088-1. PMID: 31347005
Cystic and pseudocystic pulmonary malformations in children: Clinico-pathological correlation.
Rodríguez-Velasco A, López-Jara-Zárate EJ, Vargas MH, Ramírez-Figueroa JL, Furuya MEY
Ann Diagn Pathol 2019 Apr;39:78-85. Epub 2019 Feb 16 doi: 10.1016/j.anndiagpath.2019.02.005. PMID: 30798075
Congenital pulmonary airway malformation: CT-pathologic correlation.
Shimohira M, Hara M, Kitase M, Takeuchi M, Shibamoto Y, Kurono K, Shimizu S
J Thorac Imaging 2007 May;22(2):149-53. doi: 10.1097/01.rti.0000213586.06602.d3. PMID: 17527118

Recent systematic reviews

Association between Congenital Lung Malformations and Lung Tumors in Children and Adults: A Systematic Review.
Casagrande A, Pederiva F
J Thorac Oncol 2016 Nov;11(11):1837-1845. Epub 2016 Jul 15 doi: 10.1016/j.jtho.2016.06.023. PMID: 27423390

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...