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Congenital pulmonary airway malformation type 1

MedGen UID:
1760964
•Concept ID:
C5437763
•
Congenital Abnormality
Synonyms: CCAM type 1; Congenital cystic adenomatoid malformation of the lung type 1; congenital cystic adenomatoid malformation of the lung type 1; Congenital cystic adenomatous malformation of the lung type 1; congenital cystic adenomatous malformation of the lung type 1; Congenital cystic disease of the lung type 1; congenital cystic disease of the lung type 1; congenital pulmonary airway malformation type 1; CPAM type 1; Type 1 congenital pulmonary airway malformation
SNOMED CT: Congenital pulmonary airway malformation type 1 (890392004)
 
HPO: HP:0025725
Monarch Initiative: MONDO:0017249
Orphanet: ORPHA280832

Definition

A type of congenital pulmonary airway malformation that is characterized by multiple large cysts or a single dominant cyst. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital pulmonary airway malformation type 1

Professional guidelines

PubMed

Multimodality Imaging of Pleuropulmonary Blastoma: Pearls, Pitfalls, and Differential Diagnosis.
Leung SS, Donuru A, Kandula V, Parekh MR, Saul D
Semin Ultrasound CT MR 2022 Feb;43(1):61-72. Epub 2021 May 15 doi: 10.1053/j.sult.2021.05.007. PMID: 35164911
Prenatal course and postnatal management of peripheral bronchial atresia: association with congenital cystic adenomatoid malformation of the lung.
Peranteau WH, Merchant AM, Hedrick HL, Liechty KW, Howell LJ, Flake AW, Wilson RD, Johnson MP, Bebbington MW, Adzick NS
Fetal Diagn Ther 2008;24(3):190-6. Epub 2008 Aug 28 doi: 10.1159/000151337. PMID: 18753756
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

Multimodality Imaging of Pleuropulmonary Blastoma: Pearls, Pitfalls, and Differential Diagnosis.
Leung SS, Donuru A, Kandula V, Parekh MR, Saul D
Semin Ultrasound CT MR 2022 Feb;43(1):61-72. Epub 2021 May 15 doi: 10.1053/j.sult.2021.05.007. PMID: 35164911
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
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
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
Management of congenital cystic adenomatoid malformation and bronchopulmonary sequestration in newborns.
Chen HW, Hsu WM, Lu FL, Chen PC, Jeng SF, Peng SS, Chen CY, Chou HC, Tsao PN, Hsieh WS
Pediatr Neonatol 2010 Jun;51(3):172-7. doi: 10.1016/S1875-9572(10)60032-0. PMID: 20675242

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
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
Evidence to inform decisions about maternal-fetal surgery: technical brief.
Hartmann KE, McPheeters ML, Chescheir NC, Gillam-Krakauer M, McKoy JN, Jerome R, Sathe NA, Meints L, Walsh WF
Obstet Gynecol 2011 May;117(5):1191-1204. doi: 10.1097/AOG.0b013e318216151d. PMID: 21471854
Management of congenital cystic adenomatoid malformation and bronchopulmonary sequestration in newborns.
Chen HW, Hsu WM, Lu FL, Chen PC, Jeng SF, Peng SS, Chen CY, Chou HC, Tsao PN, Hsieh WS
Pediatr Neonatol 2010 Jun;51(3):172-7. doi: 10.1016/S1875-9572(10)60032-0. PMID: 20675242

Therapy

Application and effects of an early childhood education machine on analgesia and sedation in children after cardiothoracic surgery.
Chen LL, Lei YQ, Liu JF, Cao H, Yu XR, Chen Q
J Cardiothorac Surg 2021 May 1;16(1):118. doi: 10.1186/s13019-021-01490-2. PMID: 33933112Free PMC Article
Obstetrician patterns of steroid administration for the prenatal management of congenital pulmonary airway malformations.
Aziz KB, Jelin AC, Keiser AM, Schulkin J, Jelin EB
J Neonatal Perinatal Med 2021;14(2):213-222. doi: 10.3233/NPM-200526. PMID: 33164951Free PMC Article
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
Evidence to inform decisions about maternal-fetal surgery: technical brief.
Hartmann KE, McPheeters ML, Chescheir NC, Gillam-Krakauer M, McKoy JN, Jerome R, Sathe NA, Meints L, Walsh WF
Obstet Gynecol 2011 May;117(5):1191-1204. doi: 10.1097/AOG.0b013e318216151d. PMID: 21471854
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

Prognosis

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
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 cystic adenomatoid malformation of the lung: indications and timing of surgery.
Khosa JK, Leong SL, Borzi PA
Pediatr Surg Int 2004 Jul;20(7):505-8. Epub 2004 Jun 8 doi: 10.1007/s00383-004-1225-4. PMID: 15185110
Prenatal magnetic resonance imaging enhances fetal diagnosis.
Quinn TM, Hubbard AM, Adzick NS
J Pediatr Surg 1998 Apr;33(4):553-8. doi: 10.1016/s0022-3468(98)90315-3. PMID: 9574750
Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum.
Stocker JT, Madewell JE, Drake RM
Hum Pathol 1977 Mar;8(2):155-71. doi: 10.1016/s0046-8177(77)80078-6. PMID: 856714

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
Evidence to inform decisions about maternal-fetal surgery: technical brief.
Hartmann KE, McPheeters ML, Chescheir NC, Gillam-Krakauer M, McKoy JN, Jerome R, Sathe NA, Meints L, Walsh WF
Obstet Gynecol 2011 May;117(5):1191-1204. doi: 10.1097/AOG.0b013e318216151d. PMID: 21471854
Congenital cystic adenomatoid malformation in the fetus: natural history and predictors of outcome.
Miller JA, Corteville JE, Langer JC
J Pediatr Surg 1996 Jun;31(6):805-8. doi: 10.1016/s0022-3468(96)90138-4. PMID: 8783108

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

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