CC BY 4.0 · Rev Bras Ginecol Obstet 2019; 41(11): 654-659
DOI: 10.1055/s-0039-1697983
Original Article
Thieme Revinter Publicações Ltda Rio de Janeiro, Brazil

Outcomes of Cases of Prenatally-Diagnosed Congenital Pulmonary Airway Malformation

Desfechos de casos de malformação das vias respiratórias diagnosticados no pré-natal
Mehmet Sinan Beksac
1   Division of Perinatology, Department of Obstetrics and Gynecology, Hacettepe University, Ankara, Turkey
1   Division of Perinatology, Department of Obstetrics and Gynecology, Hacettepe University, Ankara, Turkey
Atakan Tanacan
1   Division of Perinatology, Department of Obstetrics and Gynecology, Hacettepe University, Ankara, Turkey
Canan Unal
1   Division of Perinatology, Department of Obstetrics and Gynecology, Hacettepe University, Ankara, Turkey
Neslihan Bayramoglu Tepe
1   Division of Perinatology, Department of Obstetrics and Gynecology, Hacettepe University, Ankara, Turkey
Emine Aydın
1   Division of Perinatology, Department of Obstetrics and Gynecology, Hacettepe University, Ankara, Turkey
Gokcen Orgul
1   Division of Perinatology, Department of Obstetrics and Gynecology, Hacettepe University, Ankara, Turkey
Murat Yurdakok
2   Division of Neonatology, Department of Pediatrics, Hacettepe University, Ankara, Turkey
› Author Affiliations
Further Information

Publication History

24 January 2019

18 August 2019

Publication Date:
19 November 2019 (online)


Objective To evaluate the outcomes of cases of prenatally-diagnosed congenital pulmonary airway malformation (CPAM).

Methods We retrospectively evaluated cases of prenatally-diagnosed CPAM between 2004 and 2018. Ultrasonographic features such as visualization of a fetal lung mass and heterogeneous pulmonary parenchyma were used for CPAM diagnosis. Prenatal and postnatal findings were compared in terms of accuracy regarding the CPAM diagnosis.

Results The sample consisted of 27 cases. There were four cases in which the patients opted for the termination of pregnancy due to the severity of the lesion. A total of 23 neonates were delivered, and CPAM was confirmed in 15 cases. The median gestational age at delivery was 37 weeks (28–40 weeks) and the mean birth weight was 2,776 g. There were two neonatal deaths, one due to pneumothorax, and the other due to hypoplastic left heart syndrome (HLHS). A total of five patients with respiratory problems were operated in the postpartum period. There were eight misdiagnosis: bronchopulmonary sequestration (five cases), congenital lobar emphysema (two cases), and congenital diaphragm hernia (one case).

Conclusion A precise postnatal diagnosis is very important to organize the proper management of the pregnancies with fetuses with CPAM. The positive predictive value of the prenatal diagnosis of CPAM via ultrasonography is of 70.3%. The differential diagnosis of CPAM may be prolonged to the postpartum period in some cases.


All of the authors contributed with the project and data interpretation, the writing of the article, the critical review of the intellectual content, and with the final approval of the version to be published.

  • References

  • 1 Turgal M, Ozyuncu O, Boyraz G, Yazicioglu A, Sinan Beksac M. Non-immune hydrops fetalis as a diagnostic and survival problems: what do we tell the parents?. J Perinat Med 2015; 43 (03) 353-358 . Doi: 10.1515/jpm-2014-0094
  • 2 Turğal M, Yazıcıoğlu A, Özyüncü Ö, Beksaç MS. Limb body wall complex: first trimester ultrasonographic diagnosis of a case with exencephaly and megacystis. Gynecol Obstet Reprod Med 2015; 21 (02) 106-108
  • 3 Seear M, Townsend J, Hoepker A. , et al. A review of congenital lung malformations with a simplified classification system for clinical and research use. Pediatr Surg Int 2017; 33 (06) 657-664 . Doi: 10.1007/s00383-017-4062-y
  • 4 Boucherat O, Jeannotte L, Hadchouel A, Delacourt C, Benachi A. Pathomechanisms of congenital cystic lung diseases: focus on congenital cystic adenomatoid malformation and pleuropulmonary blastoma. Paediatr Respir Rev 2016; 19: 62-68 . Doi: 10.1016/j.prrv.2015.11.011
  • 5 Morotti RA, Cangiarella J, Gutierrez MC. , et al. Congenital cystic adenomatoid malformation of the lung (CCAM): evaluation of the cellular components. Hum Pathol 1999; 30 (06) 618-625 . Doi: 10.1016/s0046-8177(99)90084-9
  • 6 Kotecha S, Barbato A, Bush A. , et al. Antenatal and postnatal management of congenital cystic adenomatoid malformation. Paediatr Respir Rev 2012; 13 (03) 162-170, quiz 170–171 . Doi: 10.1016/j.prrv.2012.01.002
  • 7 Kunisaki SM, Barnewolt CE, Estroff JA. , et al. Large fetal congenital cystic adenomatoid malformations: growth trends and patient survival. J Pediatr Surg 2007; 42 (02) 404-410 . Doi: 10.1016/j.jpedsurg.2006.10.014
  • 8 Wilson RD, Hedrick HL, Liechty KW. , et al. Cystic adenomatoid malformation of the lung: review of genetics, prenatal diagnosis, and in utero treatment. Am J Med Genet A 2006; 140 (02) 151-155 . Doi: 10.1002/ajmg.a.31031
  • 9 Mann S, Wilson RD, Bebbington MW, Adzick NS, Johnson MP. Antenatal diagnosis and management of congenital cystic adenomatoid malformation. Semin Fetal Neonatal Med 2007; 12 (06) 477-481 . Doi: 10.1016/j.siny.2007.06.009
  • 10 He J, Zhou W, Lv H, Tao LJ, Chen XW, Wei M. Congenital cystic lung lesions: a clinical analysis of 28 newborns. Int J Clin Exp Med 2016; 9 (07) 12918-12924
  • 11 Davenport M, Warne SA, Cacciaguerra S, Patel S, Greenough A, Nicolaides K. Current outcome of antenally diagnosed cystic lung disease. J Pediatr Surg 2004; 39 (04) 549-556 . Doi: 10.1016/j.jpedsurg.2003.12.021
  • 12 Lecompte J-F, Gondran-Tellier B, Hery G. , et al. Congenital lung malformations: correlation between prenatal and postnatal imaging and pathological findings. Ann Pediatr Surg 2016; 12: 82-85 . Doi: 10.1097/01.XPS.0000480963.75083.91
  • 13 Chikkannaiah P, Kangle R, Hawal M. Congenital cystic adenomatoid malformation of lung: Report of two cases with review of literature. Lung India 2013; 30 (03) 215-218 . Doi: 10.4103/0970-2113.116272
  • 14 Gautam MS, Naren Satya SM, Prathyusha IS, Reddy KHC, Mayilvaganan KR, Raidu D. An atypical presentation of Congenital Pulmonary Airway Malformation (CPAM): a rare case with antenatal ultrasound findings and review of literature. Pol J Radiol 2017; 82: 299-303 . Doi: 10.12659/PJR.901447
  • 15 Bush A, Hogg J, Chitty LS. Cystic lung lesions - prenatal diagnosis and management. Prenat Diagn 2008; 28 (07) 604-611 . Doi: 10.1002/pd.2039
  • 16 Schott S, Mackensen-Haen S, Wallwiener M, Meyberg-Solomayer G, Kagan KO. Cystic adenomatoid malformation of the lung causing hydrops fetalis: case report and review of the literature. Arch Gynecol Obstet 2009; 280 (02) 293-296 . Doi: 10.1007/s00404-008-0880-4
  • 17 Duncombe GJ, Dickinson JE, Kikiros CS. Prenatal diagnosis and management of congenital cystic adenomatoid malformation of the lung. Am J Obstet Gynecol 2002; 187 (04) 950-954 . Doi: 10.1067/mob.2002.127460
  • 18 Raychaudhuri P, Pasupati A, James A, Whitehead B, Kumar R. Prospective study of antenatally diagnosed congenital cystic adenomatoid malformations. Pediatr Surg Int 2011; 27 (11) 1159-1164 . Doi: 10.1007/s00383-011-2909-1
  • 19 Stocker JT. Congenital pulmonary airway malformation: a new name and expanded classification of congenital cystic adenomatoid malformation of the lung. Histopathology 2002; 41: 424-430
  • 20 Gornall AS, Budd JL, Draper ES, Konje JC, Kurinczuk JJ. Congenital cystic adenomatoid malformation: accuracy of prenatal diagnosis, prevalence and outcome in a general population. Prenat Diagn 2003; 23 (12) 997-1002 . Doi: 10.1002/pd.739
  • 21 Desseauve D, Dugué-Marechaud M, Maurin S. , et al. [Performance of prenatal diagnosis and postnatal development of congenital lung malformations]. Gynécol Obstét Fertil 2015; 43 (04) 278-283 . Doi: 10.1016/j.gyobfe.2015.02.008
  • 22 Butterworth SA, Blair GK. Postnatal spontaneous resolution of congenital cystic adenomatoid malformations. J Pediatr Surg 2005; 40 (05) 832-834 . Doi: 10.1016/j.jpedsurg.2005.01.051
  • 23 Parikh DH, Rasiah SV. Congenital lung lesions: Postnatal management and outcome. Semin Pediatr Surg 2015; 24 (04) 160-167 . Doi: 10.1053/j.sempedsurg.2015.01.013
  • 24 Sullivan KJ, Li M, Haworth S. , et al. Optimal age for elective surgery of asymptomatic congenital pulmonary airway malformation: a meta-analysis. Pediatr Surg Int 2017; 33 (06) 665-675 . Doi: 10.1007/s00383-017-4079-2
  • 25 Ankermann T, Oppermann HC, Engler S, Leuschner I, Von Kaisenberg CS. Congenital masses of the lung, cystic adenomatoid malformation versus congenital lobar emphysema: prenatal diagnosis and implications for postnatal treatment. J Ultrasound Med 2004; 23 (10) 1379-1384 . Doi: 10.7863/jum.2004.23.10.1379
  • 26 Lee YJ, Jeong HC, Joo HJ, Kim GJ. Congenital lobar emphysema with cystic lung abnormality: antenatal ultrasound appearance. Korean J Obstet Gynecol 2012; 55 (11) 834-838 . Doi: 10.5468/KJOG.2012.55.11.834
  • 27 Babu R, Kyle P, Spicer RD. Prenatal sonographic features of congenital lobar emphysema. Fetal Diagn Ther 2001; 16 (04) 200-202 . Doi: 10.1159/000053909
  • 28 Mallmann MR, Geipel A, Bludau M. , et al. Bronchopulmonary sequestration with massive pleural effusion: pleuroamniotic shunting vs intrafetal vascular laser ablation. Ultrasound Obstet Gynecol 2014; 44 (04) 441-446 . Doi: 10.1002/uog.13304