Skip to main content

Symptom development in originally asymptomatic CPAM diagnosed prenatally: a systematic review

Pediatric Surgery International volume 34, pages 613–620 (2018)Cite this article

Abstract

Purpose

For the approximately three quarters of patients with a prenatal diagnosis of congenital pulmonary airway malformation (CPAM) who are asymptomatic at birth, the chance of eventually developing symptoms is unknown. We sought to explore the natural history of asymptomatic CPAM.

Methods

We searched EMBASE, MEDLINE, and the first 50 results from Google Scholar. Studies describing the natural history of prenatally diagnosed, initially asymptomatic CPAM were included. For asymptomatic patients initially managed non-surgically, we tabulated the proportion who went on to develop symptoms as well as the median age at symptom development.

Results

We included data from 19 retrospective studies on 353 patients. Of the 128 patients managed expectantly, 31 (24.2%) developed symptoms requiring surgical intervention. The median age at symptom development was 7.5 months (range 15 days–5 years).

Conclusion

The risk for developing respiratory symptoms exists with originally asymptomatic CPAM patients, but the exact risk is difficult to predict. Parents may be given the value of approximately 1 in 4 as an estimate of the proportion of asymptomatic CPAM patients who go on to develop symptoms, which will help them make an informed decision regarding the option of elective surgery.

This is a preview of subscription content, access via your institution.

Access options

Buy single article

Instant access to the full article PDF.

43,34 €

Price includes VAT (Finland)
Tax calculation will be finalised during checkout.

Fig. 1
Fig. 2

References

  1. Waszak P, Claris O, Lapillonne A et al (1999) Cystic adenomatoid malformation of the lung: neonatal management of 21 cases. Pediatr Surg Int 15:326–331

    Article  PubMed  CAS  Google Scholar 

  2. Laje P, Liechty KW (2008) Postnatal management and outcome of prenatally diagnosed lung lesions. Prenat Diagn 28:612–618

    Article  PubMed  Google Scholar 

  3. Laberge JM, Puligandla P, Flageole H (2005) Asymptomatic congenital lung malformations. Semin Pediatr Surg 14:16–33

    Article  PubMed  Google Scholar 

  4. Ben-Ishay O, Nicksa GA, Wilson JM et al (2012) Management of giant congenital pulmonary airway malformations requiring pneumonectomy. Ann Thorac Surg 94:1073–1078

    Article  PubMed  Google Scholar 

  5. Parikh D, Rasiah S (2015) Congenital lung lesions: postnatal management and outcome. Semin Pediatr Surg 24(4):160–167

    Article  PubMed  Google Scholar 

  6. Sauvat F, Michel J, Benachi A et al (2003) Management of asymptomatic neonatal cystic adenomatoid malformations. J Pediatr Surg 38(4):548–552

    Article  PubMed  Google Scholar 

  7. Aziz D, Langer J, Tuuha S et al (2004) Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not? J Pediatr Surg 39(3):329–334

    Article  PubMed  Google Scholar 

  8. Ruchonnet-Metrailler I, Leroy-Terquem E, Stirnemann J et al (2014) Neonatal outcomes of prenatally diagnosed congenital pulmonary malformations. Pediatrics 133(5):e1285–e1291

    Article  PubMed  Google Scholar 

  9. Baird R, Puligandla P, Laberge J (2014) Congenital lung malformations: informing best practice. Semin Pediatr Surg 23(5):270–277

    Article  PubMed  Google Scholar 

  10. Shanmugam G, MacArthur K, Pollock J (2005) Congenital lung malformations—antenatal and postnatal evaluation and management. Eur J Cardiothorac Surg 27(1):45–52

    Article  PubMed  Google Scholar 

  11. Duan M, Wang L, Cao Y et al (2005) Results of surgical treatment of congenital cystic lung disease. Thorac Cardiovasc Surg 53(1):61–64

    Article  PubMed  CAS  Google Scholar 

  12. Stanton M, Davenport M (2006) Management of congenital lung lesions. Early Human Dev 82(5):289–295

    Article  Google Scholar 

  13. Stanton M (2015) The argument for a non-operative approach to asymptomatic lung lesions. Semin Pediatr Surg 24(4):183–186

    Article  PubMed  Google Scholar 

  14. Singh R, Davenport M (2015) The argument for operative approach to asymptomatic lung lesions. Semin Pediatr Surg 24(4):187–195

    Article  PubMed  Google Scholar 

  15. Canadian Association of Paediatric Surgeons Evidence-Based Resource. https://caps.ca/evidence-based-resource

  16. Graham B, Regehr G, Wright JG (2003) Delphi as a method to establish consensus for diagnostic criteria. J Clin Epidemiol 56(12):1150–1156

    Article  PubMed  Google Scholar 

  17. Harris P, Taylor R, Thielke R et al (2009) Research electronic data capture (REDCap)—a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 42(2):377–381

    Article  PubMed  Google Scholar 

  18. Kapralik J, Wayne C, Chan E, Nasr A (2016) Surgical versus conservative management of congenital pulmonary airway malformation in children: a systematic review and meta-analysis. J Pediatr Surg 51(3):508–512

    Article  PubMed  Google Scholar 

  19. Khangura S, Konnyu K, Cushman R et al (2012) Evidence summaries: the evolution of a rapid review approach. Syst Rev 1:10

    Article  PubMed  PubMed Central  Google Scholar 

  20. Slim K, Nini E, Forestier D et al (2003) Methodological index for non-randomized studies (MINORS): development and validation of a new instrument. ANZ J Surg 73(9):712–716

    Article  PubMed  Google Scholar 

  21. Higgins JPT, Altman DG (2008) Assessing risk of bias in included studies. In: Higgins JPT, Green S (eds) Cochrane handbook for systematic review of interventions. Wiley, Oxford, pp 187–241

    Chapter  Google Scholar 

  22. Burke-McGovern S, Kraushik S, Ahmad S et al (2014) A 7 year review of cystic lung lesions in children in two inner-city hospitals [abstract]. Am J Respir Crit Care Med 189:A4671

    Google Scholar 

  23. Makhijani AV, Wong FY (2014) Congenital cystic adenomatous malformation (CCAM)—postnatal management and outcome [abstract]. J Pediatr Child Health 50(Suppl. 1):PS048

    Google Scholar 

  24. Xia B, Yu G, Liu C et al (2016) Surgical treatment of congenital cystic adenomatoid malformation: a retrospective study of single tertiary center experience. J Matern Fetal Neonat Med 30(4):416–419

    Article  CAS  Google Scholar 

  25. Aslan A, Yalcin E, Soyer T et al (2006) Prenatal period to adolescence: the variable presentations of congenital cystic adenomatoid malformation. Pediatr Int 48(6):626–630

    Article  PubMed  Google Scholar 

  26. Calvert J (2005) Outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung: 10 years’ experience 1991–2001. Arch Dis Child Fetal Neonat Ed 91(1):F26–F28

    Article  Google Scholar 

  27. Calvert J, Lakhoo K (2007) Antenatally suspected congenital cystic adenomatoid malformation of the lung: postnatal investigation and timing of surgery. J Pediatr Surg 42(2):411–414

    Article  PubMed  Google Scholar 

  28. Chow P, Lee S, Tang M et al (2007) Management and outcome of antenatally diagnosed congenital cystic adenomatoid malformation of the lung. Hong Kong Med J 13(1):31–39

    PubMed  CAS  Google Scholar 

  29. Hsieh C, Chao A, Chang Y et al (2005) Outcome of congenital cystic adenomatoid malformation of the lung after antenatal diagnosis. Int J Gynecol Obstetr 89(2):99–102

    Article  CAS  Google Scholar 

  30. Marshall K, Blane C, Teitelbaum D et al (2000) Congenital cystic adenomatoid malformation: impact of prenatal diagnosis and changing strategies in the treatment of the asymptomatic patient. Am J Roentgenol 175(6):1551–1554

    Article  CAS  Google Scholar 

  31. Miller J, Corteville J, Langer J (1996) Congenital cystic adenomatoid malformation in the fetus: natural history and predictors of outcome. J Pediatr Surg 31(6):805–808

    Article  PubMed  CAS  Google Scholar 

  32. Nagata K, Masumoto K, Tesiba R et al (2009) Outcome and treatment in an antenatally diagnosed congenital cystic adenomatoid malformation of the lung. Pediatr Surg Int 25(9):753–757

    Article  PubMed  Google Scholar 

  33. Revillon Y, Jan D, Plattner V et al (1993) Congenital cystic adenomatoid malformation of the lung: prenatal management and prognosis. J Pediatr Surg 28(8):1009–1011

    Article  PubMed  CAS  Google Scholar 

  34. Rocha G, Cristina Fernandes P, Proença E et al (2007) Congenital cystic adenomatoid malformation of the lung—the experience of five medical centres. Rev Port Pneumol (Engl Ed) 13(4):511–523

    Article  Google Scholar 

  35. Sueyoshi R, Okazaki T, Urushihara N et al (2008) Managing prenatally diagnosed asymptomatic congenital cystic adenomatoid malformation. Pediatr Surg Int 24(10):1111–1115

    Article  PubMed  Google Scholar 

  36. Tawil M, Pilling D (2004) Congenital cystic adenomatoid malformation: is there a difference between the antenatally and postnatally diagnosed cases? Pediatr Radiol 35(1):79–84

    Article  PubMed  Google Scholar 

  37. van Leeuwen K, Teitelbaum D, Hirschl R et al (1999) Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentation, surgical indications, and natural history. J Pediatr Surg 34(5):794–799

    Article  PubMed  Google Scholar 

  38. Raychaudhuri P, Pasupati A, James A et al (2011) Prospective study of antenatally diagnosed congenital cystic adenomatoid malformations. Pediatr Surg Int 27(11):1159–1164

    Article  PubMed  CAS  Google Scholar 

  39. Laberge J, Flageole H, Pugash D et al (2001) Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther 16(3):178–186

    Article  PubMed  CAS  Google Scholar 

  40. Cavoretto P, Molina F, Poggi S et al (2008) Prenatal diagnosis and outcome of echogenic fetal lung lesions. Ultrasound Obstet Gynecol 32(6):769–783

    Article  PubMed  CAS  Google Scholar 

  41. Sfakianaki A, Copel J (2012) Congenital cystic lesions of the lung: congenital cystic adenomatoid malformation and bronchopulmonary sequestration. Rev Obstetr Gynecol 5(2):85–93

    Google Scholar 

  42. MacSweeney F, Papagiannopoulos K, Goldstraw P et al (2003) An assessment of the expanded classification of congenital cystic adenomatoid malformations and their relationship to malignant transformation. Am J Surg Pathol 27(8):1139–1146

    Article  PubMed  Google Scholar 

  43. Casagrande A, Pederiva F (2017) Association between congenital lung malformations and lung tumors in children and adults: a systematic review. J Thorac Oncol 11(11):1837–1845

    Article  Google Scholar 

  44. Oliveira C, Himidan S, Pastor A, Nasr A, Manson D, Taylor G et al (2010) Discriminating preoperative features of pleuropulmonary blastomas (PPB) from congenital cystic adenomatoid malformations (CCAM): a retrospective, age-matched study. Eur J Pediatr Surg 21(01):2–7

    Article  PubMed  Google Scholar 

  45. Nasr A, Himidan S, Pastor AC et al (2010) Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma? J Pediatr Surg 45:1086–1089

    Article  PubMed  Google Scholar 

  46. Sullivan K, Li M, Haworth S et al (2017) Optimal age for elective surgery of asymptomatic congenital pulmonary airway malformation: a meta-analysis. Pediatr Surg Int 33(6):665–675

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

We thank Margaret Sampson, MLIS, PhD, AHIP (Children’s Hospital of Eastern Ontario) for developing the electronic search strategies.

Funding

University of Ottawa Faculty of Medicine Studentship.

Author information

Affiliations

  1. Department of Surgery, Children’s Hospital of Eastern Ontario, 401 Smyth Rd, Ottawa, ON, K1H 8L1, Canada

    Navot Kantor, Carolyn Wayne & Ahmed Nasr

  2. Faculty of Medicine, University of Ottawa, Ottawa, Canada

    Navot Kantor & Ahmed Nasr

Authors
  1. Navot Kantor
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Carolyn Wayne
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ahmed Nasr
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ahmed Nasr.

Ethics declarations

Conflict of interest

The authors declare that they have no conflicts of interest.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 (DOCX 18 KB)

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Kantor, N., Wayne, C. & Nasr, A. Symptom development in originally asymptomatic CPAM diagnosed prenatally: a systematic review. Pediatr Surg Int 34, 613–620 (2018). https://doi.org/10.1007/s00383-018-4264-y

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00383-018-4264-y

Keywords

  • Pulmonology
  • Surgery
  • CPAM
  • Congenital lung lesion
  • Systematic review