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Published in: Pediatric Surgery International 6/2018

01-06-2018 | Original Article

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

Authors: Navot Kantor, Carolyn Wayne, Ahmed Nasr

Published in: Pediatric Surgery International | Issue 6/2018

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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.
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Literature
1.
go back to reference Waszak P, Claris O, Lapillonne A et al (1999) Cystic adenomatoid malformation of the lung: neonatal management of 21 cases. Pediatr Surg Int 15:326–331CrossRefPubMed Waszak P, Claris O, Lapillonne A et al (1999) Cystic adenomatoid malformation of the lung: neonatal management of 21 cases. Pediatr Surg Int 15:326–331CrossRefPubMed
2.
go back to reference Laje P, Liechty KW (2008) Postnatal management and outcome of prenatally diagnosed lung lesions. Prenat Diagn 28:612–618CrossRefPubMed Laje P, Liechty KW (2008) Postnatal management and outcome of prenatally diagnosed lung lesions. Prenat Diagn 28:612–618CrossRefPubMed
3.
go back to reference Laberge JM, Puligandla P, Flageole H (2005) Asymptomatic congenital lung malformations. Semin Pediatr Surg 14:16–33CrossRefPubMed Laberge JM, Puligandla P, Flageole H (2005) Asymptomatic congenital lung malformations. Semin Pediatr Surg 14:16–33CrossRefPubMed
4.
go back to reference Ben-Ishay O, Nicksa GA, Wilson JM et al (2012) Management of giant congenital pulmonary airway malformations requiring pneumonectomy. Ann Thorac Surg 94:1073–1078CrossRefPubMed Ben-Ishay O, Nicksa GA, Wilson JM et al (2012) Management of giant congenital pulmonary airway malformations requiring pneumonectomy. Ann Thorac Surg 94:1073–1078CrossRefPubMed
5.
go back to reference Parikh D, Rasiah S (2015) Congenital lung lesions: postnatal management and outcome. Semin Pediatr Surg 24(4):160–167CrossRefPubMed Parikh D, Rasiah S (2015) Congenital lung lesions: postnatal management and outcome. Semin Pediatr Surg 24(4):160–167CrossRefPubMed
6.
go back to reference Sauvat F, Michel J, Benachi A et al (2003) Management of asymptomatic neonatal cystic adenomatoid malformations. J Pediatr Surg 38(4):548–552CrossRefPubMed Sauvat F, Michel J, Benachi A et al (2003) Management of asymptomatic neonatal cystic adenomatoid malformations. J Pediatr Surg 38(4):548–552CrossRefPubMed
7.
go back to reference 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):329–334CrossRefPubMed 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):329–334CrossRefPubMed
8.
go back to reference Ruchonnet-Metrailler I, Leroy-Terquem E, Stirnemann J et al (2014) Neonatal outcomes of prenatally diagnosed congenital pulmonary malformations. Pediatrics 133(5):e1285–e1291CrossRefPubMed Ruchonnet-Metrailler I, Leroy-Terquem E, Stirnemann J et al (2014) Neonatal outcomes of prenatally diagnosed congenital pulmonary malformations. Pediatrics 133(5):e1285–e1291CrossRefPubMed
9.
go back to reference Baird R, Puligandla P, Laberge J (2014) Congenital lung malformations: informing best practice. Semin Pediatr Surg 23(5):270–277CrossRefPubMed Baird R, Puligandla P, Laberge J (2014) Congenital lung malformations: informing best practice. Semin Pediatr Surg 23(5):270–277CrossRefPubMed
10.
go back to reference Shanmugam G, MacArthur K, Pollock J (2005) Congenital lung malformations—antenatal and postnatal evaluation and management. Eur J Cardiothorac Surg 27(1):45–52CrossRefPubMed Shanmugam G, MacArthur K, Pollock J (2005) Congenital lung malformations—antenatal and postnatal evaluation and management. Eur J Cardiothorac Surg 27(1):45–52CrossRefPubMed
11.
go back to reference Duan M, Wang L, Cao Y et al (2005) Results of surgical treatment of congenital cystic lung disease. Thorac Cardiovasc Surg 53(1):61–64CrossRefPubMed Duan M, Wang L, Cao Y et al (2005) Results of surgical treatment of congenital cystic lung disease. Thorac Cardiovasc Surg 53(1):61–64CrossRefPubMed
12.
go back to reference Stanton M, Davenport M (2006) Management of congenital lung lesions. Early Human Dev 82(5):289–295CrossRef Stanton M, Davenport M (2006) Management of congenital lung lesions. Early Human Dev 82(5):289–295CrossRef
13.
go back to reference Stanton M (2015) The argument for a non-operative approach to asymptomatic lung lesions. Semin Pediatr Surg 24(4):183–186CrossRefPubMed Stanton M (2015) The argument for a non-operative approach to asymptomatic lung lesions. Semin Pediatr Surg 24(4):183–186CrossRefPubMed
14.
go back to reference Singh R, Davenport M (2015) The argument for operative approach to asymptomatic lung lesions. Semin Pediatr Surg 24(4):187–195CrossRefPubMed Singh R, Davenport M (2015) The argument for operative approach to asymptomatic lung lesions. Semin Pediatr Surg 24(4):187–195CrossRefPubMed
16.
go back to reference Graham B, Regehr G, Wright JG (2003) Delphi as a method to establish consensus for diagnostic criteria. J Clin Epidemiol 56(12):1150–1156CrossRefPubMed Graham B, Regehr G, Wright JG (2003) Delphi as a method to establish consensus for diagnostic criteria. J Clin Epidemiol 56(12):1150–1156CrossRefPubMed
17.
go back to reference 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):377–381CrossRefPubMed 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):377–381CrossRefPubMed
18.
go back to reference 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):508–512CrossRefPubMed 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):508–512CrossRefPubMed
20.
go back to reference 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):712–716CrossRefPubMed 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):712–716CrossRefPubMed
21.
go back to reference 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 187–241CrossRef 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 187–241CrossRef
22.
go back to reference 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 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
23.
go back to reference Makhijani AV, Wong FY (2014) Congenital cystic adenomatous malformation (CCAM)—postnatal management and outcome [abstract]. J Pediatr Child Health 50(Suppl. 1):PS048 Makhijani AV, Wong FY (2014) Congenital cystic adenomatous malformation (CCAM)—postnatal management and outcome [abstract]. J Pediatr Child Health 50(Suppl. 1):PS048
24.
go back to reference 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):416–419CrossRef 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):416–419CrossRef
25.
go back to reference 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):626–630CrossRefPubMed 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):626–630CrossRefPubMed
26.
go back to reference 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–F28CrossRef 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–F28CrossRef
27.
go back to reference 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):411–414CrossRefPubMed 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):411–414CrossRefPubMed
28.
go back to reference 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):31–39PubMed 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):31–39PubMed
29.
go back to reference 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):99–102CrossRef 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):99–102CrossRef
30.
go back to reference 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):1551–1554CrossRef 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):1551–1554CrossRef
31.
go back to reference 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):805–808CrossRefPubMed 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):805–808CrossRefPubMed
32.
go back to reference 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):753–757CrossRefPubMed 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):753–757CrossRefPubMed
33.
go back to reference 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):1009–1011CrossRefPubMed 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):1009–1011CrossRefPubMed
34.
go back to reference 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):511–523CrossRef 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):511–523CrossRef
35.
go back to reference Sueyoshi R, Okazaki T, Urushihara N et al (2008) Managing prenatally diagnosed asymptomatic congenital cystic adenomatoid malformation. Pediatr Surg Int 24(10):1111–1115CrossRefPubMed Sueyoshi R, Okazaki T, Urushihara N et al (2008) Managing prenatally diagnosed asymptomatic congenital cystic adenomatoid malformation. Pediatr Surg Int 24(10):1111–1115CrossRefPubMed
36.
go back to reference Tawil M, Pilling D (2004) Congenital cystic adenomatoid malformation: is there a difference between the antenatally and postnatally diagnosed cases? Pediatr Radiol 35(1):79–84CrossRefPubMed Tawil M, Pilling D (2004) Congenital cystic adenomatoid malformation: is there a difference between the antenatally and postnatally diagnosed cases? Pediatr Radiol 35(1):79–84CrossRefPubMed
37.
go back to reference 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):794–799CrossRefPubMed 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):794–799CrossRefPubMed
38.
go back to reference Raychaudhuri P, Pasupati A, James A et al (2011) Prospective study of antenatally diagnosed congenital cystic adenomatoid malformations. Pediatr Surg Int 27(11):1159–1164CrossRefPubMed Raychaudhuri P, Pasupati A, James A et al (2011) Prospective study of antenatally diagnosed congenital cystic adenomatoid malformations. Pediatr Surg Int 27(11):1159–1164CrossRefPubMed
39.
go back to reference 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):178–186CrossRefPubMed 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):178–186CrossRefPubMed
40.
go back to reference Cavoretto P, Molina F, Poggi S et al (2008) Prenatal diagnosis and outcome of echogenic fetal lung lesions. Ultrasound Obstet Gynecol 32(6):769–783CrossRefPubMed Cavoretto P, Molina F, Poggi S et al (2008) Prenatal diagnosis and outcome of echogenic fetal lung lesions. Ultrasound Obstet Gynecol 32(6):769–783CrossRefPubMed
41.
go back to reference 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 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
42.
go back to reference 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):1139–1146CrossRefPubMed 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):1139–1146CrossRefPubMed
43.
go back to reference 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):1837–1845CrossRef 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):1837–1845CrossRef
44.
go back to reference 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):2–7CrossRefPubMed 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):2–7CrossRefPubMed
45.
go back to reference Nasr A, Himidan S, Pastor AC et al (2010) Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma? J Pediatr Surg 45:1086–1089CrossRefPubMed Nasr A, Himidan S, Pastor AC et al (2010) Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma? J Pediatr Surg 45:1086–1089CrossRefPubMed
46.
go back to reference 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):665–675CrossRefPubMed 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):665–675CrossRefPubMed
Metadata
Title
Symptom development in originally asymptomatic CPAM diagnosed prenatally: a systematic review
Authors
Navot Kantor
Carolyn Wayne
Ahmed Nasr
Publication date
01-06-2018
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Surgery International / Issue 6/2018
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI
https://doi.org/10.1007/s00383-018-4264-y

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