Current Management of Congenital Pulmonary Airway Malformations: A European Pediatric Surgeons' Association Survey

Francesco Morini, Augusto Zani, Andrea Conforti, Ernest van Heurn, Simon Eaton, Prem Puri, Risto Rintala, Marija Lukac, Joachim F. Kuebler, Florian Friedmacher, Rene Wijnen, Juan Antonio Tovar, Agostino Pierro, Pietro Bagolan

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

Aim To define current management of congenital pulmonary airway malformation (CPAM). Methods A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire. Main Results Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty-six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%). Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow-up and genetic screening, respectively. Conclusion Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear.
LanguageEnglish
Pages1-5
JournalEuropean Journal of Pediatric Surgery
Volume28
Issue number1
DOIs
Publication statusPublished - 2018

Cite this

Morini, Francesco ; Zani, Augusto ; Conforti, Andrea ; van Heurn, Ernest ; Eaton, Simon ; Puri, Prem ; Rintala, Risto ; Lukac, Marija ; Kuebler, Joachim F. ; Friedmacher, Florian ; Wijnen, Rene ; Tovar, Juan Antonio ; Pierro, Agostino ; Bagolan, Pietro. / Current Management of Congenital Pulmonary Airway Malformations: A European Pediatric Surgeons' Association Survey. In: European Journal of Pediatric Surgery. 2018 ; Vol. 28, No. 1. pp. 1-5.
@article{1e2ef8fa42cd443d87aed7acd0d8e90b,
title = "Current Management of Congenital Pulmonary Airway Malformations: A European Pediatric Surgeons' Association Survey",
abstract = "Aim To define current management of congenital pulmonary airway malformation (CPAM). Methods A total of 181 European Pediatric Surgeons' Association members (91{\%} senior) from 48 countries completed an online questionnaire. Main Results Prenatal: 93{\%} respondents work in centers with prenatal diagnosis facilities, and 27{\%} in centers offering in utero surgery. Prenatal counseling is performed by 86{\%} respondents, 22{\%} of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (<5{\%}) by more than 60{\%} of respondents. Eighty-six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87{\%}), followed by parental willingness (52{\%}). Prenatal surgery is an option for 44{\%} respondents, preferring thoracoamniotic shunt (82{\%}). Postnatal: 75{\%} respondents operate on asymptomatic patients, 18{\%} before 6 months of age, 62{\%} between 6 and 12 months of age, and 20{\%} after 12 months of age. Risk of infection (86{\%}), cancer (63{\%}), and symptoms development (62{\%}) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58{\%} respondents). Motivations against surgery include lesion <1 cm (64{\%}), risk of postoperative complications (37{\%}), and lack of evidence favoring surgery (27{\%}). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89{\%} of the cases within the CPAM. Of all the respondents, 83{\%} and 22{\%} offered dedicated follow-up and genetic screening, respectively. Conclusion Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear.",
author = "Francesco Morini and Augusto Zani and Andrea Conforti and {van Heurn}, Ernest and Simon Eaton and Prem Puri and Risto Rintala and Marija Lukac and Kuebler, {Joachim F.} and Florian Friedmacher and Rene Wijnen and Tovar, {Juan Antonio} and Agostino Pierro and Pietro Bagolan",
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doi = "10.1055/s-0037-1604020",
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Morini, F, Zani, A, Conforti, A, van Heurn, E, Eaton, S, Puri, P, Rintala, R, Lukac, M, Kuebler, JF, Friedmacher, F, Wijnen, R, Tovar, JA, Pierro, A & Bagolan, P 2018, 'Current Management of Congenital Pulmonary Airway Malformations: A European Pediatric Surgeons' Association Survey', European Journal of Pediatric Surgery, vol. 28, no. 1, pp. 1-5. https://doi.org/10.1055/s-0037-1604020

Current Management of Congenital Pulmonary Airway Malformations: A European Pediatric Surgeons' Association Survey. / Morini, Francesco; Zani, Augusto; Conforti, Andrea; van Heurn, Ernest; Eaton, Simon; Puri, Prem; Rintala, Risto; Lukac, Marija; Kuebler, Joachim F.; Friedmacher, Florian; Wijnen, Rene; Tovar, Juan Antonio; Pierro, Agostino; Bagolan, Pietro.

In: European Journal of Pediatric Surgery, Vol. 28, No. 1, 2018, p. 1-5.

Research output: Contribution to journalArticleAcademicpeer-review

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T1 - Current Management of Congenital Pulmonary Airway Malformations: A European Pediatric Surgeons' Association Survey

AU - Morini, Francesco

AU - Zani, Augusto

AU - Conforti, Andrea

AU - van Heurn, Ernest

AU - Eaton, Simon

AU - Puri, Prem

AU - Rintala, Risto

AU - Lukac, Marija

AU - Kuebler, Joachim F.

AU - Friedmacher, Florian

AU - Wijnen, Rene

AU - Tovar, Juan Antonio

AU - Pierro, Agostino

AU - Bagolan, Pietro

PY - 2018

Y1 - 2018

N2 - Aim To define current management of congenital pulmonary airway malformation (CPAM). Methods A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire. Main Results Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty-six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%). Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow-up and genetic screening, respectively. Conclusion Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear.

AB - Aim To define current management of congenital pulmonary airway malformation (CPAM). Methods A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire. Main Results Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty-six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%). Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow-up and genetic screening, respectively. Conclusion Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear.

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