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

Morini, F; Zani, A; Conforti, A; van Heurn, E; Eaton, S; Puri, P; Rintala, R; ... Bagolan, P; + view all (2018) Current Management of Congenital Pulmonary Airway Malformations: A "European Pediatric Surgeons' Association" Survey. European Journal of Pediatric Surgery , 28 (1) pp. 1-5. 10.1055/s-0037-1604020. Green open access

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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. CONCLUSIONS: 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.

Type: Article
Title: Current Management of Congenital Pulmonary Airway Malformations: A "European Pediatric Surgeons' Association" Survey
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1055/s-0037-1604020
Publisher version: http://doi.org/10.1055/s-0037-1604020
Language: English
Additional information: © Georg Thieme Verlag KG Stuttgart · New York. This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Congenital lung anomalies; observation; postnatal; prenatal; surgery
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Biology and Cancer Dept
URI: https://discovery.ucl.ac.uk/id/eprint/1558352
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