Eur J Pediatr Surg 2018; 28(01): 001-005
DOI: 10.1055/s-0037-1604020
Original Article
Georg Thieme Verlag KG Stuttgart · New York

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

Francesco Morini
1  Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy
,
Augusto Zani
2  Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada
,
Andrea Conforti
1  Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy
,
Ernest van Heurn
3  Department of Pediatric Surgery, AMC/VUMC, Amsterdam, The Netherlands
,
Simon Eaton
4  Department of Pediatric Surgery, University College London Institute of Child Health, London, United Kingdom
5  Department of Paediatric Surgery, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom
,
Prem Puri
6  Department of Paediatric Surgery, National Children's Research Centre, Dublin, Ireland
,
Risto Rintala
7  Department of Paediatric Surgery, Hospital for Children and Adolescents, Helsinki, Finland
,
Marija Lukac
8  Division of Medicine, Department of Pediatric Surgery, Belgrade, Serbia
9  Department of Neonatal Surgery, University Children's Hospital, Belgrade, Serbia
,
Joachim F. Kuebler
10  Department of Pediatric Surgery, Hannover Medical School, Hannover, Niedersachsen, Germany
,
Florian Friedmacher
11  National Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland
,
Rene Wijnen
12  Department of Pediatric Surgery, Sophia Children's Hospital, Erasmus MC, Rotterdam, The Netherlands
,
Juan Antonio Tovar
13  Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain
,
Agostino Pierro
2  Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada
,
Pietro Bagolan
1  Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy
,
on behalf of the EUPSA Network Office› Author Affiliations
Further Information

Publication History

20 April 2017

23 May 2017

Publication Date:
14 July 2017 (eFirst)

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.