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
Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy
,
Augusto Zani
Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada
,
Andrea Conforti
Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy
,
Ernest van Heurn
Department of Pediatric Surgery, AMC/VUMC, Amsterdam, The Netherlands
,
Simon Eaton
Department of Pediatric Surgery, University College London Institute of Child Health, London, United Kingdom
Department of Paediatric Surgery, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom
,
Prem Puri
Department of Paediatric Surgery, National Children's Research Centre, Dublin, Ireland
,
Risto Rintala
Department of Paediatric Surgery, Hospital for Children and Adolescents, Helsinki, Finland
,
Marija Lukac
Division of Medicine, Department of Pediatric Surgery, Belgrade, Serbia
Department of Neonatal Surgery, University Children's Hospital, Belgrade, Serbia
,
Joachim F. Kuebler
Department of Pediatric Surgery, Hannover Medical School, Hannover, Niedersachsen, Germany
,
Florian Friedmacher
National Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland
,
Rene Wijnen
Department of Pediatric Surgery, Sophia Children's Hospital, Erasmus MC, Rotterdam, The Netherlands
,
Juan Antonio Tovar
Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain
,
Agostino Pierro
Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada
,
Pietro Bagolan
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.