Francesco Morini1, Augusto Zani2, Andrea Conforti1, Ernest van Heurn3, Simon Eaton4,5, Prem Puri6, Risto Rintala7, Marija Lukac8,9, Joachim F Kuebler10, Florian Friedmacher11, Rene Wijnen12, Juan Antonio Tovar13, Agostino Pierro2, Pietro Bagolan1. 1. Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy. 2. Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada. 3. Department of Pediatric Surgery, AMC/VUMC, Amsterdam, The Netherlands. 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. 6. Department of Paediatric Surgery, National Children's Research Centre, Dublin, Ireland. 7. Department of Paediatric Surgery, Hospital for Children and Adolescents, Helsinki, Finland. 8. Division of Medicine, Department of Pediatric Surgery, Belgrade, Serbia. 9. Department of Neonatal Surgery, University Children's Hospital, Belgrade, Serbia. 10. Department of Pediatric Surgery, Hannover Medical School, Hannover, Niedersachsen, Germany. 11. National Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland. 12. Department of Pediatric Surgery, Sophia Children's Hospital, Erasmus MC, Rotterdam, The Netherlands. 13. Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
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. Georg Thieme Verlag KG Stuttgart · New York.
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. Georg Thieme Verlag KG Stuttgart · New York.
Authors: Sergei Hermelijn; Casper Kersten; Dhanya Mullassery; Nagarajan Muthialu; Nazan Cobanoglu; Silvia Gartner; Pietro Bagolan; Carmen Mesas Burgos; Alberto Sgro; Stijn Heyman; Holger Till; Janne Suominen; Maarten Schurink; Liesbeth Desender; Paul Losty; Kjetil Ertresvag; Harm A W M Tiddens; Rene M H Wijnen; Marco Schnater Journal: BMJ Open Date: 2021-04-12 Impact factor: 2.692
Authors: Casper M Kersten; Sergei M Hermelijn; Dhanya Mullassery; Nagarajan Muthialu; Nazan Cobanoglu; Silvia Gartner; Pietro Bagolan; Carmen Mesas Burgos; Alberto Sgrò; Stijn Heyman; Holger Till; Janne Suominen; Maarten Schurink; Liesbeth Desender; Paul Losty; Henri Steyaert; Suzanne Terheggen-Lagro; Martin Metzelder; Arnaud Bonnard; Rony Sfeir; Michael Singh; Iain Yardley; Noor R V M Rikkers-Mutsaerts; Cornelis K van der Ent; Niels Qvist; Des W Cox; Robert Peters; Michiel A G E Bannier; Lucas Wessel; Marijke Proesmans; Michael Stanton; Edward Hannon; Marco Zampoli; Francesco Morini; Harm A W M Tiddens; René M H Wijnen; Johannes M Schnater Journal: Children (Basel) Date: 2022-07-30