Current Management of Congenital Pulmonary Airway Malformations: A “European Pediatric Surgeons' Association” Survey
20 April 2017
23 May 2017
14 July 2017 (eFirst)
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.
- 1 Fowler DJ, Gould SJ. The pathology of congenital lung lesions. Semin Pediatr Surg 2015; 24 (04) 176-182
- 2 Thomas Bartholin. De Pulmonibus. In Marcello Malpighi Ed. Operum vol. II. Leiden, the Netherlands: Petrum van der Aa; 1687: 333-379
- 3 Fischer CC, Tropea F, Bailey CP. Congenital pulmonary cysts. Report of an infant treated by lobectomy with recovery. J Pediatr 1943; 23: 219-223
- 4 Garrett WJ, Kossoff G, Lawrence R. Gray scale echography in the diagnosis of hydrops due to fetal lung tumor. J Clin Ultrasound 1975; 3 (01) 45-50
- 5 Laberge JM, Flageole H, Pugash D. , et al. Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther 2001; 16 (03) 178-186
- 6 Duncombe GJ, Dickinson JE, Kikiros CS. Prenatal diagnosis and management of congenital cystic adenomatoid malformation of the lung. Am J Obstet Gynecol 2002; 187 (04) 950-954
- 7 Burge D, Wheeler R. Increasing incidence of detection of congenital lung lesions. Pediatr Pulmonol 2010; 45 (01) 103 , author reply 104
- 8 Belanger R, Lafleche LR, Picard J-L. Congenital cystic adenomatoid malformation of the lung. Thorax 1964; 19: 1-11
- 9 Stanton M. The argument for a non-operative approach to asymptomatic lung lesions. Semin Pediatr Surg 2015; 24 (04) 183-186
- 10 Singh R, Davenport M. The argument for operative approach to asymptomatic lung lesions. Semin Pediatr Surg 2015; 24 (04) 187-195
- 11 Lo AY, Jones S. Lack of consensus among Canadian pediatric surgeons regarding the management of congenital cystic adenomatoid malformation of the lung. J Pediatr Surg 2008; 43 (05) 797-799
- 12 Peters RT, Burge DM, Marven SS. Congenital lung malformations: an ongoing controversy. Ann R Coll Surg Engl 2013; 95 (02) 144-147
- 13 Aite L, Zaccara A, Trucchi A. , et al. Is counselling for CCAM that difficult? Learning from parental experience. J Prenat Med 2011; 5 (03) 65-68
- 14 Baird R, Puligandla PS, Laberge J-M. Congenital lung malformations: informing best practice. Semin Pediatr Surg 2014; 23 (05) 270-277