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.
| Original language | English |
|---|---|
| Article number | 174084OA |
| Pages (from-to) | 1-5 |
| Number of pages | 5 |
| Journal | European Journal of Pediatric Surgery |
| Volume | 28 |
| Issue number | 1 |
| DOIs | |
| Publication status | Published - Jul 14 2017 |
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Keywords
- congenital lung anomalies
- observation
- postnatal
- prenatal
- surgery
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Surgery
Cite this
Current Management of Congenital Pulmonary Airway Malformations : A European Pediatric Surgeons' Association Survey. / Morini, Francesco; Zani, Augusto; Conforti, Andrea; Van Heurn, Ernest; Eaton, Simon; Puri, Prem; Rintala, Risto; Lukac, Marija; Kuebler, Joachim F.; Friedmacher, Florian; Wijnen, Rene; Tovar, Juan Antonio; Pierro, Agostino; Bagolan, Pietro.
In: European Journal of Pediatric Surgery, Vol. 28, No. 1, 174084OA, 14.07.2017, p. 1-5.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Current Management of Congenital Pulmonary Airway Malformations
T2 - A European Pediatric Surgeons' Association Survey
AU - Morini, Francesco
AU - Zani, Augusto
AU - Conforti, Andrea
AU - Van Heurn, Ernest
AU - Eaton, Simon
AU - Puri, Prem
AU - Rintala, Risto
AU - Lukac, Marija
AU - Kuebler, Joachim F.
AU - Friedmacher, Florian
AU - Wijnen, Rene
AU - Tovar, Juan Antonio
AU - Pierro, Agostino
AU - Bagolan, Pietro
PY - 2017/7/14
Y1 - 2017/7/14
N2 - 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.
AB - 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.
KW - congenital lung anomalies
KW - observation
KW - postnatal
KW - prenatal
KW - surgery
UR - http://www.scopus.com/inward/record.url?scp=85024487576&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85024487576&partnerID=8YFLogxK
U2 - 10.1055/s-0037-1604020
DO - 10.1055/s-0037-1604020
M3 - Article
AN - SCOPUS:85024487576
VL - 28
SP - 1
EP - 5
JO - European Journal of Pediatric Surgery
JF - European Journal of Pediatric Surgery
SN - 0939-7248
IS - 1
M1 - 174084OA
ER -