TY - JOUR
T1 - Fetal Risk Stratification and Outcomes in Children with Prenatally Diagnosed Lung Malformations
T2 - Results from a Multi-Institutional Research Collaborative
AU - for the Midwest Pediatric Surgery Consortium
AU - Kunisaki, Shaun M.
AU - Saito, Jacqueline M.
AU - Fallat, Mary E.
AU - Peter, Shawn D.St
AU - Lal, Dave R.
AU - Karmakar, Monita
AU - Deans, Katherine J.
AU - Gadepalli, Samir K.
AU - Hirschl, Ronald B.
AU - Minneci, Peter C.
AU - Helmrath, Michael A.
N1 - Publisher Copyright:
Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.
PY - 2022/11/1
Y1 - 2022/11/1
N2 - Objective: The aim of this study was to assess current clinical outcomes in children with prenatally diagnosed congenital lung malformations (CLMs) and to identify prenatal characteristics associated with adverse outcomes. Summary Background Data: Despite a wide spectrum of clinical disease, the identification of fetal CLM subgroups at increased risk for hydrops and respiratory compromise at delivery has not been well defined. Methods: A retrospective cohort study was conducted using an operative database of prenatally diagnosed CLMs managed at 11 children's hospitals from 2009 to 2016. Statistical analyses were performed using nonparametric bivariate or multivariable logistic regression. Results: Three hundred forty-four children were analyzed. Fifteen (5.5%) fetuses were managed with maternal steroids in the setting of hydrops, and prenatal surgical intervention was uncommon (1.7%). Seventy-five (21.8%) had respiratory symptoms at birth, and 34 (10.0%) required neonatal lung resection. Congenital pulmonary airway malformation volume ratio (CVR) measurements were recorded in 169 (49.1%) cases and were significantly associated with perinatal outcome, including hydrops, respiratory distress at birth, need for supplemental oxygen, neonatal ventilator use, and neonatal resection (P < 0.001). An initial CVR ≤1.4 was significantly correlated with a reduced risk for hydrops [area under the curve (AUC), 0.93; 95% confidence interval (CI), 0.87-1.00]. A maximum CVR <0.9 (AUC, 0.72; 95% CI, 0.67-0.85) was associated with a low risk for respiratory symptoms at birth. Conclusions: In this large, multi-institutional study, an initial CVR a;circpermil 1.4 identifies fetuses at very low risk for hydrops, and a maximum CVR < 0.9 is associated with asymptomatic disease at birth. These findings represent an opportunity for standardization and quality improvement for prenatal counseling and delivery planning.
AB - Objective: The aim of this study was to assess current clinical outcomes in children with prenatally diagnosed congenital lung malformations (CLMs) and to identify prenatal characteristics associated with adverse outcomes. Summary Background Data: Despite a wide spectrum of clinical disease, the identification of fetal CLM subgroups at increased risk for hydrops and respiratory compromise at delivery has not been well defined. Methods: A retrospective cohort study was conducted using an operative database of prenatally diagnosed CLMs managed at 11 children's hospitals from 2009 to 2016. Statistical analyses were performed using nonparametric bivariate or multivariable logistic regression. Results: Three hundred forty-four children were analyzed. Fifteen (5.5%) fetuses were managed with maternal steroids in the setting of hydrops, and prenatal surgical intervention was uncommon (1.7%). Seventy-five (21.8%) had respiratory symptoms at birth, and 34 (10.0%) required neonatal lung resection. Congenital pulmonary airway malformation volume ratio (CVR) measurements were recorded in 169 (49.1%) cases and were significantly associated with perinatal outcome, including hydrops, respiratory distress at birth, need for supplemental oxygen, neonatal ventilator use, and neonatal resection (P < 0.001). An initial CVR ≤1.4 was significantly correlated with a reduced risk for hydrops [area under the curve (AUC), 0.93; 95% confidence interval (CI), 0.87-1.00]. A maximum CVR <0.9 (AUC, 0.72; 95% CI, 0.67-0.85) was associated with a low risk for respiratory symptoms at birth. Conclusions: In this large, multi-institutional study, an initial CVR a;circpermil 1.4 identifies fetuses at very low risk for hydrops, and a maximum CVR < 0.9 is associated with asymptomatic disease at birth. These findings represent an opportunity for standardization and quality improvement for prenatal counseling and delivery planning.
KW - congenital lung malformations
KW - congenital pulmonary airway malformations
KW - fetal lung lesions
UR - http://www.scopus.com/inward/record.url?scp=85139571677&partnerID=8YFLogxK
U2 - 10.1097/SLA.0000000000004566
DO - 10.1097/SLA.0000000000004566
M3 - Article
C2 - 33214447
AN - SCOPUS:85139571677
SN - 0003-4932
VL - 276
SP - E622-E630
JO - Annals of surgery
JF - Annals of surgery
IS - 5
ER -