Faculty Collaboration Database

Fetal Risk Stratification and Outcomes in Children with Prenatally Diagnosed Lung Malformations: Results from a Multi-Institutional Research Collaborative. Ann Surg 2022 Nov 01;276(5):e622-e630

Date

11/21/2020

Pubmed ID

33214447

DOI

10.1097/SLA.0000000000004566

Scopus ID

2-s2.0-85139571677 (requires institutional sign-in at Scopus site)   32 Citations

Abstract

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 ≤ 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.

Author List

Kunisaki SM, Saito JM, Fallat ME, Peter SDS, Lal DR, Karmakar M, Deans KJ, Gadepalli SK, Hirschl RB, Minneci PC, Helmrath MA, Midwest Pediatric Surgery Consortium

Author

Dave Lal MD, MPH Chief, Professor in the Surgery department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Child
Edema
Female
Humans
Infant, Newborn
Lung
Lung Diseases
Oxygen
Pregnancy
Retrospective Studies
Risk Assessment
Ultrasonography, Prenatal