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Fetal Risk Stratification and Outcomes in Children with Prenatally Diagnosed Lung Malformations: Results from a
Shaun M Kunisaki1, Jacqueline M Saito2, Mary E Fallat3
1Fetal Program, Division of General Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Johns Hopkins Children's Center, Baltimore, MD.
Insights
Prenatal diagnosis of congenital lung malformations (CLMs) using the congenital pulmonary airway malformation volume ratio (CVR) can predict outcomes. An initial CVR ≤1.4 indicates low hydrops risk, while a maximum CVR <0.9 suggests asymptomatic disease at birth.
Area of Science:
- Pediatric Surgery
- Fetal Medicine
- Neonatology
Background:
- Prenatal diagnosis of congenital lung malformations (CLMs) is established, but predicting clinical outcomes remains challenging.
- Identifying specific fetal CLM subgroups at high risk for hydrops or respiratory compromise is not well-defined.
Purpose of the Study:
- To evaluate clinical outcomes in children with prenatally diagnosed CLMs.
- To identify prenatal characteristics that predict adverse outcomes.
Main Methods:
- Retrospective cohort study of prenatally diagnosed CLMs (2009-2016) from 11 children's hospitals.
- Analysis of 344 children, with statistical analysis using logistic regression.
- Assessment of congenital pulmonary airway malformation volume ratio (CVR) in relation to perinatal outcomes.
Main Results:
- Fifteen percent of fetuses received maternal steroids for hydrops; prenatal intervention was rare (1.7%).
- Respiratory symptoms at birth affected 21.8%, and 10.0% required neonatal lung resection.
- Congenital pulmonary airway malformation volume ratio (CVR) was significantly associated with hydrops, respiratory distress, ventilation, and resection (P < 0.001).
- An initial CVR ≤1.4 correlated with reduced hydrops risk (AUC, 0.93).
- A maximum CVR <0.9 was associated with low risk for respiratory symptoms at birth (AUC, 0.72).
Conclusions:
- An initial CVR ≤1.4 identifies fetuses at very low risk for hydrops.
- A maximum CVR <0.9 is associated with asymptomatic disease at birth.
- These findings can improve prenatal counseling and delivery planning for CLMs.
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.
