Shaun M Kunisaki1, Jacqueline M Saito2, Mary E Fallat3, Shawn D St Peter4, Dave R Lal5, Monita Karmakar6, Katherine J Deans7, Samir K Gadepalli6, Ronald B Hirschl6, Peter C Minneci7, Michael A Helmrath8. 1. Fetal Program, Division of General Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Johns Hopkins Children's Center, Baltimore, MD. 2. Division of Pediatric Surgery, Department of Surgery,Washington University School of Medicine, St. Louis, MO. 3. Division of Pediatric Surgery, Hiram C. Polk, Jr., MD Department of Surgery, University of Louisville, Norton Children's Hospital, Louisville, KY. 4. Division of Pediatric Surgery, Department of Surgery, Children's Mercy Hospital, Kansas City, MO. 5. Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI. 6. Section of Pediatric Surgery, Department of Surgery, University of Michigan and Michigan Medicine, C.S. Mott Children's Hospital, Ann Arbor, MI. 7. Center for Surgical Outcomes Research, the Research Institute and Department of Surgery, Nationwide Children's Hospital, Ohio State University College of Medicine, Columbus, OH. 8. Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
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.
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.
Authors: Abigail J Engwall-Gill; Sherwin S Chan; Kevin P Boyd; Jacqueline M Saito; Mary E Fallat; Shawn D St Peter; Stephanie Bolger-Theut; Eric J Crotty; Jared R Green; Rebecca L Hulett Bowling; Sachin S Kumbhar; Mantosh S Rattan; Cody M Young; Joseph K Canner; Katherine J Deans; Samir K Gadepalli; Michael A Helmrath; Ronald B Hirschl; Rashmi Kabre; Dave R Lal; Matthew P Landman; Charles M Leys; Grace Z Mak; Peter C Minneci; Tiffany N Wright; Shaun M Kunisaki Journal: JAMA Netw Open Date: 2022-06-01
Authors: Joseph Davidson; Alena Uus; Alexia Egloff; Milou van Poppel; Jacqueline Matthew; Johannes Steinweg; Maria Deprez; Michael Aertsen; Jan Deprest; Mary Rutherford Journal: Prenat Diagn Date: 2022-03-15 Impact factor: 3.242