The aim of this study is to determine if there has been a true, absolute, or apparent relative increase in congenital diaphragmatic hernia (CDH) survival for the last 2 decades.
All neonatal Bochdalek CDH patients admitted to an Ontario pediatric surgical hospital during the period when significant improvements in CDH survival was reported (from January 1, 1992, to December 31, 1999) were analyzed. Patient characteristics were assessed for CDH population homogeneity and differences between institutional and vital statistics-based population survival outcomes. SAS 9.1 (SAS Institute, Cary, NC) was used for analysis.
Of 198 cohorts, demographic parameters including birth weight, gestational age, Apgar scores, sex, and associated congenital anomalies did not change significantly. Preoperative survival was 149 (75.2%) of 198, whereas postoperative survival was 133 (89.3%) of 149, and overall institutional survival was 133 (67.2%) of 198. Comparison of institution and population-based mortality (n = 65 vs 96) during the period yielded 32% of CDH deaths unaccounted for by institutions. Yearly analysis of hidden mortality consistently showed a significantly lower mortality in institution-based reporting than population.
A hidden mortality exists for institutionally reported CDH survival rates. Careful interpretation of research findings and more comprehensive population-based tools are needed for reliable counseling and evaluation of current and future treatments.
Perinatal management of congenital diaphragmatic hernia (CDH) and gastroschisis (GS) remains nonstandardized and institution specific. This analysis describes practice and outcome variation across a national network.
A national, prospective, disease-specific database for CDH and GS was evaluated over 4 years. Centers were evaluated individually and defined as low (low-volume center [LVC]) or high (high-volume center [HVC]) volume based on case mean.
Congenital diaphragmatic hernia. Two hundred fifteen liveborn cases were studied (mean, 14.3 cases/center) across 15 centers (8 LVCs and 7 HVCs). Significant interinstitutional practice variation was noted in rates of termination (0%-40%) and cesarean delivery (0%-61%). Centers demonstrated marked variation in ventilation strategies, vasodilator and paralytic use, timing of surgery, and rates of primary closure. Overall survival was 81.4% (LVC, 76.9%; HVC, 82.4%; P = .43). Gastroschisis. Four hundred sixteen cases were investigated (mean, 26 cases/center; range, 6-72) across 16 centers (10 LVCs and 6 HVCs). Cesarean delivery rates varied widely between centers (0%-86%) as did timing of closure (early vs delayed, 1%-100%). There was no difference in length of stay, days on total parenteral nutrition, and overall survival (94.3% vs 97.2%; P = .17) between LVCs and HVCs.
The existence of perinatal practice and outcome variation for GS and CDH suggests targets for improved delivery of care and justifies efforts to standardize treatment on a national basis.