Outcomes of Congenital Diaphragmatic Hernia Repair

Abstract

Sources: (1) Poley MJ, Elly AS, Tibboel D, et al. The cost-effectiveness of treatment for congenital diaphragmatic hernia. J Pediatr Surg. 2002;37:1245–1252. (2) Skari H, Bjornland K, Frenckner B, et al. Congenital diaphragmatic hernia in Scandinavia from 1995 to 1998: predictors of mortality. J Pediatr Surg. 2002;37:1269–1275.Two recent articles review the treatment and predictors of mortality for congenital diaphragmatic hernia (CDH). The Poley, et al article analyzed the “cost effectiveness” of neonatal surgery for congenital diaphragmatic hernia at the Sophia Children’s Hospital of Rotterdam, The Netherlands, for 168 patients who underwent CDH repair after 1969. (Of the original 285 patients who had the surgery, 24 could not be traced, 86 had died, and 7 were mentally disabled.) Patients born after 1996 were excluded to allow adequate follow-up periods. Outcome measures included hospital costs, loss of productivity, out-of-pocket expenses, and “quality adjusted years of life.” The total cost of treatment for these children averaged in excess of 42,000 Euro dollars (approximately 36,500 US dollars). Most costs were incurred with the initial hospitalization, and “productivity” losses for both parents and patients were minimal. CDH patients suffered from respiratory difficulties and abdominal pain, but quality of life did not differ between the population studied and the general population. These results suggest that CDH repair is cost-effective.A somewhat different perspective was reported in the Skari, et al article. The authors conducted a retrospective multicenter cohort review of mortality from CDH in 195 children treated for CDH in 12 of the 13 pediatric surgical centers in Scandinavia from 1995 through 1998. The overall hospital mortality from CDH was 30%. Of the 168 neonates presenting with symptoms in the first 24 hours of life, 35% (P=.001) died before discharge from the hospital and all these deaths occurred in children presenting with symptoms in the first 2 hours of life. Multi-variant analysis of these “early presenters” showed that predictors of increased risk of death included a prenatal diagnosis (Hazards Ratio [HR]=2.7 [95% CI, 1.4–5.4; P=.004]), right-sided congenital diaphragmatic hernia (HR=2.1 [95% CI, 1.03–4.2; P=.042]), and low 1-minute Apgar score (HR=.82 [95% CI, .73–.93; P=.001]).The authors found that prenatal diagnosis and right-sided congenital diaphragmatic hernia were significant independent predictors of total mortality. However, the increase in mortality for those children diagnosed prenatally may suggest a “hidden mortality” due to those fetuses (with diaphragmatic hernia) who expired in utero or following birth but before transfer to a pediatric surgical center.While these 2 articles may seem somewhat interesting in their own light, viewed in the current context of limited health care resources, the worrisome potential for care directives exists. Given that prenatal diagnosis and right-sided defects were demonstrated to be strong predictors for overall mortality, it is not difficult to hypothesize that groups with an economic interest in overall healthcare resource utilization might use this information for planning purposes. As such, transfer to a pediatric surgical center might be discouraged (or not “facilitated”) for those infants with a prenatal diagnosis of congenital diaphragmatic hernia until after birth, and early mortality adversely affected in “borderline” neonates as a result of withholding expensive treatment (ECMO). As advocates for the optimum health of every child, pediatric care providers at every level must resist external forces based on “cost-effectiveness” to dictate healthcare policy—especially in the vulnerable neonatal population.While previous reports of survival for CDH, which occurs in 1 out of every 5,800 live births, indicate about a 30–50% survival rate,1–2 the Skari, et al study examines mortality risk in more detail. In this study, mortality was restricted to neonates presenting with symptoms at <24 hours. These “early presenters” could additionally stratify3 into low, moderate and high-risk groups with a 17%, 53%, and 62% mortality rate, respectively. This study was limited by heterogeneity between centers, particularly in relation to differences in perinatal treatment strategies and the absence of a predetermined treatment protocol.