C. A. Ikedionwu1, C. C. Style1, M. A. Verla1, T. C. Lee1, C. J. Fernandes2, S. G. Keswani1, A. M. Vogel1, O. O. Olutoye1 1Baylor College Of Medicine, Texas Childrens Hospital,Department Of Surgery, Division Of Pediatric Surgery,Houston, TX, USA 2Baylor College Of Medicine, Texas Childrens Hospital,Department Of Pediatrics, Newborn Section,Houston, TX, USA
Introduction: Hyperbilirubinemia is often present in neonates with congenital anomalies, particularly congenital diaphragmatic hernia (CDH). Hence, we sought to identify risk factors for hyperbilirubinemia and the relationship with outcome in children with CDH.
Methods: An IRB approved retrospective review of all infants admitted with CDH at a single institution from January 2012 to December 2017 was performed. The cohort was categorized by the presence or absence of hyperbilirubinemia defined as either a total serum bilirubin (TSB) of >5mg/dL, direct bilirubin (DB) >1.5mg/dL or DB >20% of TSB. Patients were further stratified into physiologic vs cholestatic (conjugated) hyperbilirubinemia based on normograms for bilirubin levels and neonatal age. Data analyzed included demographics, prenatal imaging features, and postnatal clinical outcomes. Data were analyzed using chi-square, Mann-Whitney U test and Student’s t-test, and logistic regression as appropriate; a p-value of <0.05 was considered significant.
Results: A total of 122 CDH patients were identified, of which 75% were left-sided, 53% were male, and hyperbilirubinemia (HB) developed in 69% (84). The primary HB subtype, physiologic hyperbilirubinemia (PHB) was noted in 35% (43/122), while cholestatic HB (CHB) accounted for the remaining 34% (41/122). The majority of those with HB development had “liver up” morphology (81% (68/84), p=0.012) and all received total parenteral nutrition (TPN). Compared to infants with PHB, infants with CHB had prenatal findings with smaller lung volumes (O/E TFLV of 28% vs 38% p=0.007), albeit without significant difference in percent liver herniation. Postnatally, those with CHB were more likely to have had a patch repair, longer duration of TPN, developed sepsis and needed ECMO when compared to both infants with PHB and those without evidence of HB (p<0.05, Table 1). Of these variables, logistic regression revealed that ECMO was the foremost risk factor for the development of CHB (OR 0.161; CI:0.31-0.84]. Infants with CHB had a longer length of hospital stay and lower survival compared to those with PHB. However, there was no difference in the survival between those with CHB and those with no jaundice. In spite of the high incidence of CHB in the cohort, only one patient had findings of hepatic encephalopathy.
Conclusion: CDH infants are high risk for developing clinically significant hyperbilirubinemia. Cholestatic hyperbilirubinemia is associated with increased severity of CDH and portends a worse prognosis. ECMO appears to be a major factor associated with parenteral nutrition associated liver disease (PNALD) in CDH. Patients with CDH whgo undergo ECMO should be considered for management strategies to reduce PNALD.
