Less is more: 15-year audit of the evaluation of cholestatic jaundice in the neonatal intensive care unit.

OBJECTIVE

Neonatal cholestatic jaundice (cholestasis) has extensive aetiologies and occurs frequently in preterm infants in neonatal intensive care units (NICUs). Current multinational guidelines involve wide-ranging investigations suited to general newborn populations without validation in NICU settings. We retrospectively reviewed New Zealand's guidelines for investigating cholestatic jaundice in the NICU.

METHODS

Infants admitted to one tertiary NICU between 2008 and 2022 with direct bilirubin ≥20 µmol/L and percentage of total bilirubin ≥20% were identified from laboratory records. Investigations, health data and outcome data were obtained from clinical records.

RESULTS

Cholestasis was detected in 208 (1.5% admissions) with median gestational age of 27 weeks and birth weight of 942 g. Eight (3.8%) had primary liver diseases, all presenting in the early newborn period. Four (1.9%) with biliary atresia manifested cholestasis and acholic stool within 1-month postnatal age; two were born preterm and survived >5 years transplant-free post-portoenterostomy. There were no delayed or missed diagnoses of primary liver disease. The remaining 200 (96.2%) had hepatobiliary dysfunction related to preterm comorbidities, intestinal failure-associated liver disease, cytomegalovirus infection, rare extrahepatic congenital disease or unknown causes. Investigations included liver biopsy in 28 (13.5%), hepatobiliary scintigraphy in 7 (3.4%), intraoperative cholangiography in 4 (1.9%) and 1 (0.5%) percutaneous cholangiogram. Overall, invasive testing for rare congenital diseases was done infrequently.

CONCLUSIONS

Most NICU infants with cholestasis did not have congenital diseases, and the small minority who did were all distinguishable by clinical factors regardless of gestation.