Background: Management of IFALD using FO monotherapy with Omegaven has repeatedly been shown to be safe and effective in children and more recently, also in preterm neonates who are on dependent on long-term PN. In the present study we report the outcome of our expanded experience with FO monotherapy for IFALD on fish oil-based combination LE in the pediatric population, particularly in preterm low birth weight infants.

Methods: A diagnosis of IFALD was made when serum direct bilirubin levels were > 2.0 mg/dL in two consecutive measurements that were more than one week apart, without evidence of intrinsic causes of liver dysfunction. Management of IFALD was done by initially decreasing the total calories provided by PN, usually by lowering the amount of lipid to 2.0 g/kg/day. When calorie decrement did not result in alleviation of cholestasis, LE was switched from SMOF to Omegaven at 1.0 g/kg/day infused over 24 hours.

Results: Fifteen infants met the criteria for IFALD and received FO monotherapy during the study period from March 2017 to June 2018. Median gestational age was 27.5 weeks and median birth weight was 862.5 g. IFALD was successfully reversed in 10 infants (11/15, 73.3%). Four infants died of IFALD and resultant hepatic failure. Median direct bilirubin values were initially elevated and then steadily declined from the third week of treatment onward. Enteral tolerance increased in varying degrees in all but one patient during the treatment period. Mean weight gain was 26.0 g/day during FO monotherapy. Omegaven at a dose of 1.0 g/kg/day was well tolerated in these infants during the entire period of administration and no adverse events related to Omegaven use were seen. No signs of essential fatty acid deficiency (EFAD) were observed.

Conclusion: Fish oil monotherapy was safe and effective in the treatment of IFALD arising during SMOFlipid use in premature neonates on long-term PN.