Essential fatty acid deficiency in congenital biliary atresia: successful treatment to reverse deficiency.

Supplementation of lipid intake by infusion of solutions of essential fatty acid (EFA)-rich powder through Suruga II enterostomy was carried out for the treatment of EFA deficiency in nine children with postoperative congenital biliary atresia (CBA). Infusion of EFA-rich powder dissolved in excreted bile was effective in six patients except for a case who had a total bile acid concentration in the excreted bile that was less than critical micellar level. Administration of EFA-rich powder dissolved in a mixture of the patient's own bile and 1 to 2 mmol/L taurocholate (TC) solution corrected EFA deficiency in three children with total bile acid concentration lower than the critical level. Our results therefore show that infusion of EEA-rich lipid through Suruga II enterostomy after dissolving in the excreted bile is an effective treatment for EFA deficiency in postoperative patients with CBA, and that 1 to 2 mmol/L TC solution used as artificial bile facilitates lipid absorption in cases with total bile acid in the bile less than the critical micellar level.