A 2-month-old female infant with persistent jaundice is scheduled for a liver biopsy. She was born at 35 weeks' gestation.
Laboratory findings: Total bilirubin 10.3; conjugated 6.0; international normalized ratio 1.25; partial thromboplastin time 32; albumin 20.
Physiologic unconjugated hyperbilirubinemia is very common and occurs in about two-thirds of newborns. It usually becomes apparent on day 2 or 3 of life and lasts around 10 days. Persistent or severe hyperbilirubinemia warrants further evaluation. It can result from increased bilirubin production, deficiency of hepatic uptake, defects of conjugation, and increased enterohepatic circulation. Increased values of conjugated bilirubin warrant a cholestasis workup to evaluate for biliary atresia. Time is of the essence because the success of the Kasai procedure is thought to be better when it is performed early, preferably before 3 months of age.
Use general anesthesia without an endotracheal tube.
Sevoflurane mask induction and maintenance or propofol can be used.
Use opioids judiciously; the surgery is not very painful, but patients are expected to remain flat for a prolonged period postoperatively. Fentanyl 1-2 μg/kg is a good choice.
Patients must remain in the postanesthesia care unit for 4 hours for monitoring of possible hemodynamic consequences of bleeding at the site of the liver biopsy; they must take nothing by mouth for the first 2 hours.
DOs and DON’Ts
✓ Do perform brief general anesthesia. Patients must remain absolutely still.
✓ Do be prepared for significant bleeding. Have an active type and cross.
⊗ If possible, do not delay surgery.
✓ Do keep an IV in place in case bleeding develops later.
Tylenol administration (10 mg/kg) is most likely fine for patients with hyperbilirubinemia without liver failure.
Guidance of the needle biopsy is by percussion or ultrasound, especially for patients with transplanted livers. There is a small risk of significant bleeding, less so with transplanted livers, because of adhesions to surrounding structures.
The implementation of stool color cards in Taiwan in 2004 helped increase the number of patients diagnosed and treated with the Kasai operation before age 3 months.