Error Traps and Culture of Safety in Biliary Atresia

The first potential error trap in biliary atresia is not considering biliary atresia in the differential diagnosis of any newborn greater than 2 weeks of life with hyperbilirubinemia.

A direct bilirubin greater than 20% of the total or greater than 1.0 prompts further workup for biliary atresia.

Alpha-1 antitrypsin deficiency needs to be ruled out prior to proceeding to the operating room, as this can be a confusing picture intraoperatively.

Ultrasounds are obtained on all biliary atresia patients to look for abnormalities in the gallbladder as well as the triangular cord sign.

Findings on liver biopsy pathology such as bridging fibrosis, bile duct plugging, and proliferation of bile ducts are all suggestive of biliary atresia.

If workup is suggestive of biliary atresia, there is no need to wait for the result of every esoteric test prior to proceeding to the operating room.

Age at the time of Kasai is the primary determinant of outcome in biliary atresia patients.

Some gallbladders in biliary atresia do not have lumen, and in these patients further exploration and likely Kasai will proceed.

To avoid contrast extravasation during cholangiogram, the technique involves mobilizing the gallbladder off the gallbladder fossa, amputating the dome of the gallbladder, inserting the cholangiography catheter directly into the lumen, and tying it off with a silk ligature.

Vascular variants in biliary atresia may include a preduodenal portal vein, which is rare.

More commonly but still rare are confusing branching patterns of the hepatic artery and the portal vein in biliary atresia.

It is recommended to carry out dissection to the secondary branching of the right and left hepatic arteries, but sometimes this branching can be quite early and dissection needs to occur up to the substance of the liver.

The most reliable determinant of where the hilar plate exists is between the bifurcation of the right and left portal vein.

It is important to dissect the fibrous cone of the biliary remnants down into the bifurcation of the portal vein where small portal branches must be tied off.

The transection plane of the biliary remnants should be flush with the capsule of the liver so as not to dissect into the substance of the liver nor remain too shallow into the fibrous cone.

Due to the results of the START trial, steroids are no longer routinely used in post-Kasai care.

The use of antibiotics prophylactically to prevent cholangitis after Kasai is a controversial topic.

Prophylactic antibiotics are currently used on all patients after Kasai at the speaker's institution.

Biliary atresia patients require long-term follow-up care either with the pediatric surgeon or with the pediatric hepatologist.