Error Traps and Culture of Safety in Biliary Atresia

The June issue of Seminars and Pediatric Surgery focused on a topic that's important to every pediatric surgeon, improving the healthcare we deliver to children around the world. Follow along with our latest video series as we highlight articles that help enhance the culture of safety from various pediatric surgical subspecialties. And you might recognize a few well-known faces and names along the way. Here's a quick summary of error traps and culture of safety in biliary atresia with guest video reviewer Dr. Jonathan Roach. The first potential error trap in biliary atresia is not considering biliary atresia in the differential diagnosis of any newborn greater than two weeks of life with hyperbilirubinemia. We consider a direct Billy Rubin that is greater than 20% of the total or greater than 1.0 to prompt further workup for biliary atresia. The next potential error trap is an incomplete or inappropriate lab and radiographic workup for biliary atresia. Alpha one antitypsin deficiency needs to be ruled out prior to proceeding to the operating room, as this can be a confusing picture intraoperatively. Furthermore, we obtain ultrasounds on all these patients to look for abnormalities in the gallbladder as well as the triangular cord sign. Lastly, these patients will receive a percutaneous liver biopsy. Findings on pathology such as bridging fibrosis, bile duct plugging and proliferation of bile ductules are all suggestive of biliary atresia. With this workup complete, we then proceed to the operating room for cholangiogram, should the diagnosis of biliary atresia be thought of as likely. If your 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. This is because age at the time of Kasai is the primary determinant of outcome in these patients. The next potential error trap are intraoperative error traps. First, there can be difficulty performing a cholangiogram. Some gallbladders do not have a lumen, and in these patients, we will proceed with further exploration and likely Kasai. Another problem is extravasation of the contrast through the hole in the gallbladder. To avoid this, we have adopted the technique of mobilizing the gallbladder off of the gallbladder fossa, amputating the dome of the gallbladder and inserting the colangi catheter directly into the lumen of the gallbladder and simply tying this off with a silk ligature. The next potential error trap in the operating room is the failure to recognize vascular variants. There may be a pre-duodenal portal vein, which is rare. More commonly but still rare are confusing branching patterns of the hepatic artery and the portal vein. 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 the dissection needs to occur up to the substance of the liver. There can also be various branching patterns in the portal vein which can confuse the picture. The most reliable determinant of where the hiler plate exists is between the bifurcation of the right and left portal vein. It is also 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 last potential error trap within the operating room is an inappropriate transaction plane of the biliary remnants. We try to choose a plane that is flush with the capsule of the liver, so that we are not dissecting into the substance of the liver, nor remaining too shallow into the fibrous cone for the amputation of the biliary remnants. Potential post-operative error traps include the use of steroids or antibiotics. Due to the results of the start trial, we no longer routinely use steroids in our post-operative care. The use of antibiotics prophylactically to prevent cholangitis is also a controversial topic. We currently are using prophylactic antibiotics on all patients after Kasai. The last potential error trap is in long-term follow-up. These patients require long-term follow-up care either with the pediatric surgeon or with the pediatric hepatologist. Thank you very much.