Biliary Atresia-Case Presentation and Panel Discussion: Update Course 2014
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
In a Michigan series, children who had Kasai after 3 months had the best drainage and long-term outcomes
Under 60 days there was a clear difference in outcomes; procedures done on weekends if approaching day 60
French registry data show significantly better 5- and 10-year native liver survival when Kasai performed before 30 days versus after 90 days, with nearly twice the survival rate
The critical factor determining outcome is the degree of cirrhosis developed in the liver, which varies among children and does not always correlate directly with age
Biliary atresia does not start at day zero in all patients; some may begin at day 20 and progress from there
A series from Rich Ricketts showed not statistically significant better results with Kasai performed at 76 days or older versus 0-75 days
Peter Altman from Columbia, who had the largest US series, argued Kasai should be done between 60 and 75 days
Reversal of portal flow is very concerning because patients can achieve good biliary drainage and normal bilirubin but portal hypertension continues to progress, leading to transplant need
Performing Kasai can buy time and increase the donor pool potential, even if the liver looks horrible
Transplant becomes technically easier in older children with lower risk of hepatic artery thrombosis
Hepatologists can manage biliary atresia patients medically for about a year in the absence of Kasai or transplant, though nutritional status is not optimal
If patients have signs of end-stage liver disease with profound fibrosis and reversal of portal flow, Kasai may not improve outcomes and the operation may stress them unnecessarily
The Kasai operation itself is not technically demanding with low technical risk, so if uncertain, redo procedures are reasonable
Some biliary atresia cases show a race between hepatocyte regeneration and fibrosis progression; one patient's bilirubin dropped to normal 5 months post-op
If liver biopsy is suggestive of biliary atresia, additional testing is not needed
HIDA scan with phenobarbital has high sensitivity and specificity for biliary atresia
An effective HIDA requires at least 5 days of phenobarbital at therapeutic levels
In cases where initial HIDA without adequate phenobarbital was negative, about one-third showed biliary atresia when repeated with proper phenobarbital levels
A recent paper in Gastroenterology showed a scoring system combining liver biopsy with clinical parameters had almost 100% ability to predict biliary atresia
Pathologists' understanding of what to look for in biliary atresia biopsies has improved compared to 20 years ago
Interventional radiologists can perform percutaneous liver biopsies safely and routinely
Patent gallbladder in true biliary atresia is super rare
In Milwaukee, percutaneous cholangiography combined with biopsy can quickly provide answers when successful
If percutaneous cholangiography shows contrast in the gallbladder and bowel, biliary atresia is ruled out without need for surgery
Prenatal ultrasound diagnosis of biliary atresia based on absent gallbladder is unreliable and angle-dependent
Japanese centers still recommend steroids either routinely or when drainage decreases after Kasai
Japanese surgeons now perform Kasai with wider porta dissection than Dr. Kasai originally did
Japanese technique uses more superficial sutures to avoid damaging ductules and avoids placing sutures at 10 o'clock and 3 o'clock positions where normal bile ducts bifurcate
Japanese centers claim better results with modified technique, though numbers are not statistically valid
Yamataka is attempting to show laparoscopic Kasai results are as good as open, contrary to IPEG recommendations