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 the first large Michigan series, children who had Kasai performed after 3 months had the best drainage and long-term outcomes.
The critical factor determining outcome may be the degree of cirrhosis present in the liver at the time of Kasai, which does not always correlate directly with chronological age.
Reversal of portal flow is a concerning contraindication to Kasai because patients can achieve good biliary drainage but continue to develop progressive portal hypertension requiring transplant.
Even a failed Kasai can buy time by allowing the child to grow, expanding the donor pool and making transplantation technically easier with lower hepatic artery thrombosis risk.
Hepatologists can manage children with biliary atresia medically (without Kasai or transplant) for up to a year, though nutritional status is compromised.
In patients with profound portal fibrosis and reversal of portal flow, Kasai may not improve outcomes and the operative stress may worsen the patient's condition.
Some biliary atresia patients show delayed clearance of bilirubin, with bilirubin dropping to normal at 5 months post-Kasai, suggesting a race between hepatocyte regeneration and fibrosis progression.
Pathologists' understanding of biliary atresia histology has improved significantly compared to 20 years ago.
Patent gallbladder is extremely rare in true biliary atresia, making percutaneous cholangiography rarely successful.
Prenatal ultrasound diagnosis of biliary atresia based on absent gallbladder is unreliable and angle-dependent, with at least one reported case of normal outcome after consideration of abortion.
French registry data showed children who had Kasai at less than 1 month of age had nearly twice the 5- and 10-year native liver survival rate compared to those done after 90 days.
Rich Ricketts' series showed not statistically significant better results with Kasai performed at 76 days or older compared to 0-75 days.
Peter Altman from Columbia, who had the largest U.S. series, recommended performing Kasai between 60 and 75 days of age.
A Children's National Medical Center study from 1990-2011 showed HIDA scan with phenobarbital has high sensitivity for biliary atresia diagnosis.
An effective HIDA scan requires at least 5 days of phenobarbital with adequate serum levels, representing a significant time commitment.
A recent paper in Gastroenterology showed that liver biopsy plus other clinical parameters in a scoring system could predict biliary atresia with almost 100% accuracy.
Japanese surgeons still recommend postoperative steroids for biliary atresia, either routinely or when drainage decreases, based on their national registry experience.
Yamataka's modified Kasai technique uses wider dissection than the classic Kasai, 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 surgeons claim their modified technique produces better results than classic Kasai, though the numbers are not statistically valid.
Yamataka is attempting to refute IPEG's recommendation against laparoscopic Kasai by showing that laparoscopic results are as good as open procedures.