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Biliary Atresia-Case Presentation and Panel Discussion: Update Course 2014
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Biliary Atresia 26 items
Chapter 1 of 5 · Case-Based Learning
Optimal Kasai age
Case presentation and optimal age for Kasai
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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 underwent Kasai after 3 months had the best drainage and long-term outcomes, though patient selection bias (different forms of biliary atresia, late referral) may explain this finding.
Under 60 days was the threshold in the Denver series with John Lilly; cases approaching 60 days were treated as emergencies and done on weekends if necessary.
The critical factor determining outcome may be the degree of cirrhosis present at the time of Kasai, which does not always correlate directly with chronologic age.
Correlating Kasai outcomes with liver biopsy findings at the time of operation would provide more scientifically accurate data than age alone.
Reversal of portal flow on imaging is a very concerning sign; patients may achieve good biliary drainage post-Kasai but still progress to transplant due to worsening portal hypertension from ongoing fibrosis.
Even a Kasai that does not restore normal bilirubin may buy months of time, expanding the donor pool and making the child a better transplant candidate with lower risk of hepatic artery thrombosis.
Hepatologists can manage children with biliary atresia medically (without Kasai or transplant) for up to a year, though nutritional status suffers.
In patients with end-stage liver disease (profound fibrosis, reversal of portal flow), a Kasai may not improve outcomes and may stress the child more than medical management until transplant.
The Kasai operation itself is not technically demanding or high-risk, so if uncertain about candidacy, re-exploration is reasonable and sometimes results in successful drainage.
Some patients show delayed clearance of bilirubin (e.g., normalization at 5 months post-Kasai), suggesting a race between hepatocyte regeneration and fibrosis progression.
Pathologists' understanding of biliary atresia histology has improved over the past 20 years, and interventional radiologists can now perform percutaneous liver biopsies safely and quickly.
A patent gallbladder is extremely rare in true biliary atresia, making percutaneous cholangiography of limited utility.
Prenatal ultrasound diagnosis of biliary atresia based on absent gallbladder is unreliable and angle-dependent; one case was incorrectly diagnosed prenatally and the child was normal.
French registry data show significantly higher native liver survival at 5 and 10 years when Kasai is performed before 1 month of age compared to after 90 days—nearly twice the survival rate.
Peter Altman at 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 (1990–2011) found that HIDA scan with phenobarbital has high sensitivity for biliary atresia.
An effective HIDA scan requires at least 5 days of phenobarbital and therapeutic drug levels, representing a significant time commitment.
In the Children's National study, a significant number of initially negative HIDA scans converted to positive (biliary atresia) when repeated with adequate phenobarbital levels.
A recent paper in Gastroenterology described a scoring system combining liver biopsy with clinical parameters that predicted biliary atresia with almost 100% accuracy.
In Milwaukee (Jensen's center), percutaneous cholangiography is combined with liver biopsy; if contrast enters the gallbladder and drains, biliary atresia is ruled out without surgery.
Japanese centers still recommend postoperative steroids for biliary atresia, either routinely or when bile drainage decreases, despite randomized trials not supporting routine use.
Yamataka at Juntendo University has refined the Kasai technique: wider dissection than Kasai's original, more superficial sutures to avoid damaging ductules, and no stitches at 3 and 10 o'clock (normal bile duct bifurcation points). Early results suggest better outcomes, though not yet statistically validated.
Yamataka is attempting to refute IPEG's recommendation against laparoscopic Kasai by demonstrating that laparoscopic results are equivalent to open.