StayCurrentMD · Biliary Atresia-Case Presentation and Panel Discussion: Update Course 2014
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Video23 min·Published Jul 2017Older

Biliary Atresia-Case Presentation and Panel Discussion: Update Course 2014

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What the experts said10 expert statements · 10 host summaries
In the first large Michigan series, children who had Kasai performed after 3 months had the best drainage and long-term outcomes.
Epidemiological
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.
Opinion
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.
Clinical
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.
Clinical
Hepatologists can manage children with biliary atresia medically (without Kasai or transplant) for up to a year, though nutritional status is compromised.
Clinical
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.
Opinion
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.
Clinical
Pathologists' understanding of biliary atresia histology has improved significantly compared to 20 years ago.
Opinion
Patent gallbladder is extremely rare in true biliary atresia, making percutaneous cholangiography rarely successful.
Clinical
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.
Clinical
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.
Host summary
Rich Ricketts' series showed not statistically significant better results with Kasai performed at 76 days or older compared to 0-75 days.
Host summary
Peter Altman from Columbia, who had the largest U.S. series, recommended performing Kasai between 60 and 75 days of age.
Host summary
A Children's National Medical Center study from 1990-2011 showed HIDA scan with phenobarbital has high sensitivity for biliary atresia diagnosis.
Host summary
An effective HIDA scan requires at least 5 days of phenobarbital with adequate serum levels, representing a significant time commitment.
Host summary
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.
Host summary
Japanese surgeons still recommend postoperative steroids for biliary atresia, either routinely or when drainage decreases, based on their national registry experience.
Host summary
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.
Host summary
Japanese surgeons claim their modified technique produces better results than classic Kasai, though the numbers are not statistically valid.
Host summary
Yamataka is attempting to refute IPEG's recommendation against laparoscopic Kasai by showing that laparoscopic results are as good as open procedures.
Host summary