StayCurrentMD · Biliary Atresia
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Video20 min·Published Nov 2018Older

Biliary Atresia

With Dr. Greg Tiao · hosted by Dr. Todd Ponsky · StayCurrentMD
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What the experts said22 expert statements · 4 host summaries
Idiopathic neonatal hepatitis has decreased from 70% of cholestatic infants in the 1970s-1980s to approximately 10% currently due to recognition of inborn errors of bile acid metabolism and genotyping of progressive familial intrahepatic cholestasis (PFIC).
EpidemiologicalGreg Tiao
A paper published approximately one year prior demonstrated that a scoring system combining liver biopsy and clinical parameters achieves 99% accuracy in diagnosing biliary atresia.
Clinical
The jaundice chip is a microarray gene chip that tests for PFIC abnormalities (PFIC 1, 2, 3), alpha-1 antitrypsin, and JAG-1 mutation, but takes 4-6 weeks to return results.
ClinicalGreg Tiao
The absence of a gallbladder on ultrasound does not conclusively show biliary atresia, and the presence of a gallbladder does not exclude biliary atresia due to sophisticated ultrasound probe sensitivity.
ClinicalGreg Tiao
The triangular cord sign is a radiographic finding representing the fibrous plate above the portal vein where bile ducts were supposed to be, now showing inflammatory fibrous scar.
ClinicalGreg Tiao
At Cincinnati Children's Hospital, HIDA scans are not routinely used because the 3-5 day phenobarbital loading period can delay intervention into a time period potentially detrimental to Kasai outcome.
OpinionGreg Tiao
Pathognomonic findings on liver biopsy for biliary atresia include significant bile duct proliferation in portal triads and bile plugs within bile ducts, with likelihood of biliary atresia exceeding 90% when these are present.
ClinicalGreg Tiao
At Cincinnati, liver biopsies are reviewed with pathologists before taking patients to the operating room, making intraoperative decision-making easier and sometimes reducing the necessity of intraoperative cholangiogram.
ClinicalGreg Tiao
Alagille syndrome patients do not have the same bile duct proliferation as biliary atresia patients and will not have the pathognomonic finding of a bile duct plug.
ClinicalGreg Tiao
At Cincinnati, the workup for biliary atresia is limited to liver biopsy, labs, and ultrasound before posting the patient for surgery.
ClinicalGreg Tiao
The extended hilar dissection technique, going to the first bifurcation of vessels beyond the portal vein, is the proper way Kasai originally described the procedure.
ClinicalGreg Tiao
Japanese centers uniformly perform extended dissections, which may explain why their Kasai outcomes are better than those in the United States.
OpinionGreg Tiao
Polysplenia syndrome patients with biliary atresia are the most difficult cases because they often have pre-duodenal portal veins and frequently have no bile duct remnant, making Kasai nearly impossible in this population.
ClinicalGreg Tiao
Choledochal cyst patients will have pigmented stool, while cystic variant biliary atresia patients will become acholic, providing a simple clinical differentiation.
ClinicalGreg Tiao
A Roux limb length of 20 cm is inadequate because 5-8 cm of length is almost always lost during hilar dissection at the time of transplant.
ClinicalGreg Tiao
The target Roux limb length should be 30-40 cm to preserve adequate length for transplant teams, as one advantage of transplanting biliary atresia patients is having a pre-existing Roux limb.
ClinicalGreg Tiao
Anti-refluxing valves for the Roux limb have not been proven effective.
ClinicalGreg Tiao
The British health service focused all Kasai procedures at three high-volume centers, which are the only centers that can be reimbursed for the procedure.
GuidelineGreg Tiao
Despite negative trial results, there is a subset of biliary atresia patients with an inflammatory gene profile in which steroids might prove beneficial.
OpinionGreg Tiao
Cincinnati does not use MRCP for biliary atresia diagnosis due to having a high-capacity pathology team.
ClinicalGreg Tiao
Performing Kasai on Alagille syndrome patients will shorten the natural history of their native liver and result in earlier transplantation.
ClinicalGreg Tiao
For patients with cholestasis where biliary atresia diagnosis is uncertain, workup includes echocardiogram to rule out pulmonic stenosis, spine X-rays to rule out butterfly vertebrae, and eye exams, all to evaluate for Alagille syndrome.
ClinicalGreg Tiao
Data from England by Mark Davenport showed that cystic variants of biliary atresia have better outcomes if intervention occurs before 30 days of age.
Host summaryGreg Tiao · not cited in answers
UK data from approximately 15 years ago showed that centers performing more than 5 biliary atresia cases per year had statistically significantly better outcomes (approximately 20% difference) than those performing fewer cases.
Host summaryGreg Tiao · not cited in answers
Two European studies (King's Group using 2 mg/kg steroids and Klaus Peterson's German group using 10 mg/kg for 5 days) showed no benefit from postoperative corticosteroids in biliary atresia.
Host summaryGreg Tiao · not cited in answers
The Children's Network biliary atresia Research Consortium multi-center trial with approximately 70 patients in two arms showed no improvement in bile drainage with corticosteroid use and a slight increase in adverse events (primarily medical rather than surgical complications).
Host summaryGreg Tiao · not cited in answers