StayCurrentMD · Biliary Atresia Part I
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Podcast31 min·Published Jan 2022Older

Biliary Atresia Part I

With Dr. Greg Tiao & Dr. Atsuyuki Yamataka & Dr. Mark Davenport · hosted by Dr. Todd Ponsky · StayCurrentMD
Cued at 15:29 · stops at 16:14 · press play
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What the experts said25 expert statements · 5 host summaries
A high GGT is more consistent with an obstructive process and helps guide toward eliminating PFIC (progressive familial intrahepatic cholestasis) diagnoses.
ClinicalGreg Tiao
At 50 days of age, physiologic jaundice from the newborn period would have resolved.
ClinicalGreg Tiao
The key diagnostic finding is conjugated hyperbilirubinemia.
ClinicalMark Davenport
Infants who initially had yellow stool that became clay-colored have better prognosis than those with clay-colored stool immediately after passing meconium.
ClinicalAtsuyuki Yamataka
Ultrasound separates out other surgical issues including inspissated bile syndrome and spontaneous perforation of bile ducts, which should have ultrasound-positive features.
ClinicalMark Davenport
The triangular cord sign on ultrasound is not particularly discriminatory for biliary atresia.
OpinionMark Davenport
Cincinnati Children's Hospital has not used HIDA scans for biliary atresia diagnosis in approximately 25 years.
ClinicalGreg Tiao
Experienced pathologists can diagnose biliary atresia from percutaneous liver biopsy in almost 90% of cases.
ClinicalMark Davenport
King's College Hospital uses ERCP for approximately 10% of biliary atresia cases when biopsy findings are equivocal.
ClinicalMark Davenport
Histologic findings diagnostic of biliary atresia include ductular proliferation, small cell infiltrate, and bile duct plugs in proliferating bile ducts.
ClinicalMark Davenport
By 50 days of age, all histologic features of biliary atresia should be established on biopsy, whereas earlier biopsies (10-20 days) may not show consistent diagnostic findings.
ClinicalMark Davenport
Significant fibrosis on liver biopsy reflects disease progression and may guide surgical decision-making.
ClinicalGreg Tiao
Liver exteriorization during Kasai portoenterostomy can create denser, more vascularized adhesions that complicate future liver transplantation.
ClinicalGreg Tiao
Liver exteriorization with a small incision can cause kinking of hepatic veins.
ClinicalAtsuyuki Yamataka
Recent data from Juntendo shows liver function might be worse in the midterm follow-up for patients who had laparoscopic Kasai compared to open surgery.
ClinicalAtsuyuki Yamataka
The only chance a child with biliary atresia has to avoid early transplant is a well-done Kasai portoenterostomy.
OpinionGreg Tiao
Even in the best hands, long-term drainage rate after Kasai portoenterostomy is only 70-75%.
ClinicalGreg Tiao
Damage to hepatic artery branches during Kasai dissection can exacerbate the underlying liver disease process.
ClinicalGreg Tiao
The hilar plate should be transected at the level of Glisson's capsule, leaving it intact, without cutting into the liver parenchyma itself.
ClinicalGreg Tiao
In the Children's Network surgical committee, approximately one-third of centers cut into the liver during Kasai, while the rest transect at 1-2 mm of fibrous remnant.
ClinicalGreg Tiao
Coring into the liver creates an unstable interface that has never achieved good results in the literature, as biliary ductules that transgress simply scar over.
ClinicalMark Davenport
Standard Roux limb length for Kasai portoenterostomy is 40 centimeters, though Cincinnati uses 35-40 cm and measures with silk suture to 45 cm.
ClinicalMark Davenport
The Roux anastomosis suture choice does not make a difference in outcomes.
OpinionGreg Tiao
For the portoenterostomy anastomosis, 6-0 Maxon suture is used with all knots tied on the outside.
ClinicalGreg Tiao
Yamataka uses a shorter Roux limb than standard because he does not like redundant Roux loops.
ClinicalAtsuyuki Yamataka
Biliary atresia is the leading cause for liver transplant in the United States.
Host summary
NASPGHAN guidelines recommend not obtaining HIDA scans because phenobarbital loading delays treatment by 5-6 days, pushing closer to the window where Kasai efficacy deteriorates.
Host summaryGreg Tiao · not cited in answers
IPEG placed a moratorium on laparoscopic Kasai portoenterostomy because of poor outcomes.
Host summaryTodd Ponsky · not cited in answers
Peterson ran a prospective randomized trial comparing laparoscopic to open Kasai and stopped the trial early.
Host summaryGreg Tiao · not cited in answers
Dr. Neo published a paper describing different depths of hilar plate transection in Kasai portoenterostomy.
Host summaryGreg Tiao · not cited in answers