Biliary Atresia with Dr. Greg Tiao
With Dr. Greg Tiao · hosted by Dr. Em Gootee & Dr. Todd Ponsky & Dr. Ellen Encisco · Grand Rounds
Part of
Biliary Atresia 26 items
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
Video
Biliary Atresia: Where are we now? Advanced Practice Providers Pediatric...
Dr. Todd Ponsky · 48 min · Published Jul 2017
Video
Biliary Atresia - Robert Parry: Update Course 2014
24 min · Published Nov 2018
Video
Biliary Atresia-Case Presentation and Panel Discussion: Update Course 2014
23 min · Published Jul 2017
Podcast
Biliary Atresia Part II
42 min · Published Feb 2022
Video
Biliary Atresia - Clinical Practice Updates
Published Dec 2020
Podcast
Biliary Atresia Part I
31 min · Published Jan 2022
Video
Update Course Rewind: MMP-7 & Biliary Atresia Diagnosis 2024
CCHMC Pediatric Surgery · 6 min · Published Mar 2025
Video
Hepatoblastoma with Dr. Greg Tiao
CCHMC Pediatric Surgery · Published Mar 2022
Podcast
Hepatoblastoma with Dr. Greg Tiao
10 min · Published Feb 2022
Video
Biliary Atresia
CCHMC Pediatric Surgery · 20 min · Published Nov 2018
Video
Compiled Sandler Rapid Fire Sessions: Update Course 2015
29 min · Published Nov 2015
Video
GYN #4 Management of Tubal Torsion: When to Consider Salpingectomy with Dr. Lesley Breech
CCHMC Pediatric Surgery · Published Oct 2024
Podcast
Pyloric Stenosis with Dr. Alex Bondoc
16 min · Published Jun 2024
Video
GYN #3 Management of an Adnexal Torsion with a Healthy Appearing Ovary with Dr. Lesley Breech
CCHMC Pediatric Surgery · Published May 2024
Video
GYN #2 Oophoropexy in Adnexal Torsion with Dr. Lesley Breech
CCHMC Pediatric Surgery · Published May 2024
Video
GYN #1 Importance of Documenting Reproductive Anatomy with Dr. Lesley Breech
CCHMC Pediatric Surgery · Published Feb 2024
Podcast
Esophageal Replacement with Dr. Dan von Allmen
13 min · Published Nov 2023
What the experts said
Biliary atresia is an uncommon disease that results in an obstructive cholangiopathy of the biliary system
Biliary atresia is unique to infancy
Biliary atresia is the most common cause for end-stage liver disease in children
The etiology of biliary atresia is uncertain
Biliary atresia causes a biliary epithelial injury process that causes biliary obstruction
Untreated biliary atresia causes progressive cholestasis, portal fibrosis, eventually cirrhosis, portal hypertension, and can cause death by the age of 2 years
In the Far East, biliary atresia incidence is estimated at 1 in 8000 live births
In the United States, biliary atresia incidence is 1 in 15,000 live births
Infants with biliary atresia typically present with jaundice as the first clinical manifestation
Patients with biliary atresia have acholic or very pale gray stools
Patients with biliary atresia have dark urine
The liver in biliary atresia becomes quite firm and is usually palpable in the right costochondral margin
Biliary atresia typically presents between 1 to 2 months of age
Screening can pick up biliary atresia at an earlier stage and has been demonstrated to improve outcomes
The most common reason an infant has jaundice is physiologic jaundice of the newborn
Indirect hyperbilirubinemia indicates physiologic jaundice
Direct hyperbilirubinemia indicates a more pathologic process
Anatomic causes of direct hyperbilirubinemia include biliary atresia, choledochal cysts, and inspissated bile syndrome
Hepatocellular causes of direct hyperbilirubinemia include viral hepatitis, sepsis, PFIC syndromes, alpha-1 antitrypsin deficiency, tyrosinemia, and transport abnormalities
Workup for biliary atresia includes liver profile with GGT, alpha-1 antitrypsin genotype, and TORCH workup
Patients are screened for cystic fibrosis, hypothyroidism, and galactosemia as part of perinatal screening
Matrix metalloproteinase 7 (MMP-7) is a new biochemical marker with very high diagnostic sensitivity and specificity for biliary atresia
Ultrasound is the first imaging test typically obtained for biliary atresia workup
HIDA scan is fairly sensitive but has quite low specificity for biliary atresia and can delay workup and diagnosis
Liver biopsy is the gold standard for diagnosing biliary atresia
Classic biopsy findings of biliary atresia include expansion of the periportal space with mononuclear cells, bile duct proliferation, and bile duct plugs within proliferating periportal biliary ducts
Bile duct plugs are the pathognomonic finding of biliary atresia on biopsy
Treatment options for biliary atresia are surgical and include Kasai portoenterostomy or liver transplantation
As long as the patient reaches the OR before 70-75 days, the success rate of Kasai procedure justifies the small morbidity risks
In older patients with manifestations of cirrhosis including ascites and coagulopathy, consideration for primary transplant is warranted
Both Kasai and transplant procedures require an intraoperative cholangiogram to establish anatomy and visualize patency of the common hepatic duct and intrahepatic components
In Kasai procedure, the gallbladder is mobilized to identify the common bile duct and common hepatic duct, with dissection carried laterally to where arteries branch
The hilar plate in biliary atresia has a triangle-like appearance extending from the common hepatic duct to a broader base
The hilar plate is divided in one sharp transection right proximal to Glisson's capsule
A 30 to 35 centimeter Roux-en-Y limb is created and brought up in a retrocolic fashion for hilar plate reconstruction
The most worrisome complication of Kasai is vascular injury to the portal vein or hepatic artery
During Kasai, anything that is easily demonstrable should not be divided; if you think it's a vessel, don't divide it because it may be a segmental artery of importance
Other complications of Kasai include bowel obstruction and wound issues
Cholangitis is a longer-term concern after Kasai, but requires adequate bile flow to occur
Cholangitis after Kasai is presumed to be an ascending process and can be suppressed with antibiotics
In the United States, most Kasai patients are discharged home within 5 to 7 days after the procedure
Post-Kasai patients require multidisciplinary care with both surgeon and gastroenterologist follow-up
Post-Kasai patients are on prophylactic antibiotics, Actigall, and require nutritional support including fat-soluble vitamins and elemental diets
A successful Kasai is defined as direct bilirubin under 2 at 3 months of age
Immediate post-operative success indicator is a pigmented stool, which indicates the patient is draining
Biliary atresia is the most common cause of end-stage liver failure in the pediatric patient population
Biliary atresia is the number one reason for pediatric liver transplant