You refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.
You refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.
You refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.
The child or the infant is typically jaundiced. That's the first clinical manifestation. They will have acholic or very pale gray stools. They'll have dark urine. Their liver will become quite firm, so it's usually palpable in, in the right costochondral margin.
The infant develops progressive cholestasis, portal fibrosis, eventually cirrhosis, and then the manifestations of that, which include portal hypertension, and if untreated, can cause death by the age of 2 years of age.
Pretext 3 and 4 is the big game changer. This change in practice is what improves survival rates for patients with hepatoblastoma from less than 30% in the 70s and 80s to between 80 and 90% today.
clinicalThe etiology of biliary atresia is uncertain↗
▶Ep 12 · 0:56
clinicalBiliary atresia causes biliary epithelial injury that leads to biliary obstruction↗
▶Ep 12 · 1:10
quoteThe infant develops progressive cholestasis, portal fibrosis, eventually cirrhosis, and then the manifestations of that, which include portal hypertension, and if untreated, can cause death by the age of 2 years of age.↗
▶Ep 12 · 1:10
clinicalUntreated biliary atresia causes progressive cholestasis, portal fibrosis, cirrhosis, portal hypertension, and can cause death by age 2 years↗
▶Ep 12 · 1:37
epidemiologicalIn the Far East, biliary atresia incidence is 1 in 8000 live births↗
▶Ep 12 · 1:37
quoteIn the Far East, it's been estimated that 1 in 8000 live births will result in a patient with biliatresia.↗
▶Ep 12 · 1:43
quoteIn the West, or especially in the United States, it's less common where the incidence is 1 in 15,000 live births.↗
▶Ep 12 · 1:43
epidemiologicalIn the United States, biliary atresia incidence is 1 in 15,000 live births↗
▶Ep 12 · 2:02
quoteThe child or the infant is typically jaundiced. That's the first clinical manifestation. They will have acholic or very pale gray stools. They'll have dark urine. Their liver will become quite firm, so it's usually palpable in, in the right costochondral margin.↗
▶Ep 12 · 2:02
clinicalPatients with biliary atresia typically present with jaundice, acholic or pale gray stools, dark urine, and a firm palpable liver↗
▶Ep 12 · 2:21
clinicalTypical age of presentation for biliary atresia is between 1 to 2 months of age↗
▶Ep 12 · 2:26
clinicalScreening for biliary atresia can detect it at an earlier stage and has been demonstrated to improve outcomes↗
▶Ep 12 · 2:46
clinicalThe most common reason for infant jaundice is physiologic jaundice of the newborn with indirect hyperbilirubinemia↗
▶Ep 12 · 2:58
clinicalDirect hyperbilirubinemia indicates a more pathologic process↗
▶Ep 12 · 3:25
clinicalAnatomic causes of direct hyperbilirubinemia include biliary atresia, choledochal cysts, and inspissated bile syndrome↗
▶Ep 12 · 3:39
clinicalNon-anatomic causes of direct hyperbilirubinemia include viral hepatitis, sepsis, PFIC syndromes, alpha-1 antitrypsin deficiency, tyrosinemia, and transport abnormalities↗
▶Ep 12 · 5:13
clinicalMatrix metalloproteinase 7 (MMP-7) is a new biochemical marker with very high diagnostic sensitivity and specificity for biliary atresia↗
▶Ep 12 · 5:32
clinicalUltrasound is the first imaging test typically obtained for biliary atresia workup↗
▶Ep 12 · 5:42
clinicalHIDA scan is fairly sensitive but has low specificity and can delay diagnosis of biliary atresia↗
▶Ep 12 · 6:09
clinicalLiver biopsy is the gold standard for diagnosing biliary atresia↗
▶Ep 12 · 6:18
clinicalClassic biopsy findings in biliary atresia include periportal space expansion with mononuclear cells, bile duct proliferation, and bile duct plugs within proliferating periportal biliary ducts↗
▶Ep 12 · 6:45
clinicalTreatment options for biliary atresia are surgical: Kasai portoenterostomy or liver transplantation↗
▶Ep 12 · 7:00
clinicalSuccess rate of Kasai procedure justifies the small morbidity risks if performed before 70-75 days of age↗
▶Ep 12 · 7:12
clinicalManifestations of cirrhosis including ascites and coagulopathy in older patients may warrant consideration for primary transplant↗
▶Ep 12 · 7:34
clinicalIntraoperative cholangiogram is required to establish anatomy and visualize patency of the common hepatic duct and intrahepatic components↗
▶Ep 12 · 7:55
clinicalThe Kasai procedure involves mobilizing the gallbladder, dissecting laterally to where arteries branch, creating a triangle-like hilar plate appearance, and transecting the hilar plate proximal to Glisson's capsule↗
▶Ep 12 · 8:34
clinicalA 30-35 centimeter Roux-en-Y limb is created and brought up in a retrocolic fashion for reconstruction↗
▶Ep 12 · 9:04
clinicalThe most worrisome complication of Kasai procedure is vascular injury to the portal vein or hepatic artery↗
▶Ep 12 · 9:11
clinicalDuring Kasai procedure, anything that appears to be a vessel should not be divided as it may be a segmental artery of importance↗
▶Ep 12 · 9:27
clinicalOther complications of Kasai include bowel obstruction and wound issues↗
▶Ep 12 · 9:34
clinicalCholangitis is a longer-term concern but requires adequate bile flow to occur, making it paradoxically a sign of successful drainage↗
▶Ep 12 · 10:01
clinicalMost patients in the United States are discharged home within 5-7 days after Kasai procedure↗
▶Ep 12 · 10:06
clinicalBiliary atresia requires multidisciplinary care with both surgeon and gastroenterologist follow-up↗
▶Ep 12 · 10:14
clinicalPostoperative management includes prophylactic antibiotics, Actigall, and nutritional support with fat-soluble vitamins and elemental diets↗
▶Ep 12 · 10:28
clinicalA successful Kasai is defined as direct bilirubin under 2 at 3 months of age↗
▶Ep 12 · 10:28
quoteThe definition of a successful cassai is a direct bilirubin under 2 at 3 months of age.↗
▶Ep 12 · 10:35
clinicalImmediate post-operative success is indicated by pigmented stool showing the patient is draining↗
Biliary Atresia Part I
▶Ep 13 · 6:06
clinicalA high GGT is more consistent with an obstructive process and helps eliminate some of the PFIC syndromes↗
▶Ep 13 · 6:37
clinicalAt 50 days of age, physiologic jaundice from the newborn period would have resolved↗
▶Ep 13 · 13:46
opinionHIDA scan is helpful when trying to rule out biliary atresia rather than rule it in↗
▶Ep 13 · 13:46
quoteThe NASSIGN guidelines actually right now are saying its value is a little bit equivocal.↗
▶Ep 13 · 13:46
guidelineNASSGN guidelines recommend against obtaining HIDA scan because phenobarbital loading delays treatment by 5-6 days, pushing closer to the window where Kasai efficacy deteriorates↗
▶Ep 13 · 16:28
clinicalSignificant fibrosis on biopsy is a piece of information that reflects disease progression and may guide management decisions↗
▶Ep 13 · 17:38
clinicalERCP requires general anesthesia in infants, so the benefit over exploration is technical rather than avoiding anesthesia↗
▶Ep 13 · 20:26
clinicalLiver exteriorization during Kasai can create denser, more vascularized adhesions that complicate future liver transplantation↗
▶Ep 13 · 24:31
clinicalEven in the best hands, long-term drainage rate after Kasai is only 70-75%↗
▶Ep 13 · 24:31
quoteThe only chance this kid has to avoid transplant early on is a well-done casai.↗
▶Ep 13 · 24:31
opinionThe only chance a biliary atresia patient has to avoid early transplant is a well-done Kasai↗
▶Ep 13 · 24:31
clinicalPeterson ran a prospective randomized trial of laparoscopic Kasai and stopped it↗
▶Ep 13 · 25:50
clinicalThe hilar plate should be transected at the level of Glisson's capsule, leaving it intact, not cutting into the liver parenchyma↗
▶Ep 13 · 25:50
clinicalDamaging hepatic artery branches during Kasai can exacerbate the underlying liver disease process↗
▶Ep 13 · 25:50
clinicalIn the Children's Network, about one-third of centers cut into the liver during Kasai; the rest transect at 1-2 mm of fibrous remnant↗
Biliary Atresia Part II
▶Ep 14 · 9:56
quoteI think you've got to be very careful when you make those kind of statements. Because it was a properly designed randomized trial.↗
▶Ep 14 · 9:56
clinicalSTART trial was properly designed randomized trial with 110 patients in each arm, adequately powered as designed↗
▶Ep 14 · 12:31
clinicalCincinnati protocol reserves steroids for specific subsets: patients under 30 days who don't drain, or cystic variant patients who don't respond appropriately, rather than empiric use↗
▶Ep 14 · 13:40
clinicalMolecular profiling identified two biliary atresia phenotypes: inflammatory subtype that may respond to steroids, and fibrotic subtype that may not↗
▶Ep 14 · 19:48
clinicalCincinnati uses cephalosporin in immediate postoperative period, then switches to Bactrim or amoxicillin for at least 3 months (treatment dose for 2 weeks, then prophylaxis for 6 months)↗
▶Ep 14 · 24:14
clinicalCincinnati makes clinical assessment including preoperative biopsy; significant fibrosis on biopsy may lead to primary transplant decision↗
▶Ep 14 · 27:21
clinicalCholangitis requires early recognition and treatment; families instructed to see pediatrician immediately for fever, not wait at home↗
▶Ep 14 · 28:15
clinicalCincinnati uses short course of steroids for cholangitis that doesn't respond to antibiotics after several days↗
▶Ep 14 · 29:07
clinicalCincinnati offers revision Kasai only to highly selected patients who previously cleared jaundice and normalized bilirubin, then became acholic after cholangitis - can salvage native liver for 5-10+ years in some cases↗
▶Ep 14 · 31:22
clinicalPatients should be referred to transplant center by 3 months post-Kasai if bilirubin hasn't gone below 2, to allow time for evaluation before developing synthetic dysfunction↗
quoteWhat we're gonna try to touch on is a concept which is a timely diagnosis of biliatresia is critical to prolong the native liver survival.↗
▶Ep 24 · 0:53
clinicalTimely diagnosis of biliary atresia is critical to prolong native liver survival↗
▶Ep 24 · 2:47
clinicalCincinnati Children's stopped doing HIDA scans about 25 years ago↗
▶Ep 24 · 2:47
quoteAt our institution, we stopped doing HIDA scans about 25 years ago.↗
▶Ep 24 · 2:50
quoteThe challenge that we face with the, uh, a HIDA scan is it adds about 5 days.↗
▶Ep 24 · 2:50
clinicalHIDA scan adds about 5 days to the diagnostic workup↗
▶Ep 24 · 3:17
clinicalThe Children's Network is a national consortium of about 14 centers where all cholestatic liver disease children are enrolled in a registry↗
▶Ep 24 · 3:32
clinicalDr. Bezarra screened about 1000 proteins and found 70 or so that were elevated in the biliary atresia population, of which MMP-7 was the most clear↗
▶Ep 24 · 3:57
epidemiologicalThe incidence of biliary atresia is higher in the Far East↗
▶Ep 24 · 4:10
clinicalIn China, if biliary atresia patients don't get early Kasai, living donor liver transplant is their only option and it's not readily available, so those kids face a mortality risk↗
▶Ep 24 · 4:26
clinicalMMP-7 is not perfect; sensitivity is pretty good but cutoffs vary because the assay is still evolving↗
▶Ep 24 · 4:44
quoteOur commitment to the patients who come to our institution is within 7 days of them showing up. If they have it, they're in the OR.↗
▶Ep 24 · 4:44
clinicalCincinnati Children's commitment is to perform Kasai within 7 days of patient presentation if they have biliary atresia↗
▶Ep 24 · 5:03
epidemiologicalA European study showed that if Kasai is done before 45 days, it reduces the incidence of transplant need in that patient population↗
▶Ep 24 · 5:22
guidelineA Kasai portoenterostomy done before 45 days is the goal↗
▶Ep 24 · 5:27
clinicalCincinnati Children's will do Kasai before 30 days if diagnosis can be established↗
▶Ep 24 · 5:37
clinicalMore and more nurseries are screening newborns at discharge, and a direct bilirubin over 1 in the newborn period is very sensitive for biliary atresia↗
quoteSo, Hepatoblastoma, there's around 250 new cases a year. Huge changes in terms of the treatment algorithm over the last 20 years.↗
▶Ep 4 · 0:15
epidemiologicalThere are around 250 new cases of hepatoblastoma per year↗
▶Ep 4 · 0:15
clinicalTreatment algorithms for hepatoblastoma have changed dramatically over the last 20-25 years↗
▶Ep 4 · 0:51
epidemiologicalNeuroblastoma is considered by most to be the most common abdominal wall malignancy in children↗
▶Ep 4 · 1:41
quoteCommon things being common, hemangioma should be pretty high on the list.↗
▶Ep 4 · 1:41
epidemiologicalHemangioma is the most common liver lesion in children↗
▶Ep 4 · 3:00
clinicalEOVIST is an MRI contrast agent that is taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging↗
▶Ep 4 · 4:08
clinicalCouinaud labeled liver segments in a counterclockwise fashion after the districts of Paris by injecting portal vessels↗
▶Ep 4 · 4:45
clinicalThe right hepatic vein differentiates anterior sections (5 and 8) from posterior sections (6 and 7) of the right liver↗
▶Ep 4 · 5:20
clinicalThe right portal vein separates superior segments (7 and 8) from inferior segments (5 and 6) of the right liver↗
▶Ep 4 · 7:15
quotePretext 3 and 4 is the big game changer. This change in practice is what improves survival rates for patients with hepatoblastoma from less than 30% in the 70s and 80s to between 80 and 90% today.↗
▶Ep 4 · 7:15
epidemiologicalThe change in practice for PRETEXT 3 and 4 tumors improved survival rates for hepatoblastoma from less than 30% in the 1970s and 1980s to between 80 and 90% today↗
▶Ep 4 · 7:31
clinicalIndications for liver transplantation in hepatoblastoma include unresectable disease, unsafe to resect, or resection would leave inadequate liver remnant↗
▶Ep 4 · 7:31
clinicalPRETEXT 3 tumors are generally biopsied at diagnosis and then started on neoadjuvant chemotherapy↗
▶Ep 4 · 8:19
clinicalTeams are now trained to recognize tumors and send them to centers that offer transplant or can do aggressive resection↗
▶Ep 4 · 9:06
clinicalThe FIT study has been ongoing for three years↗
Hepatoblastoma with Dr. Greg Tiao
▶Ep 5 · 0:46
epidemiologicalHepatoblastoma is nowhere near the most common liver tumor or the most common abdominal tumor in children↗
▶Ep 5 · 0:46
quoteHepatoblastoma is the most common malignant liver tumor in children, but it's nowhere near the most common liver tumor or the most common abdominal tumor.↗
▶Ep 5 · 2:20
clinicalHepatoblastoma can present as an asymptomatic abdominal mass, tumor rupture, or respiratory distress due to tumor size↗
▶Ep 5 · 3:13
clinicalMost experts rely on MRI to delineate anatomy and evaluate the extent of liver involvement in hepatoblastoma↗
▶Ep 5 · 3:13
clinicalEovist is an MRI contrast agent taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging↗
▶Ep 5 · 4:25
clinicalAggressive resections include trisectionectomy, mesohepatectomy, resection with additional procedure in remnant liver, and liver transplantation↗
▶Ep 5 · 5:41
clinicalThe right hepatic vein differentiates anterior and posterior sections of the right liver↗
▶Ep 5 · 6:02
clinicalThe right portal vein separates superior and inferior segments of the right liver↗
▶Ep 5 · 6:51
clinicalPre-text 1 has three contiguous sections free of disease, pre-text 2 has two, pre-text 3 has one, and pre-text 4 has no free sections or diffuse tumor↗
▶Ep 5 · 6:51
clinicalThe pre-text staging system classifies tumors based on the number of contiguous liver sections free of disease (minus four equals pre-text stage)↗
▶Ep 5 · 8:15
clinicalPre-text 3 and 4 tumors represent a big game changer in practice that improved survival from less than 30% in the 1970s-80s to 80-90% today↗
▶Ep 5 · 8:15
clinicalPre-text 3 tumors should generally be biopsied at diagnosis, started on neoadjuvant chemotherapy, then referred to transplant center or center with expertise in advanced liver resections↗
▶Ep 5 · 9:35
clinicalIt is important to consult a transplant center early for pre-text 3 and 4 tumors↗
▶Ep 5 · 9:35
quoteYou refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.↗
▶Ep 5 · 10:27
clinicalThe PHITT study has been ongoing for three years↗
▶Ep 5 · 10:27
clinicalThe PHITT (Pediatric Hepatic International Tumor Trial) is an ongoing multinational trial to identify high and low risk features for each pre-text stage↗
epidemiologicalHepatoblastoma is nowhere near the most common liver tumor or the most common abdominal tumor in children↗
▶Ep 9 · 0:46
quoteHepatoblastoma is the most common malignant liver tumor in children, but it's nowhere near the most common liver tumor or the most common abdominal tumor.↗
▶Ep 9 · 2:20
clinicalHepatoblastoma can present as an asymptomatic abdominal mass, tumor rupture, or respiratory distress due to tumor size↗
▶Ep 9 · 3:13
clinicalMost experts rely on MRI to delineate anatomy and evaluate the extent of liver involvement in hepatoblastoma↗
▶Ep 9 · 3:13
clinicalEovist is an MRI contrast agent taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging↗
▶Ep 9 · 4:25
clinicalAggressive resections include trisectionectomy, mesohepatectomy, resection with additional procedure in remnant liver, and liver transplantation↗
▶Ep 9 · 5:41
clinicalThe right hepatic vein differentiates anterior and posterior sections of the right liver↗
▶Ep 9 · 6:02
clinicalThe right portal vein separates superior and inferior segments of the right liver↗
▶Ep 9 · 6:51
clinicalThe pre-text staging system classifies tumors based on the number of contiguous liver sections free of disease (minus four equals pre-text stage)↗
▶Ep 9 · 6:51
clinicalPre-text 1 has three contiguous sections free of disease, pre-text 2 has two, pre-text 3 has one, and pre-text 4 has no free sections or diffuse tumor↗
▶Ep 9 · 8:15
clinicalPre-text 3 and 4 tumors represent a big game changer in practice that improved survival from less than 30% in the 1970s-80s to 80-90% today↗
▶Ep 9 · 8:15
clinicalPre-text 3 tumors should generally be biopsied at diagnosis, started on neoadjuvant chemotherapy, then referred to transplant center or center with expertise in advanced liver resections↗
▶Ep 9 · 9:35
quoteYou refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.↗
▶Ep 9 · 9:35
clinicalIt is important to consult a transplant center early for pre-text 3 and 4 tumors↗
▶Ep 9 · 10:27
clinicalThe PHITT study has been ongoing for three years↗
▶Ep 9 · 10:27
clinicalThe PHITT (Pediatric Hepatic International Tumor Trial) is an ongoing multinational trial to identify high and low risk features for each pre-text stage↗
epidemiologicalHepatoblastoma is nowhere near the most common liver tumor or the most common abdominal tumor in children↗
▶Ep 11 · 0:46
quoteHepatoblastoma is the most common malignant liver tumor in children, but it's nowhere near the most common liver tumor or the most common abdominal tumor.↗
▶Ep 11 · 2:20
clinicalHepatoblastoma can present as an asymptomatic abdominal mass, tumor rupture, or respiratory distress due to tumor size↗
▶Ep 11 · 3:13
clinicalMost experts rely on MRI to delineate anatomy and evaluate the extent of liver involvement in hepatoblastoma↗
▶Ep 11 · 3:13
clinicalEovist is an MRI contrast agent taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging↗
▶Ep 11 · 4:25
clinicalAggressive resections include trisectionectomy, mesohepatectomy, resection with additional procedure in remnant liver, and liver transplantation↗
▶Ep 11 · 5:41
clinicalThe right hepatic vein differentiates anterior and posterior sections of the right liver↗
▶Ep 11 · 6:02
clinicalThe right portal vein separates superior and inferior segments of the right liver↗
▶Ep 11 · 6:51
clinicalThe pre-text staging system classifies tumors based on the number of contiguous liver sections free of disease (minus four equals pre-text stage)↗
▶Ep 11 · 6:51
clinicalPre-text 1 has three contiguous sections free of disease, pre-text 2 has two, pre-text 3 has one, and pre-text 4 has no free sections or diffuse tumor↗
▶Ep 11 · 8:15
clinicalPre-text 3 and 4 tumors represent a big game changer in practice that improved survival from less than 30% in the 1970s-80s to 80-90% today↗
▶Ep 11 · 8:15
clinicalPre-text 3 tumors should generally be biopsied at diagnosis, started on neoadjuvant chemotherapy, then referred to transplant center or center with expertise in advanced liver resections↗
▶Ep 11 · 9:35
clinicalIt is important to consult a transplant center early for pre-text 3 and 4 tumors↗
▶Ep 11 · 9:35
quoteYou refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program, uh 15 years ago because teams are are trained to recognize the tumors and send them to a center that offers transplant um um or or can and can do aggressive resection.↗
▶Ep 11 · 10:27
clinicalThe PHITT study has been ongoing for three years↗
▶Ep 11 · 10:27
clinicalThe PHITT (Pediatric Hepatic International Tumor Trial) is an ongoing multinational trial to identify high and low risk features for each pre-text stage↗
