Greg Tiao

509 statements · 12 topics · summaries given as host listed separately

Biliary Atresia · guest expert Liver Tumors (Hepatoblastoma/HCC) · guest expert Neuroblastoma · guest expert Wilms Tumor · guest expert

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The NASPGHAN guidelines, which is what most of the North American pediatric GI people are going to, in theory, follow, actually recommends not to get a HIDA scan because there's always a concern, especially in a 50-day-old, that if you tried to load the patient with phenobarb, you delay treatment by potentially five, six days.

quote · Biliary Atresia

We really try not to divide anything that is easily demonstrable. What I mean by that is if you think it's a vessel, don't divide it because you'd be surprised how oftentimes you think it's nothing and it turns out to be a segmental artery of importance.

quote · Biliary Atresia

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.

quote · Hepatoblastoma

The absence of A gallbladder on ultrasound does not conclusively show biliary atresia. The corollary to that, of course, is the presence of a gallbladder doesn't exclude biliary atresia

quote · Cholestasis

The most common lesion is hemangioma. Even though hepatoblastoma is the most common malignant liver tumor in children, it's still pretty rare with about 250 cases per year.

quote · Hepatoblastoma

EOVIST is an MRI contrast agent that is taken up by the hepatocytes and then excreted. It can provide the best definition when looking at the liver on imaging.

quote · Liver Tumors (Hepatoblastoma/HCC)

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Episode 19 - Interview with Dr Marc Levitt, Chief of Colorectal & Pelvic Reconstruction, Children‘s

Statement Dr. Stevens operated on Greg Ryan when he was a baby. Open

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Episode 19 - Interview with Dr Marc Levitt, Chief of Colorectal & Pelvic Reconstruction, Children‘s

Statement Dr. Stevens operated on Greg Ryan when he was a baby. Open

Greg's statements about Biliary Atresia 249 statements

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Biliary Atresia

Statement 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). Open

Quotation If you actually looked at studies from the 70s and 1980s, idiopathic neonatal hepatitis would be about 70% of the patients. Now it's probably down to 10% Open

Statement 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. Open

Quotation The absence of A gallbladder on ultrasound does not conclusively show biliary atresia. The corollary to that, of course, is the presence of a gallbladder doesn't exclude biliary atresia Open

Statement 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. Open

Statement 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. Open

Statement 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. Open

Quotation At our hospital we actually don't use HIDA scans and in fact if we order a HIDA scan, Dr. Ballesterri, our senior most hepatologists, looks upon it quite poorly. Open

Statement 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. Open

Quotation If you see that, the confirmatory, I mean the likelihood of it being biliotresia is well over 90+%. It's very rare that you will have a misdiagnosis at this stage. Open

Statement 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. Open

Statement 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. Open

Statement At Cincinnati, the workup for biliary atresia is limited to liver biopsy, labs, and ultrasound before posting the patient for surgery. Open

Statement 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. Open

Quotation This is what the Japanese will call an extended dissection, where they'll go to the first bifurcation of the vessels. This is actually the proper way or the way Kasai. Described it Open

Statement Japanese centers uniformly perform extended dissections, which may explain why their Kasai outcomes are better than those in the United States. Open

Statement 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. Open

Statement Data from England by Mark Davenport showed that cystic variants of biliary atresia have better outcomes if intervention occurs before 30 days of age. Open

Statement Choledochal cyst patients will have pigmented stool, while cystic variant biliary atresia patients will become acholic, providing a simple clinical differentiation. Open

Statement 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. Open

Quotation 20 is not good for a transplant team because almost always you'll lose 5 to 78 centimeters of length on your root at the time of the hilar dissection. Open

Statement 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. Open

Statement Anti-refluxing valves for the Roux limb have not been proven effective. Open

Quotation It's pretty clear cut that when they localized or focused on the group just 3 centers doing their biliatresis, that the 3 centers that did more than 5 cases a year had. Much better outcomes than those who had less. Open

Statement 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. Open

Statement 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. Open

Statement 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. Open

Statement 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). Open

Quotation The Children's Network, which is the national biliotreia Research Consortium, did a multi. Center trial in which they had about 70 patients enrolled in two different arms and they showed clearly that there was no improvement of drainage in terms of corticosteroid use between those who got it and those who didn't. Open

Statement Despite negative trial results, there is a subset of biliary atresia patients with an inflammatory gene profile in which steroids might prove beneficial. Open

Statement Cincinnati does not use MRCP for biliary atresia diagnosis due to having a high-capacity pathology team. Open

Statement Performing Kasai on Alagille syndrome patients will shorten the natural history of their native liver and result in earlier transplantation. Open

Quotation If you cassa and Alis patients, you will shorten their natural. History of that liver staying with them and they will be transplanted sooner. That's actually fairly clear cut. Open

Statement 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. Open

Biliary Atresia with Dr. Greg Tiao

Quotation Biliaryresia is an uncommon disease, results in an obstructive cholangiopathy of the biliary system. Open

Statement Biliary atresia is the most common cause for end-stage liver disease in children Open

Statement Biliary atresia is unique to infancy Open

Statement Biliary atresia is an uncommon disease that results in an obstructive cholangiopathy of the biliary system Open

Statement Biliary atresia is the most common cause for end-stage liver disease in children Open

Statement Biliary atresia is unique to infancy Open

Statement Biliary atresia is an uncommon disease that results in an obstructive cholangiopathy of the biliary system Open

Statement The etiology of biliary atresia is uncertain Open

Statement Biliary atresia causes a biliary epithelial injury process that causes biliary obstruction Open

Quotation 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. Open

Statement Untreated biliary atresia causes progressive cholestasis, portal fibrosis, eventually cirrhosis, portal hypertension, and can cause death by the age of 2 years Open

Quotation In the Far East, it's been estimated that 1 in 8000 live births will result in a patient with biliatresia. Open

Statement In the Far East, biliary atresia incidence is estimated at 1 in 8000 live births Open

Quotation In the Far East, it's been estimated that 1 in 8000 live births will result in a patient with biliatresia. Open

Statement In the Far East, biliary atresia incidence is estimated at 1 in 8000 live births Open

Statement In the United States, biliary atresia incidence is 1 in 15,000 live births Open

Quotation In the West, or especially in the United States, it's less common where the incidence is 1 in 15,000 live births. Open

Statement In the United States, biliary atresia incidence is 1 in 15,000 live births Open

Quotation In the West, or especially in the United States, it's less common where the incidence is 1 in 15,000 live births. Open

Statement Infants with biliary atresia typically present with jaundice as the first clinical manifestation Open

Quotation The child or the infant is typically jaundiced. That's the first clinical manifestation. Open

Statement Patients with biliary atresia have acholic or very pale gray stools Open

Statement Patients with biliary atresia have dark urine Open

Statement The liver in biliary atresia becomes quite firm and is usually palpable in the right costochondral margin Open

Statement Biliary atresia typically presents between 1 to 2 months of age Open

Statement Screening can pick up biliary atresia at an earlier stage and has been demonstrated to improve outcomes Open

Quotation And that screening has, has been demonstrated to improve the outcome of patients who are detected at an earlier age. Open

Statement Screening can pick up biliary atresia at an earlier stage and has been demonstrated to improve outcomes Open

Statement The most common reason an infant has jaundice is physiologic jaundice of the newborn Open

Statement Indirect hyperbilirubinemia indicates physiologic jaundice Open

Statement Direct hyperbilirubinemia indicates a more pathologic process Open

Quotation If it's a direct hyperbilirubinemia, then you actually have to be worried that there's a more pathologic process underway. Open

Statement Direct hyperbilirubinemia indicates a more pathologic process Open

Statement Anatomic causes of direct hyperbilirubinemia include biliary atresia, choledochal cysts, and inspissated bile syndrome Open

Statement Hepatocellular causes of direct hyperbilirubinemia include viral hepatitis, sepsis, PFIC syndromes, alpha-1 antitrypsin deficiency, tyrosinemia, and transport abnormalities Open

Statement Workup for biliary atresia includes liver profile with GGT, alpha-1 antitrypsin genotype, and TORCH workup Open

Statement Patients are screened for cystic fibrosis, hypothyroidism, and galactosemia as part of perinatal screening Open

Quotation More recently, from a laboratory standpoint, there's been some New studies showing that the protein MMP 7, matrix metallorotinase 7, is a new biochemical marker that has very high diagnostic sensitivity and specificity for biliary. Open

Statement Matrix metalloproteinase 7 (MMP-7) is a new biochemical marker with very high diagnostic sensitivity and specificity for biliary atresia Open

Statement Ultrasound is the first imaging test typically obtained for biliary atresia workup Open

Statement HIDA scan is fairly sensitive but has quite low specificity for biliary atresia and can delay workup and diagnosis Open

Quotation The HIDA scan was used for many years, but that test is fairly sensitive, but its specificity is quite low. Open

Statement HIDA scan is fairly sensitive but has quite low specificity for biliary atresia and can delay workup and diagnosis Open

Quotation And so it's usually not utilized because it can actually delay the workup. And diagnosis of bilioresia. Open

Quotation And then of course the gold standard for biliaryresia is a liver biopsy. Open

Statement Liver biopsy is the gold standard for diagnosing biliary atresia Open

Quotation And then of course the gold standard for biliaryresia is a liver biopsy. Open

Quotation The classic findings of biliotresia on that biopsy is expansion of the perioral space with mononuclear cells. Open

Statement 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 Open

Statement Bile duct plugs are the pathognomonic finding of biliary atresia on biopsy Open

Quotation You'll see bile duct proliferation and then the pathomnemonic finding of bilioresia is bile duct plugs within those proliferating perioral biliary ducts. Open

Statement Treatment options for biliary atresia are surgical and include Kasai portoenterostomy or liver transplantation Open

Statement As long as the patient reaches the OR before 70-75 days, the success rate of Kasai procedure justifies the small morbidity risks Open

Quotation Most teams will find that as long as they get the patient to the OR before 70, 75 days, the success rate of the procedure justifies the small morbidity risks of the procedure. Open

Statement As long as the patient reaches the OR before 70-75 days, the success rate of Kasai procedure justifies the small morbidity risks Open

Quotation Most teams will find that as long as they get the patient to the OR before 70, 75 days, the success rate of the procedure justifies the small morbidity risks of the procedure. Open

Statement In older patients with manifestations of cirrhosis including ascites and coagulopathy, consideration for primary transplant is warranted Open

Statement Both Kasai and transplant procedures require an intraoperative cholangiogram to establish anatomy and visualize patency of the common hepatic duct and intrahepatic components Open

Statement 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 Open

Statement The hilar plate in biliary atresia has a triangle-like appearance extending from the common hepatic duct to a broader base Open

Statement The hilar plate is divided in one sharp transection right proximal to Glisson's capsule Open

Statement A 30 to 35 centimeter Roux-en-Y limb is created and brought up in a retrocolic fashion for hilar plate reconstruction Open

Statement The most worrisome complication of Kasai is vascular injury to the portal vein or hepatic artery Open

Quotation We really try not to divide anything that is easily demonstrable. What I mean by that is if you think it's a vessel, don't divide it because you'd be surprised how oftentimes you think it's nothing and it turns out to be a segmental artery of importance. Open

Statement 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 Open

Statement Other complications of Kasai include bowel obstruction and wound issues Open

Statement Cholangitis is a longer-term concern after Kasai, but requires adequate bile flow to occur Open

Quotation One of the concerns we have longer term is cholangitis. But of course, for cholangitis, you actually have to have adequate bile flow to even begin to have that. Open

Statement Cholangitis after Kasai is presumed to be an ascending process and can be suppressed with antibiotics Open

Statement In the United States, most Kasai patients are discharged home within 5 to 7 days after the procedure Open

Statement Post-Kasai patients require multidisciplinary care with both surgeon and gastroenterologist follow-up Open

Statement Post-Kasai patients are on prophylactic antibiotics, Actigall, and require nutritional support including fat-soluble vitamins and elemental diets Open

Quotation The definition of a successful cassai is a direct bilirubin under 2 at 3 months of age. Open

Statement A successful Kasai is defined as direct bilirubin under 2 at 3 months of age Open

Quotation The definition of a successful cassai is a direct bilirubin under 2 at 3 months of age. Open

Statement A successful Kasai is defined as direct bilirubin under 2 at 3 months of age Open

Quotation What we're looking for immediately post-op is a pigmented stool. If we see that, we know the patient is draining Open

Statement Immediate post-operative success indicator is a pigmented stool, which indicates the patient is draining Open

Biliary Atresia Part I

Statement A high GGT is more consistent with an obstructive process and helps guide toward eliminating PFIC (progressive familial intrahepatic cholestasis) diagnoses. Open

Statement At 50 days of age, physiologic jaundice from the newborn period would have resolved. Open

Quotation We're fortunate in Cincinnati that we have an excellent ultrasound team and they will look for a triangle chord sign, but we really don't find that to be too consistent. Open

Statement Cincinnati Children's Hospital has not used HIDA scans for biliary atresia diagnosis in approximately 25 years. Open

Quotation The NASPGHAN guidelines, which is what most of the North American pediatric GI people are going to, in theory, follow, actually recommends not to get a HIDA scan because there's always a concern, especially in a 50-day-old, that if you tried to load the patient with phenobarb, you delay treatment by potentially five, six days. Open

Statement 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. Open

Statement Significant fibrosis on liver biopsy reflects disease progression and may guide surgical decision-making. Open

Statement Liver exteriorization during Kasai portoenterostomy can create denser, more vascularized adhesions that complicate future liver transplantation. Open

Quotation I've seen some of the consequences of that, where you just seem to see a little bit more dense adhesions. And again, depending on the stage of the liver disease patients, those things can become quite vascularized. Open

Statement Liver exteriorization during Kasai portoenterostomy can create denser, more vascularized adhesions that complicate future liver transplantation. Open

Statement The only chance a child with biliary atresia has to avoid early transplant is a well-done Kasai portoenterostomy. Open

Statement Even in the best hands, long-term drainage rate after Kasai portoenterostomy is only 70-75%. Open

Quotation The problem with Kasai is even open, that because the drainage rate is not so ideal, even in the best hands, it's only 70%, 75% long-term. Open

Quotation The only chance this kid has to avoid transplant early on is a well-done Kasai. And so, that's where I think you really have to be thinking about the patient's best interest when you consider this. Open

Statement Even in the best hands, long-term drainage rate after Kasai portoenterostomy is only 70-75%. Open

Statement The only chance a child with biliary atresia has to avoid early transplant is a well-done Kasai portoenterostomy. Open

Statement Peterson ran a prospective randomized trial comparing laparoscopic to open Kasai and stopped the trial early. Open

Quotation The problem with Kasai is even open, that because the drainage rate is not so ideal, even in the best hands, it's only 70%, 75% long-term. Open

Statement 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. Open

Statement Dr. Neo published a paper describing different depths of hilar plate transection in Kasai portoenterostomy. Open

Statement The hilar plate should be transected at the level of Glisson's capsule, leaving it intact, without cutting into the liver parenchyma itself. Open

Statement Damage to hepatic artery branches during Kasai dissection can exacerbate the underlying liver disease process. Open

Statement The hilar plate should be transected at the level of Glisson's capsule, leaving it intact, without cutting into the liver parenchyma itself. Open

Quotation We do not dig into the liver. I think Dr. Neo wrote a paper just a few years ago where he described the different depth of the hilar plate transection. Open

Statement The Roux anastomosis suture choice does not make a difference in outcomes. Open

Statement For the portoenterostomy anastomosis, 6-0 Maxon suture is used with all knots tied on the outside. Open

Statement The Roux anastomosis suture choice does not make a difference in outcomes. Open

Statement For the portoenterostomy anastomosis, 6-0 Maxon suture is used with all knots tied on the outside. Open

Biliary Atresia Part II

Quotation I think you've got to be very careful when you make those kind of statements. Because it was a properly designed randomized trial. I mean, Georgians work to do this. It's not a small number. It's 110 patients in each arm. Open

Statement Cincinnati molecular profiling identified two biliary atresia phenotypes: inflammatory (potentially steroid-responsive) and fibrotic (less likely to respond) Open

Statement Cincinnati antibiotic protocol: cephalosporin until taking PO, then Bactrim or amoxicillin for minimum 3 months post-op Open

Statement START trial antibiotic protocol: treatment dose for 2 weeks, then prophylaxis for 6 months Open

Statement Cincinnati approach: clinical assessment with consideration of biopsy fibrosis; will explore surgically unless significant cirrhosis on pre-op biopsy; some 120-day patients have done well while some 60-day patients had advanced disease Open

Quotation I've done 120 day olds who actually used liver was actually reasonable and the patients are still training. Open

Statement Cincinnati cholangitis protocol: IV antibiotics, consider short course of steroids if no response after several days Open

Statement Cholangitis management: early recognition critical, families instructed to seek immediate medical attention for fever, not wait at home Open

Statement Cincinnati cholangitis protocol: IV antibiotics, consider short course of steroids if no response after several days Open

Quotation If they have an episode of cholangitis, which is the biggest complication that can affect the long-term efficacy of a KASAI, we would bring them in early for antibiotics. Open

Statement Cholangitis management: early recognition critical, families instructed to seek immediate medical attention for fever, not wait at home Open

Quotation If they have an episode of cholangitis, which is the biggest complication that can affect the long-term efficacy of a KASAI, we would bring them in early for antibiotics. Open

Statement Cincinnati performs revision Kasai only in highly selected patients: those who previously cleared jaundice and normalized bilirubin, then became acholic after cholangitis; 25-patient series showed native liver salvage possible, some patients 5-10 years out with native liver Open

Statement Transplant referral timing: if bilirubin has not dropped below 2 at 3 months post-Kasai, refer to transplant center for evaluation even if not immediately listing Open

Statement Cincinnati mouse model identified peptide sequence common to rotavirus, CMV, and EBV that may govern host immune response in biliary atresia Open

Update Course Rewind: MMP-7 & Biliary Atresia Diagnosis 2024

Quotation What 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. Open

Statement Timely diagnosis of biliary atresia is critical to prolong native liver survival Open

Quotation What 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. Open

Statement Timely diagnosis of biliary atresia is critical to prolong native liver survival Open

Statement Cincinnati Children's stopped doing HIDA scans about 25 years ago Open

Statement HIDA scan adds about 5 days to the diagnostic workup Open

Statement Doctor Bezarra screened about 1000 proteins in the Children's Network registry and found 70 or so that were elevated in the biliary atresia population, of which MMP-7 was the most clear Open

Statement MMP-7 is now a validated biomarker for biliary atresia but is not perfect Open

Quotation It's not perfect. Open

Statement The sensitivity of MMP-7 is pretty good, but cutoffs vary because the assay is still evolving Open

Quotation Our 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. Open

Statement At Cincinnati Children's, the commitment is to perform Kasai within 7 days of a biliary atresia patient showing up Open

Quotation Our 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. Open

Statement At Cincinnati Children's, the commitment is to perform Kasai within 7 days of a biliary atresia patient showing up Open

Statement A European study showed that performing Kasai before 45 days reduces the incidence of transplant need Open

Statement Kasai portoenterostomy done before 45 days is the goal Open

Statement Cincinnati Children's will perform Kasai before 30 days if diagnosis can be established Open

Statement A direct bilirubin over 1 in the newborn period is very sensitive for biliary atresia Open

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