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Neonatal Jaundice Biliary Atresia

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Slide 1 :

Neonatal Jaundice & Biliary Atresia Nancy Chokshi MSIII

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Neonatal Jaundice 1) Increased hemolysis 2) Increased RBC mass 3) Decreased RBC survival 4) Enzyme deficiencies in metabolism of bilirubin

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Time Frame for Jaundice Early – 1-2 days Normal – 3-10 days Late – 14 or more days Criteria for physiologic jaundice - occurs after first day of life - rarely exceeds 12.9-15mg/dl - unconjugated - resolves by 1 week

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Treatment Physiologic jaundice - phototherapy - exchange transfusion - increase oral feeds Prevention - mental retardation, cerebral palsy hearing loss

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What is it? Inflammation of the bile ducts leading to obliteration of the extrahepatic biliary tract 2 types currently described Syndromic BA – associated with other congenital abnormalities Non-syndromic BA – biliary damage is isolated; more common Etiology of both forms remains unknown at this time Occurrence rate of 1/10,000 – 1/20,000 live births

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Anatomical Types of BA Type 1 – atresia is limited to common bile duct Type 2 – cysts in liver hilum communicate with intrahepatic bile ducts Type 3 – cystic duct, common bile duct, and gallbladder are patent Type 4 – complete extrahepatic biliary atresia

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Signs and Symptoms Jaudice which lasts greater than 2 weeks of life Acholic stools Dark urine Hepatomegaly Later findings associated with portal hypertension

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Labs Total and conjugated bilirubin ALT, AST Serum alkaline phosphatase GGPT CBC Metabolic screening Urinalysis

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Imaging Studies Ultrasound is the initial test of choice – assess any abnormalities of the heptabiliary tract Gallbladder length – accuracy 71%, sensitivity 72%, specificity 69% Triangular cord sign –sensitivity 100% and specificity 100%

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Triangular Cord Sign

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HIDA scan Infants with biliary atresia usually have normal uptake of the isotope but absent excretion into the biliary system and small intestine Enhance isotope excretion with 5 days of pretreatment with phenobarbital Liver Biopsy Recommended before surgical procedure portal tract edema, fibrosis, inflammation, intracellular and canalicular cholestasis, proliferation of bile ductules

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Laparotomy with intraoperative cholangiography Gold standard for the diagnosis of BA If intact extrahepatic biliary system is not visualized,then extrahepatic biliary atresia is evident. If an intact biliary tree is visible, then perform an intraoperative cholangiogram cannulate the bile duct through transverse abdominal incision and inject contrast to determine if the biliary ducts are patent At the porta hepatis there are microscopic bile ductules that have proliferated which communicate with the intrahepatic system

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Correctable lesion – 20% fibrosis of the distal biliary tree, however proximal biliary tree and intrahepatic bile ducts are patent Excise fibrotic area and direct drainage to bowel Non – correctable – 80% fibrosis to the level of the porta hepatis Kasai procedure

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Kasai procedure (hepatoportoenterostomy) Improves nutrition, growth, delays liver transplantation Success is dependent on age of procedure and experience of the center at where it is performed Best if performed before 10-12 weeks of life Roux-en-Y hepatoportoenterostomy where the porta hepatis is attached to a loop of bowel after resection of the fibrotic biliary system Creates an anastomoses of the liver directly to the bowel so that bile can flow from the liver into the bowel

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Prognosis Some patients produce adequate bile flow; may not need liver transplant Another group has moderate bile flow, but remains jaundiced; will need liver transplant Finally a third group of patients will not establish bile flow; will need liver transplant Survival after Kasai procedure at 5 years is 30-55% and after 10 years is 30-40% Prognosis after liver transplant is 70-80% at both 5 and 10 years

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References Abrams, Stephanie, and Robert J Shulman. “Approach to neonatal cholestasis” UpToDate. 2006 Akasaka, Yoshinobu, et al. “Sonographic Diagnosis of Biliary Atresia in Pediatric Patients Using the "Triangular Cord" Sign Versus Gallbladder Length and Contraction”. American Journal of Roentgenology 181 (2003): 1387-1390. Doherty, Gerard, and Lawrence W. Way ed. Current Surgical Diagnosis & Treatment 2003. Katb, Magd, et al. “Evaluation of the Triangular Cord Sign in the Diagnosis of Biliary Atresia.” Pediatrics 108 (2001): 416-420

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Some patients produce adequate bile flow; may not need liver transplant; Another group has moderate more Some patients produce adequate bile flow; may not need liver transplant; Another group has moderate bile flow, but remains jaundiced; will need liver … less