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Biliary Atresia

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A small presentation on Biliary atresia and Procedure of Kasai

Published in: Health & Medicine

Biliary Atresia

  1. 1. Biliary Atresia Diagnosis, Management protocols and Recent advances Dr Ravi Kanojia PGIMER, Chandigarh India
  2. 2. Objectives <ul><li>Spectrum of surgical jaundice </li></ul><ul><li>How do we diagnose EHBA </li></ul><ul><li>Pre op management by the surgical team </li></ul><ul><li>Post op management </li></ul><ul><li>Prognostic factors </li></ul><ul><li>Follow up </li></ul><ul><li>PGI experience </li></ul><ul><li>Message and lessons learnt </li></ul><ul><li>Recent advances in EHBA </li></ul><ul><li>we will not be talking on </li></ul><ul><ul><li>Surgical details and options </li></ul></ul><ul><ul><li>Liver transplant </li></ul></ul>
  3. 3. Common case scenario <ul><li>Term 60 days old female infant </li></ul><ul><li>Jaundice since birth </li></ul><ul><li>Passing pale white stools with mustard color urine </li></ul><ul><li>Palpable firm hepatomegaly </li></ul><ul><li>Referred by a Pediatrician after a exhaustive non conclusive work up spending precious prognostic days </li></ul>
  4. 4. Surgical causes of jaundice <ul><li>Biliary atresia </li></ul><ul><li>Choledochal cyst </li></ul><ul><li>Bile duct perforation </li></ul><ul><li>Inspissated bile syndrome </li></ul>
  5. 5. <ul><ul><li>Alpha1-anti-trypsin deficiency </li></ul></ul><ul><ul><li>Idiopathic neonatal hepatitis </li></ul></ul><ul><ul><li>Algillie syndrome </li></ul></ul><ul><ul><li>Inborn errors of bile acid synthesis </li></ul></ul><ul><ul><li>Nonsyndromic intrahepatic bile duct hypoplasia </li></ul></ul><ul><ul><li>Total parenteral nutrition–associated (TPN) cholestasis </li></ul></ul><ul><ul><li>Viral infections (eg, toxoplasmosis, other infections, rubella, cytomegalovirus infection, and herpes simplex [TORCH]) </li></ul></ul>Other Causes
  6. 6. Diagnosis <ul><li>Clinical Suspicion by </li></ul><ul><ul><li>Prolonged jaundice </li></ul></ul><ul><ul><li>Conjugated hyperbilirubinemia </li></ul></ul><ul><ul><li>Acholic stools dark urine </li></ul></ul><ul><ul><li>Firm to hard palpable hepatomegaly </li></ul></ul><ul><ul><li>Antenatally by abnormal maternal usg </li></ul></ul><ul><ul><ul><li>Cyst in the porta </li></ul></ul></ul><ul><ul><ul><li>Spleenic malformations </li></ul></ul></ul>
  7. 7. <ul><li>Investigations </li></ul><ul><ul><li>Non invasive & Invasive </li></ul></ul><ul><ul><ul><li>Non invasive </li></ul></ul></ul><ul><ul><ul><ul><li>Biliary USG </li></ul></ul></ul></ul><ul><ul><ul><ul><li>HIDA/Mebrofennin Scan </li></ul></ul></ul></ul><ul><ul><ul><ul><li>MRCP </li></ul></ul></ul></ul><ul><ul><ul><li>Invasive </li></ul></ul></ul><ul><ul><ul><ul><li>Liver biopsy </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Per op cholangiogram </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Serial Duodenal intubations </li></ul></ul></ul></ul><ul><ul><ul><ul><li>ERCP </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Laparoscopy </li></ul></ul></ul></ul><ul><li>Hematology </li></ul><ul><ul><li>Routine LFT </li></ul></ul><ul><ul><li>Gamma GGT </li></ul></ul><ul><ul><li>Sr Lipoprotien X </li></ul></ul><ul><ul><li>TORCH </li></ul></ul><ul><li>Minimum recommended (PGI Practice) </li></ul><ul><ul><li>USG + HIDA (with priming) + hematology (>85% sensitive for pre op diagnosis) </li></ul></ul><ul><ul><li>Per op cholangiogram </li></ul></ul>
  8. 8. Biliary USG <ul><li>Triangular Cord Sign </li></ul><ul><li>GB motility </li></ul><ul><li>GB length </li></ul>Measure GB length remains same before and after feeding All the 3 factors combined gives positive predictive value of > 90% JPS (2007) 42, 2093–2096
  9. 9. HIDA <ul><li>Hepatobiliary excretion scans </li></ul><ul><li>Pretreatment with phenobarb 5mg/kg/d for 3-5 days </li></ul><ul><li>Simultaneous administration of UDCA may be done </li></ul><ul><li>DISIDA – not reliable when sr bil levels are very high (>10) </li></ul><ul><li>99mTc-mebrofenin IDA </li></ul><ul><li>Recent reports of technetium-99m-mebrofenin iminodiacetate scans combined with estimation of gama GGT increases the negative predictive value to > 80% </li></ul>
  10. 10. MRCP <ul><li>Is done when usg demonstrates cysts at porta </li></ul><ul><li>Reliably differentiates between choledochal cyst and cystic variants of EHBA </li></ul>
  11. 11. Liver Biopsy <ul><li>90% sensitivity and specificity for biliary atresia. </li></ul><ul><li>Biopsies are not usually diagnostic in those younger than 2 weeks </li></ul><ul><li>Portal bile ductular proliferation, bile plugging, portal-portal fibrosis, and an acute inflammatory reaction </li></ul><ul><li>Not our preferred practice because most of the patients coming late and have a deranged coagulogram and practical problems </li></ul>
  12. 12. Per op cholangiogram <ul><li>Decision algorithm </li></ul>? EHBA Laparotomy Bile in GB Aspirate GB if patent with lumen White bile Obtain Cholangiogram Proceed for Kasai Small non patent fibrotic GB EHBA confirmed
  13. 13. Duodenal Intubation <ul><li>Aspiration of duodenal contents via an endoscope </li></ul><ul><li>Preferred Japanese practice </li></ul><ul><li>Reliably rules out BA if bile is seen aspirate </li></ul><ul><li>Sensitivity can be increased by combining with HIDA – if aspirated contents shows some radioactivity then BA is ruled out </li></ul>
  14. 14. ERCP <ul><li>Pediatric scope looking for ampula of vater and trying for retrograde dye study </li></ul><ul><li>Technical limitations are there with the scope as well as the skill </li></ul><ul><li>No added advantages over other investigations </li></ul>
  15. 15. Laparoscopy <ul><li>Has a role in obtaining a POC with minimally invasive technique </li></ul><ul><li>KPE procedure can be accomplished through it </li></ul><ul><li>Major advantages offered are </li></ul><ul><ul><li>Magnification while dissecting at the porta </li></ul></ul><ul><ul><li>Minimal adhesions at the time of liver transplant </li></ul></ul>
  16. 16. Diagnosis pathway followed Clinical Suspicion (conjugated jaundice), acholic stools Hepatobiliary USG Hepatic nuclear excretion scans If no bilioenteric drainage POC and proceed
  17. 17. Pre op management protocol <ul><li>Physicians role </li></ul><ul><ul><li>Identify </li></ul></ul><ul><ul><ul><li>Conjugated jaundice + pale stools  go for USG followed by HIDA </li></ul></ul></ul><ul><ul><li>EARLY REFERAL </li></ul></ul>
  18. 18. Pre op management <ul><ul><li>Phenobarbitone </li></ul></ul><ul><ul><li>Vit k </li></ul></ul><ul><ul><li>Antibiotics </li></ul></ul><ul><ul><li>Nutrition </li></ul></ul>
  19. 19. Post operative management <ul><li>Supportive </li></ul><ul><li>Pharmacological options improving outcomes </li></ul><ul><ul><li>Phenobarbitone </li></ul></ul><ul><ul><li>UDCA </li></ul></ul><ul><ul><li>Steroids </li></ul></ul><ul><ul><li>Vitamin supplements </li></ul></ul><ul><ul><li>Antibiotics </li></ul></ul>
  20. 20. Role of steroids <ul><li>Basis </li></ul><ul><ul><li>Reduces inflamation </li></ul></ul><ul><ul><li>Immune suppression </li></ul></ul><ul><ul><li>Cholerectic action- increases canalicular electrolyte transport and stimulate bile flow independent of the bile salt concentration. </li></ul></ul><ul><li>With these basis it </li></ul><ul><ul><li>Decreases protal fibrosis </li></ul></ul><ul><ul><li>Increased bile flow </li></ul></ul><ul><ul><li>Decreasing cholangitis </li></ul></ul><ul><li>Our practice </li></ul><ul><ul><li>Not routinely prescribed </li></ul></ul><ul><ul><li>In the event of cholangitis or persistent white stools 2mg/kg/d prednisolone </li></ul></ul>
  21. 21. Prognostic factors <ul><li>Age at presentation </li></ul><ul><li>Bile duct size at porta after excision of the fibrotic cord </li></ul><ul><li>Liver histology at the time of KPE </li></ul><ul><li>Presence of portal hypertension </li></ul><ul><li>Associated malformations </li></ul>MAP Score JPS, Vol31, No 10,1996: pp 1387-1390
  22. 22. Follow up protocol <ul><li>Repeat LFT at 1 mth </li></ul><ul><li>Repeat HIDA at 3 mth </li></ul><ul><li>3 monthly review subsequently </li></ul><ul><li>Registration for LTx registry </li></ul>
  23. 23. Recent Advances <ul><li>Ductal plate malformation – aberrant morphological distribution of intrahepatic bile ducts seen using immunohistochemical staining for cytokeratin 19 (CK-19) in liver biopsy </li></ul>
  24. 24. <ul><li>Recent advances in molecular pathology </li></ul><ul><ul><li>Increased expression of ICAM AND VCAM molecules in the sinusoidal epithelium </li></ul></ul><ul><ul><li>Now serum measurements of the molecules is possible </li></ul></ul><ul><ul><li>Few candidate genes </li></ul></ul><ul><ul><ul><li>jagged 1 mutation </li></ul></ul></ul><ul><ul><ul><li>CFC </li></ul></ul></ul><ul><ul><ul><li>HNF-6 </li></ul></ul></ul><ul><ul><ul><li>INV </li></ul></ul></ul>
  25. 25. PGI Experience <ul><li>15 pt in single unit in 14 mth </li></ul><ul><li>Age range 1-6 mth </li></ul><ul><ul><li>Mean age 87 days </li></ul></ul><ul><li>Mean bil levels </li></ul><ul><ul><li>10.4 (range 6-17) </li></ul></ul><ul><li>Predominant usg findings </li></ul><ul><ul><li>Small contracted GB or gb not visualised </li></ul></ul><ul><ul><li>Cysts at the porta </li></ul></ul><ul><li>HIDA scan </li></ul><ul><ul><li>Good hepatocyte uptake with absent billio enteric drainage </li></ul></ul>
  26. 26. Atretic GB with complete atresia of the EHBT Ty IIA Extrahepatic cyst with proximal atresia Per op findings 12 1 2
  27. 27. <ul><li>Outcome (short term) </li></ul><ul><ul><li>All passed yellow stools after the procedure </li></ul></ul><ul><ul><li>Persistent jaundice (levels les than pre op) </li></ul></ul><ul><ul><li>Appetite increased </li></ul></ul><ul><ul><li>Gaining weight </li></ul></ul><ul><ul><li>Mortality none </li></ul></ul>
  28. 28. Lessons learnt <ul><li>Poor prognosis even after good bile flow </li></ul><ul><li>Surgery improves quality of life in terms of feeding and thriving </li></ul><ul><li>Prognosis does not has a marked difference with early v/s late operated patients </li></ul><ul><li>Early sx reduces the incidence of complications, post op cholangitis, better response to drug treatment and delayed appearance of secondary PHT </li></ul>
  29. 29. Kasai Portoenterostomy <ul><li>Video part 1 </li></ul>
  30. 30. Kasai Portoenterostomy <ul><li>Video part 2 </li></ul>
  31. 31. Kasai Portoenterostomy <ul><li>Video part 3 </li></ul>
  32. 32. Thanks

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