EXTRAHEPATIC BILIARY ATRESIA D and M.pptx

EHBA

1. EXTRAHEPATIC BILIARY ATRESIA DIAGNOSIS AND MANAGEMENT DR. MAYANK RAJ KARN RESIDENT 1 ST YEAR ( GENERAL SURGERY)

2. CONTENT CLASSIFICATION DIFFERENTIAL DIAGNOSIS APPROACH TO EHBA MANAGEMENT TIMING OF LIVER TRANSPLANT

3. Japanese classification : ( Based on level of most proximal Obstruction)

4. DIFFERENTIAL DIAGNOSIS

5. DIAGNOSIS Children with persistent jaundice should be promptly evaluated to exclude diagnosis of EHBA. Combination of detail clinical assessment , blood test, imaging studies and pathological evaluation is used. Final diagnosis is always confirmed at surgical exploration .

6. DIAGNOSTIC APPROACH

7. BLOOD TEST Liver Function Test: Both direct and indirect Bilirubin are raised.(Direct bilirubin conc. > 1mg/dl if TSB >5mg/dl ; >20% of TSB when TSB >5mg/dl) Increased transaminases( mildly) Increased ALP Increased GGT To rule out other condition: Screening of perinatal infection (TORCH) Screening of alpha 1 antitrypsin deficiency

8. ULTRASONOGRAPHY Done in fasting state ( to allow filling of GB) Look for: Echotexture of liver Presence of ascites Patency of hepatic vasculature Anatomy of biliary structure

9. TRIANGULAR CORD SIGN Focal echogenic triangular or ovoid density just cranial to portal vein bifurcation. >4mm thickening considered positive. 74% sensitive, 97% specific .

10. GALL BLADDER GHOST TRIAD Short GB (<1.9cm ) Thin and indistinct GB wall Irregular or lobular contour 85% sensitive, 92% specific 95% sensitive and 89% specific when both sign combined.

11. HEPATIC ARTERY CHANGES Larger hepatic artery caliber (>2mm) Hepatic artery to portal vein diameter ratio >0.45 signifies hepatic artery enlargement .

12. HEPATOBILIARY SCINTIGRAPHY( HIDA SCAN) Investigation of choice Tc 99m is used Assess excretion of bile from liver into small intestine. DIDISA ( Diisopropyl iminodiacetic acid) scan : more effective .

13. LIVER BIOPSY Differentiate between N eonatal Hepatitis and EHBA

14. INTRAOPERATIVE CHOLANGIOGRAM: Gold standard

15. NORMAL INTRAOPERATIVE CHOLANGIOGRAM

16. SURGERY??? CANDIDATE FOR POSSIBLE KASAI PORTOENTEROSTOMY INTRAOP FINDING IOC FINDING

17. PREOPERATIVE PREPARATION Monitor Coagulation profile Supplimentation of fat soluble vitamins (A,D,E,K) or Inj Vit K IM (1mg) Broad spectrum antibiotic before incision

18. TREATMENT KASAI PORTOENTEROSTOMY The standard initial operation. Excision of the entire extrahepatic biliary tree with transection of the fibrous portal plate near hilum of liver. Bilioenteric continuity is then reestablished with Roux- en -Y limb. GOAL: Allow drainage of bile from liver into Roux limb via microscopic ductules in the portal plate.

19. OPERATIVE STEPS Incision : Right sided, muscle cutting to cross midline ( Kocher Incision). Mobilization of liver. Porta H epatis Dissection Right side dissection should show the bifurcation into anterior and posterior vascular pedicles. left side dissection is facilitated by dividing the spur of liver tissue linking segment IV and III to allow the origin of umbilical vein from left portal vein to be displayed.

20. ROUX LOOP Preparation Portoenterostomy : Wound closure.

21. POSTOPERATIVE ADJUVANT THERAPY Steroid Controversy: Steroids resulted in accelerated clearance of jaundice but did not improve in survival of native liver. Adverse sequelae of steroid use not commonly reported. Evidence supporting steroid use is currently stronger than the evidence against it.

22. OUTCOMES Factors to effect the outcome of Kasai Portoenterostomy : Age at operation: Goal (before 70-90 days of life). Operative Technique Severity of liver disease Gross and microscopic aspects of biliary tree and portal plate

23. Initial observation is color of stool . Pigmented stools either immediately or within 10-14 days after surgery suggest successful bile flow. Occur in 2/3 rd of patient. Among these patient half will continue to have good drainage whereas half of patient will develop jaundice and progressive liver failure . 1/3 rd of patient who never experienced clinically relevant bile drainage, jaundice continue to worsen.

24. COMPLICATIONS Ascending Bacterial Cholangitis Nutritional complication Portal hypertension and its complications Hepato pulmonary syndrome

25. LIVER TRANSPLANTATION

26. INDICATION OF LIVER TRASPLANTATION : Early failed KPE Late diagnosis of EHBA Failure to thrive Recurrent bacterial cholangitis Portal Hypertension Hepatopulmonary syndrome Hepatorenal syndrome

27. Reference : Pediatric Surgery 7 th edition : Arnold G.Coran Sabiston textbook of Surgery 21 st edition Biliary Atresia : Mark Davenport S. Roy Choudhary , Pediatric Surgery

28. THANK YOU..