BILIARY DISEASES presented by Dr.Daniel Asiimwe.pptx

Conditions affecting the Biliary tree

1. BILIARY DISEASES DR. Daniel Asiimwe Surgery Department

2. Aetiology of biliary diseases Factors causing liver failure: Include toxic accumulation of : Metabolic waste (ammonia & bilirubin ) Drugs & toxins

3. Aetiology Hepatitis Could be infective (chemical, viral or alcoholic) Cirrhosis Usu. Final end stage of many liver diseases Portal hypertension Common complication of cirrhosis associated with severe bleeding & liver failure. Stones Forms in GB & may pass into bile duct-obstruction

4. Pathogenesis Because of the large functional reserve, it takes large liver loss to cause symptoms Insults to the liver will present with; Inflammation Degeneration – fat Necrosis (infarction) Fibrosis leading to cirrhosis

5. Pathophysiology Jaundice: Occurs when bilirubin > 2mg/dl Types: Prehepatic jaundice Cause is due to hemolysis

6. Pathophysiology cont. Hepatic jaundice Major cause: Viral hepatitis Toxins (poisons) Therapeutic drugs, cirrhosis Neoplasms

7. Pathophysiology cont. Post hepatic jaundice Major cause: Choledocolithiasis Pancreatic cancer Choledocal tumors Stenosis of CBD

8. Pathophysiology cont. Cholestasis : Stagnation of bile flow. characterised by jaundice & pruritus Increased alkaline phosphatase

9. Path. cont Liver failure: May lead to death within few weeks or months Due to sudden or chronic insult to liver Follows loss of > 90% of liver function characterised by jaundice, ascites,

10. Path. Cont. Other symptoms: Bleeding disorders Hepatic encephalopathy

11. Path. Cont. Cirrhosis: Fibrotic scar tissues dividing the liver into nodules . Fibrosis is progressive Irreversible and incurable

12. Path. Cause of cirrhosis: Portal system Alcohol abuse Chronic viral hepatitis (B & C) Inherited metabolic disease Hematochromatosis Wilson disease Autoimmune hepatitis

13. Path. Biliary system : Biliary obstruction Gallstones Cystic fibrosis Autoimmune disease Primary biliary cirrhosis Sclerosing cholangitis

14. Clinical features Portal hypertension – due to obstruction of portal blood flow by cirrhosis: Esophageal & gastric varices Splenomegaly Periumbilical varices (caput medusa)

15. c/f Failure to metabolize estrogen & ammonia Thin hair Gynaecomastia palmar erythema Absent or reduced pubic hair Small testes

16. c/f. Protein synthesis failure Ascites & edema ( hypoproteinemia ) Purpura (low clotting factors) Bile excretory failure jaundice

17. Other liver diseases Hepatitis: Viral hepatitis (HBV & HCV) Chemical hepatitis Alcoholic hepatitis Liver abscesses Liver neoplasms

18. BILIARY TRACT DISEASES Broadly divided into Intra hepatic biliary tract diseases Extra hepatic biliary tract diseases

19. PRIMARY SCLEROSING CHOLANGITIS Idiopathic, fibrosis inflammatory condition aff ecting both intra/ extrahepatic ducts. Associated with hypergammaglobulinaemia , elevated markers (smooth muscle antibodies, antinuclear factor) Immunological contribution. Majority 30-60 yrs M>F Associated with IBD, inflammatory bowel disease; >>> Ulcerative colitis.

20. Presentation RUQP/discomfort Jaundice Pruritus Fever Fatigue Weight loss

21. Investigations Complete blood count LFT’s: Cholestatic pattern (raise in ALP alkaline phosphatase , GGT γ-glutamyl transferase) small raise in the aminotransferase. Bilirubin levels varies MRCP/ERCP demonstrate structuring and beading Liver biopsy confirming diagnosis, guide therapy and exclude cirrhosis.

22. Treatment PSC Antibiotics ABx Vitamin K Cholestyramine Steroids Immunosuppressants: Azathioprine though unsuccessful Endoscopic stenting Surgical resection Liver transplantation >80% 5-year survival

23. DISEASES OF EXTRA-HBDs Biliary atresia Cholangitis choledocolithiasis

24. Biliary atresia Neonatal & childhood disorder Commonest cause of neonatal cholestasis Occurs in 1:10000 live births

25. Pathology Presents with complete destruction or absence of bile duct There will be inflammation & fibrotic strictures of hepatic or CBD Common cause of death in childhood liver d’se Presents with features of biliary obstruction on biopsy.

26. Complications of EHBA Cholestasis Retention of bile componets Reduction of bile in intestine

27. Extrahepatic Biliary Atresia Type I: atresia of the CBD Type II: atresia of the CHD common hepatic duct Type III: atresia of the right and left hepatic ducts RHD/LHD

28. Clinical Features ≈⅓ jaundice at birth, progressive deepening LFT’s reveal obstruction ( raised Bilirubin, Alkaline phosphatase, pale meconium, dark urine, pruritus Clubbing, skin xanthoma (high cholesterol)

29. Treatment Direct Roux-en-Y hepaticojejunostomy achieve bile flow in ≈75% in 10% of type I In tye II, III, require Kasai procedure Kasai procedure: radical excision of all bile duct tissue unto the liver capsule Roux-en-Y jejunal loop anastomosed to the exposed live capsule above the bifurcation of the portal vein, creating a portoenterostomy.

30. BILE STONES GALLSTONES

31. Cholelithiasis Gall stones in the gall bladder

32. GALLSTONES Most common biliary pathology Affect 10-15% of western population Prevalence rate of 17% in UK Asymptomatic in >80% 1-2% of asymptomatic develop symptoms necessitating surgery

33. TYPES OF GALLSTONES Cholesterol : 80% in Europe Pigment (brown/black): 80% in Asia Mixed stones (51-99% pure cholesterol mixed with calcium salts, bile acids, bile pigments and phospholipid

34. Pigment stones Contain <30% cholesterol Black and brown Black compose of insoluble bilirubin pigment polymer + Calcium phosphate + calcium bicarbonate. 20-30% black, incidence increases with age Ass. With haemolysis (hereditary spherocytosis, SCD, cirrhosis.

35. Pigment stones Brown pigment contain calcium bilirubinate, calcium palmitate, calcium stearate, cholesterol. Rare in the gallbladder, form in the bile duct —> bile stasis, infected bile Stones form by deconjugation of bilirubin deglucuronide by bacterial β-glucuronidase ( insolube unconjugated bilirubinate precipitates) Associated with foreign bodies (prosthesis/stents, parasites Clonorchis sinuses, Ascaris lumbricoides )

36. Aetiology Risk factors Race – whites > blacks Advancing age Sex; F > M Rapid weight reduction GB stasis, Hyperlipidemia Biliary infections

37. CAUSES Increased cholesterol secretion and supersaturation of bile (Obesity, high-caloric diets, oral contraceptives Resection of terminal ileum Nucleation of cholesterol monohydrate crystals from multilamellar vesicles Abnormal gallbladder emptying.

38. Clinical presentation Asymptomatic, incidental on imaging RUQ or epigastric pain, radiating to the back: dull, constant >>> colicky 10-25% biliary colic. ± chest pain, pain severe lasting minutes - hours. Starts at night and awakes the patient Dyspepsia, flatulence, altered bowel frequency, nausea/vomiting

39. Clinical presentation Jaundice occurs if the stones migrates and obstructs the CBD Rarely bowel obstruction (biliary ileum) Diagnosis of acute cholecystitis should be made when symptoms persist.

40. Diagnosis History and examination Positive Murphy’s sign (acute inflammation, leukocytosis, raised LFT,s A palpable, non-tender gallbladder (Courvoisier’s sign) reflects a serious diagnosis Imaging with transabdominal sonography, radionuclide scans, Abdominal X-ray, Computed Tomography, MRCP

41. Treatment Observation if asymptomatic Prophylactic cholecystectomy for Diabetic, Cholecystectomy in symptomatic patients

42. Non-operative treatment >90% of acute cases subside with conservative options NPO , IV fluid therapy Analgesics Antibiotics ( cefazolin , cefuroxime, ciprofloxacillin

43. Surgery Surgery when inflammation has subsided (early 1 wk Vs delayed 6 wks)

44. Complications of gallstones Biliary c olic pain Cholecystitis : Acute and chronic Empyema gallbladde r Perforation Biliary obstruction Acute cholangitis Acute pancreatitis Intestinal obstruction (gallstone ileus)

45. CHOLECYSTITIS Acute cholecystitis Most common cplxn of gallstones and about 90% are ppt by stone obstructions and rest by infection or tumors GB will be distended and inflammed There will be pain in the right hypochondrial region Fever, nausea and vomiting.

46. Cholecystitis Chronc cholecystitis +/- H/O acute attack Almost always assoc with gallstones +/- biliary obstruction Mild to moderate RUQ pain Nausea and vomiting Intolerance to fatty foods

47. cholecystitis MGT Medical treatment Analgesics antibiotics Surgical treatment Cholecystectomy is the treatment of choice Open or Laparoscopic

48. Surgery Surgery when inflammation has subsided (early 1 wk Vs delayed 6 wks)

49. CHOLEDOCHOLITHIASIS

50. Causes/Risk factors Similar to those of Cholilithiasis but stone migrates into the CBD Post-cholecystectomy choledocholithiasis Associated with foreign bodies (prosthesis/stents, parasites Clonorchis sinuses, Ascaris lumbricoides ) Any obstruction to the flow of bile.

51. Consequences Obstruction to bile flow Infection CBD stones have 80% infection rate than gallstones

52. Presentation Asymptomatic Bouts of pain RUQP, jaundice, fevers : Charcot’s triad Cholangitis is related to Charcot’s triad Tenderness in epigastrium and RUQ

53. Management Ultrasonography , LFT’s, Liver biopsy, ERCP, MRI/MRCP to rule out cause of jaundice. Coagulation profile, FBS, RBS Supportive: rehydration, broad spectrum antibiotics

54. Treatment ERCP: Endoscopic papillotomy by sphincterotomy, stone removal using a Dormia basket or placement of stent with difficult stone removal Percutaneous transhepatic cholangiography —> drainage and later percutaneous choledochotomy. Choledochotomy is now rarely used except in resource limiting settings.