Surgical Jaundice

Definition, aetiological, investigation and management

1. Seminar Outline General Overview Surgical Anatomy of the Gall Bladder and Biliary System Common Pathologies involving Gall Bladder and Biliary System Differentiating Types of Jaundice Clinical Features in Obstructive Jaundice Investigations in Obstructive Jaundice Management of Obstructive Jaundice

2. General Overview Normal serum bilirubin – 0.2-0.8 mg/ dL Surgical jaundice – Any jaundice amenable / correctable by surgical intervention Majority due to extrahepatic biliary obstruction However not all obstructive jaundice is surgical jaundice (hepatitis) and not all surgical jaundice is due to obstruction (congenital spherocytosis).

3. Pear-shaped hollow structure with a normal capacity of 35-50ml 7 – 10 cm in length, 2.5 – 5 cm in diameter Undersurface of liver, mainly at the junction of left and right lobes of liver Surgical Anatomy of the Gall Bladder and Biliary System Gall bladder

4. Anterior view Posterior view

5. Gall bladder Divided into 4 parts: (i) Fundus (ii) Body (iii) Infundibulum - Hartmann pouch (iv) Neck - s-shaped as it joins the cystic duct

6. Blood supply Arterial blood supply -  cystic artery  which arises from the right hepatic artery The flow of the arterial blood: aorta  celiac trunk  common hepatic artery  right proper hepatic artery  cystic artery  

7. Venous drainage Cystic vein  drains blood from the gallbladder and, accompanying the cystic duct, usually ends in the right branch of the portal vein Not present, blood drains via small (cholecystohepatic) veins in the gall-bladder bed directly to the parenchyma of the liver

8. Lymphatic drainage The lymph from the gallbladder drains to the cystic lymph node , which is often enlarged when the gallbladder is inflamed Mascagni’s or Lund’s node lies in the imaginary hepatobiliary Calot’s triangle Often removed together with the inflamed gallbladder

9. Innervation Parasympathetic fibers the hepatic branch of the vagus nerve Sympathetic fibers arise from celiac plexus Sensory  innervation is provided by the right phrenic nerve

10. Calot’s triangle (hepatobiliary triangle) 3 margins Medially - common hepatic duct) Laterally - cystic duct Superiorly - inferior margin of the liver

11. 3 contents: - Cystic artery - Right hepatic artery - Cystic lymph node ( Lund’s node ) Calot’s triangle (hepatobiliary triangle)

12. Calot’s triangle ( hepatobiliary triangle) Clinical significance: Dissected during cholecystectomy Contents must be identified for ligation

13. Hepatobiliary tree

14. Main functions of gall bladder Bile reservoir Fasting resistance through sphincter is high bile diverted to gall bladder Food intake 1. Cholecystokinin (CCK) secretion stimulated by presence of fats in duodenum stimulates gallbladder contractions & common bile duct bile flows into duodenum 2. Secretin secreted in response of acid in duodenum stimulates biliary duct cells to secrete bicarbonate and water increases bile volume increase flow to duodenum

15. Main functions of gall bladder Concentration of bile Absorption of water, NaCl , and bicarbonate 5-10 times more concentrated Increase in proportion of bile salts, bile pigments, cholesterol, and calcium Secretion of mucus

16. Adult humans produce 400-800ml of bile per day Humans can actually survive without a gallbladder

17. Common Pathologies involving Gall Bladder and Biliary System Congenital : Biliary atresia , choledochal cyst . Inflammatory : Ascending cholangitis , sclerosing cholangitis. Obstructive : Cholelithiasis , Common bile duct stones , biliary strictures Neoplastic : cholangiocarcinomas , gall bladder carcinoma Extrinsic compression of common bile duct: Lymph nodes or tumours .

18. Congenital Abnormalities of the Gall Bladder and Bile Ducts

19. Extrahepatic Biliary Atresia Atresia: abnormal narrowing. Present approximately in 1:12000 live births and affect males and females equally. The aetiology is unknown. Classes Class I: atresia restricted to the common bile duct. Class II: atresia of the common bile duct. Class III: atresia of the right and left hepatic ducts.

20. Clinical feature About 1/3 of the patients are jaundice at birth. More commonly, jaundice present by the end of first week and deepens progressively. Associated with Bile stained meconium Pale stool Dark urine Severe pruritis Clubbing Skin xanthomas

21. Choledochal Cyst Congenital dilatations of the intra/ extrahepatic biliary system. Classification

22. Clinical Feature Jaundice Fever Abdominal pain Physical examination Right upper quadrant mass Investigation Ultrasonography confirm presence of an abnormal cyst MRI Reveal the anatomy. Particularly on the relationship between lower end of the bile duct and pancreatic duct. CT scan useful for delineating the extent of the intra/ extrahepatic dilatations. Treatment Radical excision of the cyst with reconstruction of the biliary tract

23. Cholelithiasis (Gall Stones) Most common biliary pathology Gall stones Cholesterol stones Pigment stones Brown pigment stones Black pigment stones Mixed stones

24. Cholesterol Stones Cholesterol is secreted from the canalicular membrane in phospholipid vesicles. Insoluble in water. Risk factors Obesity High calorie diet Abnormal gastric emptying

25. Pigment Stones Black pigment stones Insoluble bilirubin Pigment polymer Mixed with calcium phosphatase and calcium carbonate Associated with hemolysis Hereditary spherocytosis Sickle cell anemia Brown pigment stones Calcium bilirubinate Calcium palmitate Calcium stearate Cholesterol Formed in bile duct, formation of stones is associated with Bile stasis Infected bile

26. Clinical features Location : RUQ/ epigastric pain Character: Colicky but more often dull and constant Associated symptoms: Dyspepsia Flatulence Food intolerance ( fatty food ) Altered bowel habits Progression: Biliary Colic Severe RUQ pain Nuasea /Vomiting Pain radiates to the chest Pain during midnight

27. Complication In the gall bladder Biliary colic Acute cholecystitis Chronic cholecystitis Empyema of the gall bladder Mucocele In the bile ducts Biliary obstruction Acute cholangitis Acute pancreatitis In the intestine Intestinal obstruction Gall stones ileus Differential diagnosis Common Acute appendicitis Perforated gastric ulcer Acute pancreatitis Uncommon Acute pyelonephritis Myocardial infarction Pneumonia

28. Cholecystitis Inflammation of the gall bladder that occurs most commonly because of an obstruction of the cystic duct from cholelithiasis . Calculous cholecystitis Acalculous cholecystitis Obstruction of the cystic duct from cholelithiasis Risk factors Increasing age Obesity Drugs Pregnancy Associated with Biliary stasis Debilitation Major surgery Severe trauma Sepsis Long-term total parenteral nutrition Prolonged fasting Diabetes mellitus

29. Clinical Features Acute upper abdominal pain Begins in the epigastric region and then localized to the RUQ Colicky in nature Associated with fever May radiate to the shoulder or scapula On examination Tachycardia RUQ tenderness Guarding and rebound tenderness Jaundice (not very common) Complication Perforation Gangrene Peritonitis

30. Cholangitis Is an infection of the biliary tract. Pathophysiology Choledocholithiasis is the most common cause of biliary tract obstruction resulting in cholangitis Biliary tract manipulations/ stents Hepatobiliary malignancies Clinical presentations Fever with chills and rigor RUQ pain Jaundice c Charchot triad

31. Sclerosing Cholangitis Idiopathic fibrosing inflammatory condition of the biliary tree affecting both intra/ extrahepatic ducts. Strongly associated with inflammatory bowel disease, especially ulcerative colitis. May associated with Hypergammaglobulinaemia Elevated markers such as Smooth muscle antibody Anti-nuclear factors Majority patients are between 30-60 years of age.

32. Common symptoms RUQ discomfort Jaundice Pruritis Weight loss Fever Fatigue Investigations Liver function test Elevated serum alkaline phosphatase Elevated gamma- glutamyl transferase Elevated aminotransferase Ultrasonography Cholangiography Endoscopic retrograde cholangiopancreatography Demonstrating stricture and beading of the bile ducts. Treatment Vitamin K Steroids Immunosuppressant Surgery Endoscopic stenting Liver biopsy is helpful in confirming the diagnosis by excluding cirrhosis

33. Stricture of the Bile Duct Causes Congenital Biliary atresia Bile duct injury during surgery Cholecystrectomy Choledochostomy Gastrectomy Hepatic resection Transplantation Inflammation Stones Cholangitis Pancreatitis Sclerosing cholangitis Trauma Idiopathic

34. Stones in the Bile Ducts Aetiology May occur many years after a cholecystrectomy . Infection of the biliary tree Clinical features Fever RUQ pain Jaundice . Courvoisier’s law States that in obstruction of the common bile duct due to a stone, distention of the gall bladder seldom occurs. In fact, it is usually shrunken

35. Tumors of the Bile Duct Benign Papilloma and Adenoma Most common benign neoplasm arises from the glandular epithelium lining of the bile ducts. Papillomatosis Rare conditions Presence of multiple mucus secreting tumors of the biliary epithelium. Patient often presents with obstructive jaundice. Tumors have malignant potential and must be resected if possible.

36. Malignant Carcinoma can arise from any parts in the biliary tree, from common bile duct to small intrahepatic ducts. Usually is adenocarcinoma ( cholangiocarcinoma ) which predominantly located in the extrahepatic biliary system. Slow growing tumor which invade locally and metastasise to local lymph nodes. Incidence Rare malignancy Overall annual incidence is 1:100000 with 2/3 of the patients being older than 65 years old. Association History of ulcerative colitis Sclerosing cholangitis Cholecdochal cyst Clinical features Jaundice Abdominal pain Early satiety Weight loss

37. Investigation Biochemical investigation Elevated bilirubin Elevated phosphatase Elevated gamma- glutamyl transaminase Tumor Marker : CA 19-9 Ultrasonography and CT scan Detect the level of obstruction Loco regional extent of disease Presence of metastasis Percutaneous transhepatic cholangiography Percutaneous drainage for cytology Treatment Resection Prognosis Median survival is 18 months 20% of the patient survive 5 years post resection Adjuvant chemotherapy and radiotherapy has limited role.

38. Carcinoma of the Gall Bladder Rare disease Affect elder patient, 60-70 years old. Unknown aetiology Associated with calcification of the gall bladder Pathology Adenocarcinoma Tumors are nodular and infiltrative Thickening of gall bladder wall Tumor spread by Direct extension into the liver Involvement of the perihilar lymphatics Neural plexuses Clinical features Jaundice Anorexia Weight loss Palpable mass

39. Investigation Non specific investigations Anemia Leucocytosis Elevated ESR Elevated C-reactive protein Elevated CA 19-9 (80%) Diagnosis Ultrasonography and defined by multidetector row CT scan Percutaneous biopsy confirming the histological changes. Treatment Radical resection Prognosis Median survival less than 6 years

40. Tumors of the Bile Duct Gall Bladder CA Rare Present with Jaundice and weight loss Diagnosis by ultrasonography and CT scan Surgical excision is only possible in 5% of the patients Poor prognosis Rare Present as benign biliary disease(gall stones) Surgical excision is less than 10% of the patients Poor prognosis

41. Types of jaundice 1) Pre-hepatic jaundice - disruption happens before bilirubin has been transported from the blood to the liver - caused by conditions such as sickle cell anaemia and haemolytic anaemia 2) Intra-hepatic jaundice (hepatocellular jaundice) - disruption happens inside the liver - caused by conditions such as Gilbert's syndrome and liver cirrhosis 3) Post-hepatic jaundice (obstructive jaundice) - disruption prevents the bile (and the bilirubin inside it) from draining out of the gallbladder and into the digestive system - caused by conditions such as cholelithiasis (gallstones) or tumours

42. Pre-hepatic Hepatic Post-hepatic Excessive amount of bilirubin is presented to the liver due to excessive haemolysis Impaired cellular uptake, defective conjugation or abnormal secretion of bilirubin by the liver cell Impaired excretion due to mechanical obstruction to bile flow Elevated unconjugated bilirubin in serum Both conjugated and unconjugated bilirubin may be elevated in serum Elevated conjugated bilirubin in serum Types of Jaundice

43. Type Pre-hepatic Hepatic Post-hepatic Urine colour Normal Dark Dark Stool colour Normal Normal Acholic (Putty- coloured / greyish-yellow) Pruritus No No Yes Types of Jaundice

44. Pre-hepatic Hepatic Post-hepatic Haemolytic Anaemia , Sickle-cell Anaemia Hepatitis, cirrhosis, hepatocellular diseases etc. Gallstone, malignancy, inflammation Types of Jaundice

45. Types of Jaundice Other types of jaundice: Pathologic Jaundice when jaundice presents a health risk in adults / children may be pre-hepatic / hepatic / post-hepatic Gilbert Syndrome harmless hereditary condition results in mild jaundice due to low levels of bilirubin-processing enzymes in their livers does not require further medical treatment

46. Differentiating Types of Jaundice

47. Clinical Features in Obstructive Jaundice Consider: Patients' ages and associated conditions Presence or absence of pain Location and characteristics of the pain Acuteness of the symptoms Presence of systemic symptoms ( eg , fever, weight loss) Symptoms of gastric stasis ( eg , early satiety, vomiting, belching) History of anaemia Previous malignancy Known gallstone disease Gastrointestinal bleeding Hepatitis Previous biliary surgery Diabetes or diarrhoea of recent onset Commonly - pale stools , dark urine , jaundice & pruritus Explore use of any alcohol , drugs, and medications.

48. History Family history of jaundice with anaemia (haemolysis ) - Hereditary spherocytosis Gilbert’s Familial non- hemolytic hyperbilirubinemia Back Pain : 25% of patients with carcinoma pancreas (relieved by sitting Whitish clay- colored stools : suggestive of Obstructive Jaundice Melena : Periampullary carcinoma ( silver paint stool ) Charcot’s triad : Intermittent jaundice, pain, intermittent fever

49. History of infections , drug abuse , tattoos, blood transfusion (Hepatitis B ) Past History of biliary surgery (Post-operative stricture ) History of omphalitis (inflammation of the navel) Infection of Umblicus  incomplete obliterations of umbilical vein  jaundice History of drugs : Chloropromazine , Methyltestosterone

50. Examination Yellow discoloration: sclera , skin , nail bed, posterior part of the hard palate, under surface of the tongue Presence of scratch mark - in the lower limbs, chest and abdomen (accumulation of bile salts) Migratory thrombophlebitis ( Trosseau’s sign seen in carcinoma pancreas) Stigmata of liver disease – spider angioma , ascites, collateral veins on the abdomen and splenomegaly Distended gall bladder Look for supraclavicular nodal enlargement

51. Investigation of Obstructive Jaundice HEE YAN HAN BMS 14091168

52. Investigations in Obstructive Jaundice General Full Blood Count – Anaemia , signs of infection, haemoglobulinopathy Serum electrolyte, urea & creatinine Liver function test - Bilirubin (Direct  - obstruction) - Raised serum albumin (  A/G Ratio) Urinalysis – Bilirubin, urobilinogen Faecal occult blood test (Carcinoma of ampulla of pancreas) Coagulation profile – PT, PTT, INR Hepatitis serology ( HbsAg , HCV)

53. Imaging Plain radiographs – little value Abdominal ultrasonography – 1 st -line imaging in jaundice detect liver abnormalities, hepatosplenomegaly and gallstones identify extrahepatic causes of biliary obstruction Identify intrahepatic disease e.g. malignancy

54. Endoscopic ultrasound (EUS) – detailed imaging of pancreas and biliary tree, tissue sampling via fine needle aspiration (EUS-FNA) Computed tomography (CT) scan - more accurate than US to determine specific cause & level of obstruction

55. Magnetic resonance cholangiopancreatography (MRCP) – test of choice in obstructive jaundice

56. Endoscopic retrograde cholangiopancreatography (ERCP) – diagnose benign & extrahepatic obstruction, relieve obstruction

57. Percutaneous transhepatic cholangiography (PTC) – evaluates suspected biliary obstruction when ERCP is unsuccessful.

58. Liver Biopsy Laparoscopic / percutaneous To stage primary biliary cirrhosis Laparotomy

59. Management of Obstructive Jaundice Medical : Depends on underlying cause Surgical : When indicated Indications for surgery – Resectable * Palliation if unresectable

60. Pre-Operative Management Proper diagnosis and assessment Injection vitamin K IM 10 mg for 5 days Fresh Frozen plasma ‐ 6 bottles or more Blood transfusion (if anaemic) Oral neomycin, lactulose IV Mannitol 100‐200 ml BD to prevent hepatorenal syndrome Adequate hydration Repeated monitoring by doing prothrombin time, serum electrolytes Antibiotics e.g. 3 rd generation cephalosporins Calcium supplements e.g. IV Calcium chloride

61. Surgical Management Modalities Triple Bypass Whipple Procedure ERCP / Stenting CBD exploration (CBDE) + Choledochojejunostomy (CDJ) CBDE + T Tube Percutaneous transhepatic biliary drainage + Palliative Hepatojejunostomy

62. Percutaneous transhepatic biliary drainage

63. Choledocholithiasis / cholecystolithiasis – Cholecystectomy (Open / Laparoscopic) Carcinoma of head of pancreas Early: Whipple procedure, Pancreaticoduodenectomy + Pancreaticojejunostomy + Gastrojejunostomy + Cholecystojejunostomy Late: Triple bypass surgery Cholangiocarcinoma - Hepaticojejunostomy Carcinoma of ampulla of Vater – Whipples procedure Chronic pancreatitis – Subduodenal exploration, sphincterectomy , stent insertion Liver transplantation

64. Whipple Procedure Pylorus-Preserving Pancreaticoduodenectomy (PPPD)

65. Triple Bypass Consisting choledochojejunostomy ( cholecystojejunostomy ), gastrojejunostomy , and pancreaticojejunostomy

66. Cholecystectomy

67. Post-Operative Care Monitoring with prothrombin time, bilirubin, albumin,creatinine , electrolyte estimation FFP or blood transfusion Antibiotics Observe for septicaemia, haemorrhage, pneumonia, pleural effusion, bile leak Care of T-tube and drains T‐tube cholangiography in 10‐14 days TPN, CVP line, nasogastric tube, urinary catheter

68. T Tube –drainage of bile leaks post-operatively

69. References Blumgart L.H., Surgery of Liver and Biliary Tract Bailey & Love, Short Practice of Surgery, 25th edition, 2008 Boyd, Surgical Pathology Sheila Sherlock, Diseases of Liver and Biliary System Rodney Maingot , Textbook of Adbominal Operations, 11th edition, 2007 Anderson A.R., Randomized trial of endoprosthesis versus operative bypass in malignant obstructive jaundice Trede M., The Surgical treatment of pancreatic carcinoma surgery Sonnenfield , Byberg B., The effect of palliative biliodigestive operation for unresectable pancreatic cancer War-Shaw A.L., preoperative staging and assessment of resectability of pancreatic cancer Shapiro T.M., Adenocarcinoma of pancreas – a statistical analysis of biliary bypass versus Whipple resection in good risk patients, Annals of Surgery Christ D.W., Current status of pancreaticoduodenectomy for periampullary hepatogastroenterology Eastman M.C. Keene, The objective of palliative surgery in pancreatic cancer – a retrospective study of 73 cases, Australian and NewZealand journal of surgery. 13 Cotton P, Leung J ( Eds ) Advanced Digestive Endoscopy: ERCP. Oxford: Blackwell Publishing Ltd, pp. 1–8.