M Hussnain Raza, Portal Hypertension.pptx

Liver

1. International Higher School of Medicine Name: Muhammad Hussnain Raza Group: 11 Subject: Surgical Diseases Topic: Portal Hypertension

2. Portal Hypertension

3. Portal vein anatomy The portal vein is formed in front of IVC and behind the neck of the pancreas ( at the level of 2 nd lumber vertebra ) by union of the splenic & SMV. It is 7-8 cm in length & contains no valves. It courses in the lesser omentum posterior to both the common hepatic artery & common bile duct.

4. Portal vein anatomy It bifurcates into the Lt & Rt trunks in , or just below the hillum of the liver. Its main tributaries are : The coronary (Lt gastric) vein. Pyloric vein. Cystic vein. Pancreaticodudenal vein. Ligamentum teres (umbilical vein). Ligamentum venosum.

5. Portal vein anatomy

6. Portal vein anatomy The portal venous branches ramify in an arterial like pattern and end in dilated channels called sinusoids, which are equivalent to systemic capillaries. From here blood drains into the hepatic venous system. The normal portal pressure is 5-7 mmHg ( 8-12 cm of water ) . Portal hypertension is present when the portal vein pressure exceeds 12 mmHg

7. Causes of portal hypertension Increased resistance to flow A) Pre-hepatic (portal vein obstruction): 1- congenital atresia or stenosis. 2- thrombosis of portal vein. 3- thrombosis of splenic vein. 4- Extrinsic compression (e.g , tumor).

8. Causes of portal hypertension B) Intra-hepatic: 1- liver cirrhosis  obstruction is sinusoidal & post-sinusoidal. 2- bilharzial periportal fibrosis  obstruction is pre-sinusoidal.

9. Causes of portal hypertension The causes of portal hypertension in cirrhotic patients are: Diminution of the total vascular bed by obliteration, distortion, & compression of sinusoids. Compression of the tiny radicals of portal & hepatic veins by excessive fibrosis. Development of multiple arteriovenous shunts between the branches of the hepatic artery & portal vein.

10. Causes of portal hypertension C) Post-hepatic: 1- Budd-Chiari syndrome ( hepatic vein thrombosis )  prominent ascites ,hepatomegaly & abdominal pain. 2- Veno-occlusive disease. 3- Cardiac disease: a- constrictive pericarditis. b- valvuar heart disease. c- right heart failure.

11. Causes of portal hypertension Increased portal blood flow A) Arterial-portal venous fistula. B) Increased splenic flow: 1- Banti’s syndrome ( liver disease secondary to splenic disease, result from cirrhosis & other hepatic disorders) 2- Splenomegaly (e.g tropical splenomegaly ,hematologic diseases).

12. Sequelae & Clinical picture 1- Porto-systemic collaterals: In normal conditinos  collapsed. In portal hypertension  engorged  divert blood away from the portal circulation.

13. Sequelae & Clinical picture The important sites of these collaterals are: a) At the lower end of oesophagus Oesophageal tributaries of Lt gastric vein (portal) Oesophageal tributaries of hemiazygous vein (systemic). b) Around the umbilicus Para umbilical vein (portal) Superior & inferior epigastric veins (systemic)

14. Sequelae & Clinical picture c) Lower rectum & analcanal Superior rectal vein (portal) Middle & Inferior rectal veins (systemic) d) At the back of the colon Rt & Lt colic veins (portal) Rt & Lt renal veins (systemic) e) Retroperitoneum Tributaries of superior & inferior mesentric veins { Retzius } (portal) Posterior abdominal & subdiaphragmatic veins (systemic)

15. Portal vein collaterals

16. Sequelae & Clinical picture 2- Splenomegaly The most constant physical finding. In 80% of patients regardless the cause. * In Bilharzial cases : at 1 st due to reticuloendothelial hyperplasia due to absorption of bilharsial toxins. With progress of portal hypertension  due to congestion.

17. Sequelae & Clinical picture 3- Congestion of the whole GIT Leads to anorexia, dyspepsia, indigestion, and malabsorption. 4- Bleeding varices. 5- Ascites (multifactorial) Portal hypertension alone cannot cause ascites. Hypoalbuminaemia  below 3 gm/100 ml. Salt &water retention  high level of aldosterone, oestrogens & anti-duretic hormone. Increased lymphatic transudation from liver surface.

18. Investigations 1. Assessment of liver function tests (a) Hypoalbuminaemia. The liver is the only site of albumin synthesis. (b) ALT & AST are moderately raised. (c) Prothrombin time and concentration are disturbed. This test is the most sensitive liver function.

19. Investigations 2. Detection of oesophageal varices by: (a) Fibreoptic upper endoscopy (b) Barium swallow can visualize varices in 90% of cases. They appear as multiple, smooth, rounded filling defects (honey-comb appearance) (c) Duplex scan can show dilated portal vein and collaterals .

20. Detection of oesophageal varices

21. Investigations 3. Detection of splenic sequestration and hypersplenism. (a) Blood picture  anaemia, leucopenia, thrombocytopenia or pancytopenia. (b) Bone marrow examination  hypercellularity. (c) Radioactive isotope studies : using the patients own RBCs tagged with 51 Cr  diminished half life of RBCs & increased radioactivity over the spleen.

22. Investigations 4. Diagnosis of the aetiology of liver disease is performed by: (a) Immunological tests for hepatitis markers. (b) Liver biopsy after assessment of prothrombin time and concentration.

23. Child Pugh classification Points 1 2 3 Bilirubin (mg/dL) < 2 2 – 3 > 3 Albumin (g/dL) > 3.5 2.8 – 3.5 < 2.8 Prothrombin time (seconds) 1 – 3 4 – 6 > 6 Ascites None Slight Moderate Encephalopathy None Minimal Advanced Grade A, 5-6 points; Grade B, 7-9 points; Grade C, 10-15 points

24. Treatment Management of patients with actively bleeding oesophageal varices 1. Admission. The patient should be admitted to hospital. 2. Resuscitation. A wide bore cannula is inserted. A blood sample is taken. Restoration of blood volume should be rapid. Overexpansion of the circulation should be avoided . Morphine and Pethidine are contraindicated.

25. Treatment 3. Correct coagulopathy. Vitamin K is administered intravenously. 4. Prevent encephalopathy. Blood in the intestine will be fermented to ammonia and other nitrogenous products. Repeated enemas. Oral lactulose. This is a disaccharide sugar, fermented by the intestinal flora  lactic acid  combines with ammonia. Neomycin 0.5 gm every 4 hours can reduce the bacterial flora.

26. Treatment Sclerotherapy Intra- or Para- Variceal. 1-3 ml sclerosant (ethanolamine oleate). Occludes venous channels. Multiple sessions (2 weekly). Control bleeding in 80-95 %. About 50% rebleed. 30% complication rate.

27. Endoscopic Sclerotherapy Intra-variceal Para-variceal

28. Complications of Sclerotherapy LOCAL Ulceration. Stricture. Perforation. Retrosternal discomfort for few days. SYSTEMIC Fever Pneumonitis CNS

29. Treatment Endoscopic Banding Occludes venous channels Sessions < sclerotherapy Same results as sclerotherapy ↓ complications vs sclerotherapy Endoscopic treatment of choice

30. Endoscopic Banding

31. Treatment Drugs Vasopressin  vasoconstriction of the splanchnic circulation. Dose  0.2 unit/kg wt, dissolved in 200 ml of 5% dextrose, over 20 minutes. Disadvantages colicky abdominal pains, & diarrhoea . anginal pains, so it is contraindicated in the elderly. Produce temporary control of bleeding in about 80% of cases. To prolong its action it is combined with glycine ( Glypressin ) .

32. Treatment Somatostatin lower the intravariceal pressure without significant side effects. Initial bolus 100 microgram  continuous infusion of 25 microgram/ h for 24 hs. Beta blockare ↓ bleeding by ↓ cardiac output. Does 20-60 mg bid  25% ↓ in HR. Reduces 40% of bleeding episodes Does not reduce mortality

33. Treatment Balloon tamponade by Sengestaken or Linton tube. The gastric balloon is inflated first by 200 ml of air, and pulled upwards to press the gastric fundus. If bleeding continues, the oesophageal balloon is inflated. The pressure in the oesophageal balloon should not exceed 40 mm Hg. This therapy is effective in controlling bleeding in 80-90% of cases.

34. Treatment Disadvantages : Discomfort to the patient. The patient cannot swallow his saliva Liability to cause oesophageal ulceration or stricture. Once the tube is deflated, there is liability to rebleeding in 60-80% of patients. Balloon tamponade is only used as a temporary measure before sclerotherapy or surgery.

35. Balloon tamponade

36. Treatment Emergency surgery. If all the previous measures fail to stop bleeding, surgery is recommended. If the general condition of the patient is satisfactory  splenectomy, portoazygos disconnection and stapling of the oesophagus. If the patient is not very fit  stapling alone can be performed.

37. Treatment T rans-juguJar I ntra-hepatic P orto- S ystemic S hunt ( TIPSS )

38. Treatment Indications for TIPSS: Refractory bleeding Prior to transplant Child C Refractory ascites Main early complication: Perforation of liver capsule  massive haemorrhage.

39. Treatment Treatment of patients with history of bleeding oesophageal varices: 1. Repeated sclerotherapy until the varices are obliterated is the first choice. 2. Elective surgery is mainly indicated if sclerotherapy failed to stop recurrent attacks of bleeding provided that they are fit.

40. Treatment Operations for portal hypertension Shunt operations. The idea of these operations is to lower the portal pressure by shunting the portal blood away from the liver

41. Total shunt operations 1- Porta-caval operation End to side Side to side

42. Porta-caval operation very efficient in lowering the portal pressure  no bleeding occurs from the varices. disadvantages: deprives the liver of portal blood flow  accelerates the onset of liver failure. Recurrent hepatic encephalopathy in 30-50% of patients.

43. Proximal spleno-renal shunt indicated if the portal vein is thrombosed or if splenectormy is indicated due to hypersplenism . The incidence of encephalopathy is less than after porta caval shunt. it is less effective In preventing further bleeding. If the splenic vein is less than 1 cm the anastmosis is liable to thrombosis.

44. Mesocaval ( Drapanas ) shunt insertion of a a synthetic graft as dacron, or autogenic vein between the superior mesenteric vein and inferior vena cava. The incidence of thrombosis is high

45. Selective shunt ( Warren shunt ) The Rt and Lt gastric vessels are ligated. The proximal end of splenic vein is ligated while the distal end is anastomosed to the left renal vein. The short gastric veins are preserved and will selectively decompress the lower end of the oesophagus. The incidence of encephalopathy is low, and the liver functions remain normal.

46. Porta azygos disconnection operations There are many techniques for performing devascularization. Hassab Khairy operation splenectomy & ligation of the Rt and Lt gastric vessels, the short gastric vessels and the vascular arcade along the greater curvature of the stomach leaving only the right gastroepiploic vessels.

47. All vessels surrounding the lower 5-10 cm of the oesophagus are ligated. There is no encephalopathy following this operation and the portal blood flow is intact. There is a low incidence of rebleeding following the operation, but it can usually be controlled by sclerotherapy.

48. Liver Transplant Indicated for liver failure Not for variceal bleeding. 24% in USA die on waiting list

49. Control of Ascites Sodium / Water Restriction. Spironolactone. Loop Diuretic. Large Volume  Paracentesis. Peritoneal-Venous Shunt . TIPSS

50. TIPS on Portal Hypertension for Surgeons By PROF/ GOUDA ELLABBAN

51. VARICEAL BLEEDING Resuscitation Treat hemorrhagic shock Crystalloid ( Limited ) Platelets ( Rarely ) Red Cells + FFP Goal: Tissue Perfusion Monitor: Urine Output Caveat: Do NOT overload

52. VARICEAL BLEEDING Initial Treatment Continue Tx hemorrhagic shock IV therapy Sandostatin® INITIATE WHEN Dx SUSPECTED!!!

53. VARICEAL BLEEDING Diagnosis 50% UGI bleeds not variceal (MW Tear, Gastritis, Gastric/Duodenal Ulcer) Early endoscopy mandatory Variceal bleeding Dx’d: Active bleeding Stigmata Varices and NO other source

54. VARICEAL BLEEDING Initial Therapy Continue I.V. Sandostatin ® Endoscopic Therapy Sengstaaken-Blakemore tube TIPS Emergency operation

55. VARICEAL BLEEDING Supportive Therapy Correct coagulopathy FFP, vitamin K, +/- platelets Pulmonary Other infection Encephalopathy Nutrition

56. VARICEAL BLEEDING Evaluation Child class History Hepatitis profile Angiography Transplant evaluation

57. Child-Pugh Classification Points 1 2 3 Bilirubin (mg/dL) < 2 2 – 3 > 3 Albumin (g/dL) > 3.5 2.8 – 3.5 < 2.8 Prothrombin time (seconds ↑) 1 – 3 4 – 6 > 6 Ascites None Slight Moderate Encephalopathy None Minimal Advanced Grade A, 5-6 points; Grade B, 7-9 points; Grade C, 10-15 points

58. VARICEAL BLEEDING Definitive Therapy Rationale: 67% rebleed Most rebleed < 6 weeks Definitive Tx during initial stay

59. VARICEAL BLEEDING Definitive Therapy Medical Endoscopic Surgical Radiological

60. VARICEAL BLEEDING Medical Therapy Beta blockade ↓ bleeding by ↓ cardiac output Goal: 25% ↓ in heart rate Reduces # bleeding episodes Does not reduce mortality Use as adjunct

61. Endoscopic Banding Occludes venous channels Multiple sessions + surveillance >60% rebleed 1/3 fail treatment ↓ complications vs scleroTx = / ↑ efficacy vs scleroTx ENDOSCOPIC Tx OF CHOICE

62. Endoscopic Banding

63. VARICEAL BLEEDING SURGICAL OPTIONS Total Shunt Selective Shunt Partial Shunt Non-Shunt

64. Total Shunts End to Side Portocaval Side to Side Portocaval Interposition Shunts Central Splenorenal

65. Total Shunt Results Prevent rebleed > 90% Thrombosis with graft Encephalopathy rate 40%

66. Selective Shunts Goals: Prevent variceal bleeding and encephalopathy Mechanism: Decompress Varices Maintain Portal Perfusion Maintain Portal Hypertension Key: Decompress only gastrosplenic compartment

67. Distal Splenorenal Shunt

68. DSRS vs Total Shunts Six randomized trials in N.A. Mean follow-up 39 mos (1-8 yrs)

69. Partial Shunts Ease of portocaval Limited portal diversion Maintain some liver perfusion Short, straight PTFE graft

70. Partial Shunts Sarfeh Ann Surg 200:706,1986

71. Partial Shunts Randomized trial in ETOH cirrhotics Follow-up @ 20 +/- 11 mos

72. Non-Shunt Operations Options Esophageal transection Variceal ligation Devascularize +/- splenectomy Very limited role

73. Liver Transplant Indicated for liver failure Not for variceal bleeding Number ↑ > 3,500/yr in U.S. 20,000 potential recipients in U.S. 5,000 listed for transplant 24% die on waiting list

74. TIPS T ransjugular I ntrahepatic P ortocaval S hunt

75. TIPS

76. TIPS Technically feasible Complications 9 - 50% Infection Intraperitoneal Bleeding Congestive Failure Subcapsular Hematoma Acute Renal Failure Hemobilia Mortality (30 day) 3 - 13% (1) Rossie NEJM 1994;330:165, (2) Rosch Hepatology 1992;16:884, (3) LaBerge Radiology 1993;187:913.

77. Problems With TIPS Encephalopathy minimum 15% Occlusion 33 - 73% @ one year Rebleeding 18% @ one year (1) 19% @ 4.7 months (3) (1) Rossie NEJM 1994;330:165, (2) Rosch Hepatology 1992;16:884, (3) LaBerge Radiology 1993;187:913.

78. The Role For Tips Refractory bleeding Bridge to transplant Child C (all or only “D → Z” ?) ??? refractory ascites Relative contra indication: Poor f/u

79. Special Cases of Portal Hypertension

80. Splenic Vein Thrombosis Etiology: Pancreatitis - Acute or Chronic Pancreatic Carcinoma Hallmark: Isolated Gastric Varices Treatment: Splenectomy ( if bleeding)

81. Portal Vein Thrombosis Etiology: Congenital - “Cavernous Transformation” Hallmark: Normal Liver Function W/ Varices Treatment: Endo Tx OR DSRS

82. Budd-Chiari Syndrome Etiology Hypercoagulable: Estrogens, XRT, Myeloprolif, PNH IVC Occlusion: RA Myxoma, Pericarditis, Membrane Liver Mass High Dose ChemoTx Presentation: Classic Triad Abdominal Pain Ascites Hepatomegaly

83. Budd-Chiari Syndrome Diagnosis U/S, CT, Angio Treatment NOT a static disease If NO necrosis → Symptomatic Tx If necrosis → Shunt (PCS or MAS) or Transplant

84. Some Take Home Points Child A better than Child C Start Sandostatin when Dx suspected β blockade ↓ bleeding by ↓ C.O Banding safer than scleroTx TIPS: Encephalopathy & occlusion rate

85. Some Take Home Points Selective shunt: ↓ encephalopathy SV Thrombosis: Presentation & Tx Budd-Chiari: Classic triad Transplant for liver failure

86. Portal Hypertension Etiology PRE-HEPATIC Portal Vein or Splenic Vein Thrombosis INTRA-HEPATIC Cirrhosis (ETOH, Hepatitis, Other Toxins) POST-HEPATIC Budd-Chiari

87. Complications of Portal Hypertension Ascites Encephalopathy Variceal bleeding Initial management Evaluation Definitive therapy Special cases

88. Encephalopathy Etiology : ? Nitrogen compounds Induced by : Infection Dehydration Constipation Blood in gut No test is diagnostic Therapy : Hydrate Cleanse gut ↓ protein Find and treat cause

89. Ascites Origin: Sinusoidal pressure > colloid oncotic pressure Induced by: Physiologic Stress IV Fluids Complications: Spontaneous Bacterial Peritonitis “Hepatorenal Syndrome”

90. Control of Ascites Sodium / Water Restriction Spironolactone Loop Diuretic Large Volume Paracentesis Peritoneal-Venous Shunt (?) TIPS

91. VARICEAL BLEEDING General Approach Resuscitation Initial treatment Support Evaluation Definitive therapy

92. Vasopressin 8-Arginine Vasopressin (ADH) Intense constriction (all beds) + ’s ↓ Mesenteric Flow ↓ Portal Pressure Stops Bleeding in >80% - ’s Peripheral Ischemia Myocardial Ischemia NTG ↓ ’s adverse effects

93. Sandostatin® Long acting STS analogue +’s ↓ Mesenteric Flow ↓ Portal Pressure Stops bleeding in > 85% Good as VP but ↓ side effects -’s Cost DRUG OF CHOICE

94. Portal Vein Anatomy

95. Portal Vein Collaterals Five Principle Routes Veins of Retzius Umbilical Vein Hemorrhoids Adhesions Esophageal Varices

96. VARICEAL BLEEDING Sclerotherapy Intra- or Para- Variceal Occludes venous channels Multiple sessions + surveillance >60% rebleed 1/3 fail treatment 30% complication rate

97. Endoscopic Sclerotherapy Intravariceal Paravariceal

98. Complications of ScleroTx LOCAL Ulceration Stricture Perforation SYSTEMIC Fever Pneumonitis CNS

99. Total Shunts Divert most (all?) portal flow Options Portocaval Shunt (E-S or S-S; +/- Graft) Interposition Shunt Central Splenorenal Shunt

100. TIPS

101. Child’s Classification A B C Bilirubin < 2 2 – 3 > 3 Albumin > 3.5 2.8 – 3.5 < 2.8 Ascites None Controlled Un controlled Enceph None Minimal Advanced Nutrition Excellent Good Poor

102. SclTx vs TIPS Five Randomized Trials - 360 patients Mean Follow-up 15 mos (1-36) * p < 0.05 in all but one study ** p < 0.05 in all studies *** n.s. in all but one study where survival ↑ w/ SclTx

103. Thank you