This document summarizes information about portal hypertension and variceal bleeding. It discusses the causes of portal hypertension including pre-sinusoidal, sinusoidal and post-sinusoidal factors. It also describes treatments for acute variceal bleeding such as pharmacologic therapies, endoscopic therapies like band ligation, balloon tamponade and TIPS. It notes that prevention of recurrent bleeding involves pharmacotherapy, endoscopic therapy, TIPS or surgical options like portosystemic shunts or liver transplantation.

1. Dr Saeed Al-Shomimi KFHU Khobar

2. Anatomy 6 – 8 cm Splenic + s. mesenteric (behind neck of the pancreas ) i. mesenteric , Lt gastric

3. Blood Supply of the Liver Hepatic Arterial Autoregularity Vasodilatation

4. Pathophysiology Pressure = Flow X Resistance Portal pressure : 3 – 6 mm Hg Normal elevation: Eating Exercise Valsalva

5. 10 mmHg (prolonged) -> Shunting Lt Gastric -> esophageal Short Gastric -> Gastric Submucosal Lt portal -> epigastric Retroperetoneal and anorectal collateral 12 mm Hg -> Bleeding

6. Causes of Portal Hypertension Pre-sinusoidal Sinusoidal Post Sinusoidal BLOOD FLOW LIVER

7. Pre-sinusoidal Extra-hepatic: Portal vein thrombosis Splenic vein Thrombosis Intra-hepatic: Congenital hepatic fibrosis Primary biliary cirrhosis Sarcidosis Schistosomaisis

8. Sinusoidal Steatohepatits Wilson disease

9. Post Sinusoidal Extra-hepatic: Budd Chiari syndrome R heart Failure Intra-hepatic: Heamochromatosis Alcoholic cirrhosis Post-hepatitic cirrhosis

10. Variceal Bleeding Mortality associated with 1 st episode: Cirrhotic patient : 40% - 70% Non cirrhotic : 5% - 10% If bleeding resolved spontaneously 30% re-bleed , 6 weeks 70% re-bleed , 1 year (30% of the initial bleeding episodes are fatal)

11. Acute Variceal Bleeding

12. Initial evaluation & stabilization Assessment of intravascular volume status Fluid resuscitation Endotracheal intubation prior to endoscopy for: Uncontrolled bleeding Altered mental status, severe agitation Respiratory distress or depression

13. Pharmacologic Radiologic shunt TIPSS Surgical Shunt Balloon Tamponade Pharmacologic and endoscopic therapy are the usual 1 st and 2 nd interventions Endoscopic Treatment for Acute Variceal Bleeding

14. Pharmacologic Therapy Octreotide Synthetic analogue of somatostatin Decreases portal pressure and azygos blood flow Stops variceal bleed in 80% of the cases Efficacy is similar to endoscopic sclerotherapy and better than vasopressin

15. 5-day course reduces bleeding after endoscopic therapy Can cause mild hyperglycemia and abdominal cramping 250 µg Iv Bolus – followed by infusion 25 – 50 µg/h (2-4 days)

16. Vasopressin Reduces portal pressure but causes myocardial and mesenteric ischemia (more side effects) 20 u IV bolus (over 20 min) – infusion 0.2 – 0.4 u/min Control approximately 50% of acute episodes

17. Terlipressin Efficacy similar to endoscopic sclerotherapy and as effective as balloon tamponade when used with nitroglycerin Not approved for use in U.S.

18. Endoscopic Therapy Sclerosant injection Band ligation Became a standard form of therapy in acute variceal bleeding Initial control of hge in 70 – 95% Re-bleeding 20 – 50%

19. sclerotherapy 5% sodium morrhuate 5% ethanolamine oleate Intravariceally : to obliterate the varix Paravariceally : induce submucus fibrosis

20. 3 prospective randomized controlled trials studies comparing sclerotherapy and balloon temponade: Sclerotherapy achieved better initial hge control Fewer episodes of rebleeding Improved long-term survival (furthermore, routine use of balloon temponade after sclerotherapy confer no additional benefit)

21. Complications: Pulmonary complications Transient chest pain Esophageal stricture Portal vein thrombosis Esophageal perforation Bacteremia

22. Alternative to sclerotherapy Fewer rebleeding episodes Fewer endoscopic interventions Lower procedure related mortality and over all mortality Band Ligation

23. Pharmacologic versus Endoscopic Therapy 2 meta-analysis compared medical pharmacotherapy with emergency sclerotherapy as 1 st line treatment for acute bleeding: No significant difference regarding initial hge control or mortality Administration of somatostatin before and after sclerotherapy : Improve treatment efficacy Reduce blood transfusion

24. Balloon Temponade Application of direct upward pressure against varices at G-E junction Should be intubated: Prevent aspiration Prevent airway occlusion

25. Balloon positioning

26. 1. Tube inserted to 50 cm 2. Auscultate in stomach 3. Inflate gastric balloon with 50 cc 4. Stat portable film Re-confirm proximal position Inflate GB 300-400 cc air Pull to insure anchorage Recheck film 1-2 lbs of pully traction Tube Positioning and Gastric Balloon Inflation

27. Esophageal Balloon inflated to 35 - 40 mmHg Last resort Deflate periodically Use minimum effective pressure Complication - ulcer - perforation - stricture Gastric and Esophageal Balloon Inflation

28. Direct temponade therapy is 90% effective in controlling the bleeding 50% rebleeding after removal Serious potential complications (mortality 20%) Bridge therapy

29. TIPS (Transjagular Intrahepatic Portosystemic Shunting) Creating an intrahepatic portosystemic fistula to decompress the portal hypertension First performed in 1982 (non- selective side to side portosystemic shunt)

30. 1 -Cannulating the Rt hepatic vein via internal jagular vein 2 – passing needle through liver parenchyma to portal vein branch 3- guide wire 4 balloon dilatation

31. 5 – stenting the tract

33. Meta-analysis comparing TIPS with endoscopy in acute hge: Significant improvement in controlling the hge Coast : ↑rate of hepatic encephalopathy

34. Contraindications: R side heart failure Polycystic liver Portal vein thrombosis

35. Complications: Intraperitoneal bleeding due to perforation of the hepatic capsule, hepatic, or portal veins TIPS embolization Acute right heart failure due to increased venous return to right heart

36. Late: recurrent bleeding due to TIPS stenosis or thrombosis Infection hepatic encephalopathy.

37. Surgical Therapy Operative intervention is reserved for cases refractory to other modalities

38. Esophageal transection EEA stapler Operative mortality 75% Complications 25%: Perforation Stricture Esophagitis -> not useful in acute state

39. Portosystemic Shunt (side-to-side) Non-selective shunt Manipulation and dissection in porta hepatica -> Scaring and fibrosis -> complicate future liver transplant

40. DSRS Selective shunt Some cases un accompanied by refractory ascitis

41. Prevention of Recurrent Variceal Bleeding

42. Pharmacotherapy: Rebleeding without treatment 70% in 1 year Non-selective B blockers (propranalol) ↓ portal pressure Effect is variable and unpredictable Less benefit with decompesated liver

43. Endoscopic therapy: Advocated as a means for complete eradication of esophageal varices Once eliminated routine endoscopy 6-12 months Fewer rebleeding episodes than medical treatment 50 % rebleding in 1 year 30% need conversion Reserved for complaint patients

44. TIPS: Bridge therapy -> liver transplant Advantiges over surgery: No risk of general anesthesia No post-operative complications Limitations Stenosis (50% in 1 st year) Encephalopathy (1/3)

45. Surgical Therapy: Most effective method in controlling portal hypertension and recurrent bleeding 1 Portosystemic shunt procedures 2 Esophagogastric devascularization 3 Orthotopic liver transplantation

46. Portosystemic Shunt Decompressing the hypertensive portal Venus system into the low pressure systemic venous circulation Toxins -> systemic circulation -> encephalopathy

47. To minimize these effects shunting operations have evolved: Non-selective Selective partial

48. 1 – Non selective Shunts End to side portocaval (Eck fistula): Higher rate of encephalopathy among operative shunting groups Better control of rebleeding than medical treatment Eck fistula – medical therapy -> same incidence of encephalopathy

49. Side to Side portocaval shunt: Maintain the anatomic continuity of the portal vein Encephalopathy rate : no difference Decompress the sinusoidal pressure -> better ascitis control Recommended for Budd Chiari Syndrome More difficult than end to side

50. Interposition Mesocaval Shunt: Prosthetic – autogennous vien Avoid hilar dissection (future transplant) Shunt ligation in refractory post-op encephalopathy Drawback -> thrombosis (35%)

51. Proximal Spleno-Renal Shunt: Splenectomy + anastomosing proximal Splenic vein to Lt Renal vein Divert all portal flow into renal vein -> non selective Shunt occlusion 18%

52. 2 – Selective Shunts In response to post-op complications of non-selective procedures 1967 DSRS Distal Splenic vein to Lt renal Vein Selectively decompress the esophagogastric veins

53. Contraindications: Refractory ascitis Splenic vein thrombosis Previously underwent splenectomy Splenic vein diameter < 7 mm

54. Coronary – Caval Shunt: Described in Japan in 1984 Interposition graft between L Gastric and inferior vena cava Little experience with this procedure

55. 3 – Partial Shunts Small diameter interposition grafts Maintaining a degree of hepatopedal portal flow to the liver

56. Esophagogastric Devascularization The most effective non-shunt operation for preventing variceal bleeding: Devascularization + transection + splenectomy Sugiura procedure

57. Orthotopic Liver Transplantation The most definitive form of therapy for complications of portal hypertension Selective patients: Coast Unavailability Immunosuppresion

58. Child A – mild B -> non-transplant surgery Child C – advanced B -> transplant

59. Prophylaxis

60. Likehood of variceal bleeding : Alcoholic cirrhosis Active alcohol consumption Sever hepatic dysfunction Endoscopy: Variceal wall thinning Variceal tortuosity Superimposition of varices on other Gastric varicose

61. Non selective B blockers Prophylactic shunts showed no benefit , ↑morbidity

62. Portal hypertension in north Indian children Arora NK, Lodha R, Gulati S, Gupta AK, Mathur P, Joshi MS, Arora N, Mitra DK Department of Paediatrics All India Institute of Medical Sciences New Delhi.

63. cross-sectional observational study Tertiary care centre in northern India January, 1990 to December, 1994 Children below the age of 14 years with suspected portal hypertension To determine the etiology and clinical profile of portal hypertension

64. 115 patients with portal hypertension 76.5% had extrahepatic portal hypertension (EHPH) 23.5% had intrahepatic causes of portal hypertension

65. Results: Children with EHPH had a significantly earlier onset of symptoms as compared to those with intrahepatic portal hypertension (p = 0.002) And bled significantly more frequently (p = 0.00).

66. History suggestive of potential etiological factors could be elicited in only 7% of EHPH patients . The commonest site of block in splenoportal axis was at the formation of the portal vein .

67. An inverse relation of bleeding rates with duration of illness was seen in EHPH

68. Conclusion: Understanding the natural history of EHPH and portal hypertension due to other etiologies may have significant implications in choosing the appropriate intervention and predicting the outcome .

69. References ACS Surgery : Principles and Practice 2004 Web,MD Schwartz Principles of Surgery 7 th Edition Indian J Pediatr. 1998 Jul-Aug;65(4):585-91. Johns Hopkins Gastroenterology & Hepatology Resource Center http://hopkins-gi.nts.jhu.edu

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