Non cirrhotic portal hypertension- role of shunt surgery

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1. NCPH: Role of Shunt S urgery in Current E ra Dr. Harsh Shah MS, FMAS, DNB,MCh (GI) GI & HPB Surgeon Kaizen Hospital , Ahmedabad PSRS

2. Abbreviations PHTN – Portal hypertension EHPVO- Extrahepatic portal venous obstruction NCPF – Non-cirrhotic portal fibrosis EST – Endoscopic sclerotherapy EVL – Endoscopic Variceal ligation PSRS – Proximal splenorenal shunt MCS – Mesocaval shunt

3. Plan of presentation Portal hypertension EHPVO NCPF Portal biliopathy Shunt Surgery – PSRS, Rex shunt

4. Hepatic vein Sinusoids Portal vein Liver Splenic vein Coronary vein SMV IMV

5. Portal Hypertension Normal Portal venous pressure – 5-8 mm Hg Portal Hypertension >10mmHg Variceal bleeding > 12mm Hg

6. Etiology of PHTN Pre-hepatic - EHPVO Hepatic Pre-sinusoidal - Sinusoidal - Post-sinusoidal - NCPF Cirrhosis Veno -occlusive disease Post-hepatic - Budd- chiary syndrome

7. Sequalae Of PHTN Splenomegaly, Hypersplenism Enlargement of porto -systemic collaterals Esophagus, Stomach Rectum Retroperitoneum Biliary (Portal biliopathy ) Periumbilical Ascites Hepatic encephalopathy

8. Endoscopic view of Esophageal Varices Normal Low Grade High Grade

9. Endoscopic Sclerotherapy Endoscopic Variceal Ligation

10. Management of Variceal bleed After hemodynamic resuscitation all such patients should undergo endotherapy [either sclerotherapy ( EST) or band ligation (EVL) ] Controversy exists regarding prevention of further bleed Two main approaches to prevent further bleed: endoscopic intervention ( EST, EVL) and shunt surgery

11. NCPH Features of PHT without any evidence of significant liver parenchymal dysfunction Extra -hepatic PV obstruction (EHPVO ) Non - cirrhotic portal fibrosis (NCPF ) Nearly 50% of cases of PHTN in developing countries

12. NCPH

13. EHPVO(Extrahepatic Portal Venous Obstruction) Definition : A vascular disorder of liver, characterized by obstruction of the extra- hepatic PV with or without involvement of intra-hepatic PV radicles or splenic or superior mesenteric veins. Isolated occlusion of the splenic vein or superior mesenteric vein does not constitute EHPVO Prognosis of EHPVO after control of variceal bleed is good, with long term (>10 years) survival nearly 100 % Natural history is complicated by development of portal biliopathy & ectopic varices

14. Portal Cavernoma

15. Clinical Features Moderate splenomegaly, Esophageal Varices Gastric varices – 1/3 rd of cases Ectopic varices- 27-40% case Duodenal, anorectal , GB bed, biliary tree Portal biliopathy - 60-100 % (Asymptomatic) Symptomatic PB (pain, jaundice, fever)- develop in 5-14% of cases on long-term follow-up Growth retardation (?) Portal vein obstruction in children leads to growth retardation. Hepatology. 1992 Feb;15(2):229-33. Sarin et al Extra-hepatic portal vein obstruction in children is not associated with growth impairment. Nutrition & Food Science, 2013

16. NCPF (Non-cirrhotic portal Fibrosis) NCPF is a disease of uncertain etiology C haracterized by periportal fibrosis and involvement of small and medium branches of the portal vein, resulting in the development of portal hypertension. The liver functions and structure primarily remain normal, in most cases

17. NCPF ( Non-cirrhotic portal Fibrosis) Diagnostic criteria: ( APASL) Patent spleno -portal axis and hepatic veins on ultrasound Doppler Presence of moderate to massive splenomegaly Evidence of portal hypertension, varices, and/or collaterals Test results indicating normal or near-normal liver functions Normal or near-normal HVPG Liver histology-no evidence of cirrhosis or parenchymal injury Other features: Absence of signs of chronic liver disease

18. NCPF 2/3 rd of patients present with G I Bleed Uncontrolled variceal bleeding is a common cause of death Long term survival after eradication of esophagogastric varices or after a shunt surgery is nearly 80-100% Liver functions usually remain well preserved , but with course of time in 20–33% of cases, liver slowly undergoes parenchymal atrophy with subsequent decompensation

19. EHPVO Primary Prophylaxis Secondary Prophylaxis

20. EHPVO - Primary Prophylaxis 44 (untreated) children (< 12 years of age) with varices P rospectively followed up to a mean age of 20 years (range 15–34 years ) overall probability of variceal bleeding was - 49 % and 76% at ages of 16 and 24 years, respectively L ykavieris P, Gauthier F, Hadchouel P, Duche M, Bernard O. Risk of gastrointestinal bleeding during adolescence and early adulthood in children with portal vein obstruction. J. Pediatr . 2000; 136 : 805 –8 . Issues in developing countries: Poor access to endoscopic Rx in emergency Lack of blood bank facilities

21. EHPVO- Primary Prophylaxis High grade Eso varices should receive endoscopic therapy Low grade Eso varices should be followed endoscopically Consensus on extra-hepatic portal vein Obstruction. Liver International 2006 (APASL)

22. Surgery as primary prophylaxis from variceal bleeding in patients with extrahepatic portal venous obstruction Pal S,  Mangla V,  Radhakrishna P,  Sahni  P,  Pande GK,  Acharya SK,  Chattopadhyay TK,  Nundy   S. J Gastroenterol Hepatol .  2013 METHODS : Between 1976 and 2010 Selection criteria : Patients with EHPVO , who had no history of variceal bleeding but had "high-risk" esophagogastric varices or severe portal hypertensive gastropathy and/or hypersplenism , and came from remote areas with poor access to tertiary health care Prospectively followed up

23. Surgery as primary prophylaxis from variceal bleeding in patients with extrahepatic portal venous obstruction RESULTS: A total of 114 patients (mean age 19 years) underwent prophylactic operations (PSRS 98 [86% ] & esophagogastric devascularization 16(14%)) Postoperative mortality was 0.9%. Among 89(79%) patients who were followed up (mean 60 months), hypersplenism was cured, and six (6.7%) developed variceal bleeding. The latter were managed successfully by endoscopic sclerotherapy . No patient developed overwhelming post- splenectomy sepsis or encephalopathy CONCLUSION : In patients with EHPVO , prophylactic surgery is fairly safe and prevents variceal bleeding in ∼ 94% of patients with no occurrence of portosystemic encephalopathy

24. Secondary Prophylaxis E ndoscopic therapy is effective and endoscopic band ligation of varices (EVL) is preferred due to its safety and efficacy There are insufficient data to recommend B- blockers. Gastric varices: injection of glue is an effective treatment for the control of acute gastric variceal bleeding and prevention of rebleeding Shunt surgery should be considered Consensus on extra-hepatic portal vein Obstruction. Liver International 2006 (APASL)

25. EHPVO- Endoscopic Therapy Thomas(2009) Itha (2006) Poddar (2008) N 198 183 278 Treatment EST EST EST Eso Vx eradication - 89% 95% Sessions - 7.7 5 Recurrence of Eso Vx 20% 40% 14% Mean time to rebleed 5.4 yrs - - Rebleed 34(17%) 21- eso Vx 7- Gastric Vx 2- Ectopic Vx 7%(all Gvx ) 3% F/U period 20 yrs 3 yr 34 months Mortality 1.5% 0% 1.7%

26. Endoscopic Therapy Advantages Acute bleed (success rate 90-100%) Less morbid procedure Drawbacks Availability Repeated sessions(4-8) Ulcer (8 -25% ) Stricture (6 -17% ) Esophageal Perforation

27. Types of surgery Shunt PSRS(Proximal splenorenal shunt) SSLRS ( Mitra’s shunt) MCS ( Mesocaval shunt) DSRS (Distal splenorenal shunt ) Rex shunt (MLPVB) Non-shunt procedure D evascularization

28. Indications for shunt surgery Absolute Endoscopically refractory variceal haemorrhage Symptomatic hypersplenism Symptomatic Portal biliopathy Bleeding ectopic varices If patient demands one time treatment

29. Indications for shunt surgery Relative Symptomatic splenomegaly (Pain , infarction ) Large varices with poor access to health care Rare blood group Growth failure ( Z score <-2 despite nutritional rehabilitation)

30. EHPVO – Surgical Management Orloff (2002) Nundy (1994) Mitra (1993) n 200 160 81 Surgery PSRS, MCS PSRS PSRS Emergency - 20 (12.5%) - FU period 5-35 yrs 1-13 yrs 4.5 yrs Shunt patency 97.5% - 85% Rebleed 2.5% 11% 10% HE 0% 0% 0% OPSI 0% 1 case 0% Mortality 1.9% 4% -

31. Shunt surgery Advantages One time procedure Growth spurt & improved quality of life* Cost-effective in long-term Drawbacks Need for expertise Morbidity(2-15%) Shunt thrombosis (2-20%) Portosystemic encephalopathy Post- splenectomy sepsis Menon et al. Extrahepatic  portal hypertension: quality of life and somatic growth after  surgery. Eur J Pediatr Surg.  2005.

32. Management of Variceal Hemorrhage in Children with Extrahepatic Portal Venous Obstruction-Shunt Surgery Versus Endoscopic Sclerotherapy Zargar et al. Indian J Surg 2011 P rospective randomized study 61 children with bleeding esophageal varices due to EHPVO 30 received surgery and 31 patients received EST Overall incidence of rebleeding was 7(22.6%) in sclerotherapy group and 1(3.3%) in shunt surgery group (p = 0.026) Treatment failure occurred in 6 (19.4%) patients in sclerotherapy group(1 death,5 change in therapy) and 2(6.7%) in shunt surgery group(1 death,1 shunt thrombosis) (p >0.05)

33. Portal Biliopathy Morphological changes in the bile ducts have been described in 80–100% of patients clinical evidence of biliary obstruction (pain, jaundice , cholangitis ) is seen in only 5–14% of patients Etiology compression of the bile duct by the enlarged paracholedochal plexus of veins Ischemia secondary to thrombosis of small venules in the wall of the bile ducts choledocholithiasis and recurrent episodes of cholangitis may also develop inflammatory strictures

34. Portal Biliopathy

35. MRCP

36. Portal Biliopathy Endoscopy: Endoscopic Rx can be hazardous if papillotomy , dilatation or stone extraction are performed in the presence of collaterals Does not treat underlying cause Likely to require repeated stent exchange Secondary biliary cirrhosis - long term stent placement with incomplete relief of obstruction Surgery: Shunt surgery by relieving pressure in pericholedochal plexus, makes access to this region, easier & safer

37. Surgery in Portal Biliopathy Nundy (2012) Agarwal (2011) Indications Symp PB Symp PB Surgery 40 PSRS, 16 Devasc 37 PSRS FU period - 32 months Rebleed - 0% Shunt patency 88% 97% HE - - Mortality - 0% Second stage 11.6%(HJ) 35% (11HJ, 1CDD, 1CCx)

38. NCPF Primary Prophylaxis Secondary Prophylaxis

39. Primary Prophylaxis- NCPF These varices are generally large at the time of diagnosis EVL is recommended for large varices Role of non- selective beta blockers- not defined Decompressive shunt surgery is not recommended for primary prophylaxis APASL recommendation 2007

40. Prophylactic surgery in non-cirrhotic portal fibrosis : is it worthwhile ? Pal S,  Radhakrishna P,  Sahni  P,  Pande GK,  Nundy  S, Chattopadhyay TK. Indian J Gastroenterol.2005 Nov-Dec;24(6):239- 42 AIM : To study the results of prophylactic operations to prevent variceal bleeding in patients with portal hypertension due to non-cirrhotic portal fibrosis (NCPF ) METHODS Between 1976 and 2001, 45 patients with NCPF selection criteria: high -risk esophagogastric varices or symptomatic splenomegaly and hypersplenism . PSRS in 41 patients and the remaining underwent splenectomy with (2 patients) or without (2 patients) devascularization .

41. Prophylactic surgery in non-cirrhotic portal fibrosis: is it worthwhile? RESULTS : No operative mortality 38 patients were followed up for a mean 49 (range, 12-236) months Three patients bled – 1- variceal and 2 - duodenal ulcers; none died of bleeding 2 late deaths (6 weeks and 10 years after surgery), 1 from an unknown cause and 1 due to chronic renal failure The delayed morbidity was 47%. 7 - portasystemic encephalopathy, 4- glomerulonephritis and 5 - ascites requiring treatment with diuretics. Thus only 20 (53%) patients were symptom-free on follow up . CONCLUSIONS : Prophylactic surgery is safe and effective in preventing variceal bleeding in NCPF but at the cost of high delayed morbidity.

42. Secondary Prophylaxis Endoscopic therapy and elective decompressive surgery are effective and safe There should be head-to-head comparison between these two modalities APASL recommendation (2007)

43. Proximal Spleno -renal Shunt Indications EHPVO, NCPF Pre-op preparation Doppler – size of splenic vein, left renal vein Vaccination Arrange blood products Position Left subcostal or left thoraco -abdominal

44. Proximal Splenorenal Shunt Operative steps Ligation of splenic artery Splenic mobilization Splenectomy with preservation of long length of splenic vein Dissection of Left renal vein Anastomosis with Prolene 5-0 Growth factor Liver biopsy

45. Completed PSRS Pancreas Renal vein Splenic vein

46. Rex-shunt (MLPVB) Restores physiological hepatopetal blood flow Pre-requisite Patent intrahepatic left portal vein(>2mm) & SMV Venous conduits IJV, saphenous vein, coronary vein, IMV or synthetic grafts Advantages Promotes synthetic function of liver Enhances somatic growth

47. Rex-shunt(MLPVB) Follow-up - 1 and 7 years 34 patients S hunt patency rate- 91 % R ebleeding rate- 8 % Superina R, Bambini DA, Lokar J, Rigsby C, Whitington PF. Correction of extrahepatic portal vein thrombosis by the mesenteric to left portal vein bypass. Ann Surg 2006;243:515-21. Should be performed early after the diagnosis

48. Arguments against Endoscopic therapy Failure to control acute bleed in 5 -10 % of cases Rebleed rate – 5-25% Formation of new gastric varices, ectopic varices and portal hypertensive gastropathy Continued progression of portal biliopathy Repeated hospital visits- school absenteeism Splenomegaly- not addressed

49. Arguments against Shunt Surgery 10 % of patients with EHPVO have no shuntable vein or thrombosed spleno -portal and mesenterico - portal axis S hunt thrombosis (2-20%) Need for endoscopic surveillance in follow-up Rebleed Upto 7 yrs ( Nundy et al) Morbidity in NCPF patients : glomerulonephritis

50. Summary Level 1 evidence not available- for superiority of one therapy over other Surgical shunt One time, durable & cost-effective control of variceal bleed Should be offered to pt with growth retardation, symptomatic PB & ectopic variceal bleed Delayed morbidity in NCPF Endoscopy U sually the first offered treatment modality Requires repeated sessions Should be offered when no shuntable vein available Can not prevent progression of PB & ectopic varices

51. Thank You Dr Harsh Shah MS, MCh - GI & HPB Surgeon