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1. NCPH:
Role of Shunt
Surgery in Current
Era
Dr. Harsh Shah
MS, FMAS, DNB,MCh (GI)
GI & HPB Surgeon
Kaizen Hospital, AhmedabadPSRS

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

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

10. Endoscopic Sclerotherapy
Endoscopic Variceal Ligation

11. 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

12. 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

13. NCPH

14. 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

15. Portal Cavernoma

16. Clinical Features
• Moderate splenomegaly, Esophageal Varices
• Gastric varices – 1/3rd 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

17. NCPF (Non-cirrhotic portal Fibrosis)
• NCPF is a disease of uncertain etiology
• Characterized 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

18. 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

19. NCPF
• 2/3rd 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

20. EHPVO
- Primary Prophylaxis
- Secondary Prophylaxis

21. EHPVO - Primary Prophylaxis
• 44 (untreated) children(< 12 years of age) with varices
• Prospectively 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
Lykavieris 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

22. 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)

23. 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

24. 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

25. Secondary Prophylaxis
• Endoscopic 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)

26. 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%

27. 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

28. 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
 Devascularization

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

31. 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)

32. 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% -

33. 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 sur
gery. Eur J Pediatr Surg. 2005.

34. Management of Variceal Hemorrhage in Children
with Extrahepatic Portal Venous Obstruction-Shunt
Surgery Versus Endoscopic Sclerotherapy
Zargar et al. Indian J Surg 2011
• Prospective 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)

35. 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

36. Portal Biliopathy

37. MRCP

38. 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

39. 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)

40. NCPF
- Primary Prophylaxis
- Secondary Prophylaxis

41. 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

42. 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.

43. 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.

44. 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)

45. 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

46. 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

47. Completed PSRS
Pancreas
Renal vein
Splenic vein

48. 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

49. Rex-shunt(MLPVB)
• Follow-up - 1 and 7 years
• 34 patients
• Shunt patency rate- 91 %
• Rebleeding 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

50. 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

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

52. 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
 Usually the first offered treatment modality
 Requires repeated sessions
 Should be offered when no shuntable vein available
 Can not prevent progression of PB & ectopic varices

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