What are the indications for shunt surgery in patients with extra‑hepatic portal vein obstruction (EHPVO)?

Indications for Shunt Surgery in EHPVO

Shunt surgery in EHPVO is indicated primarily for recurrent variceal bleeding after failed endoscopic therapy, symptomatic portal cholangiopathy with biliary complications, and severe hypersplenism with massive splenomegaly causing growth retardation or significant cytopenias. 1

Primary Indications

Recurrent Variceal Bleeding

• Surgical portosystemic shunting should be performed when endoscopic therapy fails to control recurrent variceal hemorrhage, particularly in patients with patent superior mesenteric or splenic veins 1
• Selected patients demonstrate low mortality and rebleeding rates with surgical shunts using superior mesenteric or splenic veins 1
• Shunt surgery provides definitive control of bleeding and eliminates the need for repeated endoscopic procedures for many years 2, 3
• Rebleeding rates after shunt surgery range from 2.5-23%, with most series reporting rates under 15% 3, 4

Portal Cholangiopathy with Biliary Complications

• Only patients with clinical manifestations of portal cholangiopathy should be considered for shunt surgery 1
• Specific indications include biliary stricture associated with jaundice or bile stones that cannot be adequately managed endoscopically 1
• When superior mesenteric vein or splenic veins are patent, surgical shunt should be considered for symptomatic portal biliopathy 1
• Portal cholangiopathy-related cholestasis regresses in approximately 71% of patients following successful shunt surgery 5

Massive Splenomegaly with Severe Hypersplenism

• Massive splenomegaly with severe hypersplenism is a primary indication for shunt surgery in EHPVO, present in 95% of surgical cases in contemporary series 5
• This indication is particularly important in pediatric patients where it often coexists with growth retardation (68% of cases) 5
• Splenomegaly-related issues resolve completely following successful shunt surgery 5

Isolated Gastric and Ectopic Varices

• Isolated gastric varices (IGV) and ectopic varices that are not amenable to endoscopic therapy represent surgical indications in approximately 35% of cases 5
• These varices are less accessible to endoscopic treatment and warrant surgical intervention more frequently than esophageal varices 5

Surgical Options and Selection

Mesenterico-Rex Shunt (Pediatric)

• In children with patent superior mesenteric and left portal veins, mesenterico-Rex shunt is the preferred procedure 1
• This bypass demonstrates high feasibility and long-term patency 1, 6
• Gastrointestinal bleeding is effectively prevented, with improvement in mental status and coagulation factor levels 1
• No reports exist of adult patients treated with mesenterico-Rex shunt 1

Central Splenorenal Shunt

• Central end-to-side splenorenal shunt with splenectomy is the most commonly performed procedure in contemporary series (87% of cases) 5
• This approach addresses both portal hypertension and hypersplenism simultaneously 5

Alternative Shunt Procedures

• Side-to-side splenorenal shunt, mesocaval shunt, and inferior mesenteric vein to left renal vein shunt are viable alternatives depending on vascular anatomy 5, 3
• Portacaval shunt may be performed when other options are not feasible 3
• Gastroesophageal devascularization should be restricted to cases without shuntable portal tributaries 2

TIPS as Alternative to Surgery

Acute Portal Vein Thrombosis

• In non-cirrhotic patients with acute portal vein thrombosis, immediate anticoagulation is recommended first-line 1
• Portal vein thrombectomy/thrombolysis using transjugular approach with or without small caliber TIPS should be considered in those who fail anticoagulation 1

Chronic EHPVO

• In chronic portal hypertension secondary to non-cirrhotic EHPVO not responsive to anticoagulation, TIPS may be considered for the same indications as cirrhotic portal hypertension 1
• Covered TIPS insertion appears feasible when intrahepatic portal veins are visible, though results are available only on short-term follow-up (average 18 months) 1
• Encephalopathy occurs at similar rates as in cirrhotic patients 1
• Portal vein reconstruction-TIPS (PVRecon-TIPS) through intrahepatic collaterals is a novel technique for patients without shuntable venous anatomy 7

Outcomes and Complications

Surgical Outcomes

• Operative mortality ranges from 0-3.7% at specialized centers 3
• Shunt patency is maintained in approximately 87-89% of patients at median follow-up of 41 months 5
• Growth z-scores improve significantly following surgery in pediatric patients 5
• Hepatic encephalopathy is rare following shunt surgery in EHPVO due to preserved liver function 1, 2, 3

Common Pitfalls

• Attempting shunt surgery without adequate assessment of vascular anatomy leads to technical failure 6
• Failing to consider non-variceal sequelae (hypersplenism, growth retardation, portal cholangiopathy) as surgical indications results in suboptimal management 5
• Performing gastroesophageal devascularization when shuntable anatomy exists increases rebleeding risk compared to definitive shunt procedures 2

Management Algorithm

After endoscopic variceal eradication has been achieved:

1. Assess for surgical indications:

   • Recurrent variceal bleeding despite endoscopic therapy 1
   • Symptomatic portal cholangiopathy with biliary complications 1
   • Massive splenomegaly with severe hypersplenism 5
   • Isolated gastric or ectopic varices not amenable to endoscopy 5

2. Evaluate vascular anatomy with Doppler ultrasound and CT/MRI:

   • Identify patent superior mesenteric vein and splenic vein 1, 6
   • Assess for shuntable anatomy 5, 3

3. Select appropriate procedure:

   • If shuntable anatomy present: Perform surgical portosystemic shunt (mesenterico-Rex in children, splenorenal or mesocaval in adults) 1, 5, 3
   • If no shuntable anatomy: Consider PVRecon-TIPS or gastroesophageal devascularization 2, 7

4. Initiate anticoagulation post-procedure according to underlying prothrombotic conditions 1, 7