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Portal Hypertension Bot

Portal Vein Anatomy

  • Length: 5.5-8 cm
  • Diameter: 1 cm
  • Blood Flow Contribution: 75% of liver blood flow
  • Portal Vein Pressure: 5-10 mm Hg
  • Valves: Valveless
  • Unique Feature:
    • Only vein with tributaries and branches (Right and Left Portal Veins)
  • Formation:
    • Forms under the neck of the pancreas

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  • In cases where a patient has a Portal Cavernoma and you need to measure intraoperative Portal Vein (PV) pressure, the pressure can be measured by:

    • Cannulating the omental veins, which are part of the portal circulation.

    This method is used because direct access to the portal vein is not feasible due to the cavernoma's presence. The omental veins, being part of the portal system, provide an alternative route for accurate pressure measurement.

  • The best way to measure the efficacy of a shunt surgery is by:

    • Monitoring the drop in Portal Vein (PV) pressure after the surgery.

Etiology of Portal Hypertension

Prehepatic Causes

  • EHPVO (Extrahepatic portal venous obstruction) - HVPG is normal
  • Portal or splenic vein thrombosis
  • Arteriovenous (AV) fistula

Intrahepatic Causes

  • Pre-Sinusoidal (HVPG is normal):
    • NCPF (Non-cirrhotic portal fibrosis)
    • Schistosomiasis
    • Hepatic Fibrosis
  • Sinusoidal:
    • Only Cause: Cirrhosis due to multiple etiologies
  • Post-Sinusoidal:
    • Most Common: Veno-Occlusive Disease [due to drugs / toxins etc]

Posthepatic Causes

  • Most Common: Budd-Chiari syndrome
  • Other Cause: Constrictive pericarditis

Definition of Portal Hypertension (PHT)

  • Portal Pressure Gradient:
    • HVPG = Portal Vein Pressure (PV) - Hepatic Vein Pressure (HV)
  • Portal Hypertension:
    • Defined as HVPG > 5 mm Hg
  • Measurement:
    • Conducted via Transjugular Hepatic Vein Catheterization using a Balloon Tip Catheter.
  • Clinically Significant Portal Hypertension:
    • HVPG ≥ 10 mm Hg: Associated with the formation of varices.
  • HVPG in Presinusoidal Causes of PHT:
    • Normal HVPG in cases like EHPVO (Extrahepatic Portal Venous Obstruction) and NCPF (Non-Cirrhotic Portal Fibrosis).
Hepatic Vein Pressure HVPG Portal Pressure
Post sinusoidal Increased Increased Increased
Sinusoidal Increased Increased Increased
Pre Sinusoidal Normal Normal Increased
Pre hepatic Normal Normal Increased

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Portal Hypertension and HVPG

  • Normal HVPG:
    • 1 to 4 mm Hg
  • Portal Hypertension:
    • Develops when HVPG > 5 mm Hg
    • Clinically Significant: HVPG > 10 mm Hg (associated with the development of varices)
  • Normal Portal Venous Pressure:
    • 5 to 10 mm Hg
    • Portal Hypertension is defined as portal venous pressure > 10 mm Hg
  • Diagnostic Insights:
    • Presinusoidal Obstruction:
      • HVPG: Normal
      • WHVP (Wedged Hepatic Venous Pressure): Elevated
    • Cirrhosis:
      • Both HVPG and WHVP: Increased
  • Pediatric Considerations:
    • Data suggest similar pressure thresholds (HVPG > 5 mm Hg and HVPG > 10 mm Hg) for the development of complications as in adults, though evidence is limited.

Standard Grading of Esophageal Varices

  • Grade 0: No esophageal varices.
  • Grade 1: Small and non-tortuous esophageal varices.
  • Grade 2: Tortuous varices, limited to less than one-third of the distal esophageal radius.
  • Grade 3: Large and tortuous esophageal varices occupying greater than one-third of the distal esophageal radius.

Japanese Research Society for Portal Hypertension Grading

  • Grade 1: Varices flattened by insufflation.
  • Grade 2: Varices not flattened by insufflation.
    • Not confluent around the esophagus.
  • Grade 3: Varices not flattened by insufflation.
    • Confluent around the circumference of the esophagus.

Management of Acute Variceal Bleed

  1. Initial Stabilization:
    • A: Airway
    • B: Breathing
    • C: Circulation
  2. Transfusion Strategy:
    • Restrictive transfusion: Target Hb < 7 mg/dL
  3. Infection Prophylaxis:
    • IV Antibiotics: Due to a 50% chance of infection
  4. Pharmacological Therapy:
    • Somatostatin / Octreotide / Terlipressin: Administer for 5 days
  5. Endoscopic Treatment:
    • EVL (Endoscopic Variceal Ligation) preferred over EST (Endoscopic Sclerotherapy)
    • Should be performed within 12 hours
    • Tamponade as a temporary measure if needed
  6. Management of Non-Responders:
    • Cirrhotic Patients:
      • TIPS (Transjugular Intrahepatic Portosystemic Shunt) preferred over shunt surgery
    • Non-Cirrhotic Patients:
      • Shunt Surgery

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Transjugular Intrahepatic Portosystemic Shunt (TIPS)

Key Points:

  • Non-selective shunt: Connects the portal vein to the hepatic vein.

Indications:

  1. Acute Variceal Bleeding:
    • Uncontrollable esophageal or gastric variceal hemorrhage.
    • Variceal hemorrhage not amenable to initial or continued endoscopic therapy.
    • Prophylaxis against recurrent variceal bleeding in high-risk patients (5 days from initial bleeding).
  2. Refractory Ascites:
    • Ascites not responding to medical therapy.
  3. Hepatic Hydrothorax:
    • Management of fluid in the pleural space due to liver disease.
  4. Ectopic Variceal Bleed:
    • Bleeding from ectopic varices or stomal varices where other treatments fail.
  5. Portal Vein Recanalization (PVR-TIPS): [not a contraindication as given in shackleford; rather a new indication]
    • Indicated in Portal Vein Thrombosis [Complete Thrombosis = Relative Contraindication]
  6. Bridge to Transplant:
    • Used in patients awaiting liver transplantation.
  7. Additional Indications:
    • Hepatorenal Syndrome
    • Budd-Chiari Syndrome = TIPS is preferred modality of treatment.
    • Portal Hypertensive Gastropathy/Enteropathy: Transfusion-dependent and unresponsive to medical therapy. here we cannot use EVL
    • Decompression of Portosystemic Collaterals before abdominal surgeries.
    • Palliative Care in Portal Hypertension associated with malignancies.
    • Hepatopulmonary Syndrome: Still under study.

Technique of TIPS

  • Target:
    • Intrahepatic Portal Vein
  • Preferred Route:
    • Right Portal Vein to Right Hepatic Vein is the preferred approach.
  • DIPS (Direct Intrahepatic Portocaval Shunt):
    • Involves creating a shunt from the intrahepatic IVC (Inferior Vena Cava) into the portal vein.
  • Stent Type:
    • Covered Stent is preferred. [uncovered block very early]
      • Benefit: Lower rates of encephalopathy compared to uncovered stents.
  • Target HVPG:
    • Aim to reduce Hepatic Venous Pressure Gradient (HVPG) < 12 mm Hg.

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Survival Scores

  1. MELD Score (Model for End-Stage Liver Disease):
    • Used to assess the severity of chronic liver disease.
    • Predicts 3-month survival and prioritizes patients for liver transplantation.
    • Factors: Bilirubin, INR, Creatinine, and Sodium (in MELD-Na).
  2. CTP Score (Child-Turcotte-Pugh Score):
    • Assesses the prognosis of chronic liver disease, mainly cirrhosis.
    • Factors: Bilirubin, Albumin, INR (or Prothrombin Time), Ascites, and Hepatic Encephalopathy.
    • Classification: Class A (5-6 points), Class B (7-9 points), Class C (10-15 points).
  3. APACHE II (Acute Physiology and Chronic Health Evaluation II):
    • Used to predict mortality in critically ill patients.
    • Factors: Acute physiology score, Age, Chronic health points.
  4. Garcia-Pagan Score:
    • Specifically used for assessing prognosis in Budd-Chiari Syndrome.
    • Factors: Age, Bilirubin, INR, Ascites, and Portal Venous Thrombosis.

Key points on TIPS:

  • Encephalopathy:
    • Occurs in 20-30% of cases following TIPS.
  • Early TIPS:
    • Recommended within 24-72 hours for:
      • CTP B / CTP C (Child-Turcotte-Pugh score) patients with CTP score < 14 points.
      • MELD score > 19 or Child-Pugh C patients.
  • Stent Graft:
    • Preferred over medical and endoscopic therapy for better outcomes.

Contraindications for TIPS Procedure [blgart]

Absolute Contraindications:

  • Congestive Heart Failure:
    • Particularly right-sided heart failure.
  • Severe Tricuspid Regurgitation.
  • Severe Pulmonary Hypertension:
    • Mean pulmonary pressure > 45 mm Hg.
  • Uncontrolled Infection.
  • Biliary Obstruction.

Relative Contraindications:

  • Obstruction of All Hepatic Veins.
  • Complete Portal Vein Thrombosis.
  • Hepatocellular Carcinoma:
    • Especially if centrally located.
  • Severe Coagulopathy:
    • INR > 5.

TIPS Procedure Contraindications [Shackleford]

Absolute Contraindications:

  • Primary prevention of variceal bleeding.
  • Congestive heart failure.
  • Severe pulmonary hypertension.
  • Multiple hepatic cysts.
  • Active infections or sepsis.
  • Unrelieved biliary obstruction.

Relative Contraindications:

  • Single hepatic cyst or central hepatoma.
  • Hepatic vein thrombosis.
  • Portal vein thrombosis.
  • Severe coagulopathy or thrombocytopenia.

  • Thrombocytopenia:

    • Platelet count < 20,000/μL.
  • Moderate Pulmonary Hypertension.
  • Recurrent/Persistent Severe Spontaneous Hepatic Encephalopathy.
  • Advanced Liver Failure:
    • Bilirubin > 5 mg/dL or MELD score > 17.
  • Cardiac Dysfunction:
    • Ejection fraction < 60%, cardiac diastolic dysfunction.
  • Advanced Age:
    • 69 years old.

  • Extensive Polycystic Liver Disease.

Key Points on TIPS

  • Type:
    • Non-selective shunt.
    • Side-to-side portocaval shunt.
  • Indications:
    • Preferred when endoscopic therapy fails.
    • Used in medically refractory ascites, hydrothorax, gastric varices, Budd-Chiari syndrome.
  • Complications [from bailey]:
    • Overall Most Common Complication: Encephalopathy (40%).
    • Most Common Early Complication: Intraperitoneal hemorrhage (Bailey).
    • Most Common Late Complication: Shunt stenosis (50% at 1 year).
    • Shunt Stenosis > Shunt Thrombosis.
    • Recurrent Bleed: Often caused by shunt thrombosis.
  • Stent Considerations:
    • Covered Stent: No increased risk of encephalopathy; decreased risk of bleeding.
  • Efficacy:
    • Rebleed Rate: Less than endoscopic treatment.
    • Provides short-term portal decompression.
    • Acts as a bridge to transplant.
  • Transient arrhythmia or sustained supraventricular or ventricular tachycardia.
  • Capsular puncture or perforation.
  • Injuries of the kidney, bowel, gallbladder, or pancreas.
  • Extrahepatic puncture of the main portal vein.
  • Inadvertent puncture of the hepatic artery.
  • Arterioportal fistulae.
  • Intrahepatic biliary duct puncture.
  • Biliary fistulae, hemobilia, cholangitis, sepsis, and stent infection.
  • TIPS stent migration.
  • Undesired migration of embolization material.
  • Rupture of varices during embolization.
  • Hepatic encephalopathy.
  • Liver failure and hepatic insufficiency.
  • Segmental liver infarctions.

Secondary Prophylaxis

  • Pharmacotherapy/Endoscopic therapy/TIPS/Transplant/Shunt surgery.
  • Non-selective beta-blocker + Endoscopic therapy is more effective than endotherapy alone.
  • Non-selective beta-blocker + Isosorbide nitrate is more effective than endotherapy alone.
  • 50% rebleed rate with endotherapy alone.
  • Combination therapy (Pharmacotherapy + Endoscopic therapy) is recommended.
  • Liver transplant offers the lowest rate of rebleeding.

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Sengstaken-Blakemore Tube

  • Purpose: Provides temporary hemostasis in cases of variceal bleeding.
  • Gastric Balloon:
    • Inflated first with 300 ml of air.
  • Esophageal Balloon:
    • Inflated to a pressure of 40 mm Hg.
  • Management:
    • Temporary deflation recommended after 12 hours to reduce the risk of complications.
  • Complications:
    • Risk of esophageal necrosis.
  • Usage: Acts as a bridge therapy until definitive treatment can be provided.
    • Bridge therapy to TIPS or Endoscopic therapy

Non-Selective Shunt Overview

  • Primary Function: Divert portal flow, reducing portal pressure.
  • Risk of Encephalopathy:
    • Increased chance, particularly in cirrhotic patients due to shunting of toxins that bypass liver detoxification.
    • Used in EHPVO (Extrahepatic Portal Venous Obstruction) where the liver is normal, reducing the risk of encephalopathy.
    • NCPF (Non-Cirrhotic Portal Fibrosis) is a pre-cirrhotic state where encephalopathy can still occur due to the altered liver function.

Types of Non-Selective Shunts

  1. End-to-Side Portacaval Shunt (Eck Fistula)
    • Connects the end of the portal vein to the side of the inferior vena cava (IVC).
  2. Side-to-Side Portacaval Shunt
    • Connects the side of the portal vein to the side of the IVC.
  3. Proximal [Conventional] Splenorenal Shunt (Linton Shunt)
    • Connects the splenic vein to the left renal vein.
  4. Mesocaval Shunt
    • Connects the superior mesenteric vein to the IVC.

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Selective Shunts Overview

Selective Shunts Concept:

  • Developed to address the hemodynamic and clinical limitations of nonselective shunts.
  • Aim for selective variceal decompression, preserving portal flow while preventing variceal hemorrhage.

Warren Shunt (Distal Splenorenal Shunt - DSRS):

  • Procedure:
    • Anastomosis of the distal splenic vein to the left renal vein.
    • Interruption of collateral vessels (e.g., coronary vein, gastroepiploic veins) connecting the superior mesenteric vein and gastrosplenic venous circulation.
    • Results in a decompressed gastrosplenic venous circuit and a high-pressure superior mesenteric venous system that continues to perfuse the liver.
  • Indications:
    • Not all patients are candidates, particularly those with intractable ascites or a small splenic vein diameter (<7 mm).

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  • Challenges:
    • Aggravates ascites due to maintained sinusoidal and mesenteric hypertension and transected lymphatic pathways.
    • Gradual loss of portal flow in about 50% of patients by 1 year due to collateralization to the shunt, particularly in alcoholic cirrhosis.
    • Pancreatic siphon effect can divert portal flow to the shunt, especially in alcoholic cirrhosis.
  • Outcomes:
    • Mixed results in clinical trials, with some showing lower encephalopathy rates compared to nonselective shunts, but no clear survival advantage.
    • Effective in preventing recurrent hemorrhage but controversial due to inconsistent trial results.
    • Better outcomes noted in nonalcoholic cirrhosis and noncirrhotic portal hypertension.

Inokuchi Shunt:

  • Procedure:
    • Interposition of a vein graft between the left gastric (coronary) vein and the inferior vena cava (IVC).
    • Directly and selectively decompresses esophagogastric varices.
  • Limitations:
    • Suitable only for a minority of patients due to anatomical requirements.
    • Limited experience, primarily in Japan, with no controlled trials.

Comparisons with Other Treatments:

  • Chronic Endoscopic Therapy:
    • Selective shunting (e.g., DSRS) more effective in preventing recurrent hemorrhage compared to sclerotherapy.
    • However, sclerotherapy maintains hepatic portal perfusion better, although with similar encephalopathy rates.
  • Efficacy:
    • Equivalent to non-selective shunts for preventing recurrent bleeding.
    • Lower encephalopathy rate compared to non-selective shunts.
  • Comparison to TIPS:
    • Similar rates of rebleeding and encephalopathy.

Partial Shunts [ Sarfeh Shunt]

  • Objectives:
    • Effective decompression of varices
    • Preservation of hepatic portal perfusion
    • Maintenance of residual portal hypertension
  • Initial Attempts:
    • Small-diameter vein-to-vein anastomoses
      • Issue: Thrombosis or dilation over time, becoming nonselective shunts
  • Recent Advances:
    • Small-diameter interposition portacaval shunt using PTFE graft
      • Combined with: Ligation of the coronary vein and other collateral vessels
      • Prosthetic graft: ≤10 mm in diameter
        • Outcome: Preserves hepatic portal perfusion in most patients, especially in the early postoperative period

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  • Thrombosis Rate:
    • Less than 15% of shunts thrombosed
    • Management: Most successfully reopened via interventional radiology
  • Clinical Trials:
    • Small Prospective Randomized Trial:
      • Partial Shunt (8 mm) vs. Nonselective Shunt (16 mm)
      • Results: Lower frequency of encephalopathy with partial shunt; similar survival rates for both
    • Controlled Trial:
      • Small-diameter interposition shunt vs. TIPS
      • Outcome: Lower overall failure rate with the small-diameter interposition shunt

Non-Cirrhotic Portal Hypertension (NCPH) Overview

Key Points for Revision:

  • Most Common Cause of Portal Hypertension in Children in India:
    • Extrahepatic Portal Venous Obstruction (EHPVO)
  • Types of Non-Cirrhotic Portal Hypertension:
    • EHPVO (Extrahepatic Portal Venous Obstruction)
    • NCPF (Non-Cirrhotic Portal Fibrosis)
    • HVOTO (Hepatic Vein Outflow Obstruction) = budd chiari and veno occlusive disease.

Extrahepatic Portal Venous Obstruction (EHPVO):

  • Definition: Occlusion of the extrahepatic portal vein, regardless of whether the intrahepatic portal vein, splenic vein, or mesenteric vein is involved.
  • Exclusion of Secondary Causes:
    • Cirrhosis
    • Trauma
    • Malignancy
  • Note: Isolated splenic vein or superior mesenteric vein (SMV) involvement is not considered EHPVO.

Causes of Extrahepatic Portal Vein Obstruction (EHPVO):

  1. Idiopathic (unknown cause)
  2. Prothrombotic State:
    • Portal vein injury
    • Umbilical vein catheterization
    • Abdominal surgery
    • Trauma
    • Liver transplantation
  3. Local Inflammatory Conditions:
    • Intraabdominal abscess
    • Abdominal sepsis
    • Inflammatory bowel disease
    • Pancreatitis
    • Omphalitis (inflammation of the umbilicus)
    • Severe dehydration

Comprehensive Overview of Extrahepatic Portal Vein Obstruction (EHPVO)

Pathophysiology & Etiology

  • 95% Confluence of SMV and Splenic Vein Involved: Obstruction commonly occurs at the confluence of these major veins.
  • Causes:
    • Childhood Infections: Sepsis, cord sepsis, severe dehydration.
    • Pro-coagulant State: Protein C > Protein S deficiency.

Demographics

  • Age Group: Primarily affects children and young adults (1st and 2nd decades of life, mean age: 19 years).

Liver & Clinical Manifestations

  • Liver Function: Remains normal despite portal vein obstruction.
  • Symptoms:
    • Variceal Bleed: Common initial presentation.
    • Splenomegaly and Hypersplenism: Enlarged spleen, leading to anemia and low blood cell counts.
    • Growth Retardation: Notable in 50% of children due to decreased growth hormone and IGF.
    • Portal Biliopathy: Bile duct changes due to obstruction.
    • Ascites: Transient fluid accumulation in the abdomen.
    • Gastric Varices: Presence of varices in the stomach.
    • Chronic Anemia: Persistent anemia due to hypersplenism.

Portal Hypertensive Gastropathy/Colopathy

  • Features:
    • Hyperemic Changes: Seen in the stomach and colon.
    • Mucosal Pattern: Mosaic-like appearance with red spots.
  • Prevalence: More common in EHPVO than cirrhosis.
  • Reversibility: Condition is reversible after shunt surgery.

Notable Absence

  • No Encephalopathy: Unlike cirrhosis, EHPVO does not present with encephalopathy.

NCPF vs EHPVO

Characteristics NCPF/IPH EHPVO
Nature of Precipitating Event Mild, recurring Severe, acute, progressive
Affected Population Childhood, adolescence Neonatal, early childhood
Western countries and also Japan Eastern regions, most commonly India
Median Age 28–32 years 10–16 years
Autoimmune Features Yes No
Splenomegaly Disproportionate and massive Mild to moderate
Encephalopathy Usually absent, except in end-stage liver disease Minimal, occurs as part of natural history of disease
Growth Retardation Usually not seen Commonly seen in prepubertal disease
Portal Biliopathy Uncommon 90%–100%
Hepatic Venous Pressure Gradient Slightly elevated sometimes Normal
USG Features Patent splenoportal axis, thickened and dilated portal vein, periportal fibrosis, massive splenomegaly Portal vein thrombosis with varying degrees of thrombosis involving splenic and mesenteric veins; portal cavernoma formation is mandatory
Histology Portal sclerosis or hepatoportal sclerosis; obliterative portal venopathy. Essential for diagnosis. Unremarkable, or mild periportal fibrosis; Helpful in adult EHPVO

Portal Biliopathy

Definition:

  • Portal Cavernoma Cholangiopathy: Abnormalities in the extrahepatic biliary system including the cystic duct and gallbladder, with or without abnormalities in the 1st and 2nd generation biliary ducts in a patient with portal cavernoma.

Criteria for Diagnosis:

  1. Presence of a Portal Cavernoma.
  2. Typical Cholangiographic Changes:
    • Must be observed on Endoscopic Retrograde Cholangiography (ERC) or Magnetic Resonance Cholangiography (MRC).
  3. Exclusion of Other Causes:
    • Absence of other conditions that could cause these biliary changes, such as bile duct injury, primary sclerosing cholangitis, or cholangiocarcinoma.

Cholangiographic Abnormalities of Portal Cavernoma Cholangiopathy

  • Extrinsic impressions/indentations
  • Shallow impressions/indentations
  • Irregular ductal contour
  • Stricture
  • Filling defects
  • Bile duct angulation
  • Upstream dilatation
  • Ectasia

Stages in the Natural History of Portal Cavernoma Cholangiopathy

Stage Portal Cavernoma Cholangiopathy Liver Biochemistry Symptoms Complications
Preclinical Present Absent Normal Absent Absent
Asymptomatic Present Early changes Normal or abnormal Absent Absent
Symptomatic Present Advanced changes Abnormal Present Absent
Complicated Present Advanced changes Abnormal Present Present

Clinical Presentation:

  • Can be Asymptomatic or Symptomatic.

Asymptomatic Portal Biliopathy:

  • Diagnosis: Typically identified through radiological imaging.
  • Management: Conservative approach; no immediate intervention required.

Symptomatic Portal Biliopathy:

  • Symptoms:
    • Jaundice
    • Cholangitis
    • Pruritus
    • Elevated alkaline phosphatase (ALP) levels.
  • Treatment Options:
    • Stenting: Provides temporary relief.
    • Hepaticojejunostomy (HJ): Definitive treatment for severe cases.
    • Shunt Surgery:
      • Can be performed first, especially in patients with Portal Biliopathy + EHPVO.
      • Outcome: Around 30% of patients may not require HJ after shunt surgery.
      • Approach: Perform shunt surgery, wait 4-6 weeks, then assess if HJ is necessary.

Management of Symptomatic Portal Biliopathy (Algorithm Overview)

  1. Initial Step: Treat cholangitis if present.
  2. Assess for Shuntable Vein:
    • If Shuntable Vein is Present:
      • Perform a Surgical Shunt (e.g., Proximal Splenorenal Shunt).
      • Wait 6-8 weeks:
        • If relieved: Follow-up regularly.
        • If unrelieved: Proceed with a Biliary Drainage Procedure. = HJ
    • If Shuntable Vein is Absent:
      • Consider Endoscopic Management or proceed directly to a Biliary Drainage Procedure.

Algorithm for Managing Portal Biliopathy in Non Cirrhotic Portal HTN {EHPVO ; NCPF]

Initial Classification:

  1. Asymptomatic Portal Biliopathy (PB):
    • Normal Liver Function Tests (LFTs):
      • Management: No treatment required.
    • Persistently Deranged LFTs:
      • Evaluate further with MRCP/USG abdomen.
      • If CBD stone is found, proceed with ERCP-EPT for stone removal and stenting.
      • Follow up regularly.
  2. Symptomatic Portal Biliopathy:
    • Presenting Symptoms:
      • Jaundice, pruritus ⇒ MRCP / USG abdomen
      • Cholangitis ⇒ ERCP / Stenting
    • Initial Treatment:
      • Administer IV antibiotics for cholangitis.
      • Perform ERCP/stenting for bile duct stricture (single or multiple).
    • Assess for Shuntable Vein:
      • If Absent: Continue with endoscopic stenting and regular follow-up.
      • If Present: Consider portosystemic shunt surgery.
    • After Shunt surgery if Persistent Symptoms/Stricture:
      • If symptoms persist, consider biliary bypass (e.g., Roux-en-Y hepaticojejunostomy).

CONTD. Diagnosis of Extrahepatic Portal Venous Obstruction (EHPVO)

  1. Upper Gastrointestinal (UGI) Endoscopy:
    • Used to detect varices and other signs of portal hypertension.
  2. Ultrasound (USG) with Doppler:
    • Primary imaging modality to assess the portal vein and identify any thrombosis or obstruction.
    • Helps in evaluating the blood flow through the portal vein and its branches.
  3. CT Angiography / MR Angiography:
    • Provides detailed imaging of the portal venous system.
    • Useful for mapping the extent of the thrombosis and identifying collateral circulation.
  4. Liver Biopsy:
    • Performed if there is a suspicion of chronic liver disease (CLD).
    • Helps differentiate EHPVO from cirrhosis or other liver pathologies.

Management of Extrahepatic Portal Venous Obstruction (EHPVO)

Primary Prophylaxis of Varices:

  • Surgical Primary Prophylaxis:
    • Not advocated in general for varices.
    • Exception: In EHPVO where liver function is normal, shunt surgery can be performed, particularly in cases with:
      • High-grade varices
      • Hypersplenism
    • NCPF: Shunt surgery is not done due to the risk of postoperative encephalopathy; however, devascularization surgery can be considered.

Acute Bleeding:

  • First-Line Treatment:
    • Therapeutic Endoscopy: Preferred treatment of choice.
    • Medications: Somatostatin to reduce portal pressure and control bleeding.
  • Emergency Surgery:
    • Indicated if endoscopic treatment fails.

Prevention of Rebleeding:

  • Preferred Modality:
    • Endoscopic Variceal Ligation (EVL) is preferred over Endoscopic Sclerotherapy (EST).
  • Limitations:
    • Note: EVL does not affect portal hypertension.
    • PHG and Portal Biliopathy: These conditions typically do not resolve with EVL.
  • Follow-Up:
    • Multiple sessions and close monitoring are required for effective prevention of rebleeding.

Gastrointestinal Bleeding:

  • Preferred Management: Endoscopic Variceal Ligation (EVL).
    • Used to control acute variceal bleeding and prevent recurrent bleeding.

Indications for Surgery:

  • Multiple Bleeds: Especially when requiring frequent blood transfusions.
  • Growth Retardation: To improve overall growth and development.
  • Symptomatic Portal Biliopathy: When biliary symptoms persist or worsen.
  • Symptomatic Hypersplenism: Indicated in cases with significant anemia, leukopenia, or thrombocytopenia.
  • Rare Blood Group/Far Off Place: When regular access to medical care is challenging.

General Note: Surgery is generally preferred over Endotherapy for long-term management.

Surgical Options:

  1. Portosystemic Shunts:
    • Selective Shunts:
      • DSRS (Distal Splenorenal Shunt) - also known as Warren Shunt.
        • Note: This shunt loses its selectivity over time.
    • Non-Selective Shunts (Preferred):
      • PSRS (Proximal Splenorenal Shunt):
        • Preferred as it manages splenomegaly effectively.
        • Advantage: Encephalopathy is rare in EHPVO, making non-selective shunts more suitable.
      • Mesocaval Shunt. = SMV to IVC
      • Side-to-Side Splenorenal Shunt. = MITRA SHUNT (MCQ)
        • proposed by MITRA from PGI CHANDIGARH]
        • Splenic vein to left Renal Vein by side to side fashion
        • done in Children
        • Dont Have to remove the spleen
  2. Hepatic Blood Flow:

    • Even in non-selective shunts, hepatic blood flow is preserved in EHPVO, making these shunts safer and effective for managing the condition.

    Rationale for Proximal Splenorenal Shunt (PSRS)

    • Non-Selective Shunt:
      • PSRS is a non-selective shunt that diverts blood from the portal system to the systemic circulation.
    • Splenectomy:
      • Removes the spleen, addressing hypersplenism and reducing complications related to an enlarged spleen.
    • Treats Portal Biliopathy, Portal Hypertensive Gastropathy (PHG), and Colopathy:
      • Effective in treating complications associated with portal hypertension in EHPVO, including biliary and gastrointestinal issues.
    • Rebleeding Risk:
      • Low risk of rebleeding, with rates ranging from 0-10%.
    • Long-Term Shunt Patency:
      • Ensures sustained relief from portal hypertension and its complications.
    • Low Risk of Encephalopathy:
      • EHPVO: Minimal risk of encephalopathy, making PSRS a safer option.
      • NCPF: Higher risk of encephalopathy, hence PSRS is less favored in NCPF cases.

    Rex Shunt Overview

    • Type of Shunt:
      • Mesenterico-Left Portal Vein Bypass Shunt.
    • Initial Use:
      • Originally developed for pediatric liver transplant recipients.
    • Clinical Benefits:
      • Reverses hypersplenism and promotes the normalization of liver size by restoring portal venous flow to the liver.
    • Unsuitable Candidates:
      • Not suitable in cases with:
        • Absence of the left portal vein.
        • Superior Mesenteric Vein (SMV) thrombosis.

    Meso-Rex Bypass (MRB) Overview

    Indication:

    • Preferable Approach: According to the Baveno VI Pediatric Satellite Symposium, MRB is the preferred preprimary and primary treatment for children with Extrahepatic Portal Venous Obstruction (EHPVO).

    Procedure:

    • Bypass:
      • MRB creates a bypass between the superior mesenteric vein and the Rex recessus (a remnant of the ductus venosus).

    Criteria for MRB:

    • Absence of Underlying Liver Disease:
      • The patient should have normal Hepatic Venous Pressure Gradient (HVPG).
    • Absence of Prothrombotic State:
      • The patient should not have any known prothrombotic conditions.
    • Body Weight:
      • The child should weigh more than 8 kg to be considered for the procedure.
    • Favorable Anatomy:
      • Confirmed through a retrograde internal jugular venogram.
      • Patent Superior Mesenteric, Splenic, and Bilateral Internal Jugular Veins.
    • Absence of Significant Cardiovascular Abnormalities:
      • No significant cardiovascular issues or pulmonary hypertension should be present.

Operative Devascularization Overview

Indications:

  • Endoscopic Failure: 10-15% of patients may fail endoscopic intervention.
  • Surgical Options: Shunt or Devascularization procedures.

Procedure Objectives:

  • Directed Towards:
    • Esophagus and Stomach: The goal is to manage varices by addressing the blood vessels feeding these areas.
  • Maintains Portal Perfusion:
    • Helps in maintaining portal blood flow, which reduces the incidence of hepatic encephalopathy.
  • Shunting Limitations:
    • Extensive Thrombosis: Shunting is not feasible in cases with extensive portal vein thrombosis.
  • Applicability:
    • Can be used in both cirrhotic and non-cirrhotic patients when TIPS (Transjugular Intrahepatic Portosystemic Shunt) is not available.
  • Primary Prophylaxis:
    • Can be considered for the primary prophylaxis of variceal bleed.

Limitations:

  • Ectopic Varices:
    • Does not control bleeding from ectopic varices (varices in locations other than the gastroesophageal region).
  • Ascites:
    • Does not treat or alleviate ascites.
  • Portal Biliopathy:
    • Conflicting evidence exists, but a paper from GB Pant suggests that portal biliopathy can be resolved through devascularization surgery.

Vascular Targets:

  • Coronary Veins ———> Azygos Veins
  • Submucosal Plexus
  • Subepithelial Veins
  • Intraepithelial Veins
  • Periesophageal Veins

Types of Devascularization Procedures:

  1. Hassab Procedure:
    • Focus: Targets extramural vessels (outside the wall of the esophagus).
    • Veins Ligated: Periesophageal veins and coronary vein are ligated, which feed the gastroesophageal varices.
    • Purpose: Primarily reduces blood flow to gastroesophageal varices by addressing major feeding vessels.
  2. Sugiura Procedure:
    • Focus: Targets both extramural and intramural vessels (inside the wall of the esophagus) feeding gastroesophageal varices.
    • Preserves Longitudinal Esophageal Vessels:
      • Maintains the spontaneous portoazygos flow in the region, which is crucial for reducing portal hypertension without increasing the risk of encephalopathy.

Hassab's Procedure Overview

Original Procedure (1972):

  • Splenectomy: Removal of the spleen.
  • Devascularization:

    • Cardiac Stoma: Blood vessels around the gastric cardia are ligated.
    • Abdominal Portion of Esophagus: Devascularization extends to this area to reduce variceal bleeding risk.

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Critical Considerations in the Original Hassab Procedure

  • Main Trunk of Left Gastric Vessels:
    • Not preserved in the original Hassab procedure.
    • Division of Left Gastric Vein:
      • Can disrupt the spontaneous portacaval shunt between the left gastric vein and paraesophageal veins.
      • Impact:
        • Increases Portal Pressure: This disruption can lead to increased portal pressure.
        • Formation of New Collateral Pathways: Promotes the development of new varices due to rerouting of blood flow.
        • Recurrence of Esophageal Varices: High likelihood of variceal recurrence.
        • Exacerbation of Portal Hypertensive Gastropathy (PHG): Worsens the condition by promoting additional varices and portal hypertension.

Modified Hassab's Procedure

  • Splenectomy: Continues to be a key component.
  • Perihiatal Devascularization:
    • Targeting the lower esophagus near the hiatus.
  • Preservation of Left Gastric Vein:
    • Essential to reduce blood flow to gastroesophageal varices.

Proposed Modification of hassab

  • Selective Devascularization:
    • Focus on Branch Veins: Only the branch veins that directly enter the wall of the esophagus and stomach should be devascularized.
    • Rationale:
      • By preserving the main trunk and selectively ligating branch veins, the procedure aims to reduce the risks of increasing portal pressure and the recurrence of varices, while still effectively managing the risk of variceal bleeding.

Sugiura and Futagawa Procedure = 2 stage (Concise Overview)

  • Similarities to Hassab Procedure:

    • Splenectomy and devascularization of the parasesophagogastric area.

    image.png

  • Key Differences:

    • Selective Ligation:
      • Only transverse branches to the esophagus are ligated, preserving paraesophageal longitudinal channels and left gastric vessels.
    • Esophageal Transection:
      • Added to disrupt intramural portosystemic connections, preventing revascularization of esophageal varices.
    • Selective Vagotomy done
  • Benefits:
    • Reduces Portal Pressure by preserving the portacaval shunt between the left gastric and paraesophageal veins.
    • Lower Risk of Hepatic Decompensation compared to total shunt procedures, as portal pressure reduction is moderate.

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Modified Sugiura's Procedure (Concise Overview)

  • Description:
    • Developed by Ginsberg and Umeyama.
    • One-stage, trans-abdominal approach.
  • Key Components:
    • Devascularization of the upper two-thirds of the greater and lesser curvature of the stomach.
    • Splenectomy.
    • Devascularization of the lower 7-8 cm of the esophagus.
    • Esophageal Transection:
      • Performed 2 cm above the gastroesophageal junction (GEJ) using a circular stapler.

Mathur's Procedure (Concise Overview)

  • Transabdominal Devascularization.
  • Gastroesophageal Stapling.
  • Nissen Fundoplication (to prevent reflux).
  • +/- Splenectomy (optional, based on clinical need).

This procedure combines devascularization, stapling, and fundoplication, with the option of splenectomy, to manage complications of portal hypertension.

Complications of Devascularization Procedures

  • Portal Vein Thrombosis: A potential risk following surgery.
  • Thrombocytosis: An increase in platelet count, which can occur postoperatively.

Outcome of Devascularization Procedures

  • Immediate Control of Bleeding: Achieved in 95-100% of cases.
  • Rebleeding Rates:
    • Hassab Procedure: 6.2% to 8.3%.
    • Sugiura Procedure: 1.5% to 16%.

Budd-Chiari Syndrome Overview

  • Most Common Causes:
    • Polycythemia Vera (most common)
    • Paroxysmal Nocturnal Hemoglobinuria (PNH)
  • Etiology:
    • Eastern Variant: Membranous obstruction of the Inferior Vena Cava (IVC)
    • Thrombosis: Can affect the IVC, hepatic veins (HV), or both.
  • Pathophysiology:
    • Leads to ischemia, centrilobular necrosis, congestion, and atrophy of the liver.
    • Early Stage: Changes are reversible.
    • Prolonged Stage: Can progress to cirrhosis.
  • Clinical Features:
    • Abdominal Pain
    • Ascites
    • Hepatomegaly

Differences Between West and East in Budd-Chiari Syndrome (BCS)

Feature West East
Membranous Obstruction of the IVC Rare Frequent
Hepatic Vein Occlusion Predominates + -
IVC Occlusion Predominates - +
Acute or Subacute BCS Predominates + -
Chronic BCS Predominates - +
Pregnancy/Postpartum Uncommon Frequent
Infection Rare Common
Oral Contraceptives Frequent Uncommon
Myeloproliferative Disease Common Rare

Key Points:

  • Western BCS:
    • Hepatic vein occlusion and acute/subacute BCS are more common.
    • Myeloproliferative diseases and oral contraceptive use are frequent contributing factors.
  • Eastern BCS:
    • Membranous obstruction of the IVC and chronic BCS predominate.
    • Pregnancy/postpartum period and infection are more common triggers.

Diagnosis of Budd-Chiari Syndrome (BCS)

  • Venography:
    • Gold Standard for diagnosing BCS, providing detailed imaging of the venous system.
  • IVC Pressure Measurement:
    • Importance: Critical for determining the appropriate surgical intervention.
    • Normal IVC Pressure:
      • Indicates that a side-to-side portacaval shunt can be performed safely.
      • DIPS also can be done.
      • this shunt surgery doesnot work if IVC pressure is elevated
  • Wedge Hepatic Pressure:
    • Elevated in BCS, reflecting increased pressure within the hepatic veins.
  • Additional Diagnostic Tools:
    • Liver Biopsy: Helps assess the extent of liver damage.
    • TIPS (Transjugular Intrahepatic Portosystemic Shunt): May be considered as both a diagnostic and therapeutic option.

Treatment Approaches for Budd-Chiari Syndrome (BCS)

  • Percutaneous Interventions: Preferred over surgical approaches.
    • Options:
      • Thrombolysis: To dissolve clots.
      • Transluminal Angioplasty: To open up narrowed veins.
      • Venous Stenting: To keep veins open.
      • TIPS (Transjugular Intrahepatic Portosystemic Shunt): To reduce portal pressure and manage symptoms.
      • DIPS
  • Surgical Shunts:
    • Side-to-Side Portacaval Shunt:
      • Indicated for hepatic vein thrombosis.
      • Contraindicated in IVC thrombosis.
    • Mesoatrial Shunt: Used when there is IVC obstruction or as a combined shunt option.
  • Pressure Considerations:
    • IVC Pressure < Wedge Pressure:
      • Indicates IVC is patent, and shunts like splenorenal or interposition mesocaval (though it has a high thrombosis rate) can be considered.
    • IVC Obstruction:
      • Mesoatrial Shunt or a combined shunt is recommended.

MCQ’s On Portal HTN

Question: Most accurate definition of portal hypertension in EHPVO?

A. HVPG > 5 mmHg

B. Portal vein pressure > 15 mmHg

C. FHVP > 5 mmHg

D. Wedge hepatic vein pressure > 10 mmHg

Answer: B. Portal vein pressure > 15 mmHg

Explanation: In EHPVO, all other pressures (HVPG, FHVP, wedge hepatic vein pressure) remain normal. The most accurate measure of portal hypertension in this context is an elevated portal vein pressure > 15 mmHg.

A. 5 mm Hg

B. 6 mm Hg

C. 7 mm Hg

D. 10 mm Hg

Answer: D. 10 mm Hg

Explanation:

  • HVPG (Hepatic Venous Pressure Gradient): Difference between hepatic vein pressure and sinusoidal pressure.
  • Portal Hypertension (PHT): Defined as HVPG > 5 mm Hg.
  • Clinically Significant Portal Hypertension (CSPH): Defined as HVPG > 10 mm Hg.
  • Variceal Bleed: Occurs when HVPG > 12 mm Hg.
  • HVPG is Elevated in sinusoidal causes of cirrhosis but remains normal in conditions like NCPF and EHPVO.

Question: Mr. X, a known case of Alcoholic CLD, underwent routine endoscopy screening. UGI shows grade II esophageal varices. Which of the following is NOT true about his condition?

A. Varices are present in 40% of compensated cirrhotic patients

B. Risk of developing varices is 5-10% per year

C. Risk of symptomatic bleeding is 10% per year

D. Primary prophylaxis with EVL and beta blocker should be started

Answer: D. Primary prophylaxis with EVL and beta blocker should be started

Explanation:

  • Primary Prophylaxis: Involves either Endoscopic Variceal Ligation (EVL) or beta blockers, but not both together.
  • Natural History:
    • Varices develop in 40% of compensated cirrhosis.
    • Varices develop in 60% of decompensated cirrhosis.
    • Risk of symptomatic bleeding is 10% per year.
    • Risk of varices development without existing varices is 1% per year.
    • Risk factors for bleeding: Size of varices, severity of liver disease, HVPG, and red wale markings.

Primary Prophylactic Therapy for Esophageal Varices: Prevention of Initial Bleeding

Variceal Grade Recommended Therapy
No varices No treatment
Small, CTP class A, no red wale markings No treatment
Small, CTP class B or C, or red wale markings β-Blocker
Medium or large, CTP class A, no red wale markings β-Blocker preferred; Endoscopic Variceal Ligation if β-blocker contraindicated
Medium or large, CTP class B or C, or red wale markings β-Blocker or Endoscopic Variceal Ligation

Secondary Prophylactic Therapy for Esophageal Varices: Prevention of Rebleeding

  1. All patients should receive prophylactic therapy to prevent recurrent variceal hemorrhage.
  2. Recommended Therapy: β-blocker and Endoscopic Variceal Ligation.
  3. Alternative Strategy: β-blocker plus nitrates or Endoscopic Variceal Ligation.
  4. TIPS (Transjugular Intrahepatic Portosystemic Shunt) is recommended only in patients with recurrent variceal bleeding that is refractory to pharmacologic and endoscopic therapy.

A. PRBC transfusion to maintain Hb of 9-10

B. Administration of antibiotics

C. Terlipressin for 5 days

D. Self-expanding metal stents

Answer: A. PRBC transfusion to maintain Hb of 9-10

Explanation:

  • Restrictive Transfusion Protocol: In the management of acute variceal bleeding, a restrictive transfusion policy is followed to maintain Hb between 7-9 g/dL.
  • Other Management Steps:
    • Antibiotic prophylaxis is standard to prevent infection.
    • Terlipressin (or other vasoactive drugs) is administered for 5 days to control bleeding.
    • Endoscopy within 12 hours is crucial, with EVL (Endoscopic Variceal Ligation) preferred over EST (Endoscopic Sclerotherapy).
    • Self-expanding metal stents are not typically part of the initial management but may be considered in certain situations if standard therapies fail.

Management of Refractory Variceal Bleeding

  • Sengstaken-Blakemore Tube: Temporarily controls bleeding by applying direct pressure on varices.
  • Self-Expandable Metal Stent (SEMS): Provides immediate control of bleeding, particularly when endoscopic and pharmacologic therapies fail.
  • TIPS (Transjugular Intrahepatic Portosystemic Shunt): Creates a shunt within the liver to reduce portal pressure, often used when other measures fail.
  • Shunt Surgery: Considered in cases where TIPS is not successful or available.
  • Devascularization: Surgical removal of the blood supply to varices, used as a last resort when other methods are ineffective.

Question: Preferred modality of treatment of IGV-1 includes all except

A. EVL

B. Cyanoacrylate glue

C. TIPS

D. Splenectomy

Answer: A. EVL

Explanation:

  • Serine's Classification of Varices:
    • GOV-1: Gastroesophageal varices extending along the lesser curvature.
    • GOV-2: Gastroesophageal varices extending along the greater curvature.
    • IGV-1: Isolated gastric varices in the fundus.
    • IGV-2: Isolated gastric varices in areas other than the fundus.

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  • Treatment for IGV-1:
    • Cyanoacrylate glue: Effective in treating IGV-1 by sealing the varices.
    • TIPS: Reduces portal pressure and is effective in controlling bleeding.
    • BRTO
    • Splenectomy: Can be considered in specific cases involving hypersplenism or other related conditions.
  • EVL (Endoscopic Variceal Ligation):
    • Not effective in IGV-1 as it is primarily designed for esophageal varices, not isolated gastric varices.

Question: Indications for TIPS include all except

A. Hepatorenal syndrome

B. Portal vein thrombosis

C. Portal hypertensive gastropathy

D. None of the above

Answer: D. None of the above

Explanation:

  • TIPS (Transjugular Intrahepatic Portosystemic Shunt) is commonly indicated for:
    • Hepatorenal Syndrome: To improve renal function by reducing portal hypertension.
    • Portal Vein Thrombosis: PVR-TIPS can help manage complications associated with portal hypertension despite thrombosis.
    • Portal Hypertensive Gastropathy: To reduce portal pressure and prevent recurrent bleeding.

Question: Indications for TIPS in acute variceal bleeding are all except

A. MELD > 16

B. Gastric varices with PHG

C. Primary prophylaxis of variceal bleed

D. Child C cirrhosis

Answer: C. Primary prophylaxis of variceal bleed

Explanation:

  • TIPS (Transjugular Intrahepatic Portosystemic Shunt) is indicated in:
    • MELD > 16: High MELD score indicates severe liver disease where TIPS might be necessary.
    • Gastric Varices with PHG (Portal Hypertensive Gastropathy): TIPS is effective in managing bleeding from these varices.
    • Child C Cirrhosis: TIPS can be life-saving in decompensated cirrhosis with acute variceal bleeding.
  • Primary Prophylaxis of Variceal Bleed: TIPS is not used for primary prophylaxis; it is reserved for cases where there is a significant risk of bleeding or failure of other treatments.

Question: Not true about encephalopathy in TIPS

A. Risk is around 20-30%

B. Risk is more in stent graft TIPS

C. More in Child C

D. All of the above

Answer: B. Risk is more in stent graft TIPS

Explanation:

  • Risk of Encephalopathy:
    • 20-30% is the general risk of encephalopathy after TIPS.
    • More in Child C: Higher risk due to more advanced liver disease.
    • Stent Graft TIPS: The risk of encephalopathy is actually lower in stent graft TIPS compared to uncovered stents because liver function is preserved longer.

Question: Absolute Contraindication to TIPS

A. Unresolved biliary obstruction

B. Preexisting encephalopathy

C. Ejection fraction 50%

D. Portal vein thrombosis

Answer: A. Unresolved biliary obstruction

Explanation:

  • Unresolved Biliary Obstruction: This is an absolute contraindication to TIPS because it can lead to worsening of biliary obstruction and complications.
  • Preexisting Encephalopathy: Although a risk factor, it is not an absolute contraindication.
  • Ejection Fraction 50%: This is generally acceptable for TIPS as it indicates adequate cardiac function.
  • Portal Vein Thrombosis: TIPS can still be considered in selected cases of portal vein thrombosis.

Question: Selective shunts are all except

A. DSRS

B. Inokuchi shunt

C. 8mm Sarfeh shunt

D. Clatworthy shunt (common iliac vein to SMV)

Answer: D. Clatworthy shunt

Explanation:

  • Selective Shunts: Designed to decompress the portal vein while preserving hepatic blood flow, typically targeting specific veins:
    • DSRS (Distal Splenorenal Shunt)
    • Inokuchi Shunt
    • 8mm Sarfeh Shunt
  • Clatworthy Shunt: Involves a connection between the common iliac vein and the superior mesenteric vein (SMV), which is not selective as it does not specifically preserve hepatic perfusion.

Question: All of the following are divided during distal splenorenal shunt except

A. Short gastric vein

B. Coronary vein

C. Right gastroepiploic vein

D. All of the above

Answer: A. Short gastric vein

Explanation:

  • Distal Splenorenal Shunt (DSRS) involves:
    • Division of the coronary vein (left gastric vein).
    • Division of the right gastroepiploic vein.
  • The short gastric veins are typically preserved during the DSRS to maintain collateral circulation and reduce the risk of rebleeding from varices.

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Question: Which of the following has minimal risk of encephalopathy after PSRS?

A. Child A cirrhosis

B. NCPF

C. EHPVO

D. Budd-Chiari syndrome

Answer: C. EHPVO

Explanation:

  • Proximal Splenorenal Shunt (PSRS) involves splenectomy and an anastomosis of the distal splenic vein to the left renal vein, creating a non-selective shunt.
  • EHPVO (Extrahepatic Portal Venous Obstruction) has minimal risk of encephalopathy after PSRS because the liver function is typically normal.
  • In contrast, conditions like NCPF and Child A cirrhosis carry a higher risk of encephalopathy due to underlying liver dysfunction.

Question: A 25-year-old male presents with recurrent episodes of variceal bleed. On examination, massive splenomegaly is present. USG shows a normal liver, and the portal vein is replaced by a cavernoma. All of the following are surgical options for him except:

A. PSRS (Proximal Splenorenal Shunt)

B. Side-to-side splenorenal shunt

C. Meso-Rex shunt

D. Meso-caval shunt

Answer: B. Side-to-side splenorenal shunt

Explanation:

  • PSRS (Proximal Splenorenal Shunt): A suitable option, especially with splenomegaly.
  • Meso-Rex Shunt: Preferred for restoring normal portal flow, especially in young patients with a cavernoma.
  • Meso-Caval Shunt: Another valid option for managing portal hypertension.
  • Side-to-side splenorenal shunt is not suitable in this scenario because the presence of massive splenomegaly makes this procedure less feasible or effective.

Question: A 25-year-old male presents with pain in the left hypochondrium along with recurrent epistaxis. USG shows altered echogenicity, a dilated portal vein, and UGI shows grade II esophageal varices. Hb is 7.6, TLC is 1300, and Platelets are 25,000. What is the most appropriate treatment?

A. Observation

B. PSRS

C. Splenectomy alone

D. Hassab's procedure

Answer: D. Hassab's procedure

Explanation:

  • The patient likely has Non-Cirrhotic Portal Fibrosis (NCPF), characterized by symptomatic hypersplenism and non-bleeding varices.
  • In NCPF, shunt surgeries (like PSRS) are generally avoided due to the risk of worsening hepatic encephalopathy.
  • Hassab's procedure involves splenectomy combined with esophagogastric devascularization, which is suitable for managing symptomatic hypersplenism and reducing the risk of variceal bleeding.

Question: A 20-year-old male presents to the OPD with a routine USG report showing portal cavernoma. UGI shows grade II varices. What is the further treatment?

A. PSRS

B. EVL

C. DSRS

D. Observation

Answer: B. EVL

Explanation:

  • Diagnosis: The patient likely has EHPVO (Extrahepatic Portal Venous Obstruction) with grade II varices.
  • Management:

    • Since the patient has grade II varices without symptoms of hypersplenism or major bleeding, Endoscopic Variceal Ligation (EVL) is the appropriate treatment to prevent bleeding.
    • PSRS or other shunt surgeries would be considered in cases of high-grade varices, symptomatic hypersplenism, or major bleeding.

    Indications for Surgery in EHPVO (Concise)

    • Major Bleed: Requires transfusion/hospitalization.
    • Portal Biliopathy: To relieve biliary obstruction.
    • Growth Retardation: To improve growth.
    • Prophylactic Shunt: For high-grade varices (not in NCPF).
    • Symptomatic Hypersplenism: To manage severe anemia, thrombocytopenia, or leukopenia.

Question: A 35-year-old female presents with pain in the right hypochondrium. USG shows gallstones. The CBD is normal with no intrahepatic biliary radicle dilation (IHBRD). There is cavernomatous transformation of the portal vein. What is the diagnosis?

A. Symptomatic gallstone disease with EHPVO

B. Portal biliopathy

C. Symptomatic gallstone disease with NCPF

D. Both A and B

Answer: A. Symptomatic gallstone disease with EHPVO

Explanation:

  • The patient has gallstones causing symptomatic cholelithiasis, which explains the pain in the right hypochondrium.
  • Cavernomatous transformation of the portal vein is consistent with Extrahepatic Portal Venous Obstruction (EHPVO).
  • Since the CBD is normal and there is no IHBRD, the primary issue is the gallstone disease rather than portal biliopathy.

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Portal Cavernoma Cholangiopathy Overview

  • Definition: Abnormalities in the extrahepatic biliary system, including the cystic duct and gallbladder.
  • Prevalence: Occurs in 80-90% of patients with EHPVO.
  • Symptoms:
    • Majority are asymptomatic.
  • Most Commonly Affected Duct: Left hepatic duct.
  • Resolution: 30-40% of cases resolve with shunt surgery.

Appearance on Imaging:

  • Extrinsic Impression: The ducts appear compressed from the outside.
  • Indentation: Depressions or notches on the duct surface.
  • Irregular Ductal Contour: The ducts have an uneven or distorted outline.
  • Stenosis: Narrowing of the ducts.
  • Filling Defect: Areas within the ducts that do not fill with contrast, indicating obstruction or irregularity.

Question: A 25-year-old female presents with a lump in the left abdomen. On examination, the spleen is palpable. USG shows a portal cavernoma and bilateral mild IHBRD. LFT shows Bilirubin 2.3/1.2 and Alk Phos 400. UGI shows grade II varices, for which banding was done. There is no history of GI bleed. What is the next step?

A. PSRS

B. Meso-Rex shunt

C. Observation

D. None of the above

Answer: C. Observation

Explanation:

  • Diagnosis: The patient has EHPVO with non-bleeding grade II varices, splenomegaly without hypersplenism, and portal biliopathy with lab abnormalities but no symptoms.
  • Since banding for varices has already been performed and the patient is not symptomatic from biliopathy, surgery is not mandatory at this stage.
  • Observation or MRCP to monitor for changes in the CBD is a reasonable approach, with a wait-and-watch strategy.

Question: An 18-year-old male presents with jaundice. USG shows bilateral IHBRD with a dilated CBD along with a portal cavernoma. Bilirubin is 4.2/3.4, Alk Phos is 330. UGI shows grade III varices. What is the management?

A. Hepaticojejunostomy

B. ERCP

C. PSRS

D. DSRS

Answer: C. PSRS

Explanation:

  • The patient has EHPVO with high-grade varices and symptomatic portal biliopathy.
  • PSRS (Proximal Splenorenal Shunt) is the preferred surgical option to manage both the high-grade varices and the portal biliopathy, reducing the risk of further complications.

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Question: While performing PSRS, you realize that the splenic vein is thrombosed. Therefore, devascularization of the greater and lesser curvature of the stomach and lower third of the esophagus, along with ligation of the coronary vein and splenectomy, was done. What is the operative procedure?

A. Hassab

B. Sugiura

C. Mathur's

D. None of the above

Answer: A. Hassab

Explanation:

  • Hassab's Procedure involves splenectomy combined with devascularization of the greater and lesser curvature of the stomach and the lower third of the esophagus, along with ligation of the coronary vein.
  • This matches the description provided in the scenario.

Question: Portal hypertension is best defined as:

A. HVPG > 5 mm Hg

B. Portal pressure > 5 mm Hg

C. None of the above

D. Both A and B

Answer: A. HVPG > 5 mm Hg

Explanation:

  • Portal Hypertension is most accurately defined by an Hepatic Venous Pressure Gradient (HVPG) > 5 mm Hg. This measurement reflects the pressure difference between the portal vein and hepatic veins, which is the standard definition of portal hypertension.

Question: Complications that occur after PSRS for NCPF include all except:

A. Encephalopathy

B. Nephropathy

C. Myelopathy

D. None of the above

Answer: D. None of the above

Explanation:

  • Encephalopathy: Commonly seen after PSRS in NCPF due to altered hepatic blood flow.
  • Nephropathy: Specifically, IgA nephropathy has been reported as a complication.
  • Myelopathy: Neurological complications like myelopathy can also occur.

Question: Extrahepatic portal vein obstruction is characterized by all except:

A. Elevated portal vein pressure

B. Elevated wedged hepatic pressure

C. Normal Hepatic venous pressure gradient

D. None of the above

Answer: B. Elevated wedged hepatic pressure

Explanation:

  • Elevated Portal Vein Pressure: True in EHPVO due to the obstruction in the portal vein.
  • Normal Hepatic Venous Pressure Gradient (HVPG): True, as the obstruction is extrahepatic and does not directly affect the hepatic venous pressures.
  • Elevated Wedged Hepatic Pressure: False in EHPVO, as the obstruction is not within the liver parenchyma but outside, so the wedged hepatic pressure remains normal.

Thus, the correct answer is B. Elevated wedged hepatic pressure.

  • Most common cause of portal hypertension • A-Cirrhosis • B-EHPVO • C-Budd Chiary syndrome • D-Schistostomiasis

• All of the following is done during variceal bleed except • A-Restrictive transfusion • B-Emergency portocaval shunt • C-IV antibiotics • D-Beta blocker

  • 50 year old male Hepatitis related CLD. Upper Gl shows grade 3 varices. Next management • A-EVL • B-Beta blocker • C-Both EVL and Beta blocker • D-Both A and B

Question: A 55-year-old male presents with refractory ascites along with recurrent variceal bleed. What is the most effective therapy?

A. Side-to-side portacaval shunt

B. End-to-side portacaval shunt

C. Distal splenorenal shunt

D. Proximal splenorenal shunt

Answer: A. Side-to-side portacaval shunt

Explanation:

  • Side-to-Side Portacaval Shunt: This non-selective shunt is preferred because it allows the liver to drain into the caval system, effectively managing both refractory ascites and recurrent variceal bleeding.
  • End-to-Side Portacaval Shunt: While also non-selective, it doesn't provide the same drainage for the liver into the caval system, making it less effective.
  • Distal Splenorenal Shunt (DSRS): Selective shunt, not suitable for this case.
  • Proximal Splenorenal Shunt (PSRS): Non-selective but not required here since there is no EHPVO and splenectomy isn't necessary.

Thus, the correct answer is A. Side-to-side portacaval shunt.

Question: Not done in Warren shunt

A. Splenectomy

B. Ligation of coronary vein

C. Ligation of gastroepiploic veins

D. All of the above

Answer: A. Splenectomy

Explanation:

  • Warren Shunt (Distal Splenorenal Shunt):
    • This procedure preserves the spleen and involves creating a shunt between the splenic vein and the left renal vein.
    • Ligation of the coronary vein and ligation of gastroepiploic veins are part of the procedure to reduce blood flow to varices.

Question: Clinical triad of Budd-Chiari Syndrome (BCS) is all except:

A. Pain

B. Hepatomegaly

C. Ascites

D. Jaundice

Answer: D. Jaundice

Explanation:

  • The classic clinical triad of Budd-Chiari Syndrome (BCS) includes:
    • Pain (usually in the upper right abdomen)
    • Hepatomegaly (enlarged liver)
    • Ascites (accumulation of fluid in the abdomen)
  • Jaundice is not a typical part of the clinical triad, although it can occur in some cases.

Thus, the correct answer is D. Jaundice.

Portopulmonary Syndrome Overview

Definition:

  • Portopulmonary Syndrome encompasses two related conditions involving liver disease and pulmonary complications:
  • Hepatopulmonary Syndrome (HPS):
    • Triad:
      • Liver Disease
      • Arterial Hypoxemia
      • Intrapulmonary Vascular Dilation
    • Characteristics: Marked by hypoxemia due to intrapulmonary shunting, but without pulmonary hypertension.
  • Portopulmonary Hypertension:
    • Characteristics: Involves pulmonary hypertension in patients with chronic liver disease.
    • Prognosis: More severe with a poorer prognosis compared to HPS.

Treatment:

  • Limited success with medical treatment.
  • Liver Transplantation:
    • Indicated for patients, particularly in pediatric cases, when medical management fails.

Question: Most common cause of Budd-Chiari Syndrome (BCS) is:

A. Polycythemia vera

B. PNH

C. Protein V Leiden mutation

D. Malignancy

Answer: A. Polycythemia vera

Explanation:

  • Polycythemia vera is the most common cause of Budd-Chiari Syndrome (BCS), leading to thrombosis of the hepatic veins.
  • Other causes like Paroxysmal Nocturnal Hemoglobinuria (PNH), Protein V Leiden mutation, and malignancy are also associated but are less common.

Thus, the correct answer is A. Polycythemia vera.

Question: Which of the following surgery is advocated in Budd-Chiari Syndrome (BCS) in a patient with IVC obstruction along with hepatic vein obstruction?

A. Side-to-side portacaval

B. Mesocaval

C. Mesoatrial

D. Splenorenal

Answer: C. Mesoatrial

Explanation:

  • In cases of Budd-Chiari Syndrome with IVC obstruction and hepatic vein obstruction, a mesoatrial shunt is the preferred surgical option. This procedure bypasses the obstruction by creating a direct connection between the superior mesenteric vein and the right atrium.
  • Side-to-side portacaval, mesocaval, and splenorenal shunts are not suitable when the IVC is obstructed.

Thus, the correct answer is C. Mesoatrial.

Explanation of Mesoatrial Shunt in Budd-Chiari Syndrome

What is a Mesoatrial Shunt?

  • A mesoatrial shunt is a surgical procedure where the superior mesenteric vein (SMV) is connected directly to the right atrium of the heart.
  • This bypasses the obstructed segments of the inferior vena cava (IVC) and hepatic veins, allowing blood to flow directly from the portal system into the systemic circulation, thereby relieving portal hypertension.

Why is it the Only Option When the IVC is Obstructed?

  • IVC Obstruction: In Budd-Chiari Syndrome, if the IVC is obstructed, typical shunt procedures like side-to-side portacaval shunt, mesocaval shunt, or splenorenal shunt become ineffective because these rely on a patent (unobstructed) IVC to function properly.
  • Direct Bypass: The mesoatrial shunt bypasses the IVC entirely, providing a direct route for blood to return to the heart. This is critical in cases where the IVC is not patent, as it restores effective blood flow and reduces the pressure in the portal system.

Why Other Options Are Not Suitable:

  • Side-to-Side Portacaval Shunt: Requires a patent IVC to connect the portal vein to the IVC.
  • Mesocaval Shunt: Connects the superior mesenteric vein to the IVC, which is not feasible if the IVC is blocked.
  • Splenorenal Shunt: Connects the splenic vein to the left renal vein, which still requires a functional IVC downstream.

Portal Hypertension

July 5, 2024

  • Normal Portal pressure = 5-10 mm hg
  • Portal HTN = HVPG>5 mm hg ( normally 1-5 mm hg)
  • HVPG = [Wedge pressure ( pressure at sinusoids) - Free venous pressure]
  • Clinically Significant PHTN when HVPG is 10 mm hg
  • Varices start to bleed = 12mm hg
  • Causes:
    • Post hepatic :
      • MC = Budd Chiari
      • Constrictive pericarditis
    • Intra hepatic
      • Pre sinusoidal = HVPG will be Normal here
        • NCPF
        • Schistosomiasis
        • Hepatic Fibrosis
      • Sinusoidal
        • only cause is Cirrhosis d/t multiple etiologies
      • Post Sinusoidal:
        • MC = Veno Occlusive disease
    • Pre hepatic:
      • EHPVO =HVPG here also will be normal ‘
      • Portal or splenic vein thrombosis
      • AV fistula
  • HVPG is normal in EHPVO, NCPF ( site of obstruction is pre sinusoidal)
  • HVPG HELPS IN DIAGNOSIS OF PHTN BY:

    Hepatic Vein Pressure HVPG Portal Pressure
    Post sinusoidal Increased Increased Increased
    Sinusoidal Increased Increased Increased
    Pre Sinusoidal Normal Normal Increased
    Pre hepatic Normal Normal Increased
    - MC cause of :
    - PHTN = Cirrhosis
    - PHTN in Children = EHPVO
    - Intrahepatic Presinusoidal PHTN = Schistosomiasis

VARICEAL BLEED

  • Management of Acute bleed :

    • ABCDE
    • Resuscitate = transfusions to maintain Hb just above 7-8 gm/dl
    • IV antibiotics (50% chance of infection)
    • IV Terlipressin
    • IV Somatostatin / Octreotide ( 5days)
    • EVL >> EST = within 12 hrs [ EVL but not EST can also be done as primary prophylaxis]
    • Non responder
      • Cirrhosis = TIPS > shunt
      • Non cirrhotics [EHPVO / NCPF] = Shunt surgery
    • No role of Beta blockers in acute bleed and if the pt has successful EVL = Secondary prophylaxis can then be added [ also used in Primary prophylaxis]
    • if there is a very high CTP score then we can also directly go for Upfront TIPPS [Bavano guidelines - for PHTN]
    • Algorithm of Prevention of recurrent variceal bleeding:

      TIPS FOR CIRRHOTICS
SHUNTS FOR EHPVO AND NCPF
BRTO FOR BLEEDING GASTRIC VARICES

      TIPS FOR CIRRHOTICS SHUNTS FOR EHPVO AND NCPF BRTO FOR BLEEDING GASTRIC VARICES

      Untitled

  • Prophylaxis of Variceal Bleed:

    • EVL / Beta blocker
    • No role of TIPS , surgical shunting , EST in prophylaxis of variceal bleed.
  • Secondary Prophylaxis

    • EVL / Beta Blocker
    • TIPS can never be used as a prophylactic therapy even in decompensated liver disease with high risk varices = here also we use EVL only
    • Risk of Rebleeding is 70% within first 6 weeks
    • Beta blockers reduce Risk of rebleeding to 40%
    • Beta Blockers combined with nitrates is more effective than beta blocker alone to reduce risk of rebleeding

    Untitled

  • TIPS:

    • TIPS = Side to side Portocaval Shunt = Nonselective
    • preferred treatment when endoscopy fails
    • Not used as Primary prophylaxis d/t risk of encephalopathy
    • Can be used in
      • medical refractory ascites,
      • hydrothorax,
      • gastric varices,
      • Budd chiari sx = RxOC now a days.
    • MC early complication : Intraperitoneal H’ge ( Bailey)
    • MC late complication: Shunt stenosis ( 50% at 1 year)
    • 50% chance of Shunt stenosis > shunt thrombosis
    • Rebleed rate is less than endoscopic treatment
    • Short term portal decompression
    • Bridge to Transplant
    • Indications for TIPS:
      • Control of bleeding varices
      • Ectopic Varices
      • Refractory ascites
      • Refractory hepatic hydrothorax
      • Portal vein occlusion = cannulate PV and give anticoagulants
    • Disadvantages:
      • Increase Encephalopathy

    Untitled

    • In case of ascites with PHTN = usually managed medically = but medical refractory then best shunt is TIPS =but if a surgical shunt has to be done then it should be S-S PC Shunt ( IF we do E-S shunt then the liver will not be decompressed)

      Untitled

SELECTIVE VS NON SELECTIVE SHUNTS VS PARTIALLY SELECTIVE SHUNTS:

  • Partially selective :
    • if graft diameter = 8mm = Sarfeh Shunt = PCS
    • Linton’s = PSRS = Splenorenal shunt with Splenectomy
    • Rex shunt = Mesenterico-left Portal Bypass = SMV to LPV by passing Main PV
  • Selective:
    • DSRS = Warren’s
      • Preserve spleen
      • Connect splenic vein to Left Renal Vein
      • also cut Left Coronary Vein to decompress GE varices
      • NOT done in ascites
      • Pancreatic siphon = over time converts to Non selective
        • Loss of portal flow by 50% at 1 year
        • specially in pt’s of Alcoholic cirrhosis
      • Equivalent to Non selective shunt for recurrent bleed
      • Lower encephalopathy rates
      • Compared to TIPS = similar rebleed rate/ encephalopathy rate
    • Inokuchi shunt = Left coronary vein to IVC

Non Selective shunts:

  • Portocaval
    • S-S PC shunt
    • Eck’s Fistula = E-S PC shunt
  • Mesocaval = SMV - IVC
  • Mesorenal
  • DEVASCULARIZATION PROCEDURES
    • if there is a Small Splenic vein / damaged splenic vein during sx / non shuntable splenic vein is present then Devascularization procedures are of help
    • we devascularise the lower esophagus and stomach and also remove spleen

      1. Hassab:

        1. ligate collaterals from lower (abdominal) esophagus and stomach (cardia)
        2. ligate Left Gastric vein
        3. ligate Left Coronary vein
        4. Splenectomy

        Disadvantage : Pt may have reccurence d/t remaining PHTN

      2. Siguiara: Original - 2 stages = thorax and abdomen ( Sigiuara and Futagawa) Modified = 1 stage = all through abdomen incision ( by Ginsberg and Umeyama)

        1. Ligate the perforating veins of esophagus upto inferior pulmonary vein ( 7-8 cms)
        2. Devascularization of Upper 2/3rd of greater and lesser curve
        3. DID NOT ligate Left Coronary Vein
        4. Esophageal Transection 2 cms above GEJ done and Reanastomosis done with circular stapler
        5. Highly Selective Vagotomy +/- drainage procedures
        6. Splenectomy done

EHPVO vs NCPF

EHPVO NCPF Cirrhosis
1st or 2nd decade Child
mean age =19 yrs Middle aged pt 3rd or 4th decade
Mean age = 28-32 yrs
present with signs of Phtn
Variceal bleed present with signs of Phtn present with signs of Phtn
USG =
- Portal Vein thrombosis with involvement of Splenic & mesenteric veins
- Cavernomatous transformation of Portal vein
- Splenomegaly & Hypersplenism = mild -moderate USG =
- **Dilated Portal Vein
- Withered tree** appearance (cannot see 2nd & 3rd order branches)
-Splenomegaly is disproportionate and massive
No Cirrhosis signs = jaundice, ascites, encephalopathy No Cirrhosis signs = jaundice, ascites, encephalopathy Cirrhosis signs = jaundice, ascites, encephalopathy +nt
effects main portal vein
liver is completely normal Biopsy =
- **Portal Sclerosis of 2nd and 3rd order branches
- Obliterative portovenopathy**
-fibrosis at presinusoidal level in liver
ESSENTIAL FOR DIAGNOSIS
**Growth retardation
Portal Biliopathy in 90-100%** Autoimmune features +nt

Treatment of EHPVO & NCPF

  • These conditions are PHTN with Non Cirrhotic livers = therefore Surgical Shunts are preferred over other modalities
  • Shunts or Devascularization procedures
  • Shunts:
    • Non selective = best shunts d/t global decompression of PV
      • here splenomegaly is also a problem to be addressed; hence PSRS ( linton’s) - BEST
      • Mesocaval Shunt
    • Selective :
      • DSRS = warrens can also be done in cases of small spleen
    • Rex shunt
      • physiological shunt
      • Mesenterico - left portal bypass
      • Mostly used in cases of EHPVO
      • we should use a graft here and so risk of thrombosis
      • Splenomegaly has to be addressed separately here
  • Devascularization procedures:
    • Hassab
    • Siguiara
    • Modified Siguiara

PORTAL BILIOPATHY

Definition: abnormality in the extrahepatic biliary system including the cystic duct and gallbladder with or without abnormalities in the 1st and 2nd generation biliary ducts in a patient with portal cavernoma. For the Diagnosis to be established, all of the following criteria have to be fulfilled :

  1. presence of portal cavernoma
  2. typical cholangiographic changes in ERCP or MRCP ( table 1)
  3. absence of other causes of these biliary changes like Bile Duct Injury , PSC, Sclerosing Cholangitis, Cholangiocarcinoma etc.

    Table 1: Cholangiographic abnormalities of Portal Cavernoma Cholangiopathy

    Extrinsic impressions/ indentations
    Shallow impressions/ indentations
    Irregular ductal contour
    Stricture
    Filling defects
    Bile duct angulation
    Upstream dilatation
    Ectasia

Exp : primary diagnosis is EHPVO which lead to changes in Biliary system causing strictures which leads to PORTAL BILIOPATHY. this case is symptomatic. but there is no cholangitis

  • Most cases 70% are Asymptomatic = which needs no treatment
  • If symptomatic = look for cholangitis
    • Cholangitis present = do ERCP + Stenting
    • Cholangitis absent = Do any Non Selective Shunt ;
      • PSRS shunt = best = with follow up after 3 months = 30-40% will have resolution of symptoms with shunt alone
      • mesocaval
    • We CANNOT do a DSRS shunt in portal biliopathy because it doesnt cause global decompression
    • We dont require anything other than shunt procedures like for eg; they always give HJ in the options
    • There are 2 kinds of strictures in Portal biliopathy
      • Fibrotic strictures = Sx needed
        • we need to do Surgery but not a upfront hepaticojejunostomy
        • first do shunt surgery and repeat imaging after 3 months and if still there is a fibrotic stricture then do a HJ
      • Non fibrotic strictures = no need for additional procedure

Stages of Portal Cavernoma Cholangiopathy:

Stage Portal Cavernoma Cholangiopathy LIver Biochemistry Symptoms Complications Treatment
Pre clinical + - Normal Absent - -
Asymptomatic + early changes N/ Abnormal Absent - -
Symptomatic + Advanced changes Abnormal Present - Required
Complicated
= Biliary stricture + Advanced changes Abnormal Present + Required

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Budd Chiari Syndrome

  • In india , Most Commonly d/t sequale of Infections; eg: Amebic Liver abscess / some other abdominal infections which causes thrombosis of veins
  • In western = MC is Myeloprofilerative disease ; Polycythemia vera is Overall most common cause of thrombosis and in west also; OC pills
  • Acute BCS = MC in west = more fulminant
  • Chronic BCS = MC in east = more of subacute
  • Membranous IVC obstruction = MC in east

    Untitled

  • Clinical Triad of Budd chiari :

    • Pain
    • Hepatomegaly
    • Ascites = MC feature
      • Generally jaundice is not a part of triad; but if it is present then there is liver decompensation

Complications of Cirrhosis (1)

Electrolyte Imbalance in Cirrhosis

  • Metabolic Alkalosis and Hypokalemia
    • Associated with secondary hyperaldosteronism
  • Diarrhea
    • May be due to malabsorption secondary to splanchnic venous hypertension
  • Deleterious Effects of Metabolic Alkalosis
    • ODC (Oxygen Dissociation Curve): Shift to the left → impaired tissue oxygen delivery
    • Ammonium chlorideammoniaencephalopathy

Ascites:

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Management of Cirrhosis and Ascites

  • First-line Treatment
    • Sodium restriction: 88 mmol/day (2000 mg/day)
    • Diuretics:
      • Oral spironolactone
      • +/- Oral furosemide
  • Fluid Restriction
    • Not necessary unless serum sodium is less than 120-125 mmol/L

Refractory Ascites

  • Definition
    • Fluid overload that:
      • Is unresponsive to sodium-restricted diet and high-dose diuretic treatment (400 mg/day spironolactone and 160 mg/day furosemide)
      • Recurs rapidly after therapeutic paracentesis
  • Management Options
    • TIPS (Transjugular Intrahepatic Portosystemic Shunt)
    • Shunt procedures
    • LTP (Liver Transplantation)

Hepatic Encephalopathy

  • Ammonia Production
    • Ammonia is produced when intestinal bacteria break down blood in the GIT.
    • Neurotransmitters (NTs) are altered in the CNS.
  • Management Strategies
    • Control active bleeding and reduce dietary protein to minimize ingested blood.
    • Glucose in the diet inhibits ammonia production by bacteria.
    • Lactulose
      • Acts as a mild cathartic.
      • Its breakdown products interfere with ammonia transfer across the colonic mucosa.
    • Rifaximin
      • Changes bacterial flora.
    • L-ornithine, L-aspartate
      • Converts to favorable amino acids.

Hepatorenal Syndrome (HRS) - Criteria

  • Cirrhosis with ascites and Serum Creatinine1.5 mg/dL
  • No improvement of serum creatinine after at least 2 days with:
    • Diuretic withdrawal
    • Volume expansion with albumin (1 g/kg/day, up to a maximum of 100 g/day)
  • Absence of shock
  • No current or recent treatment with nephrotoxic drugs
  • Absence of parenchymal kidney disease evidenced by:
    • Proteinuria500 mg/day
    • Microhematuria (≤ 50 RBC/hpf)
    • Abnormal renal USG

Types of HRS

  • Type I HRS
    • Rapidly progressive reduction in renal function.
    • Doubling of initial serum creatinine to a level ≥ 2.5 mg/dL or
    • 50% reduction of the initial 24-hour creatinine clearance to a level ≤ 20 mL/min within less than 2 weeks.
  • Type II HRS
    • Does not have a rapidly progressive course.
    • Common cause of death in patients who do not succumb to other complications of cirrhosis.

image.png

Pulmonary Syndromes Associated with Cirrhosis

  • Hepatopulmonary Syndrome (HPS)
    • Pathophysiology: Long-standing cirrhosis → Intrapulmonary vascular dilatation and hypoxemia.
    • Clinical Features (C/F):
      • Orthodexia (worsening oxygenation when standing)
      • Platypnea (shortness of breath relieved by lying down)
    • Investigation of Choice (IOC): Contrast ECHO
  • Portopulmonary Hypertension (POPH)
    • Pathophysiology: Pulmonary hypertension due to Pulmonary Vasoconstriction from portal hypertension.
    • Pulmonary artery pressure will be > 25 mmHg.
    • Clinical Features (C/F): Cardiac Arrhythmias
  • Management
    • Liver Transplant (TOC): Treatment of choice for both HPS and POPH.
    • Contraindication: Portopulmonary Hypertension with Pressure > 50 mmHg is a contraindication for liver transplant.

Spontaneous Bacterial Peritonitis (SBP)

  • Definition:
    • Spontaneous bacterial infection of cirrhotic ascites without suppurative infection or bowel perforation.
    • SBP is a common and serious complication of cirrhotic ascites with prevalence ranging from 10% to 27% at the time of hospitalization.
  • Clinical Significance:
    • SBP is a potentially lethal complication and a marker of decreased survival.
    • Historically, first episodes of SBP had a mortality rate of 47%.
    • Renal failure plays a significant role in both immediate and late mortality.
  • Pathophysiology:
    • Bacterial translocation is key, commonly involving enteric gram-negative aerobic bacteria.
    • Recent trends show more gram-positive bacteria and quinolone-resistant bacteria due to increased antibiotic use.
  • Host Factors:
    • Cirrhotic patients are uniquely susceptible due to decreased reticuloendothelial function, leukocyte function, and diminished opsonic activity of ascitic fluid.
    • Low ascitic fluid protein (< 10 g/L) correlates with a higher risk of SBP.
  • Symptoms:
    • Varied and subtle symptoms, including fever, abdominal pain, acute kidney injury, hepatic encephalopathy, and jaundice.
    • GI bleeding can both promote bacterial translocation and increase SBP risk.
    • Prophylactic antibiotics during variceal bleeding reduce rebleeding risk.
  • Diagnosis:
    • Perform paracentesis in all patients with new-onset ascites or clinical deterioration.
    • Diagnostic Criteria:
      • Neutrophil count > 250 PMN cells/μL in ascitic fluid.
      • Positive culture from ascitic fluid.
    • CNNA (Culture-Negative Neutrocytic Ascites) has similar management and prognosis as SBP.
    • Bacterascites refers to positive culture with normal neutrophil count; often transient.
  • Distinguishing from Secondary Peritonitis:
    • Consider secondary peritonitis if:
      • Glucose < 50 mg/dL
      • Ascites protein > 1 g/dL
      • Ascites LDH > serum LDH
  • Treatment:
    • Empirical antibiotics promptly after diagnosis:
      • Third-generation cephalosporins (e.g., cefotaxime, ceftriaxone) are first-line.
      • Five days of therapy is generally sufficient.
    • Albumin infusion to prevent acute kidney injury:
      • Dosage: 1.5 g/kg at diagnosis, 1 g/kg on day 3.
      • Reduces renal impairment from 33% to 10% and hospital mortality from 29% to 10%.
      • Especially beneficial in patients with pre-existing kidney disease and serum bilirubin > 4 mg/dL.
  • Prophylaxis:
    • Recommended for patients with prior SBP episodes or ascites protein < 10 g/L.
    • Concerns exist regarding long-term antibiotic use and antibiotic-resistant organisms.

MCQ’s

MCQ: Porto Pulmonary Hypertension in Cirrhosis

Question: All are true statements regarding Portopulmonary hypertension in cirrhosis EXCEPT:

a) Echocardiography is used for screening

b) Pulmonary artery catheterization is required for confirmation of diagnosis

c) Its presence is a contraindication for liver transplantation

d) Prostanoid therapy is useful

Correct Answer: c) Its presence is a contraindication for liver transplantation

Explanation:

  • Echocardiography is indeed used as a screening tool for Portopulmonary hypertension.
  • Pulmonary artery catheterization is required for confirmation of the diagnosis.
  • Mild degrees of pulmonary artery hypertension (up to 35 mmHg) do not preclude liver transplantation in otherwise acceptable candidates. However, pressures greater than 35 mmHg require aggressive evaluation and treatment.
  • Prostanoid therapy is useful in managing Portopulmonary hypertension.

Therefore, presence of Portopulmonary hypertension itself is not an absolute contraindication for liver transplantation unless the pulmonary artery pressure exceeds 50 mmHg.

MCQ: Most Effective Diuretic for the Management of Ascites in Cirrhosis

Question: Most effective diuretic for the management of ascites in cirrhosis:

a) Spironolactone

b) Furosemide

c) Thiazide

d) Amiloride

Correct Answer: a) Spironolactone

Explanation:

  • Spironolactone is a potassium-sparing diuretic that acts as an aldosterone antagonist. It is considered the most effective diuretic for managing ascites in cirrhosis due to its ability to counteract the effects of secondary hyperaldosteronism, which is common in these patients.
  • Furosemide is often used in combination with spironolactone, but it is less effective when used alone for ascites.
  • Thiazide diuretics and Amiloride are not as effective as spironolactone in this context.

MCQ: Best Choice of Treatment for Recurrent Variceal Bleed and Ascites

Question: JIPMER DEC 2018: Best choice of treatment for recurrent variceal bleed and ascites:

a) SSPCS (Selective Shunt Procedure: Selective Splenorenal Shunt)

b) Devascularization

c) DSRS (Distal Splenorenal Shunt)

d) TIPS (Transjugular Intrahepatic Portosystemic Shunt)

Correct Answer: a) SSPCS

Explanation:

  • SSPCS (Selective Splenorenal Shunt) is considered the best choice for the treatment of recurrent variceal bleeding and ascites because it selectively decompresses the gastroesophageal varices while preserving portal perfusion, reducing the risk of hepatic encephalopathy compared to non-selective shunts.
  • Devascularization is usually reserved for patients who cannot undergo shunt procedures.
  • DSRS (Distal Splenorenal Shunt) is another selective shunt but is typically used for patients without significant ascites.
  • TIPS (Transjugular Intrahepatic Portosystemic Shunt) is commonly used, but SSPCS may be preferred in cases where long-term management is needed without increasing the risk of hepatic encephalopathy.

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MCQ: Management of Refractory Ascites

Question: Not true regarding the management of refractory ascites?

a) TIPS can be done as a bridge to LTP

b) DSRS helps in retaining splenic activity in those with refractory ascites without hypersplenism

c) Mesocaval shunt or PSRS are usual shunts done if liver function is relatively maintained

d) Peritoneo venous shunts do not help in long-term management due to frequent blockage

Correct Answer: b) DSRS helps in retaining splenic activity in those with refractory ascites without hypersplenism

Explanation:

  • TIPS (Transjugular Intrahepatic Portosystemic Shunt) can indeed be done as a bridge to liver transplantation (LTP), especially in patients with refractory ascites.
  • DSRS (Distal Splenorenal Shunt) is primarily used to manage portal hypertension and variceal bleeding; it is not specifically beneficial in patients with refractory ascites without hypersplenism, and its use in such cases is not standard.
  • Mesocaval shunt or PSRS (Proximal Splenorenal Shunt) are common shunts performed if liver function is relatively well-preserved.
  • Peritoneo venous shunts are associated with frequent blockage, making them less useful for long-term management.

MCQ: Spontaneous Bacterial Peritonitis (SBP)

Question: Which is true regarding Spontaneous Bacterial Peritonitis?

a) Ascitic fluid protein content > 10 g/L promotes SBP

b) Prophylactic antibiotic is recommended in the above set of patients

c) Albumin infusion prevents renal dysfunction in patients with SBP, particularly in those with bilirubin > 4 mg/dL

d) Renal dysfunction occurs in 10% of those with SBP

Correct Answer: c) Albumin infusion prevents renal dysfunction in patients with SBP, particularly in those with bilirubin > 4 mg/dL

Explanation:

  • Ascitic fluid protein content < 10 g/L (not > 10 g/L) is associated with a higher risk of developing SBP, so option a) is incorrect.
  • Prophylactic antibiotics are recommended in patients with low ascitic fluid protein (< 10 g/L), but this does not directly relate to option b) as stated.
  • Albumin infusion has been shown to prevent renal dysfunction in patients with SBP, especially in those with serum bilirubin > 4 mg/dL or serum creatinine > 1 mg/dL, making option c) correct.
  • Renal dysfunction occurs in a higher percentage of patients with SBP, typically around 30-40%, not just 10%, so option d) is incorrect.

Thus, the correct answer is c) Albumin infusion prevents renal dysfunction in patients with SBP, particularly in those with bilirubin > 4 mg/dL.

MCQ: Appropriate Maneuvers in Hepatic Encephalopathy

Question: Appropriate maneuvers in a patient with hepatic encephalopathy include all of the following EXCEPT:

a) Addition of glucose to the diet

b) Administration of lactulose

c) Construction of a side-to-side portacaval shunt

d) Limiting dietary protein

Correct Answer: c) Construction of a side-to-side portacaval shunt

Explanation:

  • Addition of glucose to the diet helps in reducing ammonia production by intestinal bacteria, which is beneficial in managing hepatic encephalopathy.
  • Administration of lactulose is a standard treatment for hepatic encephalopathy as it acts as a cathartic and reduces ammonia absorption in the gut.
  • Limiting dietary protein is recommended to decrease the production of ammonia, which exacerbates hepatic encephalopathy.
  • Construction of a side-to-side portacaval shunt is generally not recommended in patients with hepatic encephalopathy as it can worsen the condition by increasing the diversion of blood away from the liver, thus elevating ammonia levels in the blood.

MCQ: Hepatorenal Syndrome (HRS)

Question: False regarding HRS?

a) Albumin infusion in addition to antibiotic therapy in SBP patients prevents development of HRS

b) Type II HRS — mortality rate very high in spite of treatment

c) Albumin and terlipressin are recommended drugs in HRS

d) Dialysis-dependent HRS with creatinine clearance < 30 mL/min for more than 2 weeks is an indication for combined liver and kidney transplant

Correct Answer: b) Type II HRS — mortality rate very high in spite of treatment

Explanation:

  • Albumin infusion combined with antibiotic therapy in SBP patients is known to prevent the development of HRS (option a is correct).
  • Type I HRS has a high mortality rate despite treatment, whereas Type II HRS typically has a slower progression and a lower mortality rate than Type I. Therefore, the statement in option b is false.
  • Albumin and terlipressin are indeed the recommended drugs for managing HRS (option c is correct).
  • Dialysis-dependent HRS with creatinine clearance < 30 mL/min for more than 2 weeks is an indication for combined liver and kidney transplantation (option d is correct).

MCQ: Non-Hepatic Surgery in Cirrhosis

Question: Which statement is False regarding non-hepatic surgery in cirrhosis?

a) CTP А & B, MELD < 15 - safe cholecystectomy

b) Morbidity rate for CTP A, B & C - 20%, 60%, and 80% respectively

c) Elective umbilical hernia repair with mesh is preferred in compensated cirrhosis

d) Gastric banding is recommended bariatric procedure in cirrhotics

Correct Answer: d) Gastric banding is recommended bariatric procedure in cirrhotics

Explanation:

  • CTP (Child-Turcotte-Pugh) A & B and MELD < 15 are considered safe criteria for performing cholecystectomy in cirrhotic patients, making option a correct.
  • The morbidity rates for non-hepatic surgery in cirrhotic patients increase with the severity of liver disease: 20% for CTP A, 60% for CTP B, and 80% for CTP C, so option b is correct.
  • Elective umbilical hernia repair with mesh is preferred in compensated cirrhosis, as it has a lower risk of complications, so option c is correct.
  • Gastric banding is not recommended as a bariatric procedure in cirrhotic patients. Instead, sleeve gastrectomy is preferred due to better outcomes and fewer complications in these patients, making option d the false statement.

Therefore, the false statement is option d).

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MCQ: Most Common Acid-Base Disturbance in Cirrhosis and Portal Hypertension

Question: Which of the following is the most common acid-base disturbance in patients with cirrhosis and portal hypertension?

a) Metabolic acidosis

b) Respiratory alkalosis

c) Metabolic alkalosis

d) Respiratory acidosis

Correct Answer: c) Metabolic alkalosis

Explanation:

  • Metabolic alkalosis is the most common acid-base disturbance in patients with cirrhosis and portal hypertension. This is primarily due to factors like secondary hyperaldosteronism, diuretic therapy, and hypokalemia.
  • Respiratory alkalosis is also common in cirrhosis due to hyperventilation, but it is not the most common disturbance in the context of portal hypertension.
  • Metabolic acidosis and Respiratory acidosis are less common in this setting.

Thus, Metabolic alkalosis is the correct answer as the most common acid-base disturbance in these patients.

IHC of Cirrhosis (1)

Ideal Core Needle Liver Biopsy

  • Length of Biopsy Specimen: For accurate and reliable grading and staging of chronic viral hepatitis, a biopsy specimen should be:
    • At least 2 cm in length
    • Contain at least 11 complete portal tracts
  • Micronodular Cirrhosis: Characterized by:
    • ≤3 mm nodules

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Histology - Stains for Liver Disorders

  • Hemochromatosis
    • Stain: Prussian blue stain
    • Purpose: Detects iron deposition in tissues.
  • Wilson's Disease
    • Stain: Rhodanine stain (Red)
    • Purpose: Detects copper accumulation in the liver.
  • Alpha-1 Antitrypsin Deficiency
    • Characteristic: Diastase-resistant hyaline inclusions
    • Purpose: Identifies abnormal alpha-1 antitrypsin protein in liver cells.

Non Hepatic Surgery In Cirrhotics

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MCQ: Models Predicting Outcome of Non-Liver Surgeries in Cirrhotic Patients

Question: Which of the following is not a model predicting the outcome of non-liver surgeries in cirrhotic patients?

a) MELD Na

b) ADOPT-LC

c) VOCAL-Penn

d) CTP

e) none

Correct Answer: e) none

Explanation:

All the listed models are used to predict outcomes in cirrhotic patients undergoing non-liver surgeries:

  • MELD Na: Modified MELD score that includes sodium levels to predict surgical outcomes.
  • ADOPT-LC: A model that incorporates CTP score, age, Charlson comorbidity index, and duration of anesthesia.
  • VOCAL-Penn: A comprehensive model that includes the Mayo risk score, emergency status, and surgery-specific categories to predict postoperative outcomes.
  • CTP (Child-Turcotte-Pugh): A widely used score to assess the severity of liver disease and predict surgical risks.

Cholecystectomy in Cirrhotics: Key Points

  • Increased Risk of Gallstones:
    • Cirrhotic patients have twice the incidence of gallstones compared to the general population due to:
      • Increased intravascular hemolysis
      • Decreased gallbladder motility and emptying
  • Historical Context:
    • In the 1980s, open cholecystectomy in cirrhotic patients had high morbidity (35%) and mortality (25%) rates, primarily due to blood loss, sepsis, and liver failure.
    • Laparoscopic cholecystectomy was initially avoided due to fears of increased bleeding and liver failure but is now shown to have favorable outcomes in CTP class A and B patients.
  • Laparoscopic vs. Open Cholecystectomy:
    • Laparoscopic cholecystectomy offers:
      • Improved visualization
      • Less operative blood loss
      • Shorter operative time
      • Decreased length of hospital stay
    • Studies show higher morbidity (21% vs. 8%), intraoperative bleeding (26% vs. 3%), and open conversion rates (7% vs. 4%) in cirrhotics compared to non-cirrhotics but no difference in mortality.
    • Meta-analysis: Laparoscopic approach is associated with reduced complication rates and shorter hospital stay compared to the open approach.
  • Technical Considerations:
    • Umbilical port placement should avoid venous collaterals and the umbilical vein.
    • Transillumination or preoperative CT may help avoid vascular injury during trocar placement.
    • Instruments like Harmonic Scalpel and LigaSure are useful to control bleeding.
    • Subtotal cholecystectomy may be necessary in cases with large pericholecystic venous collaterals.
    • Direct visualization during trocar removal ensures adequate hemostasis.
    • Conversion to open surgery should be considered if anatomy is unclear.
  • Advanced Cirrhosis (CTP Class C):
    • Cholecystectomy is associated with very poor outcomes in advanced cirrhosis.
    • Alternative interventions:
      • Percutaneous cholecystostomy
      • Endoscopically placed cystic duct stent (less common and limited data)
  • Predictors of Outcomes:
    • Both CTP and MELD scores are valuable for predicting postoperative morbidity and mortality.
    • CTP A and B patients can typically undergo surgery with acceptable outcomes.
    • MELD score cutoff varies, but laparoscopic cholecystectomy is generally safe for patients with MELD < 15.
  • High-Risk Patients:
    • In CTP class C or high MELD score patients, surgery should be deferred if possible, and alternative therapies should be considered as a bridge to surgery when liver function improves.

MCQ: Laparoscopic Cholecystectomy in Cirrhotics

Question: Which of the following is not true about laparoscopic cholecystectomy in cirrhotics?

a) Can be performed in CTP A and B and with MELD < 15

b) Recommended in CTP C symptomatic cholelithiasis in expert centers

c) Umbilical port introduction after other ports

d) Subtotal cholecystectomy - a valid option

Correct Answer: b) Recommended in CTP C symptomatic cholelithiasis in expert centers

Explanation:

  • Option a is correct: Laparoscopic cholecystectomy can indeed be performed in patients with CTP A and B and MELD < 15 with acceptable outcomes.
  • Option c is correct: Introducing the umbilical port after other ports helps avoid venous collaterals and the umbilical vein, which is a recommended approach in cirrhotics.
  • Option d is correct: Subtotal cholecystectomy is a valid option in difficult cases, especially when there are large pericholecystic venous collaterals.
  • Option b is not true: Laparoscopic cholecystectomy is not recommended in CTP C patients due to very poor outcomes. Alternative procedures like percutaneous cholecystostomy are preferred.

Thus, option b is the false statement.

Herniorrhaphy in Cirrhotics: Key Points

  • Incidence and Pathogenesis:
    • Umbilical hernias occur in up to 20% of cirrhotic patients.
    • Causes include:
      • Increased intra-abdominal pressure from ascites.
      • Poor nutritional status leading to decreased abdominal muscle mass and fascial strength.
      • Umbilical vein dilation resulting in enlargement of the preexisting supraumbilical fascial opening.
  • Risks and Complications:
    • In cirrhotic patients, umbilical hernias pose unique risks:
      • Skin ulceration over the hernia can lead to:
        • Leakage of ascites
        • Sac rupture
        • Bacterial peritonitis
        • Evisceration
      • Flood syndrome: Spontaneous umbilical rupture and ascites leak, associated with up to 60% perioperative mortality.
  • Surgical Outcomes:
    • Historically, high mortality rates led to the recommendation against repairing uncomplicated hernias in cirrhotics.
    • Recent data show improved outcomes for elective UHR (Umbilical Hernia Repair), even in cirrhotics with end-stage liver disease.
    • Marsman et al. study: Elective UHR group had no hepatic decompensation or perioperative deaths, while the conservative management group had significantly higher complication rates, including emergency repairs and perioperative deaths.
  • Elective vs. Emergent Repair:
    • Elective UHR is associated with outcomes similar to those in non-cirrhotics, whereas emergent UHR leads to significantly worse outcomes in cirrhotic patients.
    • Perioperative management: TIPS may be considered to reduce refractory ascites before UHR, although specific studies on its efficacy for reducing complications of UHR are lacking.
  • Use of Mesh:
    • The use of nonabsorbable mesh in cirrhotic patients with umbilical hernias is debated, but recent evidence suggests it can be used successfully, even in the presence of ascites.
    • Randomized study (2010): Mesh repair led to a lower recurrence rate compared to suture repair (3% vs. 14%), despite a non-significant increase in surgical site infections.
  • Inguinal Hernia Repair:
    • Outcomes after elective inguinal hernia repair in cirrhotic patients are similar to those in non-cirrhotics.
    • Emergent repair of an incarcerated or strangulated inguinal hernia in cirrhotics results in significantly worse outcomes.
    • Mesh use in inguinal hernia repair is generally safe, with manageable local wound complications.
  • Current Recommendations:
    • Elective hernia repair (umbilical or inguinal) is advisable in cirrhotic patients, provided they are well-compensated, and ascites is controlled, to avoid complications associated with emergent repairs.

Herniorrhaphy in Cirrhotics: Key Points

  • Umbilical Hernias: Occur in up to 20% of cirrhotic patients, caused by ascites, poor nutrition, and umbilical vein dilation.
  • Risks:
    • Skin ulceration can lead to ascites leakage, rupture, and bacterial peritonitis.
    • Flood syndrome (spontaneous rupture) has up to 60% mortality.
  • Surgical Outcomes:
    • Elective UHR has better outcomes than emergent repair.
    • Mesh repair reduces hernia recurrence; infections are manageable.
  • Inguinal Hernia:
    • Elective repair is safe; outcomes worsen significantly with emergent repair.
  • Recommendation:
    • Elective hernia repair is advised in well-compensated cirrhotics [CTP A and B] with controlled ascites.

MCQ: Complications of Umbilical Hernia Repair in Cirrhotic Patients with Ascites

Question: Possible complications of umbilical hernia repair in a cirrhotic patient with marked ascites include all of the following EXCEPT:

a) Hepatic encephalopathy

b) Leakage of ascitic fluid

c) Necrosis of the abdominal wall

d) Variceal bleeding

Correct Answer: a) Hepatic encephalopathy

Explanation:

  • Leakage of ascitic fluid: Common complication that increases the risk of wound infection.
  • Necrosis of the abdominal wall: Can occur due to pressure on the incision and reforming ascites.
  • Variceal bleeding: May result from the interruption of collateral veins during surgery.
  • Hepatic encephalopathy: While possible, it is uncommon unless it occurs secondary to massive variceal bleeding.

Thus, the correct answer is a) Hepatic encephalopathy, as it is not a direct or common complication of umbilical hernia repair in cirrhotic patients with ascites.