How to manage a patient with esophageal varices and significant liver fibrosis?

Understanding Esophageal Varices with Low Liver Stiffness Values

The Apparent Discrepancy Explained

Your patient's Fibroscan values of 5.4 kPa and SWE 5.3 kPa are inconsistent with the presence of esophageal varices, as these measurements fall well below established thresholds for clinically significant portal hypertension and suggest minimal to no fibrosis rather than cirrhosis. This discordance requires immediate investigation to determine whether the varices are truly present, whether technical factors affected the elastography measurements, or whether an alternative cause of portal hypertension exists.

Critical Threshold Analysis

The liver stiffness values you report are dramatically lower than validated cutoffs for varices:

• For detecting any esophageal varices: Studies consistently show cutoffs of 19-21 kPa are needed, with values <19 kPa having 93% negative predictive value for excluding grade ≥II varices 1
• For high-risk varices: The AGA recommends a cutoff of 19.5 kPa to assess need for endoscopy screening 2
• For clinically significant portal hypertension: A threshold of 17.0 kPa is suggested for preoperative risk stratification 2
• Your patient's values of 5.3-5.4 kPa: These fall in the F0-F1 fibrosis range, typically indicating no significant fibrosis

Possible Explanations for This Discordance

Technical Factors Affecting Elastography

Fibroscan measurements can be falsely low due to:

• Inadequate fasting state (should be ≥3 hours fasting)
• Operator inexperience or inadequate probe positioning
• Excessive subcutaneous fat requiring XL probe
• Insufficient valid measurements (need ≥10 valid readings with IQR/median <30%)

Alternative Causes of Portal Hypertension Without Cirrhosis

Consider non-cirrhotic portal hypertension etiologies:

• Portal vein thrombosis (acute or chronic)
• Splenic vein thrombosis causing isolated gastric varices
• Schistosomiasis causing presinusoidal portal hypertension
• Congenital hepatic fibrosis
• Nodular regenerative hyperplasia
• Budd-Chiari syndrome
• Idiopathic non-cirrhotic portal hypertension

These conditions can produce varices with normal or near-normal liver parenchymal stiffness because the liver architecture remains relatively preserved.

Endoscopic Misidentification

Verify the endoscopic diagnosis:

• Prominent gastric folds can be mistaken for varices
• Downhill varices from superior vena cava obstruction appear similar but have different implications
• Request review of endoscopic images by experienced endoscopist

Immediate Diagnostic Workup Required

Obtain the following studies to resolve this discrepancy:

• Repeat Fibroscan with quality metrics: Ensure IQR/median <30%, ≥10 valid measurements, appropriate probe selection, and adequate fasting 2
• Cross-sectional imaging (CT or MRI abdomen with contrast): Evaluate for portal vein patency, liver morphology, splenomegaly, and collateral vessels
• Complete hepatic panel: Including hepatitis serologies, autoimmune markers, ceruloplasmin, alpha-1 antitrypsin, iron studies
• Platelet count and spleen size: Calculate platelet count/spleen diameter ratio; values <909 suggest portal hypertension 1
• Upper endoscopy review: Confirm variceal diagnosis and grade size (small vs large) and presence of red color signs 2, 3

Management Algorithm Based on Findings

If Varices Confirmed and Elastography Accurate (Non-Cirrhotic Portal Hypertension)

Initiate prophylaxis based on variceal characteristics:

• Small varices without red signs and compensated: Consider observation with surveillance endoscopy every 1-2 years 2
• Small varices with red signs OR decompensated state: Start non-selective beta-blockers (propranolol or nadolol) targeting heart rate 55-60 bpm 2, 3
• Large varices: Initiate either non-selective beta-blockers, carvedilol, or endoscopic variceal ligation; combination therapy (beta-blockers + EVL) provides enhanced protection 2

If Technical Error Identified

Repeat elastography with proper technique:

• If repeat shows values >19.5 kPa, this confirms cirrhosis with portal hypertension and management proceeds per standard guidelines 2
• If repeat remains <10 kPa, pursue alternative portal hypertension etiologies aggressively

If Alternative Diagnosis Found

Treat underlying cause:

• Portal vein thrombosis: Anticoagulation if acute, assess for underlying thrombophilia
• Splenic vein thrombosis: Consider splenectomy for symptomatic gastric varices
• Schistosomiasis: Praziquantel treatment plus variceal prophylaxis

Critical Pitfalls to Avoid

Do not dismiss the varices based solely on low elastography values - varices indicate clinically significant portal hypertension regardless of liver stiffness, and bleeding risk remains substantial with mortality rates of 20% at 6 weeks 4

Do not delay prophylactic treatment while investigating - if large varices or small varices with red signs are confirmed, initiate beta-blocker therapy immediately while diagnostic workup proceeds 2, 3

Do not assume cirrhosis is the only cause of varices - approximately 5-10% of portal hypertension cases are non-cirrhotic, and these require different long-term management strategies

Do not use elastography alone to exclude need for endoscopic surveillance - patients with known varices require ongoing endoscopic follow-up regardless of elastography values, with intervals of 6-12 months after variceal eradication 2

Prognostic Considerations

The presence of varices with low liver stiffness may actually indicate better long-term prognosis if non-cirrhotic portal hypertension is confirmed, as hepatic synthetic function remains preserved. However, bleeding risk from the varices themselves remains equivalent to cirrhotic patients and requires identical prophylactic management 3, 4. The 15% yearly bleeding risk for large varices and 20% six-week mortality from variceal hemorrhage apply regardless of underlying etiology 4.