What is the diagnosis and management plan for a 15-16 year old patient presenting with hematemesis (vomiting blood) and melena (dark stools) due to esophageal and gastric varices, likely caused by portal hypertension from a liver condition such as cirrhosis or portal vein thrombosis?

Diagnosis and Management of Variceal Bleeding in an Adolescent with Portal Hypertension

Primary Diagnosis

This adolescent patient has acute variceal hemorrhage secondary to portal hypertension, most likely from non-cirrhotic portal hypertension due to portal vein thrombosis or schistosomiasis-related periportal fibrosis, given the age, splenomegaly, and absence of chronic liver disease stigmata. 1

The key diagnostic features supporting this are:

• High-risk esophageal and gastric varices confirmed on endoscopy 1
• Splenomegaly with moderate ascites on ultrasound 1
• Recent malaria treatment (potential trigger for decompensation) 1
• Absence of jaundice, encephalopathy, or edema (suggesting preserved hepatic synthetic function) 1

Critical Distinction: Non-Cirrhotic vs. Cirrhotic Portal Hypertension

In this 15-16 year old, non-cirrhotic causes of portal hypertension are far more likely than cirrhosis. 1, 2

The most important next diagnostic step is contrast-enhanced CT or MRI to identify portal vein thrombosis or splenic vein thrombosis, as this fundamentally changes management options. 1

Key differentiating features in this case:

• Young age makes cirrhosis unlikely unless there is undiagnosed autoimmune hepatitis, Wilson's disease, or congenital hepatic fibrosis 1
• Splenomegaly is prominent, which can occur in both cirrhotic and non-cirrhotic portal hypertension 1
• Portal vein thrombosis can be associated with underlying thrombophilia or myeloproliferative disorders (though peripheral blood counts would typically show abnormalities) 2

Acute Management Already Completed (Appropriate)

The patient received appropriate acute management:

• Blood transfusion for hemodynamic stabilization 1
• Endoscopic variceal ligation (EVL) for esophageal varices 1
• Glue injection (cyanoacrylate) for gastric varices 1, 3
• Vasoactive medications (likely octreotide or terlipressin) 1, 3
• Antibiotic prophylaxis to prevent spontaneous bacterial peritonitis and reduce mortality 1, 3

Variceal hemorrhage carries at least 20% mortality at 6 weeks even with optimal treatment, making aggressive management essential. 1

Current Medical Regimen to Prevent Rebleeding

The patient should be on non-selective beta-blockers (propranolol or carvedilol) as the cornerstone of secondary prophylaxis. 1, 3

The goals of beta-blocker therapy are:

• Reduce portal pressure by decreasing cardiac output and causing splanchnic vasoconstriction 1
• Target heart rate reduction of 25% from baseline or to 55-60 bpm 1
• Reduce rebleeding risk from 60% to approximately 30-40% 1

Antibiotic prophylaxis should continue for 7 days post-bleeding (typically ceftriaxone or norfloxacin), as bacterial infections increase rebleeding risk and mortality. 1, 3

Essential Next Steps for Definitive Diagnosis

1. Advanced Imaging (Highest Priority)

Obtain contrast-enhanced CT or MRI of the abdomen immediately to evaluate:

• Portal vein patency vs. thrombosis 1, 2
• Splenic vein patency (isolated splenic vein thrombosis causes gastric varices without esophageal varices typically) 1
• Liver architecture (cirrhotic vs. non-cirrhotic) 1
• Presence of gastrorenal or splenorenal shunts (important for future interventional options) 1

If portal vein thrombosis is identified, this patient needs a complete thrombophilia workup including JAK2 mutation, Factor V Leiden, prothrombin gene mutation, protein C/S deficiency, and antiphospholipid antibodies. 2

2. Hepatic Venous Pressure Gradient (HVPG) Measurement

HVPG measurement is critical for risk stratification and should be performed when available. 1

• HVPG ≥10-12 mmHg confirms clinically significant portal hypertension 1, 4
• HVPG ≥20 mmHg within 24 hours of bleeding predicts 83% risk of early rebleeding vs. 29% with lower pressures 1
• HVPG ≥20 mmHg also predicts 64% 1-year mortality vs. 20% with lower pressures 1

3. Liver Disease Etiology Workup

Even with non-cirrhotic portal hypertension suspected, complete the following:

• Hepatitis B and C serologies 1
• Autoimmune markers (ANA, ASMA, anti-LKM, IgG levels) for autoimmune hepatitis 1
• Ceruloplasmin and 24-hour urine copper for Wilson's disease (critical in adolescents) 1
• Alpha-1 antitrypsin level 1
• Schistosomiasis serology if endemic area exposure 1

Secondary Prophylaxis Strategy

Combination therapy with endoscopic variceal ligation plus non-selective beta-blockers is superior to either alone for preventing rebleeding. 1, 3

The protocol should be:

• Continue beta-blockers indefinitely (propranolol 20-40 mg twice daily, titrated to heart rate) 1, 3
• Repeat EVL every 2-4 weeks until variceal eradication 1, 3
• Surveillance endoscopy every 3-6 months after eradication 1

For gastric varices (GOV1 or GOV2), if remnant varices persist after initial glue injection, repeat endoscopic variceal obturation (EVO) is indicated. 1

Special Considerations for Gastric Varices

The presence of both esophageal and gastric varices requires classification:

• GOV1 (gastroesophageal varices type 1) extend along lesser curvature and are managed like esophageal varices with EVL 1
• GOV2 (gastroesophageal varices type 2) extend into fundus and require cyanoacrylate injection 1

If a gastrorenal shunt is identified on imaging, balloon-occluded retrograde transvenous obliteration (BRTO) may be considered for refractory gastric varices, though this is typically reserved for recurrent bleeding despite endoscopic therapy. 1

Monitoring for Complications

Early Rebleeding (Within 6 Weeks)

Risk of early rebleeding is highest in the first 5 days, occurring in 10-20% of patients. 1

Monitor for:

• Recurrent hematemesis or melena 1
• Hemodynamic instability (tachycardia, hypotension) 1
• Dropping hemoglobin despite transfusion 1

If early rebleeding occurs despite optimal medical and endoscopic therapy, consider early TIPS (transjugular intrahepatic portosystemic shunt) placement, though this is controversial in non-cirrhotic portal hypertension and should involve multidisciplinary discussion. 1

Late Rebleeding (After 6 Weeks)

Without secondary prophylaxis, 60% of patients rebleed within 1-2 years. 1

This risk is reduced to 30-40% with combination beta-blocker and EVL therapy. 1, 3

Portal Hypertensive Gastropathy Management

The moderate ascites and splenomegaly indicate significant portal hypertension that may also cause portal hypertensive gastropathy bleeding. 1

If chronic anemia develops despite variceal control:

• Beta-blockers are first-line for portal hypertensive gastropathy 1
• Iron supplementation for chronic blood loss 1
• Consider argon plasma coagulation for active bleeding from gastropathy 1

Critical Pitfalls to Avoid

1. Over-Transfusion

Restrictive transfusion strategy (target hemoglobin 7-9 g/dL) is superior to liberal transfusion, as excessive blood products increase portal pressure and worsen bleeding. 1 The patient received 2 units initially, which is appropriate, but avoid further transfusion unless hemoglobin drops below 7 g/dL or there is active bleeding.

2. Missing Portal Vein Thrombosis

Failure to obtain cross-sectional imaging with contrast can miss portal vein thrombosis, which requires anticoagulation in addition to variceal management. 1, 2 In adolescents, this is often the underlying cause and is treatable.

3. Inadequate Beta-Blocker Dosing

Beta-blockers must be titrated to achieve 25% heart rate reduction or resting heart rate of 55-60 bpm to be effective. 1 Simply prescribing a standard dose without titration is inadequate.

4. Assuming Cirrhosis in Young Patients

In adolescents, non-cirrhotic causes (portal vein thrombosis, schistosomiasis, congenital hepatic fibrosis) are more common than cirrhosis. 1, 2 The management approach differs significantly, particularly regarding anticoagulation and interventional options.

Long-Term Management Plan

If Portal Vein Thrombosis is Confirmed:

• Anticoagulation with low molecular weight heparin or warfarin (target INR 2-3) to prevent thrombus extension 2
• Continue beta-blockers for variceal prophylaxis 1, 2
• Repeat imaging in 3-6 months to assess for recanalization 2
• Complete thrombophilia workup 2

If Non-Cirrhotic Portal Fibrosis (e.g., Schistosomiasis):

• Treat underlying infection if active 1
• Beta-blockers for variceal prophylaxis 1
• Serial EVL until eradication 1
• These patients often have preserved liver function and better prognosis than cirrhotic patients 1

If Cirrhosis is Confirmed:

• Determine Child-Pugh score (this patient appears Child A given lack of encephalopathy, controlled ascites, normal bilirubin implied) 1
• Hepatocellular carcinoma surveillance with ultrasound every 6 months 5, 4
• Liver transplant evaluation if decompensated (Child B/C) 5, 4

Prognosis and Risk Stratification

This patient's prognosis depends critically on the underlying etiology:

• Non-cirrhotic portal hypertension: Generally favorable with appropriate management, as hepatic synthetic function is preserved 1
• Cirrhotic portal hypertension: 20% mortality at 6 weeks from first bleed, 64% 1-year mortality if HVPG ≥20 mmHg 1

The absence of encephalopathy, jaundice, and significant ascites suggests preserved hepatic function, which is prognostically favorable. 1

Summary Algorithm

1. Confirm diagnosis with contrast-enhanced CT/MRI → Identify portal/splenic vein thrombosis, assess liver architecture 1
2. If portal vein thrombosis → Anticoagulate + beta-blockers + thrombophilia workup 2
3. If non-cirrhotic portal hypertension → Beta-blockers + serial EVL + treat underlying cause 1
4. If cirrhosis → Child-Pugh scoring + beta-blockers + serial EVL + transplant evaluation if Child B/C 1, 4
5. All patients → Continue beta-blockers indefinitely, repeat EVL every 2-4 weeks until eradication, surveillance endoscopy every 3-6 months 1, 3