What is the management approach for a patient with gastroesophageal varices caused by schistosomiasis?

Management of Gastroesophageal Varices in Schistosomiasis

Critical Distinction: Schistosomiasis vs. Cirrhosis

The management of gastroesophageal varices in schistosomiasis follows the same evidence-based protocols established for cirrhotic portal hypertension, with one crucial caveat: preserved hepatic synthetic function in schistosomiasis patients makes them better surgical candidates but also increases their risk of hepatic encephalopathy with non-selective shunts. 1

While the provided guidelines focus on cirrhotic varices 2, 3, 4, 5, the fundamental principles of variceal management apply equally to schistosomiasis-induced portal hypertension, with specific modifications based on preserved liver function 1.

Acute Variceal Bleeding Management

Immediate Resuscitation

• Initiate intravascular volume support with restrictive transfusion targeting hemoglobin 7-9 g/dL to avoid increasing portal pressure 3, 5
• Secure airway in patients with active hematemesis or altered mental status 5
• Establish large-bore IV access and transfer to monitored setting 5

Pharmacological Therapy (Start Immediately)

• Begin vasoactive drugs as soon as variceal bleeding is suspected, even before endoscopy 2, 3, 4
• Terlipressin is preferred (2 mg IV every 4 hours initially, then continuous infusion of 4 mg/24 hours) due to proven 34% mortality reduction 2, 5
• If terlipressin unavailable, use octreotide (50 µg IV bolus, then 50 µg/hour continuous infusion) 4, 5
• Continue vasoactive therapy for 3-5 days after endoscopic treatment to reduce early rebleeding 2, 3, 4
• Somatostatin has demonstrated efficacy in reducing portal pressure and variceal bleeding in schistosomiasis patients specifically 6

Antibiotic Prophylaxis (Mandatory)

• Administer ceftriaxone 1g IV daily for maximum 7 days in all patients with variceal hemorrhage 2, 3, 4, 5
• Ceftriaxone is superior to norfloxacin in advanced disease and high quinolone-resistance settings 2, 4
• Antibiotic prophylaxis reduces bacterial infections, decreases early rebleeding, and improves survival 2, 4

Endoscopic Management

• Perform urgent endoscopy within 12 hours once hemodynamically stabilized 2, 3, 5
• Endoscopic variceal ligation (EVL) is the first-line endoscopic therapy, achieving hemostasis in approximately 90% of cases 2, 3, 5
• EVL is superior to sclerotherapy with fewer complications 2, 3
• For gastric varices, use tissue adhesive (N-butyl-cyanoacrylate) rather than EVL, as it achieves better control with lower rebleeding rates (23% vs. 47%) 2, 3
• Combination of EVL plus vasoactive drugs is superior to either alone 5

Rescue Therapy for Treatment Failure

• TIPS is indicated when bleeding cannot be controlled or recurs despite combined pharmacological and endoscopic therapy 2, 3, 4, 5
• Early TIPS within 24-72 hours for high-risk patients (Child-Pugh C <14 or Child-Pugh B >7 with active bleeding) 4
• Balloon tamponade as temporary bridge (maximum 24 hours) while arranging definitive therapy 2, 4

Schistosomiasis-Specific Considerations

Antiparasitic Treatment

• Praziquantel is the treatment of choice for schistosomiasis and should be administered to eradicate active infection 1, 7
• Praziquantel can reverse mild to moderate hepatic fibrosis when given early 1
• Active S. mansoni infection remains highly prevalent (mean 69.9%) even in patients with established PPF, necessitating treatment 7

Surgical Considerations Unique to Schistosomiasis

• Selective shunts (distal splenorenal) or splenectomy with esophagogastric devascularization are effective options for recalcitrant bleeding in schistosomiasis 1
• Previous splenectomy significantly improves outcomes with subsequent sclerotherapy (97.3% vs. 72.7% control of rebleeding) 8
• Avoid non-selective shunts (proximal splenorenal, TIPS) in schistosomiasis due to higher risk of hepatic encephalopathy and potential hepatic impairment from reduced perfusion in patients with preserved synthetic function 1
• Schistosomiasis patients tolerate selective shunts better than cirrhotic patients due to preserved hepatic function 1

Secondary Prophylaxis (Prevention of Rebleeding)

Combination Therapy (Most Effective)

• Initiate non-selective beta-blockers plus repeat EVL sessions once recovered from acute bleeding 2, 3, 5
• This combination reduces rebleeding to 14-23% versus 38-47% with EVL alone 2, 3, 5
• Beta-blockers should be started once hemodynamically stable, NOT during acute bleeding 2, 3

Beta-Blocker Regimen

• Use propranolol or nadolol titrated to maximal tolerated dose 2, 3
• Target is maximal tolerated dose, not specific heart rate 3
• Non-selective beta-blockers reduce rebleeding and are particularly effective in schistosomiasis 7

EVL Schedule

• Repeat EVL every 7-14 days (or 2-8 weeks) until variceal obliteration, typically requiring 2-4 sessions 2, 5
• EVL is effective and safe in schistosomiasis patients, reducing variceal recurrence to 19% and bleeding to 11.9% 9
• After eradication, perform surveillance endoscopy every 3-6 months to evaluate for recurrence 2, 3, 4

Alternative: Beta-Blocker Plus Nitrate

• Combination of non-selective beta-blocker plus isosorbide mononitrate (ISMN) achieves 33-35% rebleeding rate 2
• However, this combination has significantly greater side effects and poor tolerance in clinical practice 2

Primary Prophylaxis (If No Prior Bleeding)

• Non-selective beta-blockers are indicated for medium/large varices to prevent first hemorrhage 3
• Also indicated for small varices with high bleeding risk (Child B/C cirrhosis or red wale marks) 3
• Given the high prevalence of varices in schistosomiasis PPF (average 82.1%), screening endoscopy is essential 7

Monitoring and Prognostic Factors

HVPG Response (Best Predictor)

• Patients achieving HVPG reduction to <12 mmHg or >20% from baseline have lowest rebleeding rate (10%) 2, 3
• HVPG responders may not require endoscopic therapy 2

Surveillance Schedule

• Perform endoscopy every 3-6 months after variceal eradication 2, 3, 4
• Varices recur in 19% of schistosomiasis patients treated with EVL over 29 months average follow-up 9

Critical Pitfalls to Avoid

• Never use beta-blockers during acute bleeding as they decrease blood pressure and blunt physiologic tachycardia 2, 3
• Never stop octreotide immediately after endoscopy; continue for 3-5 days to prevent early rebleeding 4
• Never perform non-selective shunts (proximal splenorenal, TIPS) in schistosomiasis due to excessive encephalopathy risk with preserved hepatic function 1
• Never use TIPS as first-line therapy; reserve for rescue after failed pharmacological/endoscopic treatment 2
• Do not overlook active schistosomiasis infection; treat with praziquantel as it can reverse fibrosis and reduce long-term complications 1, 7
• Recognize that late diagnosis with bleeding varices carries 29% mortality even with optimal care, emphasizing need for screening 7

Transplant Evaluation

• Refer to transplant center if Child-Pugh score ≥7 or MELD score ≥15 2, 5
• However, schistosomiasis patients typically have preserved synthetic function and may not meet traditional transplant criteria despite severe portal hypertension 1