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Referral and warning signs
When to refer
Referral to specialist (Gastroenterologist/Hepatologist/Infectious Diseases) for: Diagnosis confirmation (Ultrasound, Endoscopy). Management of variceal bleeding. Management of significant portal hypertension complications (ascites, hypersplenism). Consideration for specific interventions (banding, TIPS - rare).
Red flags
- Haematemesis or melaena (variceal bleeding)
- Signs of severe anaemia
- Severe thrombocytopenia
- New onset or tense ascites
Signs and symptoms
- Often asymptomatic for years.
- Symptoms related to Portal Hypertension:
- - Haematemesis/Melaena (due to bleeding oesophageal/gastric varices) - often first major presentation.
- - Splenomegaly (often massive).
- - Ascites (develops later than in cirrhosis, often related to hypoalbuminemia or advanced disease).
- - Thrombocytopenia / Pancytopenia (due to hypersplenism).
- Hepatomegaly (may be present, liver often firm).
- Abdominal discomfort.
- Symptoms related to intestinal schistosomiasis: Diarrhoea (may be bloody), abdominal pain.
- Growth retardation/stunting in children.
- Liver synthetic function (Albumin, INR) often preserved until very late stage.
Risk factors
- Living in or travel to areas endemic for Schistosoma mansoni or S. japonicum.
- Exposure to contaminated freshwater (containing infected snails).
- Repeated infections / High parasite burden.
- Genetic factors influencing fibrotic response.
Diagnosis
Clinical assessment
Suspect in individuals from endemic areas presenting with signs of portal hypertension (splenomegaly, variceal bleeding), especially if liver function appears relatively preserved. History of freshwater exposure.
Investigations
- Abdominal Ultrasound: Key diagnostic tool. Shows characteristic periportal fibrosis ('pipestem' appearance), portal vein thickening, splenomegaly. WHO Niamey protocol standardizes assessment.
- Stool Microscopy (Kato-Katz technique): Detection of Schistosoma mansoni eggs confirms infection (but may be negative in established hepatic fibrosis).
- Serology (Antibody tests for Schistosoma): Indicates exposure but not necessarily active infection or hepatic involvement.
- Upper GI Endoscopy (OGD): Essential to detect and manage oesophageal/gastric varices.
- Blood tests: CBC (thrombocytopenia, anaemia), LFTs (often near normal initially, may become abnormal late), Albumin, INR/PT.
- Liver Biopsy: Rarely needed, shows portal fibrosis, granulomas around eggs, preserved lobular architecture.
Diagnostic criteria
Characteristic findings on abdominal ultrasound (periportal fibrosis) in a patient from an endemic area, often with splenomegaly +/- evidence of portal hypertension (varices) or Schistosoma eggs in stool.
Management
Non-pharmacological management
- Management of Portal Hypertension Complications:
- - Variceal Bleeding: Acute management (resuscitation, vasoactive drugs, antibiotics, endoscopic banding) and secondary prophylaxis (non-selective beta-blockers like Propranolol +/- band ligation) as for cirrhosis (see GI020).
- - Ascites: Sodium restriction, diuretics (Spironolactone +/- Furosemide) (see GI020).
Pharmacological treatment
- Anti-schistosomal Treatment:
- - Praziquantel 40-60 mg/kg single dose (or divided into 2 doses 4-6h apart). Treats active infection, may prevent progression but unlikely to reverse established severe fibrosis.
- - Repeat treatment may be needed with re-exposure.
- Management of Portal Hypertension:
- - Non-selective Beta-blockers (Propranolol, Nadolol): For primary and secondary prophylaxis of variceal bleeding.
- - Diuretics for ascites.
Monitoring
- Monitor for complications of portal hypertension (variceal bleeding, ascites).
- Periodic CBC (platelets).
- Periodic Ultrasound to monitor fibrosis/splenomegaly.
- Endoscopic surveillance for varices.
Complications
- Variceal haemorrhage (major cause of mortality)
- Hypersplenism (thrombocytopenia, anaemia, leucopenia)
- Ascites
- Portal vein thrombosis
- Pulmonary hypertension (schistosomal associated)
- Glomerulonephritis
- Growth retardation in children
- Secondary bacterial infections
Prevention
- Mass Drug Administration (MDA) with Praziquantel in endemic areas.
- Snail control.
- Improved sanitation and access to safe water.
- Health education to avoid contact with contaminated water.
Patient education
- Explain schistosomiasis and its effect on the liver/portal system.
- Importance of Praziquantel treatment for active infection.
- Need for long-term monitoring for complications (bleeding, fluid).
- How to avoid re-exposure to contaminated water.
- Importance of adherence to medications (e.g., beta-blockers if prescribed).
- Signs of bleeding to report immediately.
Uganda-specific notes
Schistosoma mansoni is endemic in many areas around lakes and rivers. Hepatosplenic schistosomiasis is a major cause of portal hypertension and variceal bleeding in Uganda. Ultrasound is key diagnostic tool. Praziquantel is used for treatment/control. Management focuses on preventing/treating variceal bleeds.
References
- Uganda Clinical Guidelines (UCG) 2023
Chapter 6, Section 6.5.5
- Uganda Clinical Guidelines (UCG) 2023
Chapter 2 (likely section on Schistosomiasis)