Obliterative portal venopathy

Hepatoportal sclerosis (HPS) is a rare disorder characterized by sclerosis of the intrahepatic portal veins.

Histopathologic findings are

• Periportal fibrosis

• Occlusion of small portal veins

• Sclerosis of the portal venous system

• Proliferation of small vascular channels within/around portal tracts

• Exposure to toxic substances (arsenic or vinyl chloride)

• Drugs (vitamin A, 6-mercaptopurine

• Autoimmune and connective tissue diseases

• Systemic or intra abdominal infections

• Clotting factor abnormalities

Differential diagnosis from liver cirrhosis is crucial and may be difficult. A large liver biopsy specimen, together with expertise and awareness of the examiner, are requested for optimal diagnosis.

The prevalence of this disease is unknown, and the distribution is worldwide, with many cases reported in Asia.

The disease can occur at any age.

Both males and females are equally affected.

• Liver failure
• Ascites
• Encephalopathy

Current overall prognosis is good (80% survival at ten years after the diagnosis). Male patients and patients with disease onset before 40 years of age show a poorer prognosis.

Lethargy and in advanced stages symptoms of liver failure

• Jaundice

• Ascites
• Spleenomegaly
• Signs of portal hypertension

• Confusion
• Encephalopathy

• Pancytopenia
• Elevated liver enzymes
• Liver function tests

• Esophageal varices

• Evidence of sclerosis of portal tract

• Delineates the portal system and helps in the identification of lesions.

Variceal bleeding is the main cause for morbidity and mortality. Treatment of active bleeding and its prophylaxis can follow the recommendations for patients with cirrhosis. In considering spleenectomy, the high risk of thrombosis in the portal venous system should be taken into account

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