Revisiting the Rare Cause of Non-Cirrhotic Portal Hypertension in West

Abstract

Purpose: A 45-year-old man with a presumed diagnosis of cryptogenic liver cirrhosis came to our institution for a second opinion regarding need for liver transplantation. He had prior episodes of rectal bleeding. He had undergone extensive evaluation, and liver transplantation was suggested to him. He was born and raised in Egypt and moved to the United States at age 24. His physical exam was only significant for splenomegaly. His calculated MELD score was 9. Additional laboratory testing included negative serological tests for viral hepatitis, autoimmune hepatitis, PBC, PSC and normal iron studies, ceruloplasmin, and alpha-1 antitrypsin values. Serum IgG for Schistosoma mansoni was performed and returned positive (5.66, normal value < 1.0). Outside liver biopsy reviewed at our center showed non-caseating granulomas, minimal fibrosis stage 0-1. He was therefore diagnosed with non-cirrhotic portal hypertension related to schistosomiasis. He was treated with oral praziquantel. Despite prophylaxis with propranolol, the patient continued to have episodes of gastrointestinal bleeding and underwent porto-caval shunt with excellent clinical response. Liver biopsy at time of surgery revealed concentric fibrosis around bile ducts (Symmer's pipe stem fibrosis) (Figure 1) and multiple small non-caseating granulomas with eosinophils in liver parenchyma. Trichrome stain revealed subcapsular bridging fibrosis. Schistosomiasis is the second most prevalent tropical disease affecting humans, although rarely observed in the United States. It is acquired by penetration of broken skin by the parasite when in contact with contaminated fresh water. Initial infections can be asymptomatic until organ damage has occurred several years later. Adult worms lay eggs in intestinal venous drainage which migrate to the liver via the portal circulation. Granulomatous inflammation and fibrosis secondary to infection can lead to portal hypertension. The treatment of choice is oral praziquantel, but this does not reverse the fibrosis that has already developed. It is imperative to consider schistosomiasis as an etiology of liver disease in patients who originate from endemic regions and provide early treatment in order to prevent complications such as portal hypertension.Figure: No Caption available.