Hepatoportal sclerosis: a cause of portal hypertension to bear in mind

Abstract

To the editor: Hepatoportal sclerosis (HS) is an idiopathic noncirrhotic intrahepatic portal hypertension characterized by splenomegaly, hypersplenism and portal hypertension. Although it is considered uncommon in developed countries there is an increasing number of cases reported which could traduce a better knowledge of this disease and a higher level of suspicion. Our objective is to report a case of HS emphasizing diagnosis and the long-term evolution. A 47-year-old man was referred in 1992 to our institution to study a bicytopenia detected in the course of the study of epigastric complaints; he admitted a low ethanol intake (< 30 g/day). His medical history only included a left glaucoma under topic treatment with latanoprost. Laboratory tests revealed low leukocyte (3,400/μl; normal range, 4,50011,000) and platelet (59,000/μl; 150,000-350,000) counts without anaemia. Coagulation and biochemical tests including liver function tests (LFTs) and serum proteins were normal. The upper endoscopy showed a mild portal gastropathy, little esophageal varices and an Helicobacter pylori positive duodenal ulcer. Chronic infection by hepatotropic viruses was discarded and a screening of other frequent causes of chronic liver damage did not reveal infectious, autoimmune or metabolic diseases. A hypercoagulable state was discarded. A Doppler ultrasound revealed a normal liver echotexture, size and surface without portal vein thrombosis, splenomegaly and collateral vessels showing portal hypertension (PH). A percutaneous liver biopsy was then performed showing portal fibrosis without cirrhosis (fig. 1). Finally, a haemodynamic assessment revealed a near-normal hepatic venous pressure gradient of 7 mmHg (free suprahepatic pressure: 9.0 mmHg and wedged suprahepatic pressure: 16.0 mmHg). A diagnosis of HS was done. He became asymptomatic after Helicobacter eradication and refused follow-up. Fifteen years later, follow-up has been resumed; he feels well and no liver decompensation has occurred. Laboratory tests show low leukocyte (4,100/μl) and platelet (59,000/μl) counts without anaemia, and a preserved liver function. Endoscopy findings are unchanged and a computerized tomography reveal liver atrophy, no portal thrombosis, signs of PH with an open splenorenal shunt, patent portal vein and splenomegaly (fig. 2). Primary prophylaxis of variceal bleeding has been initiated. HS is a non-cirrhotic presinusoidal intrahepatic PH. Other names that seem synonymous include idiopathic portal hypertension (IPH), Banti’s disease, non-cirrhotic portal fibrosis, obliterative portal venopathy, noncirrhotic intrahepatic portal hypertension and idiopathic presinusoidal portal hypertension. IPH has been reported mainly in India and Japan being uncommon in Occident where, notwithstanding, new cases are continuously reported3-5. The diagnostic criteria include splenomegaly, normal to near-normal LFTs, demonstrable varices, decrease of one or more of the formed blood elements, liver scan not typical of cirrhosis, patent portal and hepatic veins with elevated portal pressure and a normal to slightly elevated wedged hepatic venous pressure, and marked portal fibrosis with no diffuse nodule formation. Although not all of these criteria are necessary, PH must be unequivocal with absolute exclusion of cirrhosis, schistosomiasis, and the occlusion of the hepatic and portal veins1-5. The exact cause of IPH is still obscure and toxic, autoimmune or infectious theories have been postulated. Although it usually has a benign course, liver atrophy, cirrhosis and even liver failure can occur in latter phases of the disease. In fact, some cases of liver transplantation have been performed in patients with end-stage liver disease attributed to a putative cryptogenic cirrhosis that have ultimately resul220.823