Case ReportsTransjugular intrahepatic portosystemic shunt for the treatment of portal hypertension secondary to non-cirrhotic perisinusoidal hepatic fibrosisJacobi, Davida; de Muret, Anneb; Arbeille, Brigittec; Perarnau, Jean-MarcaAuthor Information Departments of aHepatogastroenterology bPathology cElectron Microscopy and Cell Biology, Centre Hospitalier Universitaire, Tours, France Correspondence to Dr Jean-Marc Perarnau, Hepatogastroenterologie, Centre Hospitalier Universitaire Trousseau, avenue de la République, 37044 Tours Cedex 9, France Tel: +0033 (0)247 472204; fax: +0033 (0)247 478428; e-mail: [email protected] Received 1 November 2005 Accepted 6 January 2006 European Journal of Gastroenterology & Hepatology: May 2006 - Volume 18 - Issue 5 - p 549-551 Buy Abstract Non-cirrhotic perisinusoidal hepatic fibrosis is a process of imprecise pathogenesis involving collagenization of the space of Disse. Exposure to chemicals, auto-immunity, thrombophilia and/or infections are suspected primary agents. Here, we present the case of a patient who developed severe portal hypertension with histological features suggesting a non-cirrhotic perisinusoidal hepatic fibrosis. A 52-year-old man was hospitalized for oesophageal variceal haemorrhage. Liver cirrhosis or portal vein thrombosis were absent as attested by laboratory tests, duplex sonography, computed tomography scan and histological examination of a liver biopsy specimen. Presinusoidal portal hypertension was suggested by a normal wedge-free hepatic vein gradient. Only electron microscopy examination of a liver biopsy specimen could disclose perisinusoidal fibrosis. This was most probably secondary to a combined chemotherapy received 4 years earlier for non-Hodgkin large-cell lymphoma. As variceal ligation failed to control oesophageal varices while liver function tests were normal, a transjugular intrahepatic portosystemic shunt (TIPS) was performed. This dramatically improved the signs of portal hypertension. This case illustrates the use of TIPS in the treatment of portal hypertension secondary to non-cirrhotic perisinusoidal fibrosis. © 2006 Lippincott Williams & Wilkins, Inc.