[Hemorrhagic portal hypertension with soft liver]

Over 14 years the authors have observed 6 adults with a special form of portal hypertension revealed by massive gastrointestinal hemorrhages due to rupture of voluminous esophageal varices. Though first suggested by huge enlargement of the spleen (5 out of 6 cases), cirrhosis was eventually discounted by laparoscopy and laparotomy. The liver was of normal volume, smooth (never nodular) and above all soft to the touch, to the biopsy needle and to the surgeon's hand. The histologic lesions of the liver were minimal and heterogeneous: fibrosis without cirrhosis (2 cases), isolated and mild steatosis (2 cases), one non-specific lymphohistiocytic granuloma (2 cases), moderate myeloid metaplasia (2 cases), hepatic siderosis without fibrosis (1 case), sinusoidal congestion (3 cases). Normality of the portal vein was evidenced by angiography (5 cases) or inferred from the elevated porta-caval gradient (1 case), and was confirmed once on autopsy. Sus-hepatic catheterization was performed in every case and provided the following information: right atrial pressure always normal, sus-hepatic veins normal, porta-caval gradient normal or slightly elevated in 4 patients (suggesting presinusoidal block) and obviously elevated in the other two (as in post-sinusoidal block). The minimal histologic lesions of the liver, the normality of the portal vein, the lack of sus-hepatic obstruction, and the variable porta-caval gradient make it impossible to localize the obstacle (if any) and thus say what is the mechanism of portal hypertension with soft liver. In the absence of elevated splenic inflow, this hypertension has to be classified as essential or idiopathic. Soft consistency of the liver has never been reported before in such cases.