Medical Journal of The Islamic Republic of Iran (MJIRI)

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Hepatoportal sclerosis or idiopathic portal hypertension has a worldwide distribution with prevalence in developing and temperate countries. Of 64 patients with this disease seen during a twelve year period, 36 underwent splenectomy or a shunt procedure. The indications for surgical intervention were severe hypersplenism and persistent left upper abdominal pain andlorhistory of frequent episodes of esophageal bleeding. There were two operative deaths from uncontrollable bleeding. Of 34 patients, none developed esophageal bleeding or encephalopathy on follow-ups ranging from one to 12 years. Splenectomy in patients without bleeding was effective. Proximal splenorenal shunt was also satisfactory in all cases. However, in the last few cases, Warren's shunt has produced even more encouraging results. In addition to a general overview, features of this disease not previously discussed or emphasized in the literature, operative findings and surgical methods used, along with long-term results, are presented.

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