Surgical treatment of non-cirrhotic presinusoidal portal hypertension

Background/Aims: Non-cirrhotic portal hypertension has a better prognosis than other forms of portal hypertension because of a well-preserved liver function in most cases. These patients are good candidates to receive surgical treatment, which is the therapeutic choice available with the lowest rebleeding rate. Because of abnormalities in the splanchnic vessels due to the nature of the diseases, many of them cannot be shunted. An extensive esophagogastric devascularization, the complete portoazygos disconnection, was evaluated. Methodology: A retrospective review of files of 31 patients, among 491 operations between 1991 an 2001 was carried out in a tertiary care Academic University Hospital. Patients comprised those with non-cirrhotic bleeding portal hypertension treated by means of complete portoazygos disconnection. Extensive two-stage (thoracic and abdominal) esophagogastric devascularization with modified transection of the esophagus was performed. Main outcome measures: recurrence of hemorrhage, encephalopathy and survival. Results: Thirty-one patients were treated. In 17 cases (54%) a hypercoagulable state was demonstrated. No operative mortality was observed (0-30 days) with a total of 62 operations (two stages per patient). No case of encephalopathy was observed and in 3 cases (9%) rebleeding was recorded. The survival curve showed a 5-year survival of 97% and a 10-year survival of 93%. Conclusions: Complete portoazygos disconnection is an excellent surgical alternative for patients with non-cirrhotic portal hypertension, with a low morbidity and mortality as well as a low rebleeding rate and good long-term survival.