Surgical and endovascular treatment of severe complications secondary to noncirrhotic portal hypertension: experience of 56 cases.

BACKGROUND: Major complications of noncirrhotic portal hypertension (NCPH) include bleeding esophagogastric varices, hypersplenism, ascites, and bowel ischemia under acute circumstances. The aim of this article is to determine the outcomes of surgical and endovascular treatments for severe complications from NCPH.

METHODS: From January 2000 to June 2011, 56 patients with symptomatic NCPH underwent open surgery or endovascular thrombolysis. The medical records were retrospectively reviewed. Of the 56 patients, there were 39 males and 17 females. The mean age was 21 years, ranging from 2 to 54 years. Forty-one of them were diagnosed to have prehepatic portal vein obstruction (PHPVO), 9 had Budd-Chiari syndrome (BCS), and 6 had noncirrhotic portal fibrosis (NCPF). All patients were symptomatic from 5 days to 14 years (mean 25 months). Portosystemic shunt (PSS) was primarily performed in 49 patients. Shunts were as follows: 35 mesocaval; 7 splenorenal; 4 portocaval; 2 paraumbilical-jugular; and 1 portal to right atrial. Esophagogastric devascularization was performed in 3 patients, but was converted to mesocaval shunt later in 2. The remaining 4 patients with acute superior mesenteric vein (SMV) and portal vein thrombosis were treated with endovascular catheter-directed thrombolysis. Warfarin was prescribed to all the patients for at least 6 months. Mean follow-up was 57 months, ranging from 2 to 125 months. The outcomes, focusing on 30-day mortality, recurrent bleeding, and hypersplenism, were recorded.

RESULTS: In the 49 patients undergoing primary PSS, the shunts remained patent and there was no recurrent variceal bleeding during the follow-up. All 3 patients with esophagogastric devascularization had recurrent variceal bleeding at 8, 13, and 24 months postoperatively. Two of them were converted to mesocaval shunt, and 1 died before redo operation. Thrombolysis in all 4 patients with acute SMV and portal thrombosis was technically successful. Three of the 4 survived without complications and 1 died from small bowel infarction due to recurrent thrombosis 40 days later. In the 47 patients with hypersplenism, mean platelet counts increased from 43×10(9)/L to 239×10(9)/L 2 weeks after surgery. Ascites in 30 of the 31 patients disappeared within 2 months after PSS. There was no postoperative encephalopathy, and perioperative 30-day mortality was 0%.

CONCLUSIONS: PSS can be employed to treat bleeding esophagogastric varices and severe hypersplenism secondary to NCPH. Post-PSS encephalopathy is less of a concern in NCPH patients with normal liver function. Endovascular catheter-directed thrombolysis via superior mesenteric artery is a useful alternative treatment for acute portal and/or mesenteric venous thrombosis.