Recanalized umbilical vein as a conduit for mesenterico/porto-Rex bypass for patients with extrahepatic portal vein obstruction.

PURPOSE: Mesenterico-left portal vein (meso-Rex) bypass is as an effective modality for restoring intrahepatic portal perfusion in patients with extrahepatic portal vein obstruction. Achieving sufficient patency is difficult with end-to-side anastomosis of a bypass graft to a small or hypoplastic left portal vein in the Rex recessus. Here, we describe the use of a recanalized umbilical vein in the round ligament as a conduit for bypass construction in two patients. METHODS: Case 1 was an 11-year-old boy diagnosed with rupture of the esophageal varices and hypersplenism due to congenital extrahepatic portal hypertension. Because of persistent hypersplenism and thrombocytopenia, he underwent meso-Rex bypassing with a left iliac vein graft interposed between the umbilical vein and the superior mesenteric vein. Case 2 was a neonate with a large hepatic tumor (mesenchymal hamartoma) that developed abdominal compartment syndrome at birth. The tumor was removed by right hepatectomy with excision of the portal vein bifurcation at 3 days of age. Porto-Rex bypassing was accomplished by end-to-end anastomosis between the portal vein trunk and the umbilical vein. RESULTS: Sufficient hepatopetal portal flow through the umbilical vein was achieved in both patients and maintained for over 16 and 13 months, respectively. Although hypersplenism remained in Case 1, intrahepatic portal vein branches gradually widened and the cavernoma in the hepatic hilum disappeared within 2 months. Neither patient had symptoms or signs of portal hypertension at the most recent follow-up. CONCLUSION: Using the umbilical vein as a vein conduit may facilitate construction of a meso/porto-Rex bypass and restore intrahepatic portal vein perfusion in patients with extrahepatic portal vein obstruction.

Congenital absence of the portal vein with splenomegaly and hypersplenism in a young woman.

Congenital absence of portal vein (CAPV) with a systemic shunt of enteric blood is a rare malformation. Twenty-seven cases have been reported to date; with the exception of 4 patients all cases had either liver anomalies or cardiac anomalies. CAPV is usually diagnosed before the age of 18 (25/27 cases). Here we present the case of a 33-year-old woman with CAPV without further anomalies. Beside suffering from circumscript scleroderma, splenomegaly and hypersplenism, the patient was otherwise healthy. Diagnosis was based on histological findings, angiography and CT scan. The liver biopsy showed portal tracts with inconspicuous arteries and bile ducts, but with severe loss of portal vein branches and minimal proliferation of bile ductules. In addition, the perivenular sinusoids were slightly dilated with focal parenchymal atrophy. Angiography showed a missing portal vein system and a missing splenic vein with drainage of the enteric blood through dilated perisplenic and perigastric collaterals to the azygos vein system. A CT scan revealed an empty liver hilus without portal vein. Here we discuss clinical aspects of this patient, show radiologic and histopathologic findings, and compare them to other cases in the literature.