Experience with the Rex shunt (mesenterico-left portal bypass) in childrenwith extrahepatic portal hypertension

Background/Purpose: Extrahepatic portal vein thrombosis (EPVT) in children can lead to severe bleeding from gastrointestinal varices, ascites, thrombo cytopenia from hypersplenism, and other coagulation disorders. The authors have used the superior mesenteric vein to intrahepatic left portal vein (Re x) shunt in 5 children with symptomatic EPVT and report their results with this novel technique. Methods: Children with symptomatic portal hypertension were screened for th e underlying cause. All children with essentially normal livers and obstruc tion of the extrahepatic portal vein were considered for the Rex shunt. Eva luation included liver function tests, liver biopsy, and radiological evalu ation of the intrahepatic vascular anatomy. Results: Five patients between the ages of 2.8 and 10.5 years underwent eva luation for portal hypertension secondary to extrahepatic portal vein obstr uction. Three patients had idiopathic extra hepatic portal vein thrombosis with cavernous transformation, 1 had thrombosis after a living-related live r transplant, and 1 had compression and obstruction of the main portal vein from enlarged lymph nodes after treatment of systemic histoplasmosis. All patients were symptomatic. Three patients had intermittent bleeding from es ophageal and gastric varices, and all 5 had relative degrees of hyperspleni sm with enlarged spleens and thrombocytopenia (11,000 to 77,000). Th ree pa tients had significant leukopenia. Results of imaging studies suggested tha t 3 patients had inadequate intrahepatic portal veins for shunting, but all patients at exploration underwent successful shunting. There were no serio us intraoperative complications. Postoperative complications included ascit es in 2 patients that resolved within 1 month. There were no early shunt th romboses. The median postoperative length of stay was 7 days. Clinical foll ow-up ranged from 7 to 21 months. Gastrointestinal bleeding did not recur i n any patient, and ascites resolved in all. Spleen size decreased significa ntly (P <.01) from 9.4 +/- 4.0 cm to 5.0 +/- 3.7 cm below the left costal m argin. Mean platelet count and white blood cell count rose after shunting f rom 79 +/- 42 to 176 +/- 73 (P <.02) and 5.4 +/- 2.3 to 7.5 +/- 3.9 (P=.06) , respectively. All shunts were studied at 1 and 7 days, and 3 and 6 months after the procedure. Shunt patency was documented in all cases. Subsequent ly, shunt blockage occurred in 2 patients. Conclusions: The Rex shunt has proven to be an effective method of resolvin g portal hypertension caused by EPVT including thrombosis after living dono r transplantation. This shunt is preferable to other surgical procedures be cause it eliminates portal hypertension and its sequelae by restoring norma l portal flow to the liver. Copyright (C) 2000 by W.B. Saunders Company.