Extrahepatic portal vein
thrombosis (EPVT) in children can lead to severe
bleeding from gastrointestinal
varices,
ascites,
thrombocytopenia from
hypersplenism, and other coagulation disorders. The authors have used the superior mesenteric vein to intrahepatic left portal vein (Rex) shunt in 5 children with symptomatic EPVT and report their results with this novel technique.
METHODS: Children with symptomatic
portal hypertension were screened for the underlying cause. All children with essentially normal livers and obstruction of the extrahepatic portal vein were considered for the Rex shunt. Evaluation included liver function tests, liver biopsy, and radiological evaluation of the intrahepatic vascular anatomy.
RESULTS: Five patients between the ages of 2.8 and 10.5 years underwent evaluation for
portal hypertension secondary to extrahepatic portal vein obstruction. Three patients had idiopathic extra hepatic portal vein
thrombosis with cavernous transformation, 1 had
thrombosis after a living-related
liver 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
esophageal and gastric varices, and all 5 had relative degrees of
hypersplenism with enlarged spleens and
thrombocytopenia (11,000 to 77,000). Three patients had significant
leukopenia. Results of imaging studies suggested that 3 patients had inadequate intrahepatic portal veins for shunting, but all patients at exploration underwent successful shunting. There were no serious
intraoperative complications. Postoperative complications included
ascites in 2 patients that resolved within 1 month. There were no early shunt
thromboses. The median postoperative
length of stay was 7 days. Clinical follow-up ranged from 7 to 21 months. Gastrointestinal
bleeding did not recur in any patient, and
ascites resolved in all. Spleen size decreased significantly (P < .01) from 9.4 +/- 4.0 cm to 5.0 +/- 3.7 cm below the left costal margin. Mean platelet count and white blood cell count rose after shunting from 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. Subsequently, shunt blockage occurred in 2 patients.
CONCLUSIONS: