Management of hypersplenism in non-cirrhotic portal hypertension: a surgical series

BACKGROUND: Hypersplenism is commonly seen in patients with non-cirrhotic portal hypertension (NCPH). While a splenectomy alone can effectively relieve the hypersplenism, it does not address the underlying portal hypertension. The present study was undertaken to analyze the impact of shunt and non-shunt operations on the resolution of hypersplenism in patients with NCPH. The relationship of symptomatic hypersplenism, severe hypersplenism and number of peripheral cell line defects to the severity of portal hypertension and outcome was also assessed. METHODS: A retrospective analysis of NCPH patients with hypersplenism managed surgically between 1999 and 2009 at our center was done. Of 252 patients with NCPH, 64 (45 with extrahepatic portal vein obstruction and 19 with non-cirrhotic portal fibrosis) had hyperspienism and constituted the study group. Statistical analysis was done using GraphPad InStat. Categorical and continuous variables were compared using the chi-square test, ANOVA, and Student's t test. The Mann-Whitney U test and Kruskal-Wallis test were used to compare non-parametric variables. RESULTS: The mean age of patients in the study group was 21.81 +/- 6.1 years. Hypersplenism was symptomatic in 70.3% with an incidence of spontaneous bleeding at 26.5%, recurrent anemia at 34.4%, and recurrent infection at 29.7%. The mean duration of surgery was 4.16 +/- 1.9 hours, intraoperative blood loss was 457 +/- 126 (50-2000) mL, and postoperative hospital stay 5.5 +/- 1.9 days. Following surgery, normalization of hypersplenism occurred in all patients. On long-term follow-up, none of the patients developed hepatic encephalopathy and 4 had a variceal re-bleeding (2 after a splenectomy alone, 1 each after an esophago-gastric devascularization and proximal splenorenal shunt). Patients with severe hypersplenism and those with defects in all three peripheral blood cell lineages were older, had a longer duration of symptoms, and a higher incidence of variceal bleeding and postoperative morbidity. In addition, patients with triple cell line defects had elevated portal pressure (P=0.001), portal biliopathy (P=0.02), portal gastropathy (P=0.005) and intraoperative blood loss (P=0.001). CONCLUSIONS: Hypersplenism is effectively relieved by both shunt and non-shunt operations. A proximal splenorenal shunt not only relieves hypersplenism but also effectively addresses the potential complications of underlying portal hypertension and can be safely performed with good long-term outcome. Patients with hypersplenism who have defect, in all three blood cell lineages have significantly elevated portal pressures and are at increased risk of complications of variceal bleeding, portal biliopathy and gastropathy.