Spontaneous Non-Traumatic Splenic Rupture in an Adult Patient with Extrahepatic Portal Venous Obstruction (EHPVO) – A Case Report with Review of Literature

Abstract

Extrahepatic portal venous obstruction (EHPVO) is a primary vascular disorder wherein there is obstruction of the extrahepatic portal vein with or without the involvement of the intrahepatic branches, splenic vein (SV), and/or superior mesenteric vein (SMV).[1] It is a longstanding disorder and the characteristic finding is the cavernous transformation of the portal vein. The condition is distinct from acute or chronic portal vein thrombosis (PVT) occurring in the setting of liver cirrhosis or hepatocellular carcinoma. EHPVO along with obliterative portal venopathy (OPV) are two important pre-hepatic causal factors for non-cirrhotic portal hypertension (NCPH) in which the structure of liver and function is preserved until late in the disease.[2,3,4] Budd-Chiari syndrome (BCS) or hepatic venous outflow tract obstruction (HVOTO) is another important post-hepatic etiology for NCPH.[1] In the reported case the author describes a case of a spontaneous non-traumatic splenic rupture in a patient of EHPVO with massive splenomegaly and features of portal hypertension.