Noncirrhotic Portal Hypertension – A Case Report on a Sequela of Portal Vein Cavernoma

Abstract

Introduction: Increased pressures in the portal vein (portal hypertension) which occurs following portal vein thrombosis, results in cavernous transformation of the portal vein. Though portal vein thrombosis (PVT) is a frequent complication in cirrhotic patients, it may also exist as a basic vascular condition without any liver damage. Among the predisposing factors for portal vein cavernoma are deficiencies in protein C, S & antithrombin III, antiphospholipid syndrome and mutations in factor V Leiden and JAK2. Determination of the aetiology aids in the management plan to not only relieve symptoms of the patient but also to treat the underlying cause. Gastroesophageal variceal bleeding, splenomegaly, portosystemic collaterals, and ultimately hematologic abnormalities are among the prominent clinical features. Case Presentation: We present a case of a 16-year-old male with portal vein cavernoma complicated by bleeding oesophageal varices presenting with a second episode of hematemesis and melena within a 10-year period. He underwent endoscopic variceal band ligation and was put on oral warfarin and propranolol. The patient was followed up once at the outpatient clinic after discharge without the laboratory investigations we requested due to financial constraints. He has since been lost to follow up. Conclusion: Bleeding oesophageal varices from noncirrhotic causes are common and a high index of suspicion is needed to make a diagnosis. Though investigations tailored towards identifying the underlying cause presents a challenge in a resource constrained setting like ours, management of complications and symptoms to reduce morbidity and mortality cannot be over-emphasized.