A Rare Case of Chronic Extra-Hepatic Portal Vein Obstruction (EHPVO) Presenting With Variceal Bleed

Abstract

EHPVO is a vascular disorder of the liver defined by obstruction of the extra-hepatic portal vein with or without involvement of the intra-hepatic portal or splenic or superior mesenteric veins. Underlying hypercoagulable and prothrombotic states are commonly reported from the West. 21-year-old female with history of Acute lymphoblastic leukemia diagnosed 2002 requiring chemotherapy currently in remission, splenectomy 2009 presents with large volume hematemesis. Denied alcohol use, NSAID use, anticoagulation, drug use or herbal medications. Hemoglobin (Hb) 4.8, Liver function tests (LFTs) normal. EGD showed 3 columns of large esophageal varices with high-risk stigmata s/p 8 bands. Ultrasound (US) Doppler showed Heterogeneous liver echotexture otherwise normal. She presented 2 weeks later with abdominal Pain, fever and chills. Hb 4.1 (8.6 at discharge). Normal platelets, INR and LFTs. Peripheral smear unremarkable. Reticulocyte % 2.8. CT Abdomen showed occlusive thrombosis of the main portal vein extending into the right portal vein and SMV with collateral vasculature. EGD/Colonoscopy showed small Esophageal and rectal varices. Parvovirus, Ebstein bar virus, Cytomegalovirus, Bone marrow biopsy, Factor 5 Leiden, F2 202110G Variant, JAK2 V617F, ANA, Beta-2 glycoprotein and Cardiolipin negative. Hepatic venous pressure gradient (HVPG) was 6. Liver biopsy showed some enlarged and peripherally displaced portal vein branches, mild sinusoidal dilatation. No significant fibrosis, steatosis or iron accumulation. Patient was diagnosed with Idiopathic or splenectomy induced EHPVO. Chronic EHPVO presents commonly with repeated, well-tolerated bleeding episodes from esophageal varices. Gastric varices are present in about one-third of the patients with EHPVO. Moderate to massive splenomegaly is universal, and may be a presenting feature. HVPG is normal. EHPVO is diagnosed by imaging as Doppler US, CT or MRI, which demonstrate portal vein obstruction, presence of intraluminal thrombus in the portal vein and/or portal vein cavernoma. Liver biopsy is necessary in a patient with EHPVO if the liver functions are deranged. We need to pursue Investigations to evaluate for a Prothrombotic State. Upper endoscopy is warranted in these patients. For the control of acute variceal bleeding, endoscopic therapy with banding is effective. In a patient with established chronic EHPVO, anti-coagulants are recommended if there is a history of recurrent thrombotic episodes.