S5660 An Unusual Case of Portal Hypertension

Introduction: In rare cases, portal hypertension (pHTN) is presinusoidal and occurs in the absence of cirrhosis such as portosinusoidal vascular disorder (PSVD). PSVD is a clinicopathologic diagnosis with various histologic findings including hepatoportal sclerosis (HPS). Herein, we describe this rare case of portal hypertension. Case Description/Methods: A 35-year-old woman with history of untreated portal vein thrombosis presents with 2 episodes of hematemesis. Other than a palpable spleen the exam was normal. Hgb 5.9 g/dL, Platelet 66 U/L, international normalized ratio 1.2, TBili 0.4, alkaline phosphatase 63, aspartate aminotransferase 30 and alanine aminotransferase 30. Hepatitis A/B immune, hepatitis C and HIV negative. Ceruloplasmin, alpha-1 antitrypsin, celiac, ANA, ASMA, AMA negative. Ig A and IgM normal and IgG was 1743 mg/dL (600-1640). Hypercoagulable work up negative. No oral contraceptive pill use. Family history is positive for alcohol-related cirrhosis. Patient drank alcohol on occasion, smoked cannabis and tobacco, used cocaine, ecstasy and molly but denied IVDU. Abd/Pelvic CT showed a posterior notch sign, cavernous transformation of the portal vein (PV), 18.4 cm spleen, trace ascites, portal colopathy, abdominal varices, and no thrombosis. On esophagogastroduodenoscopy, there were F3 esophgeal varices with stigmata s/p EVL. Outpatient elastography was 4.8 kPa with a CAP of 230 dB/m. Due to concern for non-cirrhotic pHTN, a liver biopsy was performed and showed PV herniation beyond the portal tract borders and focal portal vein sclerosis. There was no steatosis, no advanced fibrosis or cirrhosis on trichrome and reticulin stains. A diagnosis of HPS was made. Discussion: HPS is rare in the Western world, the pathophysiology is unknown, and variceal bleeding in the absence of cirrhosis is the most common presentation. Other less common presentations include ascites, hepatic encephalopathy and PVT. A high clinical suspicion is important when imaging findings are equivocal for cirrhosis but there are signs of pHTN. In this case of variceal bleed and imaging signs of portal hypertension, normal liver enzymes as well as normal elastography helped support a low suspicion for cirrhosis and need for a diagnostic liver biopsy. While the etiology of HPS has not been elucidated multiple derangements may impact endothelial integrity and vascular remodeling complicated by thrombosis and/or fibroproliferation leading to obstruction (phlebosclerosis) and pHTN. There have been many associations including toxins, medications, and genetic disorders such as short telomeres syndrome. Treatment is focused on managing portal hypertension.