University of Massachusetts Chan Medical School - Baystate Health Springfield, MA
Syed Hamza Sohail, MD1, Pranav S. Ramamurthy, MD2, Aizaz Khan, MD3, Nha Duong, DO2 1University of Massachusetts Chan Medical School - Baystate Health, Springfield, MA; 2University of Massachusetts Chan Medical School - Baystate Medical Center, Springfield, MA; 3University of Massachusetts Chan Medical School - Baystate Medical Center, Broad Brook, CT
Introduction: Non-cirrhotic portal hypertension (NCPH) is a rare entity with a reported Western incidence of 3-6% and can be challenging to diagnose. Our case describes a patient with pre-hepatic NCPH in setting of extensive portal and mesenteric veinous thrombosis.
Case Description/Methods: An 85-year-old male has a distant history of small bowel ischemia requiring small bowel resection in 2010 due to superior mesenteric vein (SMV) and portal vein (PV) thrombosis. He carried a presumed diagnosis of liver cirrhosis and was on long term anticoagulation (AC). Patient presented to the hospital now with generalized weakness and confusion. He was found to have anemia and thrombocytopenia. CT abdomen showed a fatty liver, significant splenomegaly, extensive gastric varices, and SMV collaterals. Esophagogastroduodenoscopy revealed normal esophagus and large non-bleeding gastric varices which were managed conservatively. Cirrhosis workup was negative. His high INR was attributed to his warfarin use for AC. His albumin level and liver enzymes were normal and suggested against liver cirrhosis. Patient also developed encephalopathy. His hematology work up pointed to heterozygous prothrombin mutation as likely cause for his thrombosis.
Comparison of the recent CT images with prior films from 2010 revealed a dramatic difference in the caliber and tortuosity in the vessels, indicating extensive neovascularization from chronic occlusion with resultant NCPH. His encephalopathy was likely to be from portosystemic shunting. The patient was continued on beta-blocker therapy, started on lactulose with improvement in encephalopathy, and recommended further outpatient follow-up.
Discussion: This patient exhibited significant portal hypertension with extensive gastric varices and encephalopathy likely from portosystemic shunts. This is a case of pre-hepatic NCPH in setting of PV and SMV thrombosis secondary to heterozygous prothrombin mutation. His imaging shows dramatic neovascularization. This case highlights the importance of considering NCPH in portal hypertension and always investigating for the underlying cause before presuming cirrhosis as the etiology of portal hypertension.
Figure: Figure A: CT abdomen/pelvis from this admission with extensive neovascularization and varices. Figure B: Prior CT abdomen/pelvis from 2010 with PV and SMV thrombosis.
Disclosures:
Syed Hamza Sohail indicated no relevant financial relationships.
Pranav Ramamurthy indicated no relevant financial relationships.
Aizaz Khan indicated no relevant financial relationships.
Nha Duong indicated no relevant financial relationships.
Syed Hamza Sohail, MD1, Pranav S. Ramamurthy, MD2, Aizaz Khan, MD3, Nha Duong, DO2. P1382 - Questioning Assumptions: Non-Cirrhotic Portal Hypertension, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.
