P4827 - Porto-Sinusoidal Vascular Disease: A Case Report of PSVD in a Patient with Uncontrolled Diabetes Mellitus Type 2

Moshe Fenster, MD¹, Mahnoor Khan, DO², Pruthvi Patel, MD³, Frank Gress, MD^4
1Mount Sinai South Nassau, Valley Stream, NY; ²Mount Sinai South Nassau, Icahn School of Medicine at Mount Sinai, Oceanside, NY; ³Icahn School of Medicine at Mount Sinai, New York, NY; ⁴Mount Sinai South Nassau, New York, NY

Introduction: Porto-sinusoidal vascular disease (PSVD) is a rare form of biliary vascular disease that occurs in patients with preserved liver parenchyma. PSVD is a condition defined by typical histological lesions involving portal sinusoids or venules, with the absence of liver cirrhosis. We report a case of a female with uncontrolled DM type 2 and associated gastroparesis who was found to have evidence of PSVD on liver biopsy.

Case Description/Methods: A 58-year-old female with a PMH of uncontrolled DM type 2, gastroparesis, ESRD on HD, HTN, asthma, and pruritic anemia presented to our ED with a chief complaint of nausea and vomiting for 1 week. She reported mild, crampy epigastric pain, without radiation. Her gastroparesis was previously relatively well managed. Review of systems was negative for fever, hematemesis, diarrhea, and melena. Vital signs were notable for elevated blood pressure. Physical exam was remarkable for epigastric tenderness to palpation and pedal edema. Admission labs were remarkable for no leukocytosis, elevated glucose, and elevated LFTs. CT abdomen/pelvis showed thickened, edematous small bowel ileus with surrounding fat stranding, suggestive of severe enteritis. Patient received emergent dialysis, was placed on bowel rest and given IV antibiotics.

At her gastroenterology follow-up an upper endoscopic ultrasound showed abnormal echogenicity in the entire examined liver; fine needle biopsy was performed. Pathology report of her liver biopsy showed preserved hepatic architecture and findings consistent with PSVD.

Discussion: In rare cases, patients without liver parenchymal disease can present with findings of liver vascular disease, in a condition known as PSVD. Liver vascular disease in patients without cirrhosis is responsible for a mere 3-5% of cases of portal hypertension in the Western world. Numerous associated diseases, drugs, and toxins have been described in PSVD cases.

Our patient is unique and notable in that she did not have any of the risk factors that have previously been associated with PSVD; nonetheless, she was still found to have histological findings of PSVD on liver biopsy. Our case shows the importance of considering liver vascular disease, specifically PSVD, in a patient with abnormal liver enzymes, and gastrointestinal symptoms, especially in a patient with comorbid conditions. If other causes of liver disease have been ruled out, such a patient should be analyzed for histologic findings of PSVD on liver biopsy.


Disclosures:


Moshe Fenster, MD¹, Mahnoor Khan, DO², Pruthvi Patel, MD³, Frank Gress, MD⁴. P4827 - Porto-Sinusoidal Vascular Disease: A Case Report of PSVD in a Patient with Uncontrolled Diabetes Mellitus Type 2, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.