# Unusual Presentation of Von Hippel-Lindau Syndrome With Gastric Variceal Bleeding

## TL;DR

A rare case of gastric variceal bleeding in a patient with VHL syndrome is linked to chronic pancreatitis and liver disease.

## Contribution

This case highlights an unusual presentation of VHL syndrome involving gastric varices due to multiple underlying conditions.

## Key findings

- Gastric varices were caused by splenic vein thrombosis from chronic pancreatitis due to a pancreatic NET.
- The patient had cirrhosis from metabolic dysfunction-associated steatohepatitis.
- The case demonstrates a complex interplay of VHL syndrome and gastrointestinal complications.

## Abstract

Gastric varices are most commonly a complication of portal hypertension or splenic vein thrombosis (SVT). The presence of gastric varices due to portal hypertension is significantly less than the prevalence of esophageal varices. SVT is a known complication of pancreatitis due to inflammation or compression of the splenic vein coursing along the posterior surface of the pancreas. Occlusion of the splenic vein leads to left-sided portal hypertension. Left-sided portal hypertension results in the development of collateral vessels that bypass the splenic vein by connecting with the short gastric veins. The associated increased pressure within the gastric vessels results in gastric varices. Gastric varices due to SVT may occur in the absence of or be disproportionate to esophageal varices. We report an interesting case of gastrointestinal bleeding from gastric varices related to cirrhosis secondary to metabolic dysfunction-associated steatohepatitis and SVT secondary to chronic pancreatitis due to pancreatic neuroendocrine tumor (NET) in a patient diagnosed with von Hippel-Lindau (VHL) syndrome.