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伴有胃静脉曲张出血的冯·希佩尔-林道综合征的不寻常表现

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

作者信息

Graham David W, Thompson Blake, Mantry Parvez

机构信息

Internal Medicine, Methodist Dallas Medical Center, Dallas, USA.

Gastroenterology, Methodist Dallas Medical Center, Dallas, USA.

出版信息

Cureus. 2024 Jul 16;16(7):e64685. doi: 10.7759/cureus.64685. eCollection 2024 Jul.

DOI:10.7759/cureus.64685
PMID:39156424
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11327004/
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.

摘要

胃静脉曲张最常见于门静脉高压或脾静脉血栓形成(SVT)的并发症。由于门静脉高压导致的胃静脉曲张的发生率明显低于食管静脉曲张。SVT是胰腺炎的一种已知并发症,是由于沿胰腺后表面走行的脾静脉受到炎症或压迫所致。脾静脉阻塞导致左侧门静脉高压。左侧门静脉高压导致侧支血管形成,通过与胃短静脉相连绕过脾静脉。胃血管内压力升高导致胃静脉曲张。由SVT引起的胃静脉曲张可能在没有食管静脉曲张的情况下出现,或者与食管静脉曲张不成比例。我们报告了一例有趣的病例,一名被诊断为冯·希佩尔-林道(VHL)综合征的患者,因代谢功能障碍相关脂肪性肝炎继发肝硬化和胰腺神经内分泌肿瘤(NET)继发慢性胰腺炎导致SVT,出现胃静脉曲张引起的胃肠道出血。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a61/11327004/019cc6d4eaf0/cureus-0016-00000064685-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a61/11327004/5e72e19ce317/cureus-0016-00000064685-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a61/11327004/019cc6d4eaf0/cureus-0016-00000064685-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a61/11327004/5e72e19ce317/cureus-0016-00000064685-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a61/11327004/019cc6d4eaf0/cureus-0016-00000064685-i02.jpg

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