Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2022 Sep 7:9:970254.
doi: 10.3389/fmed.2022.970254. eCollection 2022.

Case report: Congenital arterioportal fistula: An unusual cause of variceal bleeding in adults

Junlin Xia  1 , Jingwen Zhao  2 , Bing Chang  1
Affiliations
Case Reports

Case report: Congenital arterioportal fistula: An unusual cause of variceal bleeding in adults

Junlin Xia et al. Front Med (Lausanne). .

Abstract

The aberrant vascular connecting channel that forms between the portal vein and the hepatic artery is the essence of a hepatic arterioportal fistula. Congenital hepatic arterioportal fistula more frequently occurs in early childhood rather than in adults. We describe a rare instance of a large, isolated, congenital hepatic arterioportal fistula that was successfully treated following selective hepatic arteriography and transcatheter embolization. The patient presented with significant variceal bleeding when the fistula was discovered at the age of 73. The patient's condition improved during the brief postoperative follow-up period without a recurrence. Our research suggests that in older patients with portal hypertension and an unclear etiology, selective arteriography and embolization can provide a definitive diagnosis and successfully treat symptoms.

Keywords: angiography; arteriovenous fistula; ascites; coil embolization; gastrointestinal bleeding; hepatic arterioportal fistula; occlusion; portal hypertension.

PubMed Disclaimer

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
(A) Enhanced CT shows early portal vein visualization in the arterial phase suggesting hepatic arterioportal fistula (black arrow). (B) Selective hepatic artery angiography demonstrated the dilated right hepatic arteries (black arrow). The left hepatic artery was normal. (C) Endoscopy display the tortuous varices with dilatation and positive RC sign in esophagus. (D) Endoscopy display the tortuous varices with dilatation and positive RC sign in gastric fundus.
Figure 2
Figure 2
(A) CT scan of the abdomen: CT shows occlusion of the hepatic arterioportal fistula after embolization (black arrow) and reduction of ascites. (B) Selective hepatic artery angiography showing complete closure of the fistula (black arrow) between the right hepatic artery and the right portal vein branch after coil embolization. (C) Improvement of varicose veins after endoscopic sclerotherapy. (D) Improvement of varicose veins after endoscopic sclerotherapy.
See this image and copyright information in PMC

References

    1. Guzman EA, McCahill LE, Rogers FB. Arterioportal fistulas: introduction of a novel classification with therapeutic implications. J Gastrointest Surg. (2006) 10:543–50. 10.1016/j.gassur.2005.06.022 - DOI - PubMed
    1. Gladkikh M, Parra DA. Successful embolization of a congenital intra-hepatic arterioportal fistula in a neonate with the Mvp microvascular plug system (Mvp-3q). Radiol Case Rep. (2022) 17:991–6. 10.1016/j.radcr.2021.12.065 - DOI - PMC - PubMed
    1. Zhang D-Y, Weng S-Q, Dong L, Shen X-Z, Qu X-D. Portal hypertension induced by congenital hepatic arterioportal fistula: report of four clinical cases and review of the literature. World J Gastroenterol. (2015) 21:2229–35. 10.3748/wjg.v21.i7.2229 - DOI - PMC - PubMed
    1. Norton SP, Jacobson K, Moroz SP, Culham G, Ng V, Turner J, et al. The congenital intrahepatic arterioportal fistula syndrome: elucidation and proposed classification. J Pediatr Gastroenterol Nutr. (2006) 43:248–55. 10.1097/01.mpg.0000221890.13630.ad - DOI - PubMed
    1. Ozyer U, Kirbas I, Aytekin C, Hasdogan B. Coil embolization of a congenital intrahepatic arterioportal fistula: increasing experience in management. Pediatr Radiol. (2008) 38:1253–6. 10.1007/s00247-008-0957-z - DOI - PubMed

Publication types

LinkOut - more resources