Felix Zhou, MD, Christopher Lightfoot, MD, Julie Zhu, MD Dalhousie University, Halifax, NS, Canada
Introduction: Splenic arteriovenous (AV) fistulas are exceedingly rare. To our knowledge, this is the first occurrence of splenic AV fistulas leading to development of non-cirrhotic portal hypertension and gastric varices.
Case Description/Methods: A 33-year-old male with no past medical history presented with a few months’ history of fatigue and upper abdominal discomfort, with no history of overt gastrointestinal bleeding. Laboratory tests revealed a low hemoglobin level of 74 g/L and ferritin of 3.4 ug/L with normal liver function tests. An esophagogastroduodenoscopy (EGD) revealed a cluster of isolated gastric fundal varices with stigmata of adherent clots, which was confirmed on endoscopic ultrasound. A FibroScan measured liver stiffness at 4.2 kilopascals indicating no fibrosis and chronic liver disease workup for viral hepatitis, Wilson’s, autoimmune hepatitis, and alpha-1-antitrypsin deficiency were negative. The patient denied any history of significant alcohol use.
Computerized tomography (CT) of his abdomen and pelvis revealed a dilated portal and splenic vein suggestive of portal hypertension, with no thrombosis. There was hypoattenuation of the spleen posteriorly, concerning for infarct. There was a fistula between the splenic artery and vein, and a large saccular splenic artery aneurysm (SAA) measuring approximately 2.3 x 3.2 x 3.2 cm. There was no thrombus within the portal system vasculature. The patient underwent laparoscopic splenectomy and resection of the SAA. A repeat EGD 8 months following the surgery showed complete resolution of the gastric varices.
Discussion: Splenic AV fistulas are exceedingly rare. The majority occur after rupture of an SAA. Splenic AV fistulas can also be traumatic, congenital, or infectious in origin. In our case, increased blood flow through the splenic AV fistula likely led to the development of the SAA as well as the patient’s non-cirrhotic portal hypertension and gastric varices. While the development of SAA due to splenic AV fistula has been observed in two previous case reports, our case report is the first documented occurrence of gastric varices secondary to splenic AV fistula.
Given the rarity of splenic AV fistulas and SAA, there are no published guidelines on the management of these clinical entities. Different approaches include surgical/laparoscopic resection, ligation, as well as endovascular techniques. The optimal approach will depend on various factors including patient comorbidities, size of the SAA, and location of the AV fistulas.
Figure: A) Computerized tomography revealing a splenic arteriovenous fistula (yellow arrow) with early enhancement of the splenic vein from the shunt (blue arrow) and a large saccular splenic artery aneurysm (red arrow). B) Computerized tomography revealing a splenic arteriovenous fistula C) Reconstructed image showing a diffusely enlarged splenic artery with perihilar aneurysm along with diffusely aneurysmal splenic vein outflow beyond fistulas.
Disclosures: Felix Zhou indicated no relevant financial relationships. Christopher Lightfoot indicated no relevant financial relationships. Julie Zhu indicated no relevant financial relationships.
Felix Zhou, MD, Christopher Lightfoot, MD, Julie Zhu, MD. P1568 - An Extraordinary Cause of Non-Cirrhotic Portal Hypertension and Gastric Varices - Splenic Arteriovenous Fistula, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.