A rare case of splenic arteriovenous fistula causing portal hypertension, treated by embolisation.

In the United States, most cases of portal hypertension result from intrinsic liver disease. However, when no liver disease is obvious, rarer etiologies must be carefully evaluated. Here we discuss the case of a splenic arteriovenous fistula (AVF) that led to severe portal hypertensive bleeding and gastrointestinal ischemia. A 34 y/o black female with no significant past medical history presented to our institution with 10 days of watery diarrhea and diffuse post-prandial abdominal pain. 5 days prior, she went to an outside hospital with these same complaints, where she was given a diagnosis of gastroenteritis and sent home with PPI. In our ER she underwent CT scan, which showed diffuse bowel wall thickening and significant portal hypertension (esophageal, gastric, and rectal varices and enlarged splenic and portal veins). She had no history of or significant risk factors for liver disease. While awaiting admission, she had hematemesis and hematochezia with a change in her hemodynamic status. Emergent endoscopy revealed Grade III esophageal varices, ischemic-appearing stomach and duodenum, and a large fundal pool of blood with pulsatile arterial spurting. After control of bleeding with epinephrine injection and evacuation of the fundal pool, large gastric varices were noted, but the bleeding had subsided. Given these findings, balloon tamponade of the gastric varices was achieved with a Minnesota tube. The CT and subsequent liver duplex failed to reveal evidence of portal venous ouflow obstruction. Urgent angiography was peformed to further evaluate the “idiopathic” portal hypertension. This revealed a large splenic AVF, treated with the placement of multiple coils in the mid-splenic artery. After the procedure she had no further bleeding, pain, or diarrhea and was tolerating a full diet. Repeat CT showed improvement in the bowel wall thickening. She was discharged home with surgical follow-up for consideration of splenectomy. Splenic AVF is a rare cause of forward portal hypertension in association with mesenteric ischemia. Patients can present with complications of portal hypertension, as seen in our patient. The diagnosis may be difficult to make without arteriogram. On CT scan early filling of the portal vein on the arterial phase can give a clue to this diagnosis and was seen in retrospect in our patient. The diagnosis of splenic AVF should be considered in the differential diagnosis of portal hypertension and mesenteric ischemia, especially in the absence of liver disease.

This article describes a rare case of a traumatic splenic arteriovenous fistula (AVF) causing portal hypertension in a patient presenting with abdominal pain, diarrhea, and melena. A 78-year-old was admitted to the hospital with abdominal pain. The patient's history was notable for prior laparotomy and left nephrectomy for a gunshot wound. Workup demonstrated portal hypertension with a dilated splenic vein with aneurysmal changes and a saccular celiac artery aneurysm. Celiac angiogram demonstrated a communication between celiac and portal circulation. The patient underwent laparotomy with ligation of the splenic artery and resection of the celiac artery aneurysm. In conclusion, splenic AVFs are relatively rare in clinical practice. Once the diagnosis is established, operative intervention is required to avoid complications of portal hypertension. Surgical ligation has been used in this case with a successful outcome.

A case of splenic arteriovenous fistula leading to portal hypertension and ascites is presented. The recognition of this vascular lesion is important. When portal hypertension is secondary to a splenic arteriovenous fistula, the complications of portal hypertension can be avoided by the resection of the lesion. The diagnosis can be suspected by clinical findings and confirmed by angiographic delineation of the fistula. Surgical resection of splenic arteriovenous fistulae must include the fistula and all dilated venous tributaries to prevent thrombosis and recurrent portal hypertension.

Incidental esophageal varices

Splenic Arteriovenous Fistula Treated with Percutaneous Transarterial Embolization

Fístula Arterio-Portal Congênita Em Criança - Relato De Caso

Purpose: Portal hypertension is a common condition encountered by gastroenterologists. While this is most commonly encountered in the setting of cirrhosis, other conditions affecting portal venous blood flow dynamics can present with findings consistent with portal hypertension. We present a rare case of portal hypertension caused by a splenic arteriovenous (SAV) fistula. A 69-year-old female presented with hematemesis after a prolonged bout of nausea and vomiting. Endoscopic evaluation showed large esophagogastric varices which were amenable to esophageal banding. There was no evidence for alcohol abuse, hepatotoxin exposure, or risk factors for viral hepatitis. Serologic studies for viral hepatitis, antinuclear antibody, antismooth muscle antibody, antimitochondrial antibodies, and iron studies were normal. Initial radiographic imaging did not suggest cirrhosis and a percutaneous liver biopsy was normal. She was referred to our center for further management. Following initial esophageal banding, she did not rebleed. Our review of the previous CT images suggested a possible splenic arteriovenous (SAV) fistula. CT angiography showed contrast in the splenic and portal veins during the arterial phase and aneurysmal splenic vein enlargement confirming the diagnosis of a SAV fistula. Transcatheter arterial embolization was done with only a small branch of the splenic artery still feeding the dilated splenic vein postembolization. Upon further questioning, she denied any major abdominal trauma and had two normal pregnancies. She did not experience any further bleeding and was discharged. SAV fistula is a rare cause of portal hypertension and should be suspected in female patients who present with variceal bleeding, but have no signs of chronic liver disease. In a SAV fistula, portal hypertension is due to enhanced arteriovenous flow as blood bypasses the capillary bed of the spleen. The hemodynamic changes that ensue lead to dramatic and rapid increases in portal venous pressures and subsequent clinical sequellae. The etiology of an acquired SAV fistula may be spontaneous, iatrogenic, or traumatic. A significant female predominance of spontaneous cases and relatively young presentations that are temporally associated with pregnancy have suggested that hormonal factors (perhaps associated with pregnancy) lead to arterial wall elastin weakening. Gastrointestinal hemorrhage has been the most common presentation in the literature. Our patient demonstrates that an SAV fistula should be considered as a cause of noncirrhotic portal hypertension in females even well beyond the child-bearing years.

Summary Splenic arteriovenous fistula (SAVF) is a rare but potentially curable condition. Only a few cases have been reported in the English literature. SAVF can cause portal hypertension, ascites, gastrointestinal bleeding, and heart failure. An early diagnosis is essential to avoid life threatening complications. We hereby present a case of SAVF in a young female patient, with hepatitis C liver cirrhosis who presented with recurrent severe upper gastrointestinal bleeding. Such an association of liver cirrhosis and SAVF has not been previously reported.

Splenic arteriovenous fistulas (SAVF) are a rare cause of portal hypertension (PHTN). They usually occur secondary to abdominal trauma, aneurysmal erosion, or as a complication of abdominal surgery [1]. Their clinical presentation depends on the size, location, and acuity. Indications for treatment include: hemorrhage, ascites, and worsening PHTN [2]. There are less than 50 published cases of PHTN due to SAVF based on our literature review. A 61-year-old female without prior liver disease, abdominal trauma, or surgery, complained of a 3-month history of ascites and intermittent melena. Hemoglobin was 8 g/dL. Platelet count, transaminase levels, bilirubin, albumin and INR were normal. Anti-smooth muscle, anti-mitochondrial, and anti-nuclear antibodies were all negative. The serum-ascites-albumin-gradient was 2.5 g/dL and an ascitic fluid total protein was 1.2 g/dL. Large esophageal varices were found on endoscopy and were banded prophylactically. Abdominal ultrasound with Doppler showed ascites, splenomegaly, and a large SAVF at the splenic hilum. Abdominal CT scan followed by angiography confirmed the same SAVF (Fig. ​(Fig.1A1A & B). Figure 1 (A) CT abdomen and (B) angiogram showing splenic arteriovenous malformation A two-step approach involving endovascular coil embolization (Fig. 2) followed by surgical splenectomy was performed. The resected spleen is shown with a metal pointer traversing the fistula (Fig. 3). The proximity of the arteriovenous (AV) fistula to the spleen may result in splenic infarction during coil embolization. Upfront excision of the AV fistula may result in massive hemorrhage. In order to prevent these complications, we proceeded with a two-step approach with coil embolization followed by splenectomy. The patient tolerated the surgery well and remained asymptomatic. Figure 2 Endovascular coil embolization of the arteriovenous fistula Figure 3 Gross surgical specimen of the spleen with arteriovenous fistulous tract (metal pointer)

Surgery of the liver and biliary tract: L.H. Blumgart, Y. Fong (Eds.), W.B. Saunders Company Ltd., London, Edinburgh, 2001, 2180 pages, £295. ISBM 0-7020-21011

Inferior mesenteric arteriovenous fistula associated with portal hypertension and acute ischemic colitis: Successful occlusion by intraarterial embolization with steel coils

Portal hypertension (PoH) after liver transplantation is a severe complication that results in graft loss. We investigated the characteristics and evaluated the treatment outcomes of PoH after living donor liver transplantation (LDLT). This single-center, retrospective cohort study included 325 LDLT recipients. Of the subjects, 37 (11.4%) had a PoH. The 10- and 20-year graft survival rates were significantly lower in patients with PoH than in those without PoH (69.1% vs. 90.8% and 42.1% vs. 84.7%, respectively; p < 0.0001). The types of PoH were pre-hepatic, hepatic, and post-hepatic in 16, 13, and 8 patients, respectively. Interventional radiology was performed for PoH in all post-hepatic PoH patients and in 62.5% of pre-hepatic PoH patients. Notably, 46.2% of the patients with hepatic PoH required re-transplantation. The 10-year graft survival rate was significantly worse in patients with hepatic PoH than in those with pre- and post-hepatic PoH (46.2% vs. 86.7% and 75.0%, respectively; P < 0.05). Post-transplant PoH was an independent predictor of graft loss after LDLT (hazard ratio, 5.73; 95% confidence interval: 2.43-13.55, P < 0.0005). Post-transplant PoH negatively affected the graft survival in LDLT recipients. Pre-hepatic, hepatic, and post-hepatic PoH cases had different characteristics, requiring different treatments. Therefore, an appropriate diagnosis and treatment are important.

Arteriovenous fistulas between the inferior mesenteric artery and vein are rare, with only 26 primary and secondary cases described in the literature. Secondary fistulas occur following operations of the left hemicolon and manifest as abdominal pain, abdominal mass, gastrointestinal bleeding, colonic ischemia and portal hypertension. Symptom intensities are flow-dependent, and can range from minimal symptoms to severe heart failure due to left to right shunt. Diagnosis is usually established by radiological or intraoperative examination. Treatment options include embolization and/or surgical resection. Therapeutic decisions should be adapted to the unique characteristics of the fistula on an individual basis. A new case of a primary arteriovenous fistula is described and discussed along with a complete review of the literature. The patient in this report presented with signs and symptoms of colonic ischemia without portal hypertension. The optimal treatment for this patient required a combination of embolization and surgical operation. The characteristics of these rare inferior mesenteric arteriovenous fistulas are examined and some considerations concerning diagnostic and therapeutic strategies that should be followed are presented.

Three Lumens Full of Blood—An Unusual Case of Gastrointestinal Bleeding

Introduction: Noncirrhotic portal hypertension constitutes an increase in pressure of the portal venous system in the absence of cirrhosis. This is a rare condition; however, it is insufficiently acknowledged given its decreased prevalence in North America and Europe compared to other nations. We present a rare case of noncirrhotic portal hypertension and pancreatico-gastric fistula complicating acute pancreatitis with an acute fluid collection and resultant portal vein narrowing. Case Description/Methods: A 55-year-old man with history of alcohol use disorder presented to the emergency department with worsening abdominal pain 2 weeks after discharge for uncomplicated acute pancreatitis. Lipase was 48 IU/L (normal 11-82 IU/L). CT abdomen showed increasing peripancreatic fluid collection with marked narrowing of the main portal vein, which appeared near threadlike, and narrowing of the bifurcation of the portal vein extending into the left portal vein. The splenic vein was also severely narrowed (Figure A). He was discharged home with outpatient follow-up, however, returned to the ED for intractable pain one month later. A CT abdomen showed florid changes of pancreatitis with pseudocyst formation with new foci of air within the collection concerning for fistulous tract formation with the gastric lumen (Figure B). The portal vein and splenic vein narrowing improved, but the narrowing of the right and left portal veins worsened. He underwent upper endoscopy which showed a small area of White "milky" base in the posterior wall of the incisura angularis which was suggestive of fistulous opening in the area (Figure C). Four columns of small distal esophageal varices were also noted. During admission, paracentesis was performed for ascites. Serum ascites albumin gradient was over 1.1 consistent with ascites due to portal hypertension – likely due to the portal vein narrowing. Patient was discharged in stable, improved condition. Discussion: Although portal hypertension normally presents in cirrhotic patients, it may rarely present in the absence of cirrhosis by conditions such as portal vein thrombosis or portosinusoidal vascular disease. Compression of the portal vein from an acute pancreatic fluid collection is an extremely rare cause of noncirrhotic portal hypertension. Clinicians should have a high index of suspicion for complications related to portal hypertension such as ascites or varices in patients with a prolonged course of acute pancreatitis without cirrhosis.Figure 1.: (A) Contrast CT abdomen showing marked narrowing of the main portal vein, which appears near threadlike. (B) One of 2 foci of air in the collection at the anterior superior aspect of the pancreatic body. Appears inseparable from the posterior wall of the gastric body, concerning for fistulous involvement. (C) Area of White “milky” base in the posterior wall of the incisura angularis. This area was rinsed, however re-accumulated despite no obvious opening seen.