Portal Hypertension Secondary to a Spontaneous Splenic Arteriovenous Fistula: Case Report and Review of the Literature

A 66-yr-old woman developed diarrhea, abdominal pain, and ascites which was subsequently discovered to be secondary to portal hypertension resulting from a splenic arteriovenous fistula. Splenectomy resulted in reduction of the portal pressure and resolution of the patient's symptoms. The literature pertaining to splenic arteriovenous fistulas with portal hypertension is discussed.     

Melena-associated regional portal hypertension caused by splenic arteriovenous fistula

Regional portal hypertension is a rare cause of upper gastrointestinal bleeding. We reported an extremely rare case in which regional portal hypertension was associated with both the splenic arteriovenous fistula and chronic pancreatitis. In June 2010, our patient, a 41-year-old man, was admitted to a local hospital due to a sudden melena and dizziness without haematemesis and jaundice. The splenic arteriovenous fistula in this patient was successfully occluded through transcatheter arterial embolization. At the 12-mo follow-up, our patient was in good condition.     

Iatrogenic superior mesenteric arteriovenous fistula: Ultrasonographic, CT and angiographic features and histological findings of the liver biopsy

Iatrogenic superior mesenteric arteriovenous fistula is rare. We treated a patient with this problem 6 years after small bowel resection for intestinal obstruction. The symptoms and signs were those of intestinal ischaemia and portal hypertension with an abdominal bruit. Ultrasonography and enhanced computerized tomography of the abdomen suggested the presence of superior mesenteric arteriovenous fistula, with a dilated portal vein and a communication between the dilated superior mesenteric vein and its artery. The exact location of the fistula was then determined by selective superior mesenteric arteriography. The fistula was ligated in an emergency operation to prevent cardiac or renal failure and to relieve portal hypertension. Liver biopsy showed no cirrhotic changes, but fibrosis was seen around the portal veins. We describe here not only the diagnostic arteriographic findings of superior mesenteric arteriovenous fistula, but also the interesting and suggestive ultrasonographic and computed tomographic findings. Early correction of such fistulas is recommended to prevent cardiac or liver failure.     

Arteriovenous fistula and forward hypertension in the portal circulation

The notorious predilection for rupture of splenic artery aneurysms in women of childbearing age is once more stressed by this case report. Although such ruptures usually result in a catastrophic interabdominal bleeding, in rare instances they lead to the formation of an arteriovenous fistula and consequent portal hypertension. Arterialization of the portal vein results in a progressive development of intrinsic hepatic morphologic changes and hepatoportal sclerosis, which further elevate the pressure in the portal system. This combination of pathophysiologic hemodynamic features closely resembles the original concept of Banti. He postulated that portal hypertension began with pathologic changes within the spleen which were associated with an increased blood flow through this organ. This led to increased flow and pressure in the portal venous system and ultimately cumulated in cirrhosis of the liver. This concept of the pathophysiology of portal hypertension has been universally abandoned, but it could serve as a model of the sequelae of an arteriovenous communication within the portal system.     

Successful arterial embolization of arteriovenous fistula in the portal circulation.

We successfully performed arterial embolization of an arteriovenous fistula between the left gastric artery and vein. The increased blood flow in the portal vein via the left gastric vein and the arteriovenous fistula induced severe portal hypertension. After obliteration of the left gastric artery, the arteriovenous fistula was not opacified on angiography and the portal hypertension improved.     

Inferior mesenteric arteriovenous fistula: Case report and world-literature review

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.     

Splenic arteriovenous fistula and sudden onset of portal hypertension as complications of a ruptured splenic artery aneurysm： Successful treatment with transcatheter arterial embolization. A case study and review of the literature

Splenic arteriovenous fistula (SAVF) accounts for an unusual but well-documented treatable cause of portal hypertension. A case of a 50-year-old multiparous female who developed suddenly portal hypertension due to SAVF formation is presented. The patient suffered from repeated episodes of haematemesis and melaena during the past twelve days and thus was emergently admitted to hospital for management. Clinical and laboratory investigations established the diagnosis of portal hypertension in the absence of liver parenchymal disease. Endoscopy revealed multiple esophageal bleeding varices. Abdominal computed tomography (CT) and transfemoral celiac arteriography documented the presence of a tortuous and aneurysmatic splenic artery and premature filling of an enlarged splenic vein, findings highly suggestive of an SAVF. The aforementioned vascular abnormality was successfully treated with percutaneous transcatheter embolization. Neither recurrence nor other complications were observed.     

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

The authors report a case of postoperative arteriovenous fistula between the inferior mesenteric vessels. This fistula was revealed by portal hypertension, with bleeding esophageal varices, ascites, and encephalopathy, and by acute ischemic colitis. Histologic examination of the liver was normal. All of the symptoms disappeared after transcatheter embolization of the fistula with stainless steel coils. This case report favors the reality of the so-called "forward" portal hypertension and suggests that inferior mesenteric arteriovenous fistula might be a factor predisposing to nonocclusive ischemic colitis.     

CURATIVE TREATMENT OF BLEEDING ESOPHAGEAL VARICES SECONDARY TO A SPLENIC ARTERIO-VENOUS FISTULA

A 59 year old female, who presented with abdominal pain, diarrhea, and ascites, developed major bleeding from esophageal varices. Celiac angiography demonstrated a splenic arterio-venous fistula with early filling of an enlarged splenic vein and esophageal varices (pre-sinusoidal extra hepatic portal hypertension). The patient underwent splenectomy and resection of the fistula with resultant disappearance of the varices and presenting symptoms.     

Portal hypertension induced by congenital hepatic arterioportal fistula:Report of four clinical cases and review of the literature

Intrahepatic arterioportal fistula(IAPF)can be caused by many secondary factors.We report four cases of portal hypertension that were eventually determined to be caused by congenital hepatic arterioportal fistula.The clinical manifestations included ascites,variceal hemorrhage and hepatic encephalopathy.Computed tomography scans from all of the patients revealed the early enhancement of the portal branches in the hepatic arterial phase.All patients were diagnosed using digital subtraction angiography(DSA).DSA before embolization revealed an arteriovenous fistula with immediate filling of the portal venous radicles.All four patients were treated with interventional embolization.The four patients remained in good condition throughout follow-up and at the time of publication.IAPF is frequently misdiagnosed due to its rarity;therefore,clinicians should consider IAPF as a potential cause of non-cirrhotic portal hypertension.     

Pulmonary arteriovenous fistula with bilharzial pulmonary hypertension

Congenital pulmonary arteriovenous fistulae, provided that they are not present in very large numbers, are best managed surgically, and there is no effective alternative management. Where pulmonary arteriovenous fistulae develop as a consequence of pulmonary hypertension it is rational to resect the fistulae if the cause of pulmonary hypertension - for example, mitral stenosis - is correctable. Pulmonary arteriovenous fistula in the presence of unexplained or uncorrectable pulmonary hypertension may be the safety valve on which life depends and should, therefore, not be resected. An example is reported of pulmonary arteriovenous fistula associated with bilharzial pulmonary hypertension in which resection of the fistula resulted in death.     

Pulmonary arteriovenous fistula with bilharzial pulmonary hypertension

Congenital pulmonary arteriovenous fistulae, provided that they are not present in very large numbers, are best managed surgically, and there is no effective alternative management. Where pulmonary arteriovenous fistulae develop as a consequence of pulmonary hypertension it is rational to resect the fistulae if the cause of pulmonary hypertension - for example, mitral stenosis - is correctable. Pulmonary arteriovenous fistula in the presence of unexplained or uncorrectable pulmonary hypertension may be the safety valve on which life depends and should, therefore, not be resected. An example is reported of pulmonary arteriovenous fistula associated with bilharzial pulmonary hypertension in which resection of the fistula resulted in death.     

Unusual case of digestive hemorrhage: Celiac axis-portal vein arteriovenous fistula

A case of intractable upper gastrointestinal-hemorrhage was reported in a patient with portal hypertension caused by an arterioportal fistula(APF), namely, celiac axis-portal vein arteriovenous fistula. Portal hypertension caused by extrahepatic-APFs is extremely rare. Trauma, malignancy, and hereditary causes are the common etiology of APFs; but were absent in our patient. Our patient represents an unusualcase of unexplained APF who presented with portal hypertension and was successfully managed through endovascular aortic repair.     

Arterioportal Fistula: a Rare Cause of Portal Hypertension and Abdominal Pain

Esophageal varices are commonly caused by portal hypertension secondary to cirrhosis. We report the case of a 71-year-old woman who presented with esophageal variceal bleeding due to portal hypertension caused by an arteriovenous fistula. The fistula, which was probably brought about by a liver biopsy performed 18 years previously, was complicated by bleeding. Since this event, the patient has reported right upper quadrant pain. Embolization resulted in elimination of the varices as well as abdominal discomfort.     

Arterioportal fistula: a rare cause of portal hypertension and abdominal pain.

Esophageal varices are commonly caused by portal hypertension secondary to cirrhosis. We report the case of a 71-year-old woman who presented with esophageal variceal bleeding due to portal hypertension caused by an arteriovenous fistula. The fistula, which was probably brought about by a liver biopsy performed 18 years previously, was complicated by bleeding. Since this event, the patient has reported right upper quadrant pain. Embolization resulted in elimination of the varices as well as abdominal discomfort.     

Splenic arteriovenous fistula--late complications of splenectomy

This is a case report of a 30-year-old female patient with a splenic arteriovenous fistula of rare, atypical clinical course. The patient was admitted to the hospital due to strong abdominal pains and fever. 13 years earlier, the patient underwent splenectomy due to post-traumatic rupture of the spleen. On imaging examinations prior to surgery (ultrasonography, CT), a splenic arteriovenous fistula was diagnosed. The patient was operated on due to increasing abdominal pain. The fistula was closed by splenic artery and splenic vein banding during the course of laparotomy. This case report will be discussed based on literature review.     

Frequency of intrahepatic arteriovenous fistula as a sequela to percutaneous needle puncture of the liver

Incidence of intrahepatic arteriovenous fistula after needle biopsy of the liver, percutaneous transhepatic cholangiography, and transhepatic catheterization of an intrahepatic bile duct or portal vein, was determined by studying the hepatic arteriograms obtained within 1 month of the transhepatic procedure. An arteriovenous fistula, mostly arterioportal, occurred in 5 (5.4%) of the 93 patients after biopsy, in 3 (3.8%) of 79 after percutaneous transhepatic cholangiography, and in 7 (26.2%) of 26 after catheterization. None of the fistulae was so large and close to the porta hepatis as to cause portal hypertension. In 1 patient with an intrahepatic bile duct carcinoma, the long track of a 0.7-mm caliber needle became a fistula draining arterial blood into the parenchyma. In another, an aneurysm was found after biopsy and percutaneous transhepatic cholangiography. The possibility of spontaneous closure of small fistulae is discussed.     

Endoscopic therapy in patients with Barrett's esophagus and portal hypertension

BACKGROUND: Endoscopic mucosal resection has been used to stage and treat early neoplasia in Barrett's esophagus. The ability to do this in the setting of portal hypertension has not been reported. OBJECTIVE: Our purpose was to describe the feasibility and efficacy of endoscopic mucosal resection in patients with portal hypertension and Barrett's esophagus. DESIGN: Retrospective case series. SETTING: Two tertiary referral centers. PATIENTS: Patients with Barrett's esophagus and high-grade dysplasia or adenocarcinoma in the setting of portal hypertension. INTERVENTION: Endoscopic mucosal resection of endoscopically visible lesions. MAIN OUTCOME MEASUREMENTS: Complete resection of neoplastic lesion, lack of variceal bleeding. RESULTS: Four patients were treated with endoscopic mucosal resection a total of 5 times. Endoscopic mucosal resection was successfully performed without significant GI bleeding. LIMITATIONS: This preliminary case series describes feasibility of the procedure. Whether this can be generalized remains to be determined, although it may be an option in poor surgical candidates. CONCLUSIONS: Endoscopic mucosal resection appears to be relatively safe in selected patients with portal hypertension and Barrett's esophagus. Further studies are needed to confirm these findings.     

Aneurysmal dilatation of the portal vein: a rare cause of portal hypertension

Aneurysmal dilatation of the portal vein (ADPV) is a rare cause of portal hypertension. We described a case of ADPV in a female patient who presented with ascites. Imaging studies revealed tortuosity and dilatation of the main portal vein with turbulent flow. Endoscopy revealed oesophageal varices. A liver biopsy showed no abnormalities in liver histology. This is the first case of ascites as a complication of ADPV in the absence of liver cirrhosis, arteriovenous fistula or documented portal vein thrombosis. Hyperdynamic circulation and increased portal vein flow could be implicated in the pathogenesis of ascites in this setting.     

Aneurysmal dilatation of the portal vein: A rare cause of portal hypertension

Aneurysmal dilatation of the portal vein (ADPV) is a rare cause of portal hypertension. We described a case of ADPV in a female patient who presented with ascites. Imaging studies revealed tortuosity and dilatation of the main portal vein with turbulent flow. Endoscopy revealed oesophageal varices. A liver biospy showed no abnormalities in liver histology. This is the first case of ascites as a complication of ADPV in the absence of liver cirrhosis, arteriovenous fistula or documented portal vein thrombosis. Hyperdynamic circulation and increased portal vein flow could be implicated in the pathogenesis of ascites in this setting.