Natural history of spontaneous pancreatic portal vein fistulae: A systematic review of the literature.

BACKGROUND: Spontaneous pancreatic portal vein fistula (PPVF) - a rare complication of pancreatic inflammation - varies widely in presentation and means of diagnosis but has been previously associated with bleeding complications and mortality. A systematic review of published literature was performed to assess the frequency of outcomes. METHODS: A search of electronic databases (PubMed, Ovid MEDLINE, Scopus, EMBASE, gray literature) resulted in 1667 relevant unique manuscripts; 52 met inclusion criteria. RESULTS: A total of 74 unique (male n = 47, 63.5 %) patients were included. Mean age was 53.5 (±11.9) years. History of alcohol use was reported in 55 (74.3 %). Underlying chronic pancreatitis (CP) was present in 49 (66.2 %). In cases where presenting symptoms were reported (n = 57, 77.4 %), the most frequent were abdominal pain (63.5 %), weight loss (14.9 %), rash (12.2 %), nausea/vomiting (12.2 %), and polyarthritis (9.5 %). Computed tomography was the most common imaging modality used to confirm the diagnosis (n = 20, 27.0 %), followed by magnetic resonance cholangiopancreatography (n = 14, 18.9 %). Portal vein thrombosis was reported in 57 (77.0 %), and bleeding events (luminal, variceal, or intra-pseudocyst) were reported in 13(17.6 %) patients. Younger age was associated with higher risk of bleeding events. Mortality was reported in 12 (16.2 %) patients at any time during follow up. Older age and polyarthritis at presentation were associated with mortality. CONCLUSIONS: PPVF is a rare and potentially fatal condition, though rates of bleeding complication and death were relatively low in this population. High-quality observational studies are needed to better understand the pathophysiology and natural history of this diagnosis.

Diagnosis and Management of Congenital Coronary Artery Fistulas in Infants and Children.

PURPOSE OF REVIEW: Coronary artery fistulas (CAFs) are rare coronary anomalies that most often occur as congenital malformations in children. Although most children with CAFs are asymptomatic at the time of diagnosis, some present with symptoms of congestive heart failure in the setting of large left-to-right shunts. Others may develop additional complications including coronary artery ectasia and coronary thrombosis. Surgical and transcatheter closure techniques have been previously described. This review presents the classifications of CAFs in children and the short and long-term outcomes of CAF closure in children in the reported literature. We also summarize previously-reported angiographic findings and post-treatment remodeling characteristics in pediatric patients. RECENT FINDINGS: With advancements in cross-sectional imaging technologies, anatomic delineation of CAFs via these modalities has become crucial in procedural planning. Recent reports of surgical and transcatheter closure of CAFs in children have reported good procedural success and low rates of short-term morbidity and mortality. Distal-type CAFs have elevated risk for long-term sequelae post-closure compared to proximal-type CAFs. A recent report of a multi-institutional cohort also describes post-closure remodeling classifications which may predict long-term outcomes in these patients as well as guide individualized anticoagulation management. Invasive closure of significant CAFs via surgical or transcatheter techniques is feasible and safe in most children with good short and intermediate-term outcomes. However, close clinical and imaging follow-up is required to monitor for late complications even after successful closure. Antiplatelet and anticoagulation regimens remain important aspects of post-closure management, but the necessary intensity and duration of such therapy remains unknown.

Aortoesophageal fistula as a fatal complication of esophageal perforation.

Aortoesophageal fistula is a very rare cause of upper gastrointestinal bleeding, however its interest lies in the high mortality rate associated with it. Due to this, early diagnosis and treatment of this entity is essential to increase survival. The typical symptoms known as the Chiari´s triad are only present in 45% of reported cases. We present the case of a patient with upper gastrointestinal bleeding due to an aortoesophageal fistula as well as the importance of endoscopic use for its differential diagnosis.

Endoscopic diagnosis of primary aortoesophageal fistula: An extremely rare cause of acute upper gastrointestinal bleeding.

Primary aortoesophageal fistula, an abnormal communication between native aorta and oesophagus, is an extremely rare cause of upper gastrointestinal bleeding. The typical symptoms, known as the Chiari´s triad, are only present in 45% of cases. It has a high mortality, so early diagnosis is essential to increase the probability of survival. We present a case report of a patient with massive upper gastrointestinal bleeding due to primary aortoesophageal fistula.

[Left Main Coronary Artery-to-left Atrium Fistula with Aneurysmal Dilatation:Report of a Case].

Coronary artery fistula (CAF) is a relatively rare disease, many of which are asymptomatic and are not indicated for surgery. However, CAF's patients with angina, heart failure due to shunt, and fistula hemangiomas are usually indicated for surgical treatment. A 49-year-old woman with severe congestive heart failure was found to have a fistula from the main trunk of the left coronary artery to the left atrium, and a fistula aneurysm. This patient had heart failure due to shunt blood flow, and also had a fistula aneurysm, so surgical treatment was indicated. During surgery, the fistula aneurysm was incised and the origin and opening of the fistula were closed without any problems. The postoperative course is good, and as of three years after the operation, the patient is being followed up at an outpatient clinic without recurrence of shunt flow and heart failure.

Spontaneous spinal cerebrospinal fluid-venous fistulas in patients with orthostatic headaches and normal conventional brain and spine imaging.

OBJECTIVE: To determine the occurrence of cerebrospinal fluid (CSF)-venous fistulas, a type of spinal CSF leak that cannot be detected with routine computerized tomography myelography, among patients with orthostatic headaches but normal brain and spine magnetic resonance imaging. BACKGROUND: Spontaneous spinal CSF leaks cause orthostatic headaches but their detection may require sophisticated spinal imaging techniques. METHODS: A prospective cohort study of patients with orthostatic headaches and normal brain and conventional spine imaging who underwent digital subtraction myelography (DSM) to look for CSF-venous fistulas, between May 2018 and May 2020, at a quaternary referral center for spontaneous intracranial hypotension. RESULTS: The mean age of the 60 consecutive patients (46 women and 14 men) was 46 years (range, 13-83 years), who had been suffering from orthostatic headaches between 1 and 180 months (mean, 43 months). DSM demonstrated a spinal CSF-venous fistula in 6 (10.0%; 95% confidence interval [CI]: 3.8-20.5%) of the 60 patients. The mean age of these five women and one man was 50 years (range, 41-59 years). Spinal CSF-venous fistulas were identified in 6 (19.4%; 95% CI: 7.5-37.5%) of 31 patients with spinal meningeal diverticula but in none (0%; 95% CI: 0-11.9%) of the 29 patients without spinal meningeal diverticula (p = 0.024). All CSF-venous fistulas were located in the thoracic spine. All patients underwent uneventful surgical ligation of the fistula. Complete and sustained resolution of symptoms was obtained in five patients, while in one patient, partial recurrence of symptoms was noted 3 months postoperatively. CONCLUSION: Concerns about a spinal CSF leak should not be dismissed in patients suffering from orthostatic headaches when conventional imaging turns out to be normal, even though the yield of identifying a CSF-venous fistula is low.

Left circumflex artery pericardia fistula combined with huge pseudoaneurysm: a rare case report.

BACKGROUND: Coronary artery fistula refers to an abnormal communication between a coronary artery and great vessel, a cardiac chamber or other structure. The left circumflex artery (LCX) pericardia fistula combined with huge pseudoaneurysm is extremely rare. CASE PRESENTATION: A 39-year-old young female was admitted into our hospital because of palpitation and shortness of breath. Coronary computed tomography angiography (CCTA) showed a huge pseudoaneurysm located in pericardium. Coronary angiography revealed the LCX pericardia fistula. Then surgical treatment was performed. She was in good condition without complications after surgery. CONCLUSIONS: Coronary artery fistula combined with pseudoaneurysm can be caused by congenital factors. Early surgical treatment can relieve the patient's symptoms and prevent the occurrence of adverse cardiovascular events.

Ureteral Arterial Fistula - A Role for Open Operation in the 21st Century.

BACKGROUND: Ureteral arterial fistula is a rare and challenging clinical entity. The diagnosis and management of ureteral arterial fistula present a unique disease process that requires a dual specialty approach, involving both vascular and urologic surgeons. There are different options for repair, including both open and endovascular techniques. METHODS AND RESULTS: A 78-year-old male presented to the emergency department (ED) in septic shock secondary to a urinary tract infection and was admitted to the intensive care unit (ICU) for further management and resuscitation. The patient had previously undergone radical cystoprostatectomy with ileal conduit creation in 2011 for recurrent bladder cancer. Following creation of his ileal conduit, he required long-term indwelling ureteral stents bilaterally due to recurrent stricturing at the ureteroneocystostomy with stent exchanges performed 2-3 times per year due to frequent urinary tract infections. During his hospitalization for sepsis, the urology service performed an exchange of his left indwelling ureteral stent. However, pulsatile bleeding was observed from the junction of the ileal conduit and left ureter. The stent was replaced at the bedside, and the bleeding ceased. Vascular surgery consultation and a computed tomography angiogram (CTA) gave support to the diagnosis of a ureteral arterial fistula. A plan was developed to exchange the stent in the operating room with vascular surgery assistance. It was determined that a definitive open repair with excision of the fistula would be the most appropriate course. CONCLUSIONS: In the setting of hemodynamically significant bleeding, we recommend an endovascular approach to obtain hemostasis. However, an open approach provides both reconstruction and infectious resistance in an already soiled field. Open repair may provide a more definitive reconstruction.

Extraabdominal Rupture of an Inflammatory Abdominal Aortic Aneurysm in a Patient With Aortodecubital Fistula: A Case Report.

Aortodecubital fistula is a pathologic communication between aorta and a decubitus ulcer. It is very rarely encountered vascular condition in abdominal aortic aneurysms (AAA), with difficult diagnostics and high mortality. Patients often present with systemic and local infection and are at risk for hemorrhage. We present a paraplegic patient with fistulous communication between an inflamed abdominal aortic aneurysm and a sacral decubitus ulcer, leading to intermittent bleeding episodes and finally to exsanguination. While extremely rare, this case emphasizes the need for early, accurate diagnosis and salvage intervention when possible.

Primary Aorto-Duodenal Fistula in Association with Chronic Ischemic Enteritis: A Case Report.

BACKGROUND: Primary aorto-duodenal fistula (PADF) is a rare but life-threatening condition that should be taken into account when considering upper gastrointestinal bleeding in elderly patients with history of abdominal aortic aneurysm. Unfortunately, its diagnosis is often unsuspected until surgery or at postmortem. PRESENTATION OF CASE: We report a case of a 69 years old man with massive gastrointestinal bleeding secondary to a primary aortic duodenal fistula without a history of abdominal aortic aneurysm and with a misleading diagnosis of chronic ischemic enteritis. Repeated endoscopies and a prior CT angiography failed to document a proper diagnosis. Finally, the aorto-duodenal fistula was identified with a further abdominal CT angiography. Despite a prompt endovascular treatment with aortic endoprosthesis placement, the patient died due to a severe hemorrhagic shock consequent to the massive blood loss. DISCUSSION: Primary aorto-duodenal fistula represents a very rare (<0.1% of incidence) cause of severe upper gastrointestinal bleeding most often leading to patient's death for hemorrhagic shock. It is frequently associated to aortic atherosclerosis. Its prompt diagnosis with endoscopy and CT angiography is very often difficult and almost never immediate. Furthermore, these exams may be misleading. In case of massive upper GI bleeding without a certain diagnosis in patients with severe aortic atherosclerosis, laparotomy with careful inspection of the distal duodenum is strongly recommended for aortic repair and bowel suture. CONCLUSIONS: The diagnosis of PADF should be taken into account in patients with upper gastrointestinal bleeding associated with aortic atherosclerosis with strong suspect of penetrating ulcer.

COVID-19 and aorto-enteric fistula.

We present the case of a 72-year-old male with upper gastrointestinal bleeding. He had been discharged from hospital two weeks before after severe COVID-19 infection, treated with lopinavir-ritonavir (L-R), hydroxychloroquine, tocilizumab, and methylprednisolone. On presentation, he was in hypovolemic shock. Esophagogastroduodenoscopy showed an ulcer in the third duodenal portion, which was sclerosed and hemodynamic stability was recovered. A scan was performed as it was in an atypical location for ulcers, showing an aortic aneurysm in close relationship to the duodenum, suggesting a primary aortoenteric fistula (PAEF).

[Rupture of abdominal aortic aneurysm into the duodenum: uncommon cause of massive gastrointestinal bleeding]. / Aneurisma de aorta abdominal roto al duodeno: causa infrecuente de hemorragia digestiva masiva.

Primary aortoenteric fistula is the spontaneous communication between the lumen of the aorta and a portion of the digestive tract. The most common cause is the erosion of an abdominal aortic aneurysm into the 3rd or 4th portion of the duodenum. It manifests clinically as gastrointestinal bleeding, with or without abdominal pain and a pulsatile abdominal mass on physical exam. Gastrointestinal bleeding is initially recurrent and self-limiting and progresses to fatal exsanguinating hemorrhage. Endoscopic examination diagnoses only 25% of aortoenteric fistulas because these are usually located in the distal duodenum. Contrast computed tomography of the abdomen and pelvis is diagnostic in only 60% of cases. We report three cases with this condition. A 67-year-old male presenting with an upper gastrointestinal bleeding. He was operated and a communication between an aortic aneurysm and the duodenum was found and surgically repaired. The patient is well. A 67-year-old male with an abdominal aortic aneurysm presenting with abdominal pain. He was operated and anticoagulated. In the postoperative period he had a massive gastrointestinal bleeding and a new CAT scan revealed an aorto enteric fistula that was surgically repaired. The patient is well. An 82-year-old male with an abdominal aortic aneurysm presenting with hematochezia. A CAT scan revealed a communication between the aneurysm and the third portion of the duodenum, that was surgically repaired. The patient died in the eighth postoperative day.

Giant coronary artery fistula complicated with coronary artery aneurysm and acute myocardial infarction: a case report.

BACKGROUND: Coronary artery fistula (CAF) is an abnormal connection between a coronary artery and either a cardiac chamber or the great vessels. Although most patients are asymptomatic, potential complications such as heart failure, angina pectoris or acute myocardial infarction can be fatal. CASE PRESENTATION: We present here a 62-year-old man diagnosed with giant coronary artery fistula complicated with gross coronary artery aneurysm and acute myocardial infarction. He underwent intravenous thrombolysis treatment at a local hospital, coronary angiography at a regional hospital and complex surgery at a national centre for cardiovascular disease. The patient had no major adverse cardiac events during the 3-year follow-up. CONCLUSION: Early diagnosis of CAF patients and an appropriate treatment plan are the key factors for avoiding serious complications. Because of the rare incidence of this disease, it is necessary to discover and discuss management strategies, including medical management, percutaneous interventions or surgical treatment, for a successful outcome.

Recurrent Aortic Infections Due to Unrecognized Aorto-Enteric Fistula.

We report a case of an infrarenal abdominal aortic aneurysm (AAA) with unrecognized primary aortoduodenal fistula (ADF), treated by endovascular aortic repair (EVAR). Endograft infection was diagnosed 12 months thereafter. The associated ADF was uncovered during open surgery, which included endograft extraction, in situ aortic reconstruction with a cryopreserved homograft (CHG) and duodenal repair. The patient was urgently reoperated in the early postoperative course, due to CHG rupture and subsequent hemorrhagic shock. After establishing control of hemorrhage, CHG was explanted, followed by aortic ligation and extraanatomical reconstruction with axillofemoral bypass. The importance of timely diagnosis of primary ADF prior to AAA repair, as well as treatment options and optimal materials for simultaneous aortic and bowel reconstruction in the setting of primary or secondary ADF, are discussed.

Right coronary artery-right ventricular fistula complicated with infective endocarditis.

Coronary artery fistula (CAF) is a congenital disease in which a communication forms between one or more coronary arteries and a cardiac chamber or great vessel. We describe an infrequent case of right coronary artery (RCA) fistula into the right ventricle (RV) complicated by infective endocarditis in a child. The patient received echocardiography and contrast-enhanced multidetector computed tomography (MDCT). Surgical treatment was performed after management of the infection. Unfortunately, a residual fistula formed after surgery. However, interestingly, the residual fistula spontaneously resolved at one year after surgery. He is now in good condition and totally asymptomatic.

Long-Term Follow-Up of Pediatric Patients After Congenital Coronary Artery Fistula Closure.

The surgical closure of congenital coronary artery fistulas (CAF) is associated with excellent immediate outcomes. Few studies have investigated the long-term prognosis in patients who have undergone surgery for the closure of CAF or differentiated among types of CAF or types of surgical procedures. In this study, we performed clinical examinations and computed tomography angiography (CTA) to characterize outcomes after CAF closure in pediatric patients. The medical records of 79 pediatric patients who underwent surgical closure of CAF were retrospectively reviewed. The median age of the patients included in the study at the time of surgery was 3.4 years (range 0.2 to 15.3 years). The patients had been followed up for 11 years (range 1 to 17 years) with electrocardiography, echocardiography, and coronary CTA. There were 67 medium-to-large CAF and 12 small CAF. Twenty-six (32.9%) CAF arose from the branch coronary artery (proximal type); the others arose from the parent coronary artery (distal type). The surgical procedure included endocardial closure in 16 cases, epicardial distal ligation in 51 cases, epicardial proximal and distal ligation in 12 cases. There was no instance of perioperative death among the cases included in the study. Twenty-eight patients were treated with antiplatelet medication postoperatively. No patient required re-operation during the follow-up period. Coronary thrombi were detected in 27 patients (34.2%). There was no instance of myocardial ischemia related to thrombosis. Among the patients with thrombosis, 26 had medium-to-large CAF (96.3%), and 23 had distal-type CAF (85.2%). Average age at surgery was higher among the patients with thrombosis than among the patients without thrombosis (7.4 years vs. 3.3 years, t = 5.509, P = 0.000). Among the patients with distal-type CAF, thrombosis was more common among the patients treated with ligation than treated with endocardial closure (41.5% vs. 16.7%, χ2 = 3.742, P = 0.043). There was no difference in risk for thrombosis between the patients who did vs. did not receive antiplatelet therapy (P = 0.436). The most common complication after CAF closure was thrombosis. Increased risk for thrombosis was associated with large fistulae, distal-type CAF, and older age at presentation. Antiplatelet treatment did not appear to decrease the risk of thrombosis. Among patients with distal-type CAF, risk for thrombosis was lower among patients treated with endocardial closure, compared with patients treated with epicardial ligation.

Severe Dilated Cardiomyopathy Resulted from a Large Single Coronary Artery Fistula Drained Into the Left Ventricle.

BACKGROUND: Coronary artery fistula (CAF) draining into the left ventricle (LV) is a rare condition and dilated cardiomyopathy (DCMP) that results from single coronary artery (SCA) accompanied by CAF also is extremely rare. CASE REPORT: We report the case of a 36-year-old man, who presented with severe DCMP that resulted from SCA with CAF draining into the LV. Transthoracic echocardiogram (TTE) showed severe diffuse hypokinesia of the LV with ejection fraction (EF) of 15-20%. Coronary angiography (CAG) revealed SCA connected between left anterior descending artery (LAD) and posterior descending artery (PDA), course of the PDA was very tortuous from apex to base of the LV, and connected to posterior lateral (PL) branch, which was drained into the LV at distal part of the PL. Coronary artery computed tomography (CACT) showed LAD ran over the apex of the LV and connected to PDA, which was drained into the mid portion of lateral wall of the LV. Cardiac magnetic resonance imaging (CMRI) showed no evidence of irreversible myocardial change in global wall of the LV. The patient underwent surgical ligation of PDA near the base of the posterior wall of the LV as close to the entry of CAF to the LV as possible without any surgery-related complications. Three months after the surgical ligation, follow-up TEE  showed much improved EF of 45-50%. He has been doing well without congestive heart failure (CHF) until now. DISCUSSION: Symptomatic CAF with hemodynamic deterioration may need mechanical correction of CAF, including surgical ligation or percutaneous interventional occlusion. How to treat this condition in terms of methodology is a very difficult issue. The detailed methods related to surgical or interventional correction of CAF have to be determined based on anatomical characteristics of CAF, underlying comorbidities, and relevant complications risk.

Gastrointestinal bleeding due to a primary aortoenteric fistula.

We present the case of a 80 years-old male patient who underwent a CT angiogram due to hematemesis and hypovolemic shock. An upper gastrointestinal endoscopy revealed a large clot that closed the antrum and abundant red blood in the pyloric-antrum region. Sclerosis was performed blindly and the bleeding origin was not identified and the success of the sclerosis could not be evaluated. A primary aorta-duodenal fistula was observed by CT angiography, which was treated with an endograft and femoro-femoral bypass. The patient was discharged 14 days after admission.

Spontaneous Aortoesophageal Fistula And Ruptured Aortic Aneurysm - A Case Report On Combined Aortic And Esophageal Prosrhesis Palliative Treatment.

Aortoesophageal fistulas are uncommon, dreadful vascular events, most frequently found in the setting of thoracic aorta aneurysms. Patients usually present with thoracic pain, dysphagia and sentinel hematemesis - the Chiari triad - followed by life threatening hematemesis. Emergent open surgery with debridement of necrotic tissue and in situ aortic graft repair is currently the best strategy. However, in patients which cannot withstand surgery, endovascular repair is currently gaining acceptance as a palliative treatment or as a bridge to surgery. We present a case of a 55-year-old female with a past of heavy alcohol abuse and a previously unknown massive aortic aneurysm, who presented to the emergency department complai- ning of acute dysphagia and epigastric pain. An abdominal ultrasound revealed left pleural effusion and suspected clots in the pleural space. A thoracic CTA was promptly done, where a spontaneous ruptured aortic aneurysm with aortoesophageal fistula was discovered. The team, fearing open surgery due to poor cardiac function, opted for a thoracic endovascular aortic repair. The aortoesophageal fistula dissected the esophageal wall in all of its thickness without rupture into the lumen. This was complicated with esophageal ischemia, aneurysmal sac infection and mediastinitis. Because the patient was in shock, in order to help control the infection, an esophageal prosthesis was placed, followed by proximal esophagostomy, distal esophageal closure and gastrostomy. Six months after initial presentation, the patient died at the emergency room, shortly after reentering with massive hematemesis and hypovolemic shock of undetermined origin.

Thoracic endovascular aortic repair for esophageal cancer invading the thoracic aorta: a questionnaire survey study.

BACKGROUND: Locally advanced esophageal cancer occasionally invades the aorta, and hemorrhage from the esophagoaortic fistula can cause sudden death. Thoracic endovascular aortic repair (TEVAR) enables hemostasis in such cases, and prophylactic TEVAR can prevent fatal hemorrhagic events during treatment. However, its efficacy in Japan has not been evaluated. This study aimed to clarify the clinical significance of TEVAR in esophageal cancer patients. METHODS: The Japan Esophageal Society conducted a questionnaire survey targeting authorized or semi-authorized member institutes of the Authorized Institutes for Board Certified Esophageal Surgeons. Patients who underwent TEVAR for esophageal cancer were identified from 19 institutes. Data on patient demographics, treatment performed, and survival rate were obtained using the questionnaire. The Kaplan-Meier method was used for survival analysis and to compare differences in survival rates between those who underwent TEVAR for hemorrhage and those for preoperative prophylaxis. RESULTS: Of the 41 patients identified, 20 patients underwent TEVAR for hemorrhage or impending hemorrhage from the esophagoaortic fistula, while 21 patients underwent TEVAR as preoperative prophylaxis. The median survival time after TEVAR was 135 days in the hemorrhage or impending hemorrhage group and 378 days in the preoperative prophylaxis group. Eighteen patients underwent esophagectomy after TEVAR. No hemorrhagic event was observed during the perioperative period. The median survival time of the patients who underwent esophagectomy was 373 days. Some patients who achieved R0 resection obtained long-term survival. CONCLUSION: TEVAR is an efficacious modality to control a life-threatening hemorrhage from esophagoaortic fistula and helps to prolong the survival of patients with locally advanced esophageal cancer invading the aorta.