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.

Aorto-left atrial fistula secondary to aortic infective endocarditis in a dog with a bicuspid aortic valve.

An 11-year-old male neutered American bulldog was presented for evaluation of thrombocytopenia, acute onset of ataxia, and vomiting. A new murmur was auscultated on physical examination. Transthoracic echocardiographic examination revealed a bicuspid aortic valve, vegetative lesions on the aortic valve, and continuous shunting from the aortic root to the left atrium through an aorta to left atrial fistula. The dog was euthanized due to its guarded prognosis and critical condition. Pathological examination confirmed presence of a bicuspid aortic valve, aorto-left atrial fistula, and aortic infective endocarditis. Antemortem blood culture revealed two unusual organisms: Achromobacter xylosoxidans and Fusobacterium mortiferum.

"Empty Cyst Sign" Appearance of CSF-Venous Fistula on Digital Spinal Myelography.

Cerebrospinal fluid venous fistulas (CSF-VFs) are an uncommon, yet increasingly recognized, cause of spontaneous intracranial hypotension.1-5 The workup involves magnetic resonance imaging (MRI) of the brain with and without contrast and MRI of the neuroaxis without contrast before dynamic myelography, either computed tomography or digital subtraction.6 The present case of an older woman with symptomatic intracranial hypotension is notable for the specific appearance of CSF-VFs on digital spinal myelography (Video 1). Among her numerous perineural cysts, it was the "disappearing" or "empty" cyst from which the fistula originated. The diagnosis was made using a second lateral fluoroscopy view, not typically used in digital spinal myelography, which demonstrated emptying of contrast from the T6 perineural cyst into the segmental vein at this level, or the "empty cyst sign." The patient then underwent transvenous onyx embolization with resolution of her orthostatic headaches and improvement of contrast-enhanced MRI of the brain with the Bern score decreasing from 7 to 0 at 3 months of follow-up.7 Because transvenous embolization of CSF-VFs is a relatively new procedure, the long-term outcomes of the procedure are not yet known.

Echocardiographic and pathologic identification of an aorto-left atrial fistula secondary to infective endocarditis in a canine patient.

A dog was presented for lameness, fever, and extreme lethargy. On physical exam, a new heart murmur, arrhythmia, and joint effusion were detected. These findings were not detected two months prior. A diagnostic work-up confirmed septic suppurative inflammation in multiple joints. Echocardiogram revealed aortic valvular endocarditis along with a communication, as a consequence of a fistula, that extended from just below the aortic sinotubular junction to the left atrial lumen. Due to a poor prognosis, humane euthanasia was elected. Necropsy and histopathology confirmed infective endocarditis of the aortic valve and an aorto-left atrial fistulous tract extending from the left coronary sinus of the aortic valve to the lumen of left atrium.

A Rare Cause of a Continuous Murmur in a Newborn.

We describe what we believe to be the first reported case of a neonate with right coronary ostial atresia with the right coronary artery originating from the left circumflex coronary artery, in conjunction with a right coronary artery to right ventricle fistula in a patient with otherwise normal cardiac anatomy. This was found following an evaluation for a continuous murmur at 2 weeks of life with elevated troponin and abnormal electrocardiogram. Thus far the child has required no intervention and is asymptomatic at 17 months of age, but he will require long-term follow-up to monitor the size of the fistula and potential for myocardial insufficiency.

Massive gastrointestinal bleed due to a primary aortoenteric fistula.

A man in his 60s attended emergency for acute-onset abdominal pain and haematemesis. Requiring resuscitation, a CT abdomen/pelvis revealed a primary aortoenteric fistula actively bleeding into the duodenum. His background included a previous severe Q-fever infection and a heavy smoking history. Despite attempts at resuscitation and an emergent surgical attempt at haemostasis, the patient did not survive the massive gastrointestinal haemorrhage.Even in less severe cases, management of aortoenteric fistulas is tricky. Blood cultures and angiographic imaging are important investigations in guiding surgical approach. The pathology tends to have a significant rate of mortality even at tertiary-level vascular surgical centres.

Multifaceted surgical approach of combined thoracoretroperitoneal incision and midline abdominal incision for a secondary aortoenteric fistula.

BACKGROUND: We report a one-stage surgery to the case of secondary aortoenteric fistula (sAEF) after prosthetic reconstruction of abdominal aortic aneurysm, by multifaceted approach. CASE PRESENTATION: A 63-year-old male was admitted to our unit under diagnosed of sAEF after prosthetic reconstruction of abdominal aortic aneurysm, and a pseudoaneurysm of thoracoabdominal aorta due to infection. The patient underwent emergency operation. Firstly, we placed the patient in a modified right lateral decubitus position and performed thoracoabdominal aortic replacement with retroperitoneal approach by thoracoretroperitoneal incision which combined thoracotomy and pararectal incision, and secondly, we changed to a supine position and performed closure of the duodenal fistula and omental flap transposition by midline abdominal incision. The patient was doing well without complications. CONCLUSIONS: A one-stage, multifaceted surgical approach covering both prosthetic reconstruction of thoracoabdominal aorta and closure of sAEF with omentopexy is reasonable and useful strategy.

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.

Colorectal Malignancy Complicated by Arterioenteric Fistula.

Arterioenteric fistulas (AEF) are rare and devastating complications of colorectal/pelvic malignancies. These fistulas can be seen following neoadjuvant or adjuvant therapy but are exceptionally rare de novo. The reported incidence of AEF is less than 1% and iliac artery-enteric fistulas make up less than .1% of all AEF. Here we present a patient in hemorrhagic shock secondary to an advanced colorectal malignancy without adjuvant therapies with local invasion of the right external iliac artery. Following initial resuscitation and hemorrhage control with coil embolization, definitive control with ligation and excision of the involved artery, end colostomy, and ureteral stent placement was achieved. It is important to consider malignancy as the source of lower gastrointestinal bleeds, especially in elderly patients without current colonoscopy studies. The management of this unfortunate diagnosis often involves a multidisciplinary approach with early and frequent goals of care discussions.

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.

[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.

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.

A strategy to prevent tracheo-innominate artery fistula in the course of laryngotracheal separation: 9-year experience in a children's hospital.

AIM OF THE STUDY: Laryngotracheal separation (LTS) is known to be the definitive solution for intractable aspiration pneumonia in neurologically impaired children. Postoperatively, a tracheostomy cannula is usually required. However, there are fatal cannula related complications such as a tracheo-innominate artery fistula (TIAF). We present our methods of preventing TIAF. METHODS: A retrospective review in a single center from 2011 to 2019 identified 57 cases treated with LTS. We divided them into three groups: no pre-existing tracheostomy (n = 26), pre-existing tracheostomy with preservation of the pre-existing fistula (n = 20), and pre-existing tracheostomy without preservation of the pre-existing fistula (n = 11). The first group underwent traditional modified Lindeman's procedure. The second received transection of the trachea above the tracheostomy site, while the third had transection of the trachea at the tracheostomy site and creation of a distal end tracheostomy. Proper length and the angle of the cannula were selected to prevent damaging the innominate artery by the tip of the cannula. If the innominate artery compressed the trachea anteriorly, prophylactic arterial transection was considered. RESULTS: Three patients (5.3%) died from causes unrelated to the surgical treatment. Only one patient had a postoperative TIAF followed by LTS (1.8%). Other postoperative complications were: wound infection (8.8%), intratracheal granuloma (12.3%), intratracheal minor bleeding (10.5%), wound granuloma (43.9%), leakage (1.8%). No one required revision of LTS. CONCLUSION: Success rates of LTS were high without major complications in all three groups and implies a safe operation and a definitive solution to intractable aspiration.

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.