Diagnosis and Management of Congenital Coronary Artery Fistulas in Adults.

PURPOSE OF REVIEW: This review describes the presentation, diagnosis, and management of congenital coronary artery fistulas (CAFs) in adults. RECENT FINDINGS: CAFs are classified as coronary-cameral or coronary arteriovenous fistulas. Fistulous connections at the distal coronary bed are more likely to be aneurysmal with higher risk of thrombosis and myocardial infarction (MI). Medium-to-large or symptomatic CAFs can manifest as ischemia, heart failure, and arrhythmias. CAF closure is recommended when there are attributable symptoms or evidence of adverse coronary remodeling. Closure is usually achievable using transcatheter techniques, though large fistulas may require surgical ligation with bypass. Given their anatomic complexity, cardiac CT with multiplanar 3-D reconstruction can enhance procedural planning of CAF closure. Antiplatelet and anticoagulation are essential therapies in CAF management. CAFs are rare cardiac anomalies with variable presentations and complex anatomy. CAF management strategies include indefinite medical therapy, percutaneous or surgical CAF closure, and lifelong patient surveillance.

Erección prolongada dolorosa / Prolonged painful erection

El priapismo es una erección peneana prolongada y dolorosa, que ocurre sin estímulo sexual previo. Existen dos tipos principales, el priapismo de alto flujo y el priapismo de bajo flujo. Aunque en la mayoría de las ocasiones la causa subyacente será desconocida, puede ser la primera manifestación de una enfermedad grave. En el paciente pediátrico con una erección prolongada se debe diferenciar entre la erección peneana recurrente y los distintos tipos de priapismo, puesto que cada entidad requiere un manejo concreto e implica un pronóstico diferente. (AU)

Priapism is a prolonged and painful penile erection, which occurs without prior sexual stimulation. There are two main types, high-flow priapism and low-flow priapism. Although on most occasions the underlying cause will be unknown, it may be the first manifestation of serious disease. In the pediatric patient with prolonged erection we must differentiate between recurrent penile erection and the different types of priapism since each entity requires a specific management and implies a different prognosis. (AU)

Transcatheter closure of a large coronary cameral fistula presenting as a calcified cystic right atrial mass.

A 51-year-old patient with progressive right heart dysfunction was found to have a large calcified right atrial mass on echocardiography. As part of the work up for an intracardiac mass he had a cardiac computed tomogram which detailed a large coronary cameral fistula from the circumflex coronary artery to the right atrium associated with a spherical calcific pseudo-aneurysmal sac. Transcatheter occlusion of the exit point into the atrium with a vascular plug was performed directly from a right atrial approach without the need for an arteriovenous wire loop. This case details a unique presentation of a coronary cameral fistula to an unusual position within the right atrium which facilitated the rare ability to occlude the fistula from a venous approach without creating an arteriovenous wire rail.

Endovascular Management of Aortic Stump Blowout by Parallel Grafting and Coil Embolization of Visceral Aorta.

Aortic graft and endograft infections remain a significant source of morbidity and mortality after abdominal aortic aneurysm repair. With graft excision and extra-anatomic bypass, an infrarenal aortic stump remains which can have suture line dehiscence and catastrophic stump blowout. Treatment of this is extremely challenging, especially for severely co-morbid patients who cannot undergo major surgery, or in patients with a hostile abdomen. We present a case study of a 74-year-old male found to have an aortoenteric fistula (AEF). This case broadens operative options for this type of patient population by demonstrating an endovascular technique for addressing aortic stump blowout by parallel grafting and coil embolization of the visceral aorta.

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.

Early diagnosis and management of arterio-ureteral fistulas: A literature review.

OBJECTIVES: Arterio-ureteralfistula (AUF) is an infrequent but potentially life-threatening condition. The aim of this study was reviewing the literature to build a flow-chart useful for an early and effective diagnosis and treatment of this pathology. MATERIALS AND METHODS: A literature search in PubMed was conducted. In addition, retrieved articles were cross-referenced. Data parameters included oncologic, vascular and urological history, diagnostics, treatment, and follow up were collected using a standard template by 2 independent reviewers. RESULTS: A total of 140 cases of AUF out of 172 available in the literature at the time of the review, were considered. All patients presented gross hematuria. Chronic indwelling ureteral catheter (CIUC); history of pelvic surgery (HPS) and history of pelvic radiotherapy (HRT) were present respectively in 81%, 62.1%and 58.6% of the sample. The most predominant location of AUF was at the common iliac artery ureteral crossing. Angiography with provocative measures had the highest diagnostic sensitivity (50%) and endovascular treatment with stent-graft placement across the fistula is the current state of the art treatment choice. CONCLUSIONS: Failure to diagnose can postpone a potentially life-saving targeted therapy and lead to complications. The identifi-cation of the Trifecta hematuria, history of pelvic surgery (HPS) and history of pelvic radiotherapy (HPR) would allow the identity-fication of patients at high risk of AUF, who may benefit from more sensitive early diagnostic investigations such as CT angiography and provocative angiography. The treatment of choice in case of AUF to date consist in endovascular prosthesis placement.

Esophago-aortic fistula of esophageal cancer after chemotherapy, proton therapy and salvage photodynamic therapy: a rescued case.

We describe a case of esophageal cancer after proton therapy that resulted in an esophagoaortic fistula after photodynamic therapy (PDT). A 49-year-old woman with esophageal cancer (cT1bN0M0, cStage I) underwent chemotherapy (5-FU and cisplatin) and radiotherapy (proton therapy to the cancer lesion after X-ray radiotherapy to the regional lymph nodes). Despite a complete response of the primary tumor, local recurrence was observed 10 months after treatment. PDT was performed as a salvage treatment. She was transported to the emergency department in a state of hemorrhagic shock due to hematemesis 50 days after PDT. We diagnosed an esophagoaortic fistula caused by esophageal perforation, and resuscitative endovascular balloon occlusion of the aorta and thoracic endovascular aortic repair were performed. The patient was successfully rescued after three surgeries (esophagectomy, extraesophageal fistula, aortic vascular replacement, and gastrointestinal reconstruction). In addition to X-ray radiotherapy before photodynamic therapy, proton therapy in combination with the vascular shutdown effects of PDT may have caused ischemia of the esophagus, resulting in an esophagoaortic fistula. When performing PDT, the type of radiation therapy and the location of the lesion should be examined to assess the risk of penetration or perforation.

Endovascular coil embolization of inferior mesenteric artery to ileal-conduit fistula: a case report.

BACKGROUND: Uretero-arterial fistulas (UAFs) are uncommon and pose a diagnostic dilemma, making them life threatening if not recognized and treated expediently. UAFs to small arteries such as a branch of the inferior mesenteric artery (IMA) are very uncommon and present a further diagnostic and treatment challenge. There should be a high index of suspicion for UAFs when intervening on patients with history of treated pelvic cancers and long-standing ureteric stents experiencing hematuria not attributable to another cause. CASE PRESENTATION: We present a case of a fistula formed between a distal branch of the IMA-superior rectal artery-and an ileal-conduit in a patient with a long-standing reverse nephroureterostomy (Hobbs) catheter presenting with abdominal pain and hematuria through the conduit. During a tube exchange, contrast injection demonstrated a fistula with the superior rectal artery, multiple ileal intraluminal blood clots, and active extravasation. The patient became tachycardic and hypotensive, actively bleeding through the ileal-conduit, prompting a massive transfusion protocol. Successful endovascular coiling of the superior rectal artery was performed with resolution of active extravasation and stabilization of the patient. The patient recovered and was discharged in stable condition 10 days later. CONCLUSIONS: Although UAFs are uncommon, our case demonstrated key predisposing risk factors to fistula development; pelvic cancer surgery, pelvic radiation, and a prolonged ureteric stent through the ileal-conduit. Typically, UAFs arise from communication with the iliac arterial system, however in this instance we have demonstrated that fistulization to other arterial vessels is also possible. Endovascular management has become the preferred method of therapy, typically involving the placement of covered stents when involving the iliac arterial system. In this instance stent grafting was not possible due to the small caliber vessel and therefore had to be embolized.

Arterioureteral fistula: overview of clinical characteristics, endovascular management, and outcomes.

BACKGROUND: Arterioureteral fistula (AUF) is a condition resulting from the pathologic connection between the ureter and the artery. Despite the low incidence, it can lead to devastating clinical consequences due to massive hematuria with a considerable mortality rate. MATERIAL AND METHODS: From January 2009 to December 2018, eight patients with AUF from two tertiary referral centers were included. Clinical data including presenting symptoms, previous pelvic surgery or radiotherapy, indwelling ureteral stents, primary vascular pathology, angiographic findings, type of treatment, survival, and recurrence were analyzed. RESULTS: All eight patients (six women, mean age 62.4 ± 14.5 years) presented with macroscopic hematuria and were successfully treated by endovascular management. One patient developed AUF due to an underlying iliac artery aneurysm, and the rest were due to secondary causes. Six patients had a history of an indwelling ureteral stent for a median of 5.5 months (1-84 months). All of the patients were successfully treated by endovascular management. For the median follow up of 987 days, three patients had recurrence of hematuria in a mean of 6.3 months, two patients were treated by surgery, while one was treated by endovascular treatment. CONCLUSION: AUF should be confirmed through a purposeful iliac angiogram or ureterography when suspected based on a relevant history or CT findings. AUF can be successfully treated by endovascular management. The surgical option should be considered in cases of recurrence. ABBREVIATIONS: AUF: arterioureteral fistula; CIA: common iliac artery; DJ: double J; EIA: external iliac artery; IIA: internal iliac artery; NBCA: N-butyl cyanoacrylate; PCN: percutaneous nephrostomy.

Arterio-Ureteral Fistula: Systematic Review of 445 Patients.

PURPOSE: Arterio-ureteral fistula (AUF) is an uncommon diagnosis, but increasingly reported and potentially lethal. This systematic review comprehensively presents risk factors, pathophysiology, location and clinical presentation of AUF aiming to increase clinical awareness of this rare but life-threatening condition, and to put this entity into a contemporary perspective with modern diagnostic tools and treatment strategies. MATERIALS AND METHODS: This review was performed according to the PRISMA (Preferred Reporting Items for a Systematic Review and Meta-Analysis of Individual Participant Data) guidelines. A literature search in PubMed® and EMBASE™ was conducted. In addition, retrieved articles were cross-referenced. Data parameters included oncologic, vascular and urological history, diagnostics, treatment, and followup, and were collected using a standard template by 2 independent reviewers. RESULTS: A total of 245 articles with 445 patients and 470 AUFs were included. Most patients had chronic indwelling ureteral stents (80%) and history of pelvic oncology (70%). Hematuria was observed in 99% of the patients, of whom 76% presented with massive hematuria with or without previous episodes of (micro)hematuria. For diagnosis, angiography had a sensitivity of 62%. The most predominant location of AUF was at the common iliac artery ureteral crossing. AUF-specific mortality before 2000 vs after 2000 is 19% vs 7%, coinciding with increasing use of endovascular stents. CONCLUSIONS: AUF should be considered in patients with a medical history of vascular surgery, pelvic oncologic surgery, irradiation and/or chronic indwelling ureteral stents presenting with intermittent (micro)hematuria. A multidisciplinary consultation is necessary for diagnosis and treatment. The most sensitive test is angiography and the preferred initial treatment is endovascular.

Coronary Artery Fistulas: Indications, Techniques, Outcomes, and Complications of Transcatheter Fistula Closure.

Coronary artery fistulas (CAFs) are rare coronary anomalies that are usually diagnosed incidentally with cardiac imaging. Small CAFs are generally asymptomatic and can close over time, while some untreated medium or large CAFs can enlarge, leading to clinical sequelae such as cardiac chamber enlargement or myocardial ischemia. With the advancement of transcatheter equipment and techniques, CAFs have been increasingly closed using a percutaneous approach. However, the procedure is not free of limitations given the risk for myocardial infarction, device embolization, and fistula recanalization. In this review, the authors illustrate the contemporary procedural considerations, techniques, and outcomes of transcatheter CAF closure.

Midterm follow up of transcatheter closure of coronary artery fistula with Nit-Occlud® patent ductus arteriosus coil.

BACKGROUND: Coronary artery fistula (CAF) is a rare congenital anomaly with a challenging scenario in children. This study reports our experience in transcatheter closure of CAF with Nit-Occlude PDA coil and midterm clinical and imaging follow-up. METHODS: Twelve children with congenital CAF between 2009 and 2019, mean age 2.05 ± 2.05 years (4 days to 7.2 years), mean weight 8.8 ± 4.83 (2.8-17 kg), who underwent transcatheter closure with PFM coil at the Namazi hospital, Shiraz, Iran, were reported. Echocardiography and electrocardiogram were done before and after the procedure (early, 3, and 6 months after), and Multi-slice computerized tomography or conventional coronary angiography was performed at least one year after closure. RESULTS: In a median follow-up of 5.5 years (range 13 months to 8 years), retrogradely closed fistula had no residual, and the fistula tract was wholly occluded, but in most anterogradely closed fistula, had a small residual, which made the fistula tract open and need additional coil closure. CONCLUSIONS: Transcatheter closure of CAF with PFM coil is feasible and effective with low mortality and morbidity, although antegrade closure with this device may be accompanied by residual shunt and need for multiple coil insertion.

Management of an Aortoenteric Fistula in a Patient with End Stage Renal and Liver Disease, Prior Endovascular Aortic Repair With Type II Endoleak.

Primary aortoenteric fistula (AEF) is an uncommon but life-threatening condition. We present a case of primary AEF in an octogenarian with previous endovascular aortic repair, type II endoleak and end stage liver and renal disease. He was successfully treated with accessory renal artery ligation, duodenojejunostomy, aneurysm sac debridement and irrigation and closure of the aneurysm sac over a drain. The patient made an excellent recovery and was discharged on POD #7, with no complications noted after over a year of follow up. This approach may represent a valuable option to manage primary AEF versus open endograft explant, particularly in severely ill patients.

Massive Hemoptysis Caused by an Aortobronchial Fistula Related to Pedicle Screw Impingement.

We report a case of a 50-year-old man with a 10-year history of pedicle screw internal fixation in the thoracic spine and heroin abuse, who presented with sudden-onset massive hemoptysis with hemorrhagic shock and asphyxia. Urgent contrast-enhanced chest computed tomography (CT) characteristically showed thoracic aortic perforation, a paravertebral pseudoaneurysm, and an intrapulmonary hematoma. Emergency percutaneous thoracic endovascular aortic repair (pTEVAR) with the preclose technique using a vascular closure device under local anesthesia achieved success without any complications. The current case highlights the importance of understanding massive hemoptysis caused by an aortobronchial fistula related to pedicle screw impingement in clinical practice and the value of pTEVAR with the preclose technique under local anesthesia in the emergency setting.

Severe complications of chronic cholelithiasis treatment.

BACKGROUND: Gallstone disease is a burden affecting about 15% percent of the population around the world. The complications of gallstone disease are numerous and many require emergency care. Severe complications are not uncommon and require special attention, as lethal outcome is possible. CASE PRESENTATION: We present a retrospective analysis of eight cases describing severe complications of gallstones in patients undergoing endoscopic treatment of chronic gallstones conditions. All patients were admitted to our emergency care department following symptoms onset. The diagnostic difficulties, treatment strategies and outcomes are presented. The associated risk factors and preventative measures are discussed. Two patients developed profuse bleeding, two developed acute pancreatitis, two patients had perforation related complications. One rare case of bilioma and one case of iatrogenic injury are presented. All patients had severe condition, in two cases lethal outcome was a result of co-morbidity and difficulties in management. CONCLUSION: Special care should be taken in patients with risk factors of severe complications in order to improve outcome and prevent the development of life-threatening conditions.

CT-guided Fibrin Glue Occlusion of Cerebrospinal Fluid-Venous Fistulas.

Background Cerebrospinal fluid-venous fistulas (CVFs) are one of the less common etiologic causes of spontaneous intracranial hypotension. CVFs are most commonly treated with open surgical ligation and have reportedly not responded well to percutaneous treatments. Purpose To study treatment outcomes of CT-guided fibrin glue occlusion for CVFs. Materials and Methods Retrospective review of medical records from two institutions was performed for all patients with CVFs who underwent CT-guided percutaneous fibrin glue occlusion from March to October 2020. CVFs were assessed for resolution or persistence at posttreatment decubitus CT myelography (CTM). Pre- and posttreatment brain MRI scans were reviewed for principal signs of spontaneous intracranial hypotension. Clinical symptoms were documented before and immediately after therapy, and the current symptoms to date after fibrin glue occlusion were documented. Results CT-guided fibrin glue occlusion was performed in 13 patients (mean age, 62 years ± 14 [standard deviation]; eight women) with CVFs. Ten of 10 patients who underwent final posttreatment decubitus CTM examinations showed CVF resolution. All 13 patients showed improvement on posttreatment brain MRI scans. All 13 patients are currently asymptomatic, although three patients were asymptomatic before fibrin glue occlusion. Conclusion CT-guided fibrin glue occlusion is an effective treatment for patients with cerebrospinal fluid-venous fistulas (CVFs). Direct fibrin glue administration within the CVF may be one of the key factors for success. Further studies are needed to determine the long-term efficacy of this treatment. © RSNA, 2021.