DOCSLIB.ORG

Arteriovenous Fistula of the Kidney: a Case Report of 47-Year-Old Female Patient Treated by Embolisation

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Arteriovenous Fistula of the Kidney: a Case Report of 47-Year-Old Female Patient Treated by Embolisation Journal of Human Hypertension (2003) 17, 293–296 & 2003 Nature Publishing Group All rights reserved 0950-9240/03 $25.00 www.nature.com/jhh CASE REPORT Arteriovenous fistula of the kidney: a case report of 47-year-old female patient treated by embolisation K Paschalis-Purtak1, M Januszewicz2, A Rokicki3, B Puciłowska1, J Imiela4, I Cybulska1, W Cies´la5, A Prejbisz1, M Szostek5 and A Januszewicz1 1Department of Hypertension, Institute of Cardiology, Warsaw, Poland; 2II Department of Clinical Radiology, Medical University of Warsaw, Poland; 3Department of Radiology, Central Railway Hospital, Warsaw, Poland; 4Department of Internal Medicine, Central Railway Hospital, Warsaw, Poland; 5Department of General Surgery and Chest Diseases, Medical University of Warsaw, Poland Arteriovenous fistulas of the kidney are rare. They may embolised arteriovenous fistula. Diagnosis was made be acquired, idiopathic or arise in congenital arteriove- on the basis of colour duplex Doppler examination and nous malformations. There are only few reports in the this method enabled further successful embolisation of current literature describing the successful embolisa- the fistula. tion of idiopathic arteriovenous fistulas. We report a 47- Journal of Human Hypertension (2003) 17, 293–296. year-old hypertensive female patient with a successfully doi:10.1038/sj.jhh.1001544 Keywords: arteriovenous fistula; kidney; embolisation Introduction Case report Arteriovenous fistulas of the kidney are rare and are A 47-year-old hypertensive female patient (known classified as acquired, idiopathic or arising in a duration of hypertension: 5 years) was admitted to congenital arteriovenous malformation.1 In addi- the Department of Internal Medicine, Central Railway tion, with the more widespread use of interventional Hospital in Warsaw because of poor control of blood procedures such as percutaneous needle biopsy and pressure (BP). Prior to admission, she was found to percutaneous nephrostomy, iatrogenic fistulas have have a blood pressure of up to 240/120 mmHg. become more frequent.2 During the hospitalisation, colour duplex Doppler Congenital arteriovenous malformations have a sonography showed a vascular malformation in the cirsoid or angiomatous configuration and comprise right renal hilum. The cirsoid vessel with turbulent 14–27% of arteriovenous malformations. The term fast flow was connected to an interlobar branch of idiopathic is used to describe those fistulas in which the renal artery and efferent vein. There was the exact aetiology cannot be precisely determined. pulsatile flow detected in the renal efferent vein Idiopathic arteriovenous fistulas comprise between that gave indication of the presence of arteriovenous 2 and 5% of all arteriovenous fistulas.1 fistula. Renal angiography confirmed the diagnosis. It has been reported that duplex Doppler sono- The patient’s treatment was continued in the graphy is very effective in diagnosing arteriovenous Department of Hypertension, Institute of Cardiology, fistulas and helpful in the long-term follow-up after Warsaw. The patient complained of headaches and surgery or percutaneous embolisation.1 denied any other symptoms, including haematuria. However, in contrast to acquired fistulas, there are Past medical history was unremarkable, but there only a few reports describing successful embolisa- was a family history of hypertension. tion of an idiopathic arteriovenous fistula. On admission, physical examination was normal except for a mild tachycardia (85–90 bpm), and there was no abdominal bruit. The patient had no signs of congestive heart failure, jugular vein was not seen in the upright position, hepatojugular reflux was Correspondence: Dr K Paschalis-Purtak, Department of Hyperten- positive. Fundoscopy demonstrated hypertensive sion, Institute of Cardiology, 04-628 Warsaw, Alpejska 42, Poland. E-mail: [email protected] retinopathy, grade II. Received 25 May 2002; revised 15 December 2002; accepted 31 ABPM (before medication was started): showed a December 2002 mean BP 150/100 mmHg during the day and mean Arteriovenous fistula of the kidney K Paschalis-Purtak et al 294 BP of 125/77 mmHg during the night. The patient was treated with amlodipine 5 mg/day, bisoprolol 5 mg/day and indapamide 1.5 mg/day, and good control of BP was achieved. Laboratory studies were within normal ranges. Urinary cate- cholamines, metoxycatecholamines, plasma aldos- terone and plasma renin activity were within normal ranges. Chest X-ray was normal. Electrocardiography showed left ventricular hypertrophy. Transthoracic echocardiogram demonstrated moderate mitral and tricuspid regurgitation, the posterior wall diameter (PWD) and intraventricular septal diameter (ISD) were 1.1 and 1.2 cm, respectively. Left ventricular diastolic diameter, left atrial diameter and right Figure 1 Renal angiography show an enlarged renal artery branch ventricular diastolic diameter were 5, 4 and 5 cm, and cirsoid vessel located in the renal hilar connecting artery with respectively. The left ventricular mass, calculated renal vein. following the equation by Devereux, was 220.3 g, then normalised for body surface areaFleft ventri- cular mass indexF121 g/m2. Abdominal ultrasonography demonstrated the size of right and left kidney being 97 and 107 mm, respectively. Doppler duplex of carotid arteries showed no atherosclerotic lesions. Although the arteriovenous fistula was asympto- matic and the patient had no heart failure or haematuria, we decided to manage the arteriove- nous fistula with embolisation. Pretreatment angiography of the right renal artery showed a curved, elongated vessel connecting the medial interlobal renal artery branch with the renal vein. There was an early filling of renal vein and poor parenchymal opacification after contrast ad- ministration (see Figure 1). Then the catheter was Figure 2 Postembolisation angiography show metalic coils in the placed at the entrance of the fistula and two metalic entrance of the fistula. No flow through the fistula is present. coils (Cook Inc.) were positioned in the first segment of the cirsoid vessel. The postembolisation angio- graphy showed a direct flow through the renal artery Discussion branches to the renal parenchyma and a regular back-flow to the renal vein. Blood flow through the Since the first description of renal arteriovenous fistula was no longer seen (see Figure 2). fistulas by Varela in 1928, their exact classification On duplex Doppler (ATL 5000 HDI) examination, varies, but generally separates acquired malforma- there was no flow through the vascular malforma- tions from congenital malformations. The term tion and there were no disturbances in renal artery idiopathic is used by some authors to describe those and renal vein flow. malformations in which the exact aetiology cannot The early postinterventional period was unevent- be determined.1–4 ful and follow-up has been for 8 months. There was It should be noted that since most of arteriovenous no effect of the embolisation on BP. ABPM (anti- fistulas are asymptomatic and remain undetected, hypertensive medication was discontinued for 2 their real incidence is unknown. However, if weeks) showed a mean BP of 140/99 mmHg during diagnosed they have an important impact on clinical the day and 130/87 mmHg during the night. There- management. It should also be kept in mind that fore antihypertensive therapy was continued. arteriovenous fistulas may cause severe cardiologic Control echocardiogram disclosed mild mitral manifestations, including congestive heart failure or regurgitation and no tricuspid regurgitation; left hypertension. ventricular diastolic diameter: 4.2 cm, left atrial Since in our patient there was no clinical diameter: 3.6 cm. These suggest that there has been evidence indicating the development of iatrogenic normalisation of left ventricular volume because of or traumatic arteriovenous fistula, taking into con- the successful procedure. sideration angiographic appearance, the lesion was The long-term successful effect of the embolisa- classified as idiopathic. There was no trauma or tion was observed in repeat duplex Doppler exam- renal biopsy history and no evidence of arterio- inations. sclerosis or arteritis. Journal of Human Hypertension Arteriovenous fistula of the kidney K Paschalis-Purtak et al 295 Clinical manifestations of the arteriovenous fistu- Hypertension is found in about 40–50% of las depend on their localisation and size. The most patients with renal fistulae and may be caused by frequent clinical signs are: widened pulse pressure, local renal ischaemia resulting from a short circuit brisk arterial pulsation, mild tachycardia and a bruit of blood with increased renin secretion. However, over the region where the fistula is localised. most studies show that renal vein renin levels are Haematuria and symptoms of congestive heart fail- within normal range with no lateralisation. There- ure are often reported. It has been reported that a fore, renal vein renin sampling may be of little bruit is less frequent in cirsoid arteriovenous diagnostic value.1,14 malformations and haematuria is more common in It has also been reported that congenital arterio- congenital malformations.1,5–7 venous fistulas may cause refractory hypertension. Recently, Lekuona et al8 presented a 56-year-old Ullian and Malitoris14 described a 55-year-old female with high-output heart failure secondary to patient with severe hypertension and bilateral large congenital arteriovenous fistula successfully congenital renal arteriovenous
Load more

Recommended publications
  • Evicore Pediatric PVD Imaging Guidelines
    CLINICAL GUIDELINES Pediatric Peripheral Vascular Disease (PVD) Imaging Guidelines Version 1.0 Effective January 1, 2021 eviCore healthcare Clinical Decision Support Tool Diagnostic Strategies: This tool addresses common symptoms and symptom complexes. Imaging requests for individuals with atypical symptoms or clinical presentations that are not specifically addressed will require physician review. Consultation with the referring physician, specialist and/or individual’s Primary Care Physician (PCP) may provide additional insight. CPT® (Current Procedural Terminology) is a registered trademark of the American Medical Association (AMA). CPT® five digit codes, nomenclature and other data are copyright 2020 American Medical Association. All Rights Reserved. No fee schedules, basic units, relative values or related listings are included in the CPT® book. AMA does not directly or indirectly practice medicine or dispense medical services. AMA assumes no liability for the data contained herein or not contained herein. © 2020 eviCore healthcare. All rights reserved. Pediatric PVD Imaging Guidelines V1.0 Pediatric Peripheral Vascular Disease (PVD) Imaging Guidelines Procedure Codes Associated with PVD Imaging 3 PEDPVD-1: General Guidelines 5 PEDPVD-2: Vascular Anomalies 10 PEDPVD-3: Vasculitis 15 PEDPVD-4: Disorders of the Aorta and Visceral Arteries 19 PEDPVD-5: Infantile Hemangiomas 25 ______________________________________________________________________________________________________ ©2020 eviCore healthcare. All Rights Reserved. Page 2 of
    [Show full text]
  • The Arteriovenous Fistula
    DISEASE OF THE MONTH J Am Soc Nephrol 14: 1669–1680, 2003 Eberhard Ritz, Guest Editor The Arteriovenous Fistula KLAUS KONNER,* BARBARA NONNAST-DANIEL,† and EBERHARD RITZ† *Merheim Hospital, Medical Faculty, University of Cologne, Germany; †Department of Nephrology, Med. Klinik IV, Erlangen-Nu¨rnberg, Germany; and ‡Department Internal Medicine, Ruperto Carola University, Heidelberg, Germany. The ground-breaking article by Brescia and Cimino in 1966 (1) side fistula. Flow increased from 21.6 Ϯ 20.8 ml/min to 208 Ϯ revolutionized the creation of the vascular access, and the 175 ml/min immediately after operation. In well-developed fistu- Cimino fistula was soon used in almost all dialysis patients. lae, flow rates may ultimately reach values of 600 to 1200 ml/min. Unfortunately, subsequent wide-spread use of PTFE grafts Flow increases as a result of both vasodilation and vascular instead of AV fistulae occurred because of the ease of the remodeling. The latter has been studied using echo-tracking surgical technique, the immediate availability of the graft for techniques (8). It was found that the diameter of the proximal puncture, the need of high blood flow for high-efficiency, antecubital vein increased progressively while the intima me- short-duration hemodialysis sessions, and because of financial dia thickness remained unchanged. Venous dilation caused disincentives against the AV fistula. PTFE grafts currently reduction of mean shear stress, which had returned to normal account for 80% of primary vascular accesses created in the values by 3 mo. The venous limb of the AV fistula underwent United States (2,3), but they are less frequently used in other excentric hypertrophy as documented by increased wall cross- countries.
    [Show full text]
  • Ens Manifestations of Hereditary Hemorrhagic Telangiectasia
    569 eNS Manifestations of Hereditary Hemorrhagic Telangiectasia David Sobel, ·2 Hereditary hemorrhagic telangiectasia (HHT) is a familial angiodysplastic disorder. David Norman' Dermal, mucosal, and visceral vascular lesions of this disorder are well known. However, central nervous system (CNS) manifestations, occurring in as many as one-third of patients, have not been well appreciated until recently. The etiology of neurologic symptomatology includes hypoxemia or ischemia secondary to pulmonary arteriovenous shunting, vascular lesions of the brain and spinal cord ranging from aneurysms to arteriovenous malformations, brain abscesses secondary to pulmonary arteriovenous fistulas, and portal systemic encephalopathy. Angiographic and computed tomographic findings in four patients with CNS involvement in HHT are reported. Hereditary hemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber disease, is an uncommon genetic angiodysplastic disorder transmitted as a simple mendel ian dominant character. Characteristic findings first become evident at puberty and include widely scattered dermal and mucosal telangiectases, most common on the skin of the face and neck and on buccal and nasopharyngeal mucous membranes; diffuse visceral vascular lesions; recurrent bleeding; and absence of hematologic disorders other than those secondary to bleeding and/or arteriovenous fistulas (AVFs) [1, 2). The most common presenting symptom is epistaxis from nasomu­ cosal lesions. Gastrointestinal, genitourinary, pulmonary, and cerebral hemorrhage may all occur. More than 300 families with HHT have been recorded in the literature. Pulmonary AVFs have been reported in 15.4% of patients [4) and are the most frequent visceral lesions. Fifty percent of patients with pulmonary AVFs are cited as having HHT [5) . Vascular malformations of the brain in HHT are much less common and have rarely been documented either angiographically or pathologically [6) .
    [Show full text]
  • Presentation and Treatment of Arteriovenous Fistula, Arteriovenous Malformation, and Pseudoaneurysm of the Kidney in Ramathibodi Hospital
    42 Insight UROLOGY : Vol. 41 No. 2 July - December 2020 Original Article Presentation and treatment of arteriovenous fistula, arteriovenous malformation, and pseudoaneurysm of the kidney in Ramathibodi Hospital Dussadee Nuktong, Pokket Sirisreetreerux, Pocharapong Jenjitranant, Wit Viseshsindh Division of Urology, Department of Surgery, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand Keywords: Abstract Renal arteriovenous Objective: To review the presentation, predisposing factors, treatment and outcome fistula, renal of renal vascular malformation, including arteriovenous malformation (AVM), arteriovenous arteriovenous fistula (AVF) and pseudoaneurysm of the kidney in Ramathibodi malformation, Hospital. renal pseudoaneurysm, Material and Method: In-patient medical records from January 2007 to January embolization 2017 were retrospectively reviewed. Patients admitted and diagnosed with any type of vascular malformation of the kidney, comprising AVM, AVF and pseudoaneurysm in Ramathibodi Hospital were included in the study. Baseline characteristics of the patients, including gender, age at diagnosis, and underlying disease were recorded. Vascular malformation, clinical presentation, imaging data, predisposing factors of the disease, treatment and the outcome of patients were summarized and reported. Results: Seventeen patients were diagnosed with vascular malformation; 9 patients were males and 8 females. The most common comorbidity was hypertension, followed by chronic kidney disease. Nine patients had AVF (52.94%), 3 had AVM (17.65%), 2 had pseudoaneurysm (11.76%), and 3 had AVF with pseudoaneurysm (17.65%). Common presentations were gross hematuria, flank pain, anemia, and hypovolemic shock. Previous surgery and history of renal biopsy were mutual predisposing factors. Embolization was the most common treatment option. All patients were asymptomatic on follow-up visit with a median follow-up of 90 days.
    [Show full text]
  • Congenital Renal Arteriovenous Malformation: a Rare but Treatable Cause of Hypertension
    e-ISSN 1941-5923 © Am J Case Rep, 2019; 20: 314-317 DOI: 10.12659/AJCR.912727 Received: 2018.08.13 Accepted: 2018.11.22 Congenital Renal Arteriovenous Malformation: Published: 2019.03.10 A Rare but Treatable Cause of Hypertension Authors’ Contribution: BE 1 Nicholas Isom 1 Department of Internal Medicine, University of Kansas Medical Center, Kansas Study Design A ABCE 2 Reza Masoomi City, KS, U.S.A. Data Collection B 2 Department of Cardiovascular Diseases, University of Kansas Medical Center, Statistical Analysis C BD 3 Adam Alli Kansas City, KS, U.S.A. Data Interpretation D ABCDE 2 Kamal Gupta 3 Department of Radiology, University of Kansas Medical Center, Kansas City, KS, Manuscript Preparation E U.S.A. Literature Search F Funds Collection G Corresponding Author: Kamal Gupta, e-mail: [email protected] Conflict of interest: None declared Patient: Female, 29 Final Diagnosis: Renal arteriovenous malformation Symptoms: Hypertension Medication: — Clinical Procedure: Angiography Specialty: Cardiology Objective: Rare disease Background: Congenital renal vascular anomalies have been classified into 3 categories: cirsoid, angiomatous, and aneu- rysmal. These classifications are based on the size, location, and number of vessels involved. Aneurysmal mal- formations, such as the one reported here, have a single (and dilated) feeding and draining vessel. The preva- lence of renal AVMs is estimated at less than 0.04%, making them rare causes of secondary hypertension. Case Report: A 29-year-old white woman was seen in the hypertension clinic as a referral from high-risk obstetric clinic for management of hypertension (HTN). A secondary hypertension workup with Doppler waveforms of the re- nal arteries revealed prominent diastolic flow in the left compared to the right.
    [Show full text]
  • Intercostal Was Performed, and the Patient Arteriovenous Fistula Recovered Uneventfully
    976 Bilton,. Webb, Foster, Mulvenna, Dodd of factor VIII and it also releases plasminogen tion in haemaglobin than when he had activator from endothelial cells.9 previously been admitted for haematemesis. Vasopressin has been used to control bleed- Severe haemoptysis in chronic lung disease ing from oesophageal varices. Its plasma half is uncommon and pressor agents should not Thorax: first published as 10.1136/thx.45.12.976 on 1 December 1990. Downloaded from life is about 24 minutes and it is most effective be used routinely owing to the side effects of when given by infusion. The site of action is water retention and bronchoconstriction. probably arteriolar smooth muscle, through They may, however, have a useful con- an increase in the intracellular concentration servative role in the management of patients of inositol phosphates, which mobilise with cystic fibrosis who have severe lung and intracellular calcium, causing contraction. liver disease. The bronchial and mesenteric arteries both arise directly from the aorta. We hoped to reproduce the effect of pressor agents on the 1 Penketh ARL, Wise A, Mearns MB, Hodson M, Batten JC. mesenteric vasculature in the bronchial cir- Cystic Fibrosis in adolescents and adults. Thorax 1987; culation. The effect of the pressor agents in 42:526-32. 2 King AD, Cumberland DC, Brennan SR. Management of stopping pulmonary bleeding may have been severe haemoptysis by bronchial artery embolisation in a fortuitous; but the immediate termination of patient with cystic fibrosis. Thorax 1989;44:523-4. occasions, 3 Sweezey NB, Fellows K. Bronchial artery embolisation for profuse bleeding on separate severe Hemoptysis in Cystic Fibrosis.
    [Show full text]
  • Aortoiliac Arteriovenous Fistulae Simulating Deep Vein Thrombosis
    Case Report J Cardiol & Cardiovasc Ther - Volume 10 Issue 2 April 2018 Copyright © All rights are reserved by Germán J Chaud DOI: 10.19080/JOCCT.2018.10.555782 Aortoiliac Arteriovenous Fistulae Simulating Deep Vein Thrombosis Germán J Chaud*, Filippa A Pablo, Wainscheinker Ezequiel, Parisi Andrés, Guillermo Paladini and Alejandro M Martínez Colombres Department of Cardiovascular Surgery at Hospital Privado Universitario de Córdoba, Argentina Submission: March 05, 2018; Published: April 20, 2018 *Corresponding author: Chaud Germán J, Department of Cardiovascular Surgery at Hospital Privado Universitario de Córdoba, Córdoba, Naciones Unidas 346, Argentina, Tel: ; Email: Abstract Incidence of aorto-caval fistulae is quite low, ranging from 0.22 to 6.04% of all abdominal aortic aneurysm. One of the rare forms of abdominal aortic aneurysm rupture is rupture into great abdominal veins, such as the inferior vein cava (IVC) or the iliac veins. The typical ofclinical atherosclerosis. presentation Correct includes operative abdominal management pain, a pulsatile includes abdominal expeditious mass, control an abdominal of the bleeding, bruit and greater acute care dyspnea. to avoid Morbidity embolization and mortality through willthe be affected by the acute presentation, preoperative recognition of the fistula, the extent of cardiac failure, coronary disease and other risk factors fistula, use of blood salvage, and only selective caval interruption. Introduction Arteriovenous fistula (AVF) of the infrarenal aorta is a well- The control of the venous bleeding from fistula is challenging. useful manoeuvre. The transfemoral insertion of an occlusive known clinical entity. Incidence of aorto-caval fistulae is quite Manual compression distally and proximally to the fistula is a low, ranging from 0.22 to 6.04% of all abdominal aortic aneurysm (A.A.A).
    [Show full text]
  • Clostridial Mycotic Aneurysm Leading to Emphysematous Aortitis
    Open Access Case Report DOI: 10.7759/cureus.14136 Clostridial Mycotic Aneurysm Leading to Emphysematous Aortitis Thomas G. Ng 1 , Usha Trivedi 1 , Kajol Shah 1 , Pierre Maldjian 2 1. Internal Medicine, Rutgers University, Newark, USA 2. Radiology, Rutgers University, Newark, USA Corresponding author: Thomas G. Ng, [email protected] Abstract Mycotic aneurysms account for less than 5% of all aneurysms of the aorta, with most cases linked to infection with either Staphylococcus or Salmonella species. Emphysematous aortitis is a rare consequence of mycotic aneurysms and is associated with high morbidity and mortality. It typically occurs from infection superimposed on already damaged endothelium, which is commonly seen in conditions such as atherosclerosis. This report discusses the presentation and relevant imaging findings of a unique case of emphysematous aortitis from Clostridial infection of the thoracic aorta. The patient was a 66-year-old male with a past medical history of end-stage renal disease, arteriovenous fistula for dialysis, hypertension, and diabetes, who presented with tachycardia and tachypnea. Computed tomography of the chest showed inflammatory changes of the thoracic aorta with gas bubbles along the aortic wall, and post-mortem aortic tissue cultures were positive for Clostridium innocuum. Although emphysematous aortitis is rare, the radiographic findings are strikingly characteristic and should prompt immediate and aggressive management. Categories: Cardiac/Thoracic/Vascular Surgery, Infectious Disease, Anatomy Keywords: emphysematous aortitis, clostridium, mycotic aneurysm, rutgers njms, cardiothoracic surgery Introduction Emphysematous aortitis is a rare consequence of underlying mycotic aneurysm, an uncommon cause of arterial dilation resulting from infection of damaged vessel endothelium. A misnomer, mycotic aneurysm is usually caused by bacterial rather than fungal infection, and accounts for 0.7%-1.3% of all surgically treated aneurysms.
    [Show full text]
  • Aneurysms Associated with Arteriovenous Malformations Beatrice Gardenghi, MD, Carlo Bortolotti, MD, and Giuseppe Lanzino, MD
    VOLUMEVOLUME 3636 •• NUMBERNUMBER 122 JanuaryNovember 31, 1,2014 2014 A BIWEEKLY PUBLICATION FOR CLINICAL NEUROSURGICAL CONTINUING MEDICAL EDUCATION Aneurysms Associated With Arteriovenous Malformations Beatrice Gardenghi, MD, Carlo Bortolotti, MD, and Giuseppe Lanzino, MD Learning Objectives: After participating in this CME activity, the neurosurgeon should be better able to: 1. Assess the various classifi cations of cerebral aneurysms associated with arteriovenous malformations (AVMs). 2. Compare the various hypotheses about the pathogenesis of the cerebral aneurysms associated with AVMs. 3. Evaluate the role of surgery, endovascular therapy, and radiosurgery in the treatment of intracranial aneurysms associated with AVMs. Intracranial aneurysms can occur in patients with brain Classifi cation arteriovenous malformations (AVMs), and this not uncom- A clear understanding of the various types of aneurysms mon association poses important therapeutic challenges. In and aneurysm-like dilations that occur in patients with AVMs patients presenting with intracranial hemorrhage, the AVM is paramount to clarify their pathophysiology and clinical is responsible for bleeding in 93% of cases, with the remaining signifi cance. These aneurysms can be classifi ed on the basis 7% related to associated intracranial aneurysms. The inci- of location, histopathologic characteristics, and hemodynamic dence of aneurysms in patients with AVMs is higher than features. expected on the basis of the frequency of each lesion indi- vidually. This observation suggests that factors such as Location increased regional fl ow (hence hemodynamic stress) may Aneurysms associated with AVMs can occur on the arterial play a causative role in the formation of aneurysms associated side (arterial aneurysms) or the venous side (venous aneurysms). with AVMs, although other causative factors such as genetic In relation to the nidus of the AVM, aneurysms and aneurysm- predisposition cannot be excluded.
    [Show full text]
  • Clinically Suspected Vascular Malformation of the Extremities
    New 2019 American College of Radiology ACR Appropriateness Criteria® Clinically Suspected Vascular Malformation of the Extremities Variant 1: Upper or lower extremity. Suspected vascular malformation presenting with pain or findings of physical deformity including soft-tissue mass, diffuse or focal enlargement, discoloration, or ulceration. Initial imaging. Procedure Appropriateness Category Relative Radiation Level MRA extremity area of interest without and Usually Appropriate with IV contrast O MRI extremity area of interest without and Usually Appropriate with IV contrast O CTA extremity area of interest with IV Usually Appropriate Varies contrast US duplex Doppler extremity area of interest Usually Appropriate O MRA extremity area of interest without IV May Be Appropriate contrast O CT extremity area of interest with IV contrast May Be Appropriate Varies MRI extremity area of interest without IV May Be Appropriate contrast O US extremity area of interest with IV contrast May Be Appropriate O CT extremity area of interest without IV May Be Appropriate Varies contrast CT extremity area of interest without and with Usually Not Appropriate Varies IV contrast Radiography extremity area of interest Usually Not Appropriate Varies Arteriography extremity area of interest Usually Not Appropriate Varies Variant 2: Upper or lower extremity. Vascular murmur (bruit or thrill). Initial imaging. Procedure Appropriateness Category Relative Radiation Level MRA extremity area of interest without and Usually Appropriate with IV contrast O MRI extremity
    [Show full text]
  • Genetic Syndromes with Vascular Malformations – Update on Molecular Background and Diagnostics
    State of the art paper Genetics Genetic syndromes with vascular malformations – update on molecular background and diagnostics Adam Ustaszewski1,2, Joanna Janowska-Głowacka2, Katarzyna Wołyńska2, Anna Pietrzak3, Magdalena Badura-Stronka2 1Institute of Human Genetics, Polish Academy of Sciences, Poznan, Poland Corresponding author: 2Department of Medical Genetics, Poznan University of Medical Sciences, Poznan, Adam Ustaszewski Poland Institute of Human Genetics 3Department of Neurology, Poznan University of Medical Sciences, Poznan, Poland Polish Academy of Sciences 32 Strzeszynska St Submitted: 19 April 2018; Accepted: 9 September 2018 60-479 Poznan, Poland Online publication: 25 February 2020 Phone: +48 61 65 79 223 E-mail: adam.ustaszewski@ Arch Med Sci 2021; 17 (4): 965–991 igcz.poznan.pl DOI: https://doi.org/10.5114/aoms.2020.93260 Copyright © 2020 Termedia & Banach Abstract Vascular malformations are present in a great variety of congenital syn- dromes, either as the predominant or additional feature. They pose a major challenge to the clinician: due to significant phenotype overlap, a precise diagnosis is often difficult to obtain, some of the malformations carry a risk of life threatening complications and, for many entities, treatment is not well established. To facilitate their recognition and aid in differentiation, we present a selection of notable congenital disorders of vascular system development, distinguishing between the heritable germinal and sporadic somatic mutations as their causes. Clinical features, genetic background and comprehensible description of molecular mechanisms is provided for each entity. Key words: arteriovenous malformation, vascular malformation, capillary malformation, venous malformation, arterial malformation, lymphatic malformation. Introduction Congenital vascular malformations (VMs) are disorders of vascular architecture development.
    [Show full text]
  • Hypopharyngeal Venous Malformation Presenting with Foreign Body Sensation and Dysphagia
    AMERICAN JOURNAL OF OTOLARYNGOLOGY– HEAD AND NECK MEDICINE AND SURGERY 37 (2016) 34– 37 Available online at www.sciencedirect.com ScienceDirect www.elsevier.com/locate/amjoto Hypopharyngeal venous malformation presenting with foreign body sensation and dysphagia Andrew M. Vahabzadeh-Hagh, MD a,⁎, Ali R. Sepahdari, MD b, Jayson Fitter a, Elliot Abemayor, MD, PhD a a Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA USA b Department of Radiological Sciences, David Geffen School of Medicine at UCLA, Los Angeles, CA USA ARTICLE INFO ABSTRACT Article history: Objective: Review the importance of imaging selection and clinicoanatomic correlation for a Received 26 August 2015 vascular malformations presenting with unique symptomatology. Methods: Case study and literature review. Results: A 64-year-old female presented with globus and dysphagia ongoing for 40 years. Esophagogastroduodenoscopy discovered a hypopharyngeal mass. A CT scan showed a soft tissue mass with shotty calcifications. Flexible laryngoscopy revealed a bluish compressible mass. MRI showed T2 hyperintensity with heterogeneous enhancement resulting in the diagnosis of a low-flow vascular malformation. Conclusions: All globus is not equal. Attention to symptoms, anatomy, and imaging selection is crucial for the diagnosis and treatment of vascular malformations uniquely presenting with dysphagia. © 2016 Elsevier Inc. All rights reserved. 1. Introduction mass and may be seen within the muscles of mastication, lips, tongue, or elsewhere within the upper aerodigestive Vascular anomalies, including vasoproliferative/vascular tract. Imaging is critical in the diagnosis and management of neoplasms and vascular malformations remain a diagnostic vascular malformations. See Table 1 for the importance of and therapeutic challenge.
    [Show full text]