Arteriovenous Malformation Presenting Research Articles

Neurovascular lesions in parturients: Anesthetic management for cesarean section

Cerebral arteriovenous malformation (AVM) or aneurysm in pregnancy is a complex situation and there is no definite recommendation regarding mode of anesthesia for patient with this type of intracranial pathology. We present a case series on the anesthetic management in two pregnant patients with either cerebral AVM or aneurysm presenting for elective cesarean section. Our case series highlights the following: (1) team working and collaboration with neurosurgeon and obstetrician to improve patient outcome; (2) crucial role of anesthetic management in reducing perioperative complications; (3) anesthetic management goals so as to minimize the risk of hemorrhage from an AVM or aneurysm.

The characteristics of transcranial color-coded duplex sonography in children with cerebral arteriovenous malformation presenting with headache.

Cerebral arteriovenous malformations (AVM) are uncommon lesions. They are most often presented in childhood as intracranial hemorrhage. The aim of this report is to present the use of transcranial color-coded duplex sonography (TCCS) in detection of AVMs in children suffering headache. This report describes five pediatric patients with headache and cerebral AVM which were initially discovered by TCCS. Diagnosis was confirmed by magnetic resonance imaging and digital subtraction angiography. In all patients, TCCS showed saccular enlargement of the vessels with a multicolored pattern corresponding to the different directions of blood flow. Spectral analysis showed significantly high flow systolic and diastolic velocities and low resistance index. In this report, we describe TCCS as a valuable non-invasive, harmless, low-cost, widely available method for the detection and follow-up of hemodynamic changes of AVMs in children with headache, before and after treatment.

Arteriovenous malformations (AVM) of the corpus uteri

Introduction: The arteriovenous malformations (AVM) are sporadic lesions and they consist in a large number of tortuous, dilated, and thick-walled vessels with different sizes. AVM can be divided into two distinct types, in relation to their site: the superficial type and the deep type. Uterine AVM are precisely labelable as deep AVM and they are well-known in the gynaecological and radiological practice, but rarely reported in the histopathological literature. Materials And Methods: To fill this gap, the authors have retrospectively examined 25 cases of incidental uterine AVM from post-menopausal Caucasian women with a mean age of 68 years (range 63 and 74 years), who underwent hysterectomy with bilateral salpingo-oophorectomy for uterine prolapse. Surprisingly, in the anamnestic records, all the patients suffered from dysmenorrhea during their life. Moreover, they have reviewed about 300 cases of uterine AVM, reported in the gynaecological and radiological English literature, correlating them with their observations. Results: From their results, it emerged that the use of the term ‘acquired’ to describe uterine AVM should be avoided, because all the present lesions show a malformative morphology, related to a developmental disorder. Since no criteria exist to differentiate between AVM and placental bed sub-involution, a descriptive nomenclature should be preferred in the radiologic terminology. Conclusion: AVM should be routinely remarked in the histopathological reports, because their presence could be correlated with an explainable history of dysmenorrhea. Even if embolization remains an acceptable form of treatment in order to avoid hysterectomy in those patients presenting with heavy life-threatening bleeding, a vasoconstrictive therapy could be considered when other possible causes of disabling dysmenorrhea are excluded and the presence of AVM at high flow has been ascertained by eco-colour Doppler.

Pulmonary Arterio-Venous Malformation (PVAM) Presenting as Recurrent Hemoptysis

Pulmonary Arterio-Venous Malformation (PVAM) Presenting as Recurrent Hemoptysis

Brain Arterio-Venous Malformations Presenting with Seizures and Diagnosed on CT Scan: A Case Report

Brain Arterio-Venous Malformations Presenting with Seizures and Diagnosed on CT Scan: A Case Report

Inferior Mesenteric Arteriovenous Malformation Presenting as Ischemic Colitis After an Acute Episode of Campylobacter Jejuni Colitis

Inferior Mesenteric Arteriovenous Malformation Presenting as Ischemic Colitis After an Acute Episode of Campylobacter Jejuni Colitis

Contemporary Imaging of Cerebral Arteriovenous Malformations.

Brain arteriovenous malformation (AVM) rupture results in substantial morbidity and mortality. The goal of AVM treatment is eradication of the AVM, but the risk of treatment must be weighed against the risk of future hemorrhage. Imaging plays a vital role by providing the information necessary for AVM management. Here, we discuss the background, natural history, clinical presentation, and imaging of AVMs. In addition, we explain advances in techniques for imaging AVMs.

Vascular malformations of the maxillofacial skeleton — an overview of presentation and management

Background: Vascular disorders of the maxillofacial skeleton vary from benign localised lesions to aggressive, proliferative high-flow malformations that present enormous challenges for the clinician. Objectives: This presentation will describe the classification of vascular malformations within the maxillofacial complex, their characteristics and management. The distinction between arteriovenous malformations (AVM) and haemangiomas will also be highlighted to emphasise that these lesions are separate pathological entities. Methods: A retrospective review of patients (adults and paediatric patients) with a diagnosis of a vascular lesion of the facial skeleton was undertaken in an attempt to classify the main categories. The implications for elective and curative surgery were to be considered and treatment protocols outlined. Findings: Of 13 patients with AVM's, 6 were syndromic low-flow arteriovenous malformations, 4 were nonsyndromic low-flow lesions and 3 were high-flow malformations. Sturge-Weber syndrome is a phakomatosis due to an embryonal developmental anomaly (mutations in the GNAQ gene) with several subtypes. Nonsyndromic low-flow intraosseous lesions can be incidentally diagnosed on routine imaging and some may present with contour deformities or the displacement of teeth. However, high-flow lesions are rare and potentially life threatening. They may present with a pulsatile, expanding bony cortex, interdental bleeding or massive socket haemorrhage. For low-flow lesions, resection with a small margin may be sufficient for resolution of the lesion. However, high-flow lesions may require embolisation, sclerosing solutions, surgical resection or a combination of these interventions. A number of cases will be presented to illustrate the presentation of AVM's and the approach to management.

Dyspnoea and Cyanosis in Pregnancy: An Extremely Rare Cause

Pulmonary arteriovenous malformation first presenting with platypnea and orthodeoxia in the 3rd trimester of pregnancy is an extremely rare entity and poses a great diagnostic challenge to the treating physician. We hereby report a patient with pulmonary arteriovenous malformation presenting for the first time in third trimester of pregnancy diagnosed by pulmonary angiogram with subsequent improvement in symptoms post-delivery. Key words: Pulmonary Arteriovenous Malformation, Pregnancy, Platypnea, Orthodeoxia, Pulmonary Angiogram.

Causes and Severity of Anemia in Hereditary Hemorrhagic Telangiectasia

Introduction: Hereditary hemorrhagic telangiectasia (HHT) is characterized by the abnormal development of blood vessels leading to the formation of mucocutaneous telangiectasias and arteriovenous malformations (AVMs) in visceral organs. Anemia is a frequent complication of HHT secondary to blood loss from recurrent epistaxis, gastrointestinal bleeding, or both. The purpose of this study was to determine presence and severity of anemia at initial evaluation for HHT, and identify predictors of anemia.Methods: We conducted a retrospective chart review of HHT patients evaluated at the University of North Carolina HHT Center of Excellence from 2008-2015. Data abstracted from the medical record included diagnosis of anemia, hemoglobin, source of bleeding, HHT genotype, epistaxis severity score (ESS), presence of visceral organ AVMs, history of treatment of AVMs, iron therapy, and need for blood transfusions. All data were collected from the patient’s first visit to the HHT center. Source of bleeding was recorded as epistaxis, GI bleeding, or both. Epistaxis severity was determined by the ESS, with a higher score indicating more severe bleeding. GI bleeding had to be confirmed by patient history, presence of bleeding telangiectasias on endoscopy, or ongoing symptoms (bright red or dark stools). Presence of anemia required verification with complete blood count results. Anemia severity was defined as mild if hemoglobin ≥ 10, moderate if 8-10, and severe if <8 g/dl. Screening for visceral organ AVMs was conducted as per the international guidelines for management of HHT. All patients were screened for pulmonary (pAVM) and brain (bAVM) AVMs, and screening for GI and liver (lAVM) AVMs was pursued only in symptomatic patients. Cramer’s V and chi squared goodness of fit test determined if overall source or severity was linked to gender, genotype, and AVM location. A test of multinomial proportions determined statistical significance between individual gender, genotype, and AVM location with source and severity.Results: A total of 168 patients were included, of which 84 had documented anemia. In patients with anemia, the majority were female (72%), Caucasian (79%), and had ALK-1 genotype (50%). Mild anemia was most common (52%), followed by moderate (37%), and severe anemia (11%). Neither gender nor genotype was significantly associated with anemia severity. In patients with mild anemia, pAVMs were the most common visceral organ affected (45%), while GI telangiectasias were most common in the severe anemia group (67%). In moderate anemia, there was an equal proportion of GI and pAVM involvement (44%). Overall the presence of AVMs was linked to anemia severity (p-value: 0.0006), but there was no association between individual AVM site and anemia severity. The majority of patients with mild anemia were or had been on oral iron (76%), while patients with moderate (75%) and severe anemia (100%) had or were currently on IV iron replacement.Epistaxis was the most common cause of anemia in the mild and moderate groups (75% and 59% respectively), while both GI bleeding and epistaxis were present in the majority of patients with severe anemia (44%). The mean ESS in patients with mild anemia was 5.18 compared to 6.93 in those with moderate anemia. The ALK-1 genotype was a significant predictor of anemia related to epistaxis. LAVMs also significantly increased the risk for anemia from recurrent epistaxis while GI telangiectasias were associated with anemia from both GI bleeding and epistaxis.Conclusion:Iron deficiency anemia can be associated with significant morbidity. This is the first study to analyze the causes and severity of anemia in HHT patients at the time of initial evaluation to an HHT Center of Excellence. A high prevalence of anemia was found in this HHT population, with 50% of patients having documented anemia. We found that patients with ALK-1 mutations and liver AVMs are more likely to have epistaxis as the source of their anemia. Further, we demonstrate that patients with severe anemia more frequently present with GI AVMs. Understanding disease related predictors of anemia could help identify patients that are at highest risk and thereby facilitate aggressive screening in high-risk groups. Our findings are timely given the increasing awareness of HHT within the hematology community. DisclosuresNo relevant conflicts of interest to declare.

Legal Briefs: Arteriovenous Malformation Presenting as Intracranial Hemorrhage in a Preterm Infant

A 1,910-g, 35-4/7 weeks’ gestation male infant was born by cesarean delivery due to breech position to a 33-year-old mother. He required vigorous stimulation and blow-by oxygen at birth. He was admitted to the newborn intensive care unit because of immaturity. His physical examination yielded normal results. He was always on room air and nippled all feeds. Except for some mild apnea, his course was unremarkable. Because of his prematurity, the neonatologist ordered cranial ultrasonography, which showed a parenchymal hemorrhage in the right parietal lobe. A computed tomography (CT) scan was obtained a few days later and confirmed the presence of the intracranial hemorrhage (ICH). The neonatologist requested a consultation with a neurologist because of the ICH. The neurologist did not view the scan or examine the baby but left word that he would see the infant as an outpatient. The infant was discharged from the hospital. Three months after discharge, the neurologist saw the infant in his office …

Prevalence and characteristics of brain arteriovenous malformations in hereditary hemorrhagic telangiectasia: a systematic review and meta-analysis.

OBJECTIVE Patients with hereditary hemorrhagic telangiectasia (HHT) are known to suffer from high rates of cerebral arteriovenous malformations (AVMs). The authors performed a systematic review and meta-analysis of the literature examining prevalence rates, characteristics, and clinical presentation of cerebral AVMs in the HHT population. METHODS To identify studies on AVM prevalence and characteristics in the HHT population, 4 databases (MEDLINE, EMBASE, Scopus and Web of Science) were searched by a reference librarian with over 30 years experience in systematic reviews and meta-analysis. The search period was January 1, 1990-March 2016. The following search terms were used: hereditary hemorrhagic telangiectasia, Osler-Weber-Rendu syndrome, AVM, brain AVM, arteriovenous malformation, arteriovenous fistula, prevalence, and epidemiology. The authors identified studies that examined the prevalence rates, characteristics, and clinical presentation of cerebral AVMs in patients with HHT. They assessed overall AVM prevalence rates as well as prevalence rates by age, sex, HHT type, and country/region. They also systematically reviewed the characteristics of AVMs, including rupture status, location, clinical presentation, angioarchitecture, and Spetzler-Martin grade. Data were analyzed using a random-effects meta-analysis model. RESULTS Thirty-nine studies were included in this meta-analysis. Thirty studies examined brain AVM prevalence rates in various HHT patient populations, and 18 studies examined AVM clinical and angiographic characteristics (9 studies examined both prevalence rates and AVM characteristics). The prevalence of brain AVMs in HHT patients was 10.4% (95% CI 7.9%-13.0%) with no significant difference between males (8.5%, 95% CI 4.9%-12.0%) and females (11.0%, 95% CI 5.9%-16.1%). Patients with HHT Type 1 (HHT1) had a significantly higher brain AVM prevalence (13.4%, 95% CI 9.5%-17.4%) compared with those with HHT Type 2 (HHT2) (2.4%, 95% CI 1.0%-3.8%) (p < 0.0001). In 55.2% (95% CI 38.3%-72.1%) of cases, the AVMs were symptomatic. Spetzler-Martin grade was 2 or less in 86.9% (95% CI 67.5%-95.2%) of patients. CONCLUSIONS The prevalence of brain AVMs in the HHT population is about 10%. HHT1 patients are significantly more likely to have brain AVMs than HHT2 patients. Most AVMs in the HHT population are symptomatic. The Spetzler-Martin grade for these lesions is 2 or less in nearly 90% of patients.

Abstract 50: Evaluation of Candidate Polymorphisms for Association With Brain Arteriovenous Malformations and Intracranial Hemorrhage in Hereditary Hemorrhagic Telangiectasia

Introduction: Brain arteriovenous malformations (AVM) are an important cause of intracranial hemorrhage (ICH) in young adults. Most are sporadic, but also occur in inherited diseases such as hereditary hemorrhagic telangiectasia (HHT). ICH presentation of brain AVM in both sporadic and HHT cases is a marker of high ICH risk. In order to investigate whether the same genetic modifiers influence sporadic and HHT brain AVM, we evaluated candidate genetic polymorphisms reported as associated with sporadic brain AVM, with ICH presentation or ICH during clinical course, in HHT patients. Methods: We genotyped 8 polymorphisms ( APOE E2/3/4 [rs7412, rs429358], ANGPTL rs116724, EPHB4 rs314308, IL6 -174G&gt;C [rs1800795], IL1B -31T&gt;C [rs1143627], ITGB8 rs10486391, TNF -238G&gt;A[rs361525]) in 753 Caucasian HHT patients enrolled by the Brian Vascular Malformation Consortium (BVMC). Genotypes were collapsed into risk allele carriers vs. other for analysis, as published for sporadic AVM. APOE E2/3/4 haplotypes were assigned based on genotypes of the 2 APOE polymorphisms. Association of genotype with phenotype was evaluated by multivariable logistic regression adjusted for age, gender and accounting for family clustering. We used a nominal significance threshold of p=0.05, requiring the same direction of effect as in sporadic brain AVM (odds ratio for risk genotype [OR]&gt;1). Results: Among 753 HHT patients, 155 (21%) had brain AVM, of whom 26 (17%) presented with ICH. Two additional brain AVM patients had ICH during follow-up. None of the 7 variants (6 single nucleotide polymorphisms and APOE haplotype) were significantly associated with brain AVM (OR=0.6-1.3), with ICH presentation of brain AVM (OR=0.4-1.9), or with any brain AVM ICH in HHT patients (OR=0.5-2.1). Conclusions: Common genetic variants previously reported to be associated with sporadic brain AVM were not associated with brain AVM nor with ICH in the BVMC HHT cohort, suggesting different genetic modifiers may influence sporadic and HHT brain AVM. However, the number of ICH cases in the cohort is small, so the confidence intervals are wide and we cannot rule out clinically important associations. The BVMC is enrolling additional HHT patients to expand the cohort and increase power for association analyses.

Anesthetic concern during cesarean delivery in patient with ruptured cerebral arteriovenous malformation

Vascular malformations of the brain are a rare cause of intracranial hemorrhage during pregnancy. The presentation of arteriovenous malformations during pregnancy is usually a result of hemorrhage following rupture. Once hemorrhage occurs, it accounts for 5–12% of all the maternal deaths and remains the third most common nonobstetric cause of maternal morbidity. Successful anesthetic management during cesarean delivery requires close monitoring to address both the varying maternal and fetal needs. The fundamental aims are to maintain oxygenation and stable systemic, cerebral, and placental hemodynamics and to avoid increase in intracranial pressure.

Hypervascular glioblastoma multiforme or arteriovenous malformation associated Glioma? A diagnostic and therapeutic challenge: A case report

Background:Simultaneous presentation of arteriovenous malformation (AVM) and glioblastoma multiforme (GBM) is rarely reported in the literature and needs to be differentiated from “angioglioma”, a highly vascular glioma and other differential diagnosis such as hypervascular glioblastoma. Incorporating critical features of both, malignant glioma and AVM, such lesions lack a standard algorithm for diagnosis and therapy due to their rare incidence as well as their complex radiological and highly individualized clinical presentation.Case Description:We present a case of a 71-year-old female with newly developing motor deficits and radiographic findings of a heterogeneously contrast enhancing right-sided thalamic lesion with highly prominent vasculature. While computed tomography angiogram and cerebral digital subtraction angiography supported the diagnosis of AVM, contrast-enhancing magnetic resonance imaging (MRI) and MR-spectroscopy was suggestive of malignant glioma. A stereotactic biopsy revealed the diagnosis of a GBM (WHO IV) and the patient was treated accordingly.Conclusion:The coincidental presentation of vascular lesions such as AVM and malignant glioma is rare and presents a major challenge when establishing a diagnosis. The respective treatment decision is complicated by the fact that available treatment modalities (e.g. radiosurgery and/or open resection) carry disease specific complications for each entity. Finding a suitable solution for such cases requires standardization of early diagnostic and therapeutic management.

Transcatheter Arterial Embolization of Pancreatic Arteriovenous Malformation Presenting as Retroperitoneal Bleeding: A Case Report

Pancreatic arteriovenous malformation (AVM) is a rare condition defined as a tumorous formation or vascular anomaly arising from an aberrant bypass anastomosis of the arterial and venous systems in the pancreas (1). Esophageal varix bleeding by portal venous hypertension is the most common complication of pancreatic AVM. Duodenal ulcer bleeding or bleeding from the pancreatic AVM itself are other causes of bleeding associated with this disease entity. Pancreatic AVM presenting as retroperitoneal bleeding is an extremely rare clinical condition and is often misdiagnosed as a pseudoaneurysm. Treatment may include ligation of the afferent artery, embolization of feeding vessels, portacaval shunting, or surgical resection of the affected part or the entire affected organ (2). We report a case of pancreatic AVM presenting as a mesenteric hemorrhage that was successfully treated with transcatheter arterial embolization using n-butyl-2-cyanoacrylate (NBCA). CASE REPORT

Spinal arteriovenous malformation presenting with urinary retention

We present a case of a young female patient presenting predominantly with recurrent episodes of urinary retention. Extensive urological workup was unremarkable. There were some subtle and long-standing neurological findings which prompted us to investigate further with a spinal magnetic resonance imaging scan. The scan demonstrated a spinal arteriovenous malformation (AVM) beginning from the L4/L5 level to the S1/S2 level. Spinal AVM can be a rare cause of urinary retention, and we would like to highlight the importance of having that in the list of our differential when other more common causes have been excluded, and the patient remains symptomatic.

Endovascular treatment of posterior fossa arteriovenous malformations

Infratentorial arteriovenous malformations (AVM) are rare, representing only 7–15% of cerebral AVM. The concentration of eloquent neurological structures and the high rate of bleeding presentation of AVM in this location complicate the management of such lesions. New therapeutic options, especially in endovascular therapy, have fundamentally modified the treatment strategy and also the outcome of posterior fossa AVM. Between 1999 and 2013, baseline, clinical and angiographic data of cerebral AVM were prospectively collected. We analyzed data from patients treated for a posterior fossa AVM, focusing on risk factors for bleeding, and clinical and angiographic outcomes. Sixty-nine patients (mean age 34years, male to female ratio 2:1) were consecutively treated for an infratentorial AVM. Fifty-seven presented with hemorrhage, six with focal neurologic deficits, and the remaining six patients were diagnosed incidentally. The Spetzler–Martin grade was <3 in 39 (56.5%) patients. Associated aneurysms were noted in 43.5% of patients. All patients were treated using endovascular procedures, associated with microsurgical resection in nine patients and with stereotactic radiosurgery in six. Mean follow-up was 28.5months, with angiographic exclusion of the AVM in 72.5% of patients; 21.7% of patients presented a modified Rankin Score ⩾3 at follow-up. Endovascular embolization seems to be a secure approach for posterior fossa AVM although a large number of sessions are necessary to achieve complete obliteration. Multi-disciplinary discussion and management is crucial to obtain the best cure rate without increasing procedural risks.

Anatomic and angiographic findings of cerebellar arteriovenous malformations: Report of a single center experience

Cerebellar arteriovenous malformations (AVM) are rare and comprise only 5–10% of cerebral AVM. The concentration of eloquent neurological systems and the high rate of bleeding presentation of AVMs particularly in this location complicate the management of such lesions. New therapeutic options, especially in endovascular neurosurgery, have fundamentally modified the strategy and, also, the outcome of cerebellar AVMs. Between 1995 and 2013, demographic, clinical and angiographic data of cerebral AVMs have been prospectively collected. We analyzed data of patients treated for a cerebellar AVM, focusing on the angiographic anatomy. Fifty-nine patients (mean age : 35years, male to female ratio : 2) were consecutively treated for a cerebellar AVM. 81.4% of them presented bleeding at admission. 20 AVMs (33.9%) were in eloquent areas. The Spetzler–Martin grade was I or II in 36 cases (51%). An associated aneurysm was noted in more than 40% of cases and a venous drainage anomaly in 70%. The vast majority of cases of this series presented an anatomical risk factor of bleeding. Patients with cerebellar AVMs presented with bleeding more often than patients with supratentorial AVMs, justifying an aggressive management. The analysis of angio-architecture highlighted the high rate of associated aneurysm and/or venous drainage anomalies that could explain the tendency to bleed of such lesion.

Angiographic factors associated with haemorrhagic presentation of brain arteriovenous malformation in a Chinese paediatric population.

To identify specific angiographic factors associated with haemorrhagic presentation of brain arteriovenous malformation in Chinese paediatric patients. Retrospective cross-sectional observational study. Four locoregional tertiary neurosurgical centres in Hong Kong: Queen Elizabeth Hospital, Tuen Mun Hospital, Kwong Wah Hospital, and Pamela Youde Nethersole Eastern Hospital. Patients aged 18 years or younger who underwent pretreatment digital subtraction angiography for brain arteriovenous malformation between 1 January 2005 and 31 July 2013 were included. Patients were divided into haemorrhagic and non-haemorrhagic groups based on the initial presentation. Pretreatment digital subtraction angiographies were independently reviewed by two experienced neuroradiologists. The following parameters were evaluated for their association with haemorrhagic presentation by univariate and multivariate analyses: nidus location, nidus size, nidus morphology (diffuse or compact); origin and number of arterial feeders; venous drainage; number of draining veins; presence of aneurysms, venous varices, and venous stenosis. A total of 67 children and adolescents (28 male, 39 female) with a mean age of 12 years were included. Of them, 52 (78%) presented with haemorrhage. Arteriovenous malformation size (P=0.004) and morphology (P=0.05) were found to be associated with haemorrhagic presentation by univariate analysis. Small arteriovenous malformation nidus size and diffuse nidal morphology were identified as independent risk factors for haemorrhage by multivariate analysis. Smaller arteriovenous malformation size and diffuse nidal morphology are angiographic factors independently associated with haemorrhagic presentation. Bleeding risk is important in determining the therapeutic approach (aggressive vs conservative) and timeframe, particularly in paediatric patients.