Vascular Malformations of the Head and Neck in Children.

BACKGROUND/AIM: Vascular malformations are congenital abnormalities that result from disturbances in the embryologic development of the vascular system. A retrospective study at a single institution was performed to determine the localization and treatment patterns for vascular malformations in children. PATIENTS AND METHODS: A total of 198 pediatric patients were identified. Age at diagnosis and presentation, sex, localization, diagnostics, and therapy were described. RESULTS: The most common diagnosis was lymphatic malformation (LM, 58.6%), followed by venous (VM, 31.8%) and arteriovenous malformation (AVM, 4.5%). The mean age at diagnosis was 2.2 years, while the mean age at presentation at our hospital was 7.2 years. The sex ratio showed a female predominance (1.44:1), which was most evident in children with AVM. The neck, cheek/parotid gland and oral cavity were the most predominant locations. Half of the patients required at least one intervention at our hospital. Especially, CM and LM were managed by watch-and wait, whereas lymphovenous malformation (LVM) and AVM were most often treated. Treatment differed between the various malformation types, the most common used treatment was conventional surgery followed by laser therapy. In case of treatment, the average number of procedures in our hospital was 1.58 for VM, 1.53 for LM, 1.33 for AVM, and 1.0 for LVM. CONCLUSION: In children with vascular malformations interventional treatment is often necessary, in many cases more than one treatment step is needed. Correct identification of the malformation type is important for optimal treatment and appropriate care of patients with vascular malformations.

Head and neck arteriovenous malformations: University of Tennessee experience, 2012-2022.

OBJECTIVE: Head and neck arteriovenous malformations (AVMs) are complex lesions that represent a subset of vascular anomalies (VAs). The authors present an analysis of their institutional experience managing these lesions as a multidisciplinary team. METHODS: A retrospective chart review was performed of head and neck AVM patients treated at the authors' institution from 2012 to 2022. Recorded data included patient demographic characteristics, details of clinical presentation, Schöbinger clinical scale and Yakes AVM classification results, and details of all endovascular and surgical treatments. The primary outcome of the study was clinical response to treatment. Angiographic occlusion and complication rates were reported. Chi-square tests were used for comparative statistics. RESULTS: Sixteen patients (9 female, 56%) with AVMs of the head and neck presented from age 3 to 77 years. The Schöbinger stage was stage II in 56% of patients (n = 9) and stage III in 44% of patients (n = 7). The Yakes AVM classification was nidus type (2a, 2b, or 4) in 7 patients (43%) and fistula type (1, 3a, or 3b) in 9 patients (57%). The majority of patients (n = 11, 69.0%) were managed with embolization as the only treatment modality, with an average of 1.5 embolizations/patient (range 1-3). Surgical resection was employed in 5 patients (4 in combination with embolization). Symptom resolution and symptom control were achieved in 69% and 31% of patients, respectively, in the entire cohort. A radiographic cure was demonstrated in 50% of patients. There were no statistical differences in clinical outcomes or radiographic cure rates between patients treated with different modalities. CONCLUSIONS: Head and neck AVMs can be treated successfully with a primarily endovascular management strategy by a multidisciplinary team with the goal of symptomatic control.

The trend of percutaneous and open surgical procedures for peripheral arteriovenous malformations in the National Health Service England.

INTRODUCTION: This study aimed to assess the trend of percutaneous and open surgical procedures for peripheral arteriovenous malformations (AVMs) performed in NHS hospitals in England between 2012 and 2018. METHODS: Hospital Episode Statistics (HES) is a freely available data warehouse that represents the whole population of England served by the NHS. Data from the HES database was obtained and analysed for all hospital episodes between 2012 and 2018 for the total number and trend of 'primary diagnosis', and 'primary procedures and interventions' identified for peripheral AVMs. RESULTS: Over the period studied, there was an increase in the total number of admissions for peripheral AVMs; total primary diagnosis increased from 2242 to 2857 per year. Open surgery remained more commonly performed than percutaneous procedures throughout the studied period. However, the overall percentage of primary procedures and interventions being percutaneous in this period increased from 29.8% to 41.0% per year. The increase in the number of percutaneous procedures per year seemed to occur in both children (from 43 to 124) and adults (from 408 to 492) over the course of the study period. CONCLUSIONS: This study concluded that open surgery remained the most commonly performed primary procedure for peripheral AVMs, although there was an increasing trend for percutaneous procedures in NHS hospitals in England. The increase in the number and percentage of percutaneous procedures for peripheral AVMs was likely to have significant resource implications for the provision of care for patients with peripheral AVMs in NHS hospitals.

Ischemic Stroke and Pulmonary Arteriovenous Malformations: A Review.

The potential of covert pulmonary arteriovenous malformations (PAVMs) to cause early onset, preventable ischemic strokes is not well known to neurologists. This is evident by their lack of mention in serial American Heart Association/American Stroke Association (AHA/ASA) Guidelines and the single case report biased literature of recent years. We performed PubMed and Cochrane database searches for major studies on ischemic stroke and PAVMs published from January 1, 1974, through April 3, 2021. This identified 24 major observational studies, 3 societal guidelines, 1 nationwide analysis, 3 systematic reviews, 21 other review/opinion articles, and 18 recent (2017-2021) case reports/series that were synthesized. Key points are that patients with PAVMs have ischemic stroke a decade earlier than routine stroke, losing 9 extra healthy life-years per patient in the recent US nationwide analysis (2005-2014). Large-scale thoracic CT screens of the general population in Japan estimate PAVM prevalence to be 38/100,000 (95% confidence interval 18-76), with ischemic stroke rates exceeding 10% across PAVM series dating back to the 1950s, with most PAVMs remaining undiagnosed until the time of clinical stroke. Notably, the rate of PAVM diagnoses doubled in US ischemic stroke hospitalizations between 2005 and 2014. The burden of silent cerebral infarction approximates to twice that of clinical stroke. More than 80% of patients have underlying hereditary hemorrhagic telangiectasia. The predominant stroke mechanism is paradoxical embolization of platelet-rich emboli, with iron deficiency emerging as a modifiable risk factor. PAVM-related ischemic strokes may be cortical or subcortical, but very rarely cause proximal large vessel occlusions. Single antiplatelet therapy may be effective for secondary stroke prophylaxis, with dual antiplatelet or anticoagulation therapy requiring nuanced risk-benefit analysis given their risk of aggravating iron deficiency. This review summarizes the ischemic stroke burden from PAVMs, the implicative pathophysiology, and relevant diagnostic and treatment overviews to facilitate future incorporation into AHA/ASA guidelines.

Spinal Neurovascular Malformations in Klippel-Trenaunay Syndrome: A Single Center Study.

BACKGROUND: A number of studies have demonstrated spinal anomalies associated with Klippel-Trenaunay syndrome (KTS). To date, there are no large consecutive series examining the prevalence and subtype distribution of spinal neurovascular malformations in patients with KTS. OBJECTIVE: To report the spectrum and incidence of spinal neurovascular manifestations in the KTS population. METHODS: This was a cross-sectional study. Consecutive patients with definite KTS as defined by International Society for the Study of Vascular Anomalies criteria who underwent spinal neuroimaging at our institution were included. All studies were evaluated by a staff neuroradiologist and a senior radiology resident for the presence of developmental venous anomalies, cavernous malformations (CMs), and arteriovenous shunts (AVS). RESULTS: A total of 116 patients with definite KTS who underwent spinal neuroimaging were included. A total of 23 neurovascular anomalies were found in 19 patients (16.4%), including 4 patients with multiple anomalies. These included 5 patients with spinal cord CMs (4.3%), 14 patients with a paraspinal or epidural venous malformation (12.1%), and 4 patients with an AVS (3.4%). Of the AVS, 3 were epidural arteriovenous fistulas, 1 of which likely formed de novo in an epidural venous malformation. One was a conus medullaris arteriovenous malformation. CONCLUSION: Our study cohort of 116 KTS patients demonstrated a wide spectrum of spinal neurovascular anomalies with a relatively high prevalence. Potential phenotypic descriptions of KTS should include the possibility for spinal neurovascular anomalies.

Capillary malformation-arteriovenous malformation syndrome: a multicentre study.

BACKGROUND: Capillary malformation-arteriovenous malformation (CM-AVM) syndrome is a rare syndrome with characteristic skin lesions that are associated with fast-flow vascular malformations (FFVMs) in one-third of patients. Few case series have been described, and none in Spain. AIM: To identify the prevalence of dermatological parameters, FFVMs and associated features in a large series of patients with CM-AVM. METHODS: We conducted an observational study of patients with CM-AVM syndrome diagnosed in 15 Spanish hospitals over 3 years. The main clinical, radiological, genetic findings and associated diseases were analysed. RESULTS: In total, 64 patients were assessed. In 26.5% of cases, the diagnosis was incidental. In 75% of patients, there was one significantly larger macule, which we termed the 'herald patch'. FFVMs were detected in 34% of the patients, with 30% located on the skin, 7.8% in the brain and in 1.5% in the spine. There was a positive family history in 65% of the 64 patients. Genetic analysis was performed for RASA1 mutations in 57 patients, of whom 42 (73%) had a positive result. All 4 patients tested for EPHB4 mutations had a positive result. No tumour lesions were detected in the series, except for five infantile haemangiomas. CONCLUSIONS: Our data on clinical lesions, associated FFVM, family history and genetics are similar to those previously published in the literature. An extensive data analysis failed to demonstrate any statistically significant association between the presence of an FFVM and any clinical, familial or genetic parameter that could predict its onset, although a link between the presence of a herald patch on the midline face and the presence of a brain FFVM was observed. We did not detect any genotype-phenotype correlation.

RASopathies: A significant cause of polyhydramnios?

OBJECTIVE: The aim of the study is to determine the prevalence of RASopathies in a polyhydramnios cohort selected by postnatal medical genetics evaluation. METHODS: In this retrospective study, we reviewed 622 pregnancies with polyhydramnios seen at Lucile Packard Children's Hospital between 2008 and 2017. The findings from 131 cases evaluated by Medical Genetics were included in our final analysis. Genetic testing information was extracted to determine the rate of chromosomal or single gene conditions focusing on the RASopathies. Additional variables collected were: maternal characteristics, ultrasound findings, and the severity and timing of diagnosis of polyhydramnios. RESULTS: Postnatal genetic testing or clinical examination identified a genetic disorder in 63 (48.1%) cases, more than half (n = 33) of which had a single gene condition. Postnatal testing revealed an underlying RASopathy in 15 (11.5%) cases. An underlying RASopathy was significantly associated with the severity and timing of polyhydramnios (p < 0.05). CONCLUSION: Focusing on a selected cohort postnatally evaluated by Medical Genetics, our study identified a chromosomal or genetic disorder in almost half of pregnancies complicated by polyhydramnios. Specifically, an underlying RASopathy was found in 11.5% of cases with 13/15 of these cases having additional ultrasound findings.

Pulmonary Arteriovenous Malformations in Non-hereditary Hemorrhagic Telangiectasia Patients: An 18-Year Retrospective Study.

PURPOSE: Pulmonary arteriovenous malformations (PAVMs) are most commonly associated with hereditary hemorrhagic telangiectasia (HHT). Patients with PAVMs can present with serious complications including stroke, transient ischemic attack (TIA), and brain abscess. PAVMs are rare in non-HHT patients and little is known about this patient population. The aim of this retrospective study is to better understand clinical presentation and outcomes of PAVMs occurring exclusively in non-HHT patients. METHODS: Non-HHT patients with PAVMs at the Mayo Clinic-Rochester between 01/01/2000 and 12/31/2018 were reviewed. Patients with Curacao score > 1 were excluded. Demographics, imaging characteristics, neurological complications, and follow-up imaging were analyzed. RESULTS: Seventy-seven patients with PAVMs were identified. The mean age at diagnosis was 48.2 ± 18.3 years with female preponderance (59.7%). The majority of PAVMs had lower lobe predominance (66.7%) and were simple and single in 75.3% and 89.6% of cases, respectively. Most patients were asymptomatic (46.8%) with dyspnea being the most common symptom (28.6%). Neurologic complications occurred in 19.5% of patients. The majority of PAVMs were idiopathic (61%). Thirty patients (39%) had one or more possible risk factors including previous thoracic surgery (23.4%), congenital heart disease (19.5%), and chest trauma (10.4%). Embolization was performed in 37 (48.1%) patients and only 4 (5.2%) underwent surgical resection. CONCLUSIONS: Non-HHT PAVMs occur more commonly in females, are most commonly simple and single, and have lower lobe predominance and a high rate of neurologic complications. Potential predisposing risk factors were identified in about 40% of the cases. Clinicians should be aware of the risk of PAVM development in patients with history of chest trauma, congenital heart disease, lung infection/abscess, and thoracic surgery.

Hereditary haemorrhagic telangiectasia and pulmonary arteriovenous malformations in brain abscess patients: a nationwide, population-based matched cohort study.

OBJECTIVES: The extent of hereditary haemorrhagic telangiectasia (HHT) and pulmonary arteriovenous malformations (PAVMs) as a risk factor for brain abscess is unknown. METHODS: Nationwide and population-based registries were used to identify persons with first-time hospitalization for brain abscess (index date) and population controls matched by age, sex and residence (1:10). Accounting for competing risks, cumulative incidence curves of new diagnosis of HHT/PAVM after brain abscess were constructed. Next, Cox regression was used for computation of cause-specific hazard rate ratios (HRRs) adjusted for severe liver disease and congenital heart disease as potential confounders. RESULTS: HHT/PAVM was prevalent before the index date in 2/1384 (0.1%; 95% CI 0.02-0.52) brain abscess patients and 6/13 838 (0.04%; 95% CI 0.02-0.09) matched population controls. After the index date, a new diagnosis of hereditary haemorrhagic telangiectasia or pulmonary arteriovenous malformations was made in 15/1384 brain abscess patients (range 0 days to 17 years) compared with 7/13 812 population controls yielding an adjusted hazard rate ratio of 31.4 (95% CI 9.95-98.9). Cumulative incidence was 1.5% for brain abscess patients and 0.1% for population controls. DISCUSSION: HHT/PAVM should be considered in patients with cryptogenic brain abscess, although absolute risk is low.

Hereditary haemorrhagic telangiectasia and pregnancy: a review of the literature.

BACKGROUND: Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited genetic vascular disorder that has prevalence of 1:5000 to 1:8000, and which is characterised by recurrent epistaxis, cutaneous telangiectasia, and arteriovenous malformations (AVMs) that affect many organs including the lungs, gastrointestinal tract, liver, and central nervous system. The aim here was to carry out a review of the literature on HHT complications during pregnancy in order to guide management decisions. MAIN BODY: A literature review was carried out to analyse all publications on complications that occurred during pregnancy in women with HHT. The PubMed/Medline and Scopus databases were searched. The complications observed in HHT women during pregnancy were then described. The authors identified 5 case series and 31 case reports that describe the evolution of 1577 pregnancies in 630 women with HHT. The overall maternal death rate described in the case series was estimated at 1.0% of pregnancies in the case series and 2 maternal deaths occurred in 31 pregnancy case reports. Severe maternal complications occurred in 2.7 to 6.8% of pregnancies in the case series. Severe complications occurred mostly in the second and third trimester in non-diagnosed and non-screened HHT patients. Severe complications were related to visceral involvement. The most frequent complications were related to pulmonary arteriovenous malformations (PAVMs) (haemothorax (n = 10), haemoptysis (n = 4), and severe hypoxaemia (n = 3)). Neurological complications were related to PAVMs in one case (right to left shunt) and to cerebral arteriovenous malformations (CAVM) and intracranial haemorrhage in 2 cases. Complications were related to hepatic arteriovenous malformations (HAVMs) in 8 cases (acutely decompensated heart failure due to hepatic involvement (n = 1), dyspnoea related to heart failure (n = 5), and hepatobiliary necrosis (n = 2)). CONCLUSION: Based on the literature review, most pregnancies in HHT women occur normally. However, these pregnancies should be considered high-risk, given the potential life-threatening events related to AVM rupture. Furthermore, there is currently no international consensus regarding the medical follow-up of pregnancy in women with HHT and the aim here was to carry out a review of the literature in order to guide screening and management decisions for this rare disease.

Management of extracranial arteriovenous malformations of the head and neck.

The purpose of this study was to review the outcomes of the different therapies for extracranial head and neck arteriovenous malformations (AVMs). AVMs are high-flow congenital vascular anomalies. They are composed of a complex system of vessels directly connecting feeding arteries to draining veins forming a nidus. They may be potentially life-threatening due to progressive symptoms and infiltrative disease. Extracranial AVMs most commonly affect the head and neck area (47.4%) followed by the extremities (28.5%). AVMs are best characterized as being either focal or diffuse. Focal AVMs have good outcomes following adequate treatment. Diffuse lesions have multiple feeding vessel, which results in high rates of recurrence despite treatment. The management of AVMs includes conventional surgery and endovascular techniques. A combination of embolization and surgical resection has become the treatment of choice over the last years. The main goal of both forms of treatment being the complete blockage or resection of the nidus. Transcatheter embolization of vessels has evolved over the years and new embolic agents have emerged. The types of materials available for embolization are classified into mechanical devices, liquid agents and particulates. Efficacy, rate of recurrence and most common complications were evaluated. AVMs recurrence after embolization or resection is reported in up to 80% of cases. Incomplete resection and embolization can induce aggressive growth of the remaining nidus and the risk of progression is up to 50% within the first 5 years and recurrences can occur up to 10 years later. Although ethanol seems to be associated with the highest degree of cure and permanent occlusion, the overall complication rate reported was 48%. Other materials, such as cyanoacrylate, have obtained modest rates of complete remission, while the reported rates of complete regression of AVMs with Fibrin glue and Polyvinyl alcohol are above 50%. At present, there are no unified agreement on the ideal embolic agent. Therefore, a multidisciplinary approach is recommended to support decision making about the best therapeutic approach and to achieve optimal outcome. A long-term post-treatment follow-up is recommended to recognize early recurrence.

Diagnostic Yield of Rescreening Adults for Pulmonary Arteriovenous Malformations.

PURPOSE: To determine the yield of rescreening adult hereditary hemorrhagic telangiectasia (HHT) patients with initial negative screening CT for pulmonary arteriovenous malformations (PAVMs). MATERIALS AND METHODS: Patients with a definite diagnosis of HHT were identified in the University of Toronto, Université de Montréal, and Mayo Clinic HHT databases. Inclusion criteria were: (i) definite diagnosis of HHT; (ii) initial negative PAVM screening based on bubble echocardiography and/or chest CT; and (iii) minimum 2-year imaging follow-up. A positive rescreen was defined as a newly detected PAVM on follow-up CT. Frequency of new PAVMs was calculated at 3 ± 1 years, 5 ± 1 years, 7-9 years, and ≥10 years. The primary endpoint was the rate of new PAVMs at 5 ± 1 years. RESULTS: One hundred seventy-two patients (mean age, 49.6 ± 16.7 years; 59% female) were followed for a median of 7 years. Nine patients (5.2%) had newly detected PAVMs. At the 3-, 5-, 7-, and ≥10-year time points, the cumulative rate of newly detected PAVMs was 1.8% (3/166), 5.0% (7/140), 8.8% (8/91), and 13.8% (9/65), respectively. Median feeding artery diameter was 1.3 mm. One patient had a feeding artery larger than 3 mm discovered after 6 years and was treated with embolization. The overall rate of newly detected PAVMs was 0.7%/patient-year. CONCLUSIONS: There is a definite but low rate of newly detected PAVMs in HHT patients with initial negative screening studies. No new treatable PAVMs were identified at the 5-year mark, although 1 treatable case was identified after 6 years. These findings suggest that a longer screening interval may be warranted.

A Descriptive Analysis of Spinal Cord Arteriovenous Malformations: Clinical Features, Outcomes, and Trends in Management.

BACKGROUND: Spinal arteriovenous malformations (AVM) are an abnormal interconnection of vasculature in the spine than can lead to significant neurologic deficit if left untreated. OBJECTIVE: The objective of this study was to characterize how patients with spinal AVM initially presented, what treatment options were used, and their overall outcomes on a national scale. METHODS: The MarketScan database was queried to identify adult patients diagnosed with a spinal AVM from 2007 to 2015. Trends in management, postoperative complication rates, and costs were determined. RESULTS: In total, 976 patients were identified with having a diagnosis of a spinal AVM. Patients were more commonly treated with an open incision than an embolization (40.1% vs. 15.4%). The overall complication rate was 33.61%. Spinal AVM admissions have been stable over the past decade, and mean cost of hospitalization has risen from of $48,700 in 2007 to $71,292 in 2015. Patients who underwent open surgery had a greater complication rate than those treated with embolization (31.15% vs. 18.25%, P < 0.005); however, this may be strongly influenced by complexity of spinal AVM pathology and not treatment modality. CONCLUSIONS: Costs of spinal AVM management continue to rise, even when treatment modalities have reduced length of stay significantly. Open surgery may lead to more postoperative complications and a greater length of stay than endovascular approaches. Further studies should look to identify the efficacy of endovascular approaches for spinal cord AVMs, particularly in complex spinal AVM traditionally treated with open surgery and to isolate factors leading to the elevated hospitalization costs.

Pulmonary gas exchange in hereditary hemorrhagic telangiectasia patients with liver arteriovenous malformations.

BACKGROUND: The severity of Hereditary Hemorrhagic Telangiectasia (HHT) disease is generally related to vascular visceral involvement represented by arteriovenous malformations (AVMs). Pulmonary function tests (PFTs) remain normal in HHT patients without Pulmonary AVMs (PAVMs) and respiratory comorbidity. The aim of our study was to compare the diffusing capacity of the lung for carbon monoxide (DLCO) and nitric oxide (DLNO) and its 2 components: the pulmonary capillary blood volume (Vc) and the alveolar-capillary membrane conductance (Dm), in HHT patients with PAVMs, PAVMs and liver AVMs (LAVMs), LAVMs without PAVM, no PAVM and LAVM, and controls. METHODS: Sixty one consecutive adult patients (HHT without PAVM and LAVM: n = 7; HHT with PAVMs: n = 8; HHT with PAVMs and LAVMs: n = 25; HHT with LAVMs: n = 21) and controls matched for age and sex ratio without respiratory, heart and liver pathology (n = 15) were non-invasively evaluated using PFTs, combined DLCO/DLNO, arterial blood gas at rest, contrast echocardiography and enhanced computed tomography scan of the liver and chest the day of pulmonary function testing. RESULTS: We found that patients with LAVMs but without PAVMs exhibited increased Vc/Dm ratio. Interestingly, HHT patients with hepatic artery enlargement showed higher Vc/Dm ratio than HHT patients with normal hepatic artery diameter. CONCLUSION: Vc/Dm ratio may have practical impact in HHT patients' management to detect precociously the occurrence of LVAMs. However, further studies are needed to assess the accuracy and potential prognostic value of pulmonary gas exchange measurements in HHT patients with LVAMs.

Pulmonary arteriovenous malformations.

Pulmonary arteriovenous malformation, a condition most commonly associated with hereditary hemorrhagic telangiectasia, is an abnormal communication between the pulmonary artery and pulmonary vein without an intervening capillary communication. Although asymptomatic in ~ 50% individuals, it can present with the dreaded complications of stroke or intracranial abscess in high-risk individuals including pregnant women, if untreated. The mainstay of treatment is now endovascular embolization of the feeding artery which can alleviate the symptoms and prevent these complications. In this review, we describe the pathophysiology, methods of screening, diagnostic workup and treatment of these vascular lesions with a particular focus on the currently used embolization techniques and their outcomes.

Ischemic Stroke Secondary to Paradoxical Embolism Through a Pulmonary Arteriovenous Malformation: Case Report and Review of the Literature.

Paradoxical embolism due to isolated pulmonary arteriovenous malformation (AVM) is an uncommon cause of ischemic stroke, with the majority occurring in patients who have not yet been diagnosed with their malformation. We report a 32-year-old man who presented with an abrupt onset of right facial weakness and expressive aphasia. Brain magnetic resonance imaging revealed an acute infarct in the left middle cerebral artery territory and chronic infarcts in the bilateral cerebellar hemispheres. A cardioembolic mechanism was initially considered in the setting of perimyocarditis diagnosed a few months earlier. Transthoracic and transesophageal echocardiograms revealed high volume right to left shunting, but no septal defects. A pulmonary AVM was confirmed with computed tomography angiography and fistualization was successfully treated with embolization. This report highlights a case of undiagnosed pulmonary AVM leading to recurrent paradoxical emboli to the brain. We review the epidemiology, pathophysiology, and management of pulmonary AVMs in relation to stroke risk.

The Incidence of Cranial Arteriovenous Shunts in Patients With Pulsatile Tinnitus: A Prospective Observational Study.

OBJECTIVES: Finding the underlying cause for pulsatile tinnitus can be challenging. We aimed to determine the incidence of arteriovenous shunts, i.e., arteriovenous malformations (AVMs) or dural arteriovenous fistulas (dAVFs), in patients referred for catheter angiography (digital subtraction angiography [DSA]). Furthermore, we assessed which clinical features were predictive for the presence of such a lesion. STUDY DESIGN AND METHODS: Fifty-one patients with pulsatile tinnitus, who were referred to us for DSA to exclude an arteriovenous shunt, were enrolled, prospectively. MAIN OUTCOME MEASURES: DSA determined the presence of a dAVF or AVM. Clinical characteristics were recorded systematically and all patients underwent a physical examination. RESULTS: Fifty patients were included in the final analyses. While no AVMs were found, a dAVF was found in 12 cases (24%). Three of these demonstrated cortical venous reflux, thus requiring treatment due to the risk of hemorrhage. In three cases (6%), DSA demonstrated a non-arteriovenous-shunt abnormality, likely causing the tinnitus. The odds of having a dAVF were significantly raised by unilaterality, objective bruit, and the ability to influence the tinnitus with compression. Unilaterality even had a negative predictive value of 1 and, if used as selection criterion, would have raised dAVF prevalence from 24 to 32%. CONCLUSION: In a tertiary care setting, the prevalence of dAVFs in patients with pulsatile tinnitus is not negligible. Thus, patients with unilateral pulsatile tinnitus should be offered dynamic vascular imaging to rule out a dAVF. Especially, since some of these patients are at risk of intracranial hemorrhage and treatment options exist.

Unilateral Aplasia versus Bilateral Aplasia of the Vertebral Artery: A Review of Associated Abnormalities.

Morphological characteristics of 108 cases of uni- and bilateral aplasia of the vertebral artery (VA) in reports or images of retrospective studies, including one recent case, published between 1967 and 2016 are analyzed. Incidence, gender, persistence of carotid-vertebrobasilar anastomosis (CVBA), associated with other vascular variants, and vascular pathology in each group of uni- and bilateral VA aplasia are mutually compared. Most of the cases of VA aplasia in ages 31 to 80 were discovered in USA, Japan, and India. The bilateral VA aplasia is more common in the male gender than in the female one. The side of the VA aplasia had a significant effect on the side of CVBA persistence. Associated aplasia of other arteries was more common in cases of unilateral VA aplasia. The left VA was more commonly hypoplastic in cases of single right VA aplasia than the right VA in cases of single left VA aplasia. Aneurysms of definitive arteries were more frequent in cases of single right VA aplasia than in cases of single left VA aplasia. We claim that the aplasia of the VA probably depends on genetic factors in some races, while diseases are expressed usually in persons over 30 years of age.

Pulmonary Arteriovenous Malformations Are Associated with Silent Brain Infarcts in Hereditary Hemorrhagic Telangiectasia Patients.

BACKGROUND AND PURPOSE: There is a high prevalence of right-to-left shunting pulmonary arteriovenous malformations (PAVMs), which are stroke risk factors, in hereditary hemorrhagic telangiectasia (HHT) patients. While the prevalence of ischemic complications in HHT patients is known, the prevalence of silent brain infarcts (SBI) remains unknown. The purpose of this study was to determine the prevalence and risk factors for SBI in HHT patients. MATERIALS AND METHODS: Our institutional HHT database was queried to identify HHT patients who received a baseline screening brain MRI from January 2000 to February 2017. This study group was further refined by excluding patients who had a history of clinical ischemic disease as defined by having a stroke or transient ischemic attack (TIA). Brain MRIs were reviewed for SBI. Baseline data on demographics, Curacao criteria, presence of PAVMs, and cardiovascular risk factors were collected. The primary outcome was SBI prevalence. We also examined which baseline patient characteristics were associated with SBI through univariate chi-square and Student t tests and multivariate logistic regression analyses. RESULTS: Three hundred fifty three consecutive HHT patients from January 2000 to February 2017 with a screening brain MRI and no prior history of stroke/TIA were included. SBI prevalence was 9.9% (35/353). SBI patients were more likely to have PAVMs than non-SBI patients (80.6 vs. 53.1%, p = 0.005). The median age was 66 in the SBI group and 52 in the non-SBI group (p = 0.006). SBI patients had higher prevalence of hyperlipidemia (34.3 vs. 9.8%, p < 0.0001), hypertension (48.6 vs. 22.0%, p = 0.005), and tobacco use (25.7 vs. 9.8%, p = 0.005). No patients under 30 had SBI. In the 60-69 age group, the prevalence of SBI was 18.8% with rates of 28.6% in the PAVM group and 10.5% in the non-PAVM group. For patients ≥70 years old, the prevalence of SBI was 21.4% overall and 27.6% in the PAVM group and 10.5% in the non-PAVM group. On multivariate analysis, PAVMs (OR 3.62, 95% CI 1.46-10.40) and increasing age (OR 1.04, 95% CI 1.01-1.07) were independently associated with SBI. CONCLUSIONS: Overall, a similar 10% SBI prevalence in the HHT cohort was noted as compared to the general population. However, the prevalence of SBI was higher in HHT patients with PAVMs when compared to that of the general population, particularly among patients than 60 years old. These findings highlight the need to accurately identify, and when appropriate, treat PAVMs in the HHT population especially given the multiple significant, clinical consequences of SBI.

Surgery versus percutaneous transcatheter embolization for pulmonary arteriovenous malformation: Analysis of a national inpatient database in Japan.

OBJECTIVE: Although the treatment options for pulmonary arteriovenous malformation are surgery and percutaneous transcatheter embolization, no study has compared the outcomes between these 2 treatments. METHODS: From the Japanese Diagnosis Procedure Combination database, the medical records of 996 patients who received treatment for pulmonary arteriovenous malformation between 2010 and 2015 were retrospectively reviewed. We created balanced groups for surgery or percutaneous transcatheter embolization using propensity scoring. The primary outcome was the rate of reintervention for pulmonary arteriovenous malformation, and the secondary outcomes were composite complications and postoperative length of stay. Patients who had any 1 of the complications during hospitalization were considered to have experienced a composite complication. RESULTS: Of the total sample, 211 patients underwent surgery and 785 patients underwent percutaneous transcatheter embolization. By using 1-to-1 propensity score matching, 202 pairs were selected. Compared with percutaneous transcatheter embolization, surgery was associated with a significantly higher proportion of composite complications (6.9% vs 2.0%, P = .027) and longer postoperative length of hospital stay (median, 6 vs 2 days, P < .01). However, surgery resulted in a significantly lower rate of reintervention for pulmonary arteriovenous malformation (2.1% vs 8.3% at 2 years; P < .01). CONCLUSIONS: Percutaneous transcatheter embolization had the advantage in composite complications and shorter postoperative length of stay compared with surgery, but surgery had higher curability than percutaneous transcatheter embolization. Surgery may be considered as a therapeutic option for patients with lesions that can be completely resected and are difficult to treat with percutaneous transcatheter embolization.