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Intraoperative spinal angiography via the popliteal artery for patients in the prone position has been reported only twice in 4 patients. This study aimed to clarify the safety precautions to be taken with this technique in a larger patient cohort.Seven patients with spinal vascular disorders underwent intraoperative spinal angiography in the prone position via the popliteal artery. Ultrasound was used to evaluate the neurovascular anatomy in the popliteal fossa and guide the arterial puncture. Patient characteristics, features of angiography devices, puncture attempts, and angiography-related complications, such as hematoma formation and injury to the neurovascular bundle, were analyzed.The average number of arterial puncture attempts was 1.3 times (range: 1-2). Sheaths (4 and 4.5 Fr) with different ineffective lengths were used. In 1 case, a 4.5-Fr sheath was replaced with a 4-Fr sheath with a shorter noneffective length as the length of the catheter limited access to the target. Catheters with Type-JB2 tip shapes were used for craniocervical junction lesions, and those with Type-KAGAWA tip shapes were used for thoracic and lumbar spinal lesions. No puncture site complications were observed in any patient.Intraoperative spinal angiography via the popliteal artery was an effective tool in surgeries for spinal vascular diseases. The introduction of the ultrasound enabled atraumatic puncture of the popliteal artery. Spinal targets above T5 to T6 may be inaccessible from the popliteal fossa when using a 100-cm-long catheter.

Arteriovenous malformations (AVMs) are a group of high-flow congenital vascular malformations. They are characterized by abnormal shunting of the blood supply from fast-flow feeding arteries to low-resistance draining veins via a cluster of aberrant blood vessels termed a central nidus. They are often sporadic but can be associated with syndromes. AVMs are of the most challenging vascular malformations to diagnose and manage and often lead to significant morbidity and mortality. Early diagnosis by recognizing clinical features and experienced multidisciplinary team management is essential to minimize and avoid later complications. This article focuses on clinical findings and natural history of AVMs.

The treatment strategy for ruptured brain arteriovenous malformations (bAVMs) in the acute phase is still controversial. We describe five consecutive cases of successful emergent endoscopic evacuation (EEE) of intracerebral hematoma (ICH) caused by ruptured bAVMs with the electromagnetic (EM)-neuronavigation system to avoid damage to the bAVMs intended to save valuable time in the emergent phase. A single-institution retrospective analysis was performed in patients with ruptured bAVMs treated by the EM-navigated EEE as part of the strategic multimodality therapy. EM-navigated EEE was performed as follows: 1) obtaining three-dimensional computed tomography to identify the location of the nidus, large draining vein, feeding artery, and hematoma; 2) using a supine position without rigid head fixation for both supra- and infratentorial hematoma; 3) planning the entry point and trajectory of the endoscope as far as possible from the location of the nidus using the EM-navigation system; 4) designing a linear skin incision line suitable for the endoscopic surgery as well as possible decompressive craniectomy; and 5) performing EM-navigated endoscopic partial evacuation of ICH. EM-navigated EEE of ICH was successfully performed for all 5 patients, resulting in partial removal of the ICH without rebleeding from bAVMs. The mean surgical time was 37 min. Subsequent strategic endovascular embolization and curative resection of bAVMs could be performed for all patients, achieving Glasgow Coma Scale score of 15. EM-navigated EEE of partial ICH may be valuable in the emergent phase of ruptured bAVMs with massive life-threatening ICH to reduce the intracranial pressure and to obtain better prognosis.

Background Cirsoid aneurysms, also known as arteriovenous malformations (AVMs), of the scalp are relatively rare lesions. They may be found incidentally or with symptoms such as an enlarging pulsatile mass, headache, or bleeding. Methods This retrospective case series comprised 10 cases of scalp AVMs that were treated with surgical excision from January 2010 to January 2020. Diagnosis was made with simple palpation and computed tomography angiography. Scalp AVMs were categorized according to the Schobinger classification. Results There were 10 patients, 8 males and 2 females, with a mean age of 22.6 years (range, 10–40 years). All patients underwent ligation of the feeding artery with total excision of the AVM. There were no postoperative complications or recurrences during a mean follow-up of 21.6 months. Conclusions Preoperative embolization reduces vascularity and helps in easy identification as well as complete excision of cirsoid aneurysms during surgery. However, surgical excision alone of cirsoid aneurysms also results in excellent outcomes.

A 54-year-old man with hereditary hemorrhagic telangiectasia and severe hypoxemia was referred for lung transplantation. Embolization had not been performed because of numerous bilateral small pulmonary arteriovenous malformations. Although he appeared to be qualified for lung transplantation, we instead performed bilateral thoracoscopic multiple wide wedge resections because of his age, lifestyle as a farmer, and relatively clustered distribution of arteriovenous malformations. Intermittent bilateral ventilation was needed because of poor oxygenation in the early stages of the operation, but his oxygenation improved as the resection progressed. His postoperative oxygenation improved significantly, and lung transplantation was avoided.

PURPOSE To compare coil embolotherapy outcomes of feeding-artery-only versus nidus-plus-feeding-artery technique for treating pulmonary arteriovenous malformations (PAVMs). MATERIALS AND METHODS A total of 219 treatment-naive PAVMs embolized in 90 patients at a single center from 2008 to 2018 met inclusion criteria for retrospective evaluation. Of the patients, 87% had a diagnosis of hereditary hemorrhagic telangiectasia (HHT). Feeding artery (FA) diameters ≥2 mm were treated. Coil embolization techniques were classified on the basis of embolic deployment zone: (i) distal feeding artery (DFA) technique (coil-to-nidus distance ≤ 1 cm) or (ii) nidus plus feeding artery (NiFA) technique. Successful embolization predictors were assessed using a multivariate linear regression model with input from patient- and PAVM-specific variables. RESULTS Treatment success was achieved in 192 of 219 PAVMs (87.7%) over a mean follow-up period of 19 months. Statistically significant predictors of success in the linear regression model included simple angioarchitecture, NiFA embolization technique, and shorter follow-up duration. Stratified by technique, success rates were 99 of 105 (94.3%) and 93 of 114 (81.6%) PAVMs for NiFA and DFA, respectively (P = .007). On average, NiFA-embolized PAVMs had a larger FA diameter (3.6 mm vs 2.7 mm, P < .001) and comprised more complex PAVMs (48% vs 22%, P < .001) than DFA. Treatment success was not significantly associated with sac size or FA diameter. CONCLUSIONS Coil embolization of both the nidus and FA was associated with a higher persistent occlusion rate than FA embolization alone.

BACKGROUND: It is crucial to identify a shunt point for spinal arteriovenous malformation (AVM) treatment. For this purpose, some intraoperative supports have been reported & mdash;intravenous injection of indocyanine green (ICG), selective arterial injection of ICG, and selective arterial injection of saline with a high frame rate digital camera. However, there are difficulties in accurately identifying the shunt point, especially if the lesion has multiple feeders. The aim of this technical note was to report a novel method, selective arterial injection of saline to subtract signals of ICG, to precisely identify perimedullary arteriovenous fistula shunt points having multiple feeding arteries. METHODS: After exposing the lesion, a 4-F catheter was cannulated into the origins of the segmental artery. ICG was injected intravenously as a first step, and then heparinized saline solution was flushed from the catheter. RESULTS: Compared with other methods, this method could identify the exact shunt point and was effective for certain shunt point obliterations. CONCLUSIONS: Despite having similar invasiveness, selective arterial injection of saline to subtract signals of ICG is superior to previously described techniques, such as selective arterial injection of ICG. Therefore, it will be useful in spinal arteriovenous malformation surgical treatment.

Uterine arteriovenous malformations are rare but may represent a life-threatening cause of vaginal bleeding. The typical patient affected is a multiparous woman during her thirties. The origin can be congenital or acquired, with the latter being more common after uterine surgery and presenting mainly as arteriovenous fistulous connections into the myometrium supplied by uterine arteries. The correct diagnosis of uterine arteriovenous malformations requires imaging findings of tubular and tortuous structures with mixed signal from arterial and venous flows; transvaginal color-Doppler ultrasound is the initial technique applied, then integrated with contrast-enhanced magnetic resonance or computed tomography. Multiple treatment approaches are available, including conservative-medical, endovascular embolization and surgery. Transarterial embolization represents the most applied, preserving childbearing capacity with negligible procedural complications; clinical and technical success rates are elevated, up to 90%. The goal of embolization is to occlude the point of fistula or the nidus and the application of multiple embolizing agents has been reported: despite there is no clear superiority of one over the others, liquids, especially those related to the dymethil-sulfoxide family, present relevant technical advantages. Surgery is nowadays to be considered when the endovascular approach fails and in these cases hysterectomy remains the common recommendation.

Mesenteric arteriovenous vasculopathy (MAVD/V) is an extremely rare and poorly understood disease and its incidence is probably underestimated. It is an uncommon, non-inflammatory and non-atherosclerotic form of mesenteric vascular injury, first reported in 2016, with characteristic histopathologic evidence of fibromuscular dysplasia-like vascular changes. We present the case of a chronically ill 84-year-old female with a 5 year history of recurrent small bowel obstruction, who underwent segmental resection of the small bowel. Intraoperative examination showed bowel stricture with fibrosis, intraluminal pill fragments and creeping mesenteric adipose tissue clinically compatible with Crohn's disease. Histological examination showed acute and chronic mucosal injury characterized by crypt distortion, ulcerations with granulation tissue, pseudo-pyloric metaplasia, areas of fibrosis and serosal adhesions. Multiple blood vessels (including both veins and arteries) demonstrated wall hyalinization, elastic degeneration and non-atherosclerotic luminal occlusion. The pattern of the mucosal injury is, in this case, potentially a consequence of acute and chronic ischemic processes secondary to mesenteric arteriovenous vasculopathy.

Contains fulltext : 229345.pdf (Publisher’s version ) (Closed access) PURPOSE: To evaluate treatment outcomes of embolization for peripheral arteriovenous malformations (AVMs) in a tertiary referral center where ethanol is the primary agent of choice. METHODS: A retrospective study was performed of 93 patients (median age, 31 years; range, 2-66 years) with peripheral AVMs treated with embolization (n = 442; median, 2 per patient; range, 1-82) between January 2010 and July 2016. Ethanol was used in most cases (n = 428; 97%). AVMs were classified as type I (n = 3), type II (n = 57), type IIIa (n = 5), type IIIb (n = 15), and type IV (n = 13) according to the Yakes classification system. Effectiveness of embolization was based on AVM devascularization on angiography: 100% (total), 90%-99% (near-total), 70%-90% (substantial), 30%-70% (partial), and 0%-30% (failure). Complications were graded according to the Society of Interventional Radiology classification. RESULTS: In 69% of patients, 70%-100% devascularization was achieved. Total and near-total occlusion of the nidus were more often achieved in AVMs of types I and IIIa (both 100%) than in AVMs of types II, IIIb, and IV (56%, 67%, and 39%, respectively; P = .019). A total of 109 complications were identified: 101 minor (22.9%) and 8 major (1.8%). Major complications included wounds (n = 5), false aneurysm (n = 1), finger contracture (n = 1), and severe pain (n = 1) requiring therapy. The patient complication risk was significantly affected by the number of procedures (relative risk = 2.0; P < .001). Age, AVM location, and angioarchitecture type did not significantly affect complication risk. CONCLUSIONS: AVM embolization resulted in 70%-100% devascularization in 69% of patients, with few major complications. This study indicates that the type of AVM angioarchitecture affects the number of procedures needed and the achievability of AVM devascularization.

Background Multiple high-flow arteriovenous malformations (AVMs) are the hallmark of Parkes-Weber syndrome (PWS). Surgical resection has historically shown poor outcomes with PWS. The aim of this study was to assess the management of PWS in the current era. Methods A retrospective review was performed from 2004 to 2017 on all patients presenting to a single institution for the management of PWS. Presentation, techniques, complications, and outcomes were reviewed. Results Fourteen patients (50% male) with PWS were seen at our institution, and mean age was 19.9 years (range, 4.7–68.8). The lower extremity was affected in 12 (86%) and the upper extremity in 2 (14%) patients. All patients presented with pain and swelling in the affected limb. Seven (50%) patients presented with ulcers, of which 3 (43%) had extensive wounds. Five (36%) patients had echocardiographic evidence of high-output cardiac failure. All patients underwent angiography with the intention to treat. Three (21%) patients were found to have diffuse arteriovenous communication with no discrete AVM nidus and thus did not undergo intervention. The remaining 11 (79%) patients underwent transcatheter embolization of the AVM's arterial inflow. Six (55%) patients required multiple or staged inflow embolization procedures, with a mean of 3.3 (range, 1–10) interventions. Thirty-two arterial embolization procedures were performed in total. n-Butyl-2-cyanoacrylate (nBCA) adhesive was used in 22 (69%), microspheres in 8 (25%), and a combination of coils and nBCA adhesive in 2 (6%) cases. Technical angiographic success was seen in all patients. Six (55%) patients also had interventions to treat the venous component of the malformation, either concomitantly or during a separate procedure. This included radiofrequency ablation in 1 (17%), coil embolization in 1 (17%), sodium tetradecyl sulfate (STS) sclerotherapy in 2 (33%), and a combination of STS, coil embolization, and vein stripping in 2 (33%) patients. Ten (91%) patients experienced a partial response and 1 (9%) patient experienced no response to treatment. No patients had a complete response, as expected with the diffuse nature of this disease. There were no periprocedural complications. Two of 3 patients with complex wounds required major amputations for gangrene, including one above-knee and one below-knee amputation at 128 months and 66 months after the index procedure, respectively. Conclusions AVMs in PWS can be successfully treated by a transcatheter approach. Multiple interventions are usually required. Patients with extensive wounds remain at risk for loss of limb.

Background Spinal dural arteriovenous fistulas (DAVFs) and spinal arteriovenous malformations (AVMs) are relatively rare. Moreover, multiple spinal cord vascular lesions in an individual, such as double DAVFs and DAVF with AVM, are extremely rare. To date, there are only several reported cases of concomitant spinal DAVFs and AVMs. Case Description A 25-year-old woman presented with pain of the right lower limb persisting for 1 month. Spinal magnetic resonance imaging showed prominent signal flow voids in the lumbosacral canal. Spinal digital subtraction angiography (DSA) showed a sacral DAVF fed by the right lateral sacral artery and a spinal intradural AVM at the conus medullaris fed by the anterior spinal artery from the left T11 intercostal artery. The drainage veins in these 2 lesions were respective and without common anastomosis. The diagnosis of concomitant sacral DAVF and conus AVM was made. Microsurgery was performed to excise these lesions successfully in the same operation. The patient's symptoms gradually improved after surgery. Postoperative spinal DSA confirmed the complete disappearance of the 2 lesions. Conclusions The concomitant presence of sacral DAVF and conus AVM is extremely rare. Nonetheless, clinicians should be aware of the possibility of the coexistence of multiple types of spinal vascular lesions in a single patient.

Background Radiation-induced spinal cord cavernous malformations (CMs) are rare pathologies compared with radiation-induced cerebral CMs. We present a case of a radiation-induced spinal cord CM developed 31 years after radiation therapy for medulloblastoma. Case Description A 37-year-old man developed a symptomatic spinal hemorrhagic lesion 31 years after radiation therapy for medulloblastoma. Magnetic resonance imaging revealed an intramedullary cystic lesion with a fluid-fluid level in the C7 area. Surgery was performed leading to an unclear diagnosis. Two years later, the patient had a relapse and underwent a second operation, allowing a definitive diagnosis of radiation-induced spinal cord CM. This is believed to be the second case of de novo intramedullary CM formation following spinal radiation therapy for medulloblastoma. Conclusions Radiation-induced spinal cord CMs should be recognized as a possible late adverse effect in patients treated with radiation therapy for medulloblastoma.

Objective The objectives of this study were to document the results of using fibrin glue (FG) combined with pingyangmycin (PYM) for the embolism and sclerotherapy of maxillofacial arteriovenous malformations (AVMs). Study Design We reviewed the associated clinical data from December 2012 to June 2017 for 25 patients with maxillofacial AVMs. The major treatment method was direct percutaneous puncture and injection of FG combined with PYM. Treatment outcomes were assessed through physical examination, Doppler ultrasonography, computed tomography, and 3-dimensional computed tomography angiography scans. Follow-up time ranged from 12 months to 3 years after the last treatment (mean 21 months). Results Of the 25 lesions, 80% showed greater than 90% reduction, 12% showed greater than 75% reduction, and 8% showed greater than 50% reduction. Superficial skin necrosis or mucous ulcer occurred in 3 patients and healed without intervention. Regrowth was observed in 3 patients with extensive lesions involving multiple anatomic regions. Conclusions These data suggest that embolization and sclerotherapy with the use of FG combined with PYM are safe and effective for the treatment of small- to medium-sized, locally dilated maxillofacial AVMs. For AVMs involving multiple anatomic regions, combined application of this approach with other options should be considered.

Cerebral artery fenestrations detected incidentally during computed tomographic angiography (CTA) or magnetic resonance angiography (MRA) are reported to be associated with aneurysmal dilatation, which may cause cerebrovascular diseases, arteriovenous malformations, or, rarely, ischemic symptoms.We retrospectively analyzed CTA and MRA of patients with cerebral artery fenestration examined between January 2014 and December 2017. The location, shape, and other associated vascular diseases were described.Two hundred eleven cerebral artery fenestrations were found in 208 patients for a detection rate of 1.13% (208/18,360). Basilar artery fenestrations were most common, accounting for 50.2% (106/211). The fenestration was5 mm in 115 patients (54.5%), 5-10 mm in 63 (29.9%), and ≥10 mm in 33 (15.6%). Forty-one patients had other vascular malformations, including 29 aneurysms. Except for one aneurysm, which was at the site of the fenestration, all other aneurysms were separate from the fenestrations. 26 patients had cerebral infarctions; among them, 11 had cerebral infarctions in the blood supply area of the arterial fenestration.Cerebral artery fenestration is an uncommon finding at cerebral imaging, but mostly affects the basilar artery. Cerebral artery fenestrations could be associated with cerebrovascular diseases and malformations, but the present study could not evaluate the cause-to-effect relationship.

Background Verification of complete occlusion or resection of neurovascular lesions is often performed using intraoperative angiography. Surgery for spinal vascular lesions such as arteriovenous malformations (AVMs) and arteriovenous fistulas (AVFs) is typically performed with the patient in the prone position, making intraoperative angiography difficult. No standardized protocol is available for intraoperative angiography during spinal surgery with the patient in the prone position. We have described our experience using radial artery access for intraoperative angiography in thoracic spinal neurovascular procedures performed with the patient in the prone position. Methods We reviewed the data from all patients who had undergone surgical resection of spinal vascular lesions in the prone position with radial artery vascular access for intraoperative angiography. The patients were treated in a hybrid endovascular operating room. Results A total of 4 patients were treated in the prone position using transradial artery access intraoperative angiography for confirmation of complete resection of the vascular lesions. Of the 4 patients, 2 had undergone surgery for a dural AVF, 1 for a pial AVF, and 1 for an AVM of the filum terminale. None of the patients had experienced any procedural complications. Conclusion Radial artery access for intraoperative angiography during spinal neurovascular procedures in which selective catheterization of a thoracic branch is necessary is feasible, safe, and practical.

Purpose To prospectively evaluate the efficacy and safety of embolization using hydrogel-coated coils for the treatment of pulmonary arteriovenous malformations (PAVMs). Materials and methods The outcomes of 21 PAVMs in 19 patients (3 men and 16 women; mean age, 58.8 ± 15.2 [SD] years; age range 14–78 years) treated by venous sac embolization (VSE) with additional feeding artery embolization were prospectively evaluated. For VSE, using one or more 0.018-inch hydrogel-coated coils was mandatory. Recanalization and/or reperfusion were evaluated by pulmonary arteriography 1 year after embolization. Results The mean feeding artery and venous sac sizes were 4.0 mm and 8.5 mm, respectively. Embolization was successfully completed in 20/21 PAVMs, yielding a technical success rate of 95%. The feeding artery was also embolized in 17/20 successful PAVMs (85%). A technical failure occurred in one PAVM, where embolization was abandoned because of migration of one bare coil to the left ventricle. The mean numbers of hydrogel-coated coils and bare platinum detachable coils used for VSE were 3.3 ± 2.1 (SD) (range, 1–8) and 4.4 ± 3.9 (SD) (range, 1–17), respectively. The mean percentages of hydrogel-coated coils in number, length, and estimated volume were 42.9%, 33.3%, and 72.7% respectively. One patient with one PAVM was lost to follow-up after 3 months. Neither recanalization nor reperfusion was noted in the remaining 19 PAVMs (success rate, 19/19 [100%]). One grade 4 (coil migration) adverse event occurred, and it was treated without any sequelae. Conclusion VSE using hydrogel-coated coils with additional feeding artery embolization is a safe and effective treatment for PAVM.

Introduction Persistent trigeminal artery (PTA) is the most common remnant of primitive circulation communicating the developing carotid and vertebrobasilar junction. Although discovered incidentally, an altered hemodynamic may lead to an increased association of aneurysms, vascular malformations, and stroke. Neurosurgeons should be aware of the presence and significance of PTA when interpreting imaging and planning interventions. Methods We retrospectively reviewed all magnetic resonance angiography and cerebral digital subtraction angiography performed between 2012 and 2017 for the presence of PTA. The radiologic and anatomic details were noted and analyzed along with the clinical profiles. We categorized the radiologic findings with respect to the available classification systems. A review of the available literature was done comparing our results. Results We found 33 cases of incidentally detected PTA. The average age of the patients was 45.42 years. The lateral surface of the proximal cavernous internal carotid artery was the most common origin (n = 23). Only 3 cases had a medial/transsellar course. Most cases were Saltzman/Weon type I (19/33). Intracranial aneurysms were associated with 6 patients (18.18%). Trigeminal neuralgia (TN) was a presenting feature in 5 patients. None had a direct neurovascular conflict at the root entry zone. Conclusions Our study is one of the largest to describe the incidence of PTA. We emphasized the importance of PTA to the neurosurgeons; increased association of aneurysms, as a route for intervention in occlusive disease of the posterior fossa; risk of injury and bleeding during transsphenoidal surgery; and the association with TN. However, we found that only PTA variants are likely to be associated with TN because of their cisternal course causing NV conflict.

Highlights • A massive arterio-venous malformation (AVM) on the buttock of a young girl. • Recurrent embolisation and surgery made total resection possible. • This is the first report from the Middle East of such a case in a child. • This AVM was sucessfully managed by repeated embolization and resection. • Management should not be postponed from financial disadvantge., Background Arteriovenous malformations (AVMs) are rare congenital lesions that affect multiple regions. AVMs often affect the brain and the buttock is the least common. They are usually congenital, but discovered later in life as they are asymptomatic, small and resemble a benign lesion. However, they can abruptly grow and become symptomatic and life-threatening as they can bleed and get infected. Presentation of case An eight-year-old girl presented with ulcerated and bleeding AVM at the sacro gluteal region. However, due to financial difficulty, it grew to reach an enormous size of (15*15*2 cm) in the buttock. It was later managed by multiple embolisation followed by total surgical resection. Discussion This case reflects the first case of enormous AVM in the sacro-gluteal region in a child in the Middle East as they rarely reach such huge sizes. Total resection was possible by repeat embolisation and surgery. Conclusion Embolisation can render even huge AVM operable. AVMs should be treated as early as possible as they continue to grow and drastically decrease the quality of life of the patient.

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, Universite de Montreal, 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.

Objective Spinal arteriovenous malformations (SAVMs) are a very rare and complex spinal cord pathology that require high clinical acumen to diagnose and treat. Management includes both nonoperative and operative paradigms. A review of the literature yields a paucity of data regarding the surgical outcomes of SAVMs, with the majority of data limited to single-center outcomes and/or small sample sizes. The purpose of this study was to use a multi-institutional international database to study the natural history of SAVMs. Methods We used the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP) database to select patients that underwent laminectomy for surgical excision of a SAVM between 2008 and 2017. Results The data from 196 patients were studied (65.8% male, 34.2% female). A majority of cases were in the thoracic region (53.6%), followed by thoracolumbar (31.6%) and cervical (14.8%) regions. The mean age was 57.4 years and 52.5% patients were graded ASA class 3–5 before the operation. The mean operation time was 215 minutes, with a significantly lower operative time for thoracic arteriovenous malformations (195.6 minutes) when compared with cervical (266.6 minutes) and thoracolumbar (223.7 minutes). The mean length of hospital stay was 6.4 days. Patients had a 6.6% readmission rate and a 4.6% reoperation rate within 30 days. Conclusions This study presents the largest analysis of patients undergoing surgery for SAVMs and 30-day postoperative outcomes. Operative time differed based on SAVM location. The three most frequent complications (deep vein thrombosis, wound infection, and UTI) occurred at rates of 3.6% or less.

Brain arteriovenous malformations (AVMs) are a disorder wherein abnormal, enlarged blood vessels connect arteries directly to veins, without an intervening capillary bed. AVMs are one of the leading causes of hemorrhagic stroke in children and young adults. Most human sporadic brain AVMs are associated with genetic activating mutations in the KRAS gene. Our goal was to develop an in vitro model that would allow for simultaneous morphological and functional phenotypic data capture in real time during AVM disease progression. By generating human endothelial cells harboring a clinically relevant mutation found in most human patients (activating mutations within the small GTPase KRAS) and seeding them in a dynamic microfluidic cell culture system that enables vessel formation and perfusion, we demonstrate that vessels formed by KRAS4A(G12V) mutant endothelial cells (ECs) were significantly wider and more leaky than vascular beds formed by wild-type ECs, recapitulating key structural and functional hallmarks of human AVM pathogenesis. Immunofluorescence staining revealed a breakdown of adherens junctions in mutant KRAS vessels, leading to increased vascular permeability, a hallmark of hemorrhagic stroke. Finally, pharmacological blockade of MEK kinase activity, but not PI3K inhibition, improved endothelial barrier function (decreased permeability) without affecting vessel diameter. Collectively, our studies describe the creation of human KRAS-dependent AVM-like vessels in vitro in a self-assembling microvessel platform that is amenable to phenotypic observation and drug delivery.

Hereditary hemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber syndrome, is an autosomal dominant vascular disorder with low prevalence that presents a wide variety of clinical manifestations due to multiple vascular lesions in a wide variety of organs and tissues. Orthotopic liver transplantation is the only treatment option for arteriovenous malformation in HHT. These patients require complicated anesthetic management. We present the case of a 63-year-old woman with HHT of 18 years' evolution, intrahepatic portal systemic shunts, and severe pulmonary hypertension, with dyspnea at minimum effort.

Background Renal arteriovenous malformation (AVM) is a rare cause of massive hematuria, and patients with renal AVM may present with symptoms like urinary tract infections in the emergency department. Case Report A 37-year-old woman presented to the emergency department with symptoms of hematuria, urinary hesitancy, and severe suprapubic pain that had been present for a few hours. A urine examination revealed no pyuria, but urine occult blood for 3+ and a red blood cell count of >100 per high-power field. Bedside echocardiography revealed right kidney hydronephrosis and a distended bladder with a blood clot. A 3-way Foley catheter was inserted and drained 800 mL of bloody urine. A contrast-enhanced computed tomography scan was ordered that showed a 1.1-cm hypervascular tumor in the lower pole of right kidney, with active bleeding and rupture into the adjacent collecting system. Active renal tumor bleeding or renal AVM was suspected. The patient was transferred to a tertiary medical center where right renal artery angiography was arranged and disclosed an AVM with aneurysm formation at the right renal lower pole. Transarterial embolization was performed immediately to embolize the 3 feeders of the AVM. Why Should Emergency Physicians Be Aware of This? Renal AVM is a rare but potentially life-threatening cause of massive hematuria. Delayed or missed diagnosis is possible because renal AVM may present with symptoms like urinary tract infection, especially in young females. Renal artery angiography is the diagnosis of choice, and emergent transarterial embolization is now the standard of treatment.

Hereditary hemorrhagic telangiectasia (HHT) is a Mendelian disease characterized by vascular malformations (VMs) including visceral arteriovenous malformations and mucosal telangiectasia. HHT is caused by loss-of-function (LoF) mutations in one of three genes, ENG, ACVRL1, or SMAD4, and is inherited as an autosomal-dominant condition. Intriguingly, the constitutional mutation causing HHT is present throughout the body, yet the multiple VMs in individuals with HHT occur focally, rather than manifesting as a systemic vascular defect. This disconnect between genotype and phenotype suggests that a local event is necessary for the development of VMs. We investigated the hypothesis that local somatic mutations seed the formation HHT-related telangiectasia in a genetic two-hit mechanism. We identified low-frequency somatic mutations in 9/19 telangiectasia through the use of next-generation sequencing. We established phase for seven of nine samples, which confirms that the germline and somatic mutations in all seven samples exist in trans configuration; this is consistent with a genetic two-hit mechanism. These combined data suggest that bi-allelic loss of ENG or ACVRL1 may be a required event in the development of telangiectasia, and that rather than haploinsufficiency, VMs in HHT are caused by a Knudsonian two-hit mechanism.

Background Navigated transcranial magnetic stimulation (nTMS) provides a reliable identification of “eloquent” cortical brain areas. Moreover, it can be used for diffusion tensor imaging fiber tracking of eloquent subcortical tracts. We describe the use of nTMS-based cortical mapping and diffusion tensor imaging fiber tracking for defining the “eloquence” of areas surrounding brain arteriovenous malformations (BAVMs), aiming to improve patient stratification and treatment. Methods We collected data of BAVMs suspected to be in eloquent areas treated between 2017 and 2019, and submitted to nTMS-based reconstruction of motor, language, and visual pathways for the definition of the eloquence of the surrounding brain areas. We describe the nTMS-based approach and analyze its impact on patient stratification and allocation to treatment in comparison with the standard assessment of eloquence based on anatomical landmarks. Results Ten patients were included in the study. Preliminarily, 9 BAVMs were suspected to be located in an eloquent area. After nTMS-based mapping, only 5 BAVMs were confirmed to be close to eloquent structures, thus leading to a change of the score for eloquence and of the final BAVMs grading in 60% of patients. Treatment was customized according to nTMS information, and no cases of neurological worsening were observed. Radiological obliteration was complete in 7 cases microsurgically treated, and accounted for about 70% in the remaining 3 patients 1 year after radiosurgical treatment. Conclusions The nTMS-based information allows an accurate stratification and allocation of patients with BAVMs to the most effective treatment according to a modern, customized, neurophysiological identification of the adjacent eloquent brain networks.

Purpose To assess pulmonary arteriovenous malformation (PAVM) growth among patients with untreated PAVMs using imaging from long-term follow-up per hereditary hemorrhagic telangiectasia international guidelines. Materials and Methods Analysis included 88 untreated PAVMs from 21 patients (6 male;15 female; mean age at presentation 47 y; range, 12–68 y). Two CT studies with the longest interval between were evaluated (mean 8.4 y; median 8.8 y; range, 3.1–14.1 y). Measurement of feeding artery diameter and anteroposterior (AP) and mediolateral (ML) sac dimensions for each PAVM was performed separately by 2 radiologists blinded to patient and CT order. Statistical analysis was performed to determine change in size between earliest and follow-up imaging. Results Mean feeding artery diameter, AP sac dimension, and ML sac dimension were 1.4 mm (range, 0.8–3.7 mm), 4.3 mm (range, 2.1–11.1 mm), and 4.1 mm (range, 1.8–9.2 mm) on earliest imaging and 1.4 mm (range, 0.9–2.6 mm), 4.5 mm (range, 2.2–12.2 mm), and 4.3 mm (range, 2.0–9.6 mm) on follow-up. Model-based mean analysis showed no statistically significant change in dimension of any variable between earliest and follow-up imaging. Secondary analysis including age also found no statistically significant difference (feeding diameter, P = .09; AP sac dimension, P = .9; ML sac dimension, P = .1). Analysis including time between measurements found no significant relationship between change in variables and time (feeding artery diameter, P = .4; AP sac dimension, P = .3; ML sac dimension, P = .06). Conclusions Untreated PAVMs grew slowly, if at all, in a near-decade span, and any demonstrated growth was minimal. These findings challenge the current recommendation of 3- to 5-year CT follow-up.

Purpose To assess the efficacy and safety of the AMPLATZER Vascular Plug type IV for pulmonary arteriovenous malformation (PAVM) treatment. Materials and Methods Between June 2013 and January 2018, 13 patients with 26 PAVMs were treated with the type IV AVP. Patients without follow-up computed tomography (CT) were excluded. Technical success was defined as flow occlusion on angiography. Plug-to-sac distance was measured on angiographic images. Feeding artery and venous sac diameter changes were measured on preprocedural and follow-up CT. Successful embolization was defined as > 70% sac size regression. Procedure time, device migration, and complications were evaluated. Results Nine female patients (mean age, 49 y; range, 40–71 y) with 19 PAVMs were enrolled. Four patients with 7 PAVMs were lost to follow-up. Nineteen PAVMs were treated in 11 sessions, and the mean procedure time was 29 min. The technical success rate was 100%. Mean feeding artery diameter was 3.1 mm ± 0.7 (range, 2.1–4.9 mm). Mean plug-to-sac distance was 5.4 mm ± 4.9 (range, 0–13.3 mm). The mean CT follow-up period was 14 months ± 7 (range, 6–30 mo). Sixteen of 19 PAVMs (84%) were successfully embolized. Minor complications (tachycardia and chest discomfort) arose in 2 of 11 sessions. No device migrations or major complications occurred. Conclusions The type IV AVP showed an 84% treatment success rate based on 70% sac size regression criteria in small PAVMs. There were no device migrations or major complications.

Purpose To evaluate effectiveness of the polytetrafluoroethylene-covered nitinol mesh microvascular plug (MVP) and compare it with other devices in pulmonary arteriovenous malformation (PAVM) embolization in patients with hereditary hemorrhagic telangiectasia (HHT). Materials and Methods Twenty-five patients (average age 35 y; range, 15–56 y) with hereditary hemorrhagic telangiectasia (HHT) and de novo PAVM embolization with at least 1 MVP between November 2015 and May 2017 were retrospectively evaluated. Retrospective data were also obtained from prior embolization procedures in the same patient population with other embolic devices dating back to 2008. Technical success, complications, PAVM persistence rates, and category of persistence were analyzed. Results In 25 patients, 157 PAVMs were treated: 92 with MVP, 35 with AMPLATZER vascular plug (AVP), 6 with AVP plus coils, and 24 with coils. The per-PAVM technical success rates were 100% with MVP; 97%, AVP; 100%, AVP plus coils; and 100%, coils. PAVM persistence rates and median follow-up were as follows: MVP, 2% (1/92) (510 d); AVP, 15% (3/20) (1,447 d); AVP plus coils, 20% (1/5) (1,141 d); coils, 46.7% (7/15) (1,141 d). Persistence owing to recanalization for MVP, AVP, AVP plus coils, and coils was 2%, 15%, 0%, and 33%. No difference was found between persistence rates of MVP vs AVP (P = .098). Embolization with a vascular plug (MVP or AVP) with or without coils had a statistically significant lower persistence rate (5.4%) than embolization with coils alone (46.7%) (P = .022). Conclusions PAVM embolization with MVP had a high technical success rate and a low persistence rate comparable to AVP and lower than coil embolization alone.

Background Advances in acute and long-term poststroke care have resulted in improved survival and functional outcomes for patients who have suffered large vessel ischemic strokes. For years, tissue plasminogen activator was the mainstay of treatment for acute stroke. Its use was previously limited to patients without known comorbid intracranial vascular pathology because of concern for bleeding risk. More recently, however, the use of tissue plasminogen activator in select patients with vascular anomalies has increased and is now largely thought to be safe. With the safety and efficacy of mechanical thrombectomy now proven for large vessel occlusions (LVOs), similar investigation is needed to assess procedural safety in patients with concomitant arteriovenous (AV) malformations or fistulae. Methods We reviewed patients treated for LVOs at our institution and those of our collaborators and identified 6 patients who were treated for LVO with either known or incidentally identified concomitant AV malformations or dural AV fistulae. Results We present a case series of 6 patients with nonaneurysmal intracranial vascular lesions who underwent mechanical thrombectomy for LVO without complications related to these lesions. Conclusions Although limited by small size, our series adds to the literature evidence that mechanical thrombectomy for LVO can safely be performed with concomitant dural AV fistulae and AV malformations.

Purpose The purpose of this study was to evaluate the management, outcomes, and technical and clinical safety of coil-assisted dominant outflow vein (DOV) occlusion for the ethanol embolization of high-flow arteriovenous malformations (AVMs) in the hands. Materials and Methods Between March 2013 and October 2016, 12 consecutive patients with AVMs with DOVs underwent ethanol embolization combined with detachable and pushable coil-assisted DOV occlusion. All patients completed the course of clinical follow-up (range: 14–57 months; mean: 36.7 months), and imaging follow-up (range: 8–25 months; mean: 16.6 months) results from the final treatment session were available for 8 patients. The therapeutic effects, degree of devascularization, and complications at the time of follow-up arteriography were evaluated as the clinical outcomes. Results The patients underwent 23 ethanol embolization procedures (range: 1–3; mean, 1.9) with 24 detachable coils and 223 pushable coils. The average stretched length of the total coils per patient was 320.17 cm. Seven of 12 patients (58.3%) exhibited complete responses, and 5 patients (41.7%) exhibited partial responses. Minor complications, including blistering and focal swelling, occurred in all 12 patients (100%) but showed spontaneous and complete recovery. No major complications occurred. Conclusions Ethanol embolization has the potential to control high-flow hand AVMs by using coil-assisted DOV occlusion with an acceptable risk of minor and major complications.

Objective This study investigated the efficacy of GnRH agonists concomitantly with transient aromatase inhibitor and tranexamic acid to treat women with uterine arteriovenous malformations (AVMs) associated with abnormal uterine bleeding (AUB) to preserve fertility and determine reproductive outcome. Methods This was a prospective cohort study in a tertiary centre. Doppler ultrasound demonstrated AVM in 19 women with AUB 1–28 weeks following spontaneous or therapeutic abortion and in one woman 4 years after normal pregnancy while taking an oral contraceptive. On the basis of experience from the first three cases, 17 women were treated with tranexamic acid (1 g three times daily orally for 5 days), a GnRH agonist (3.75–11.25 mg, for 1–3 months), plus an aromatase inhibitor (letrozole 2.5 mg once daily for 5days) with the initial injection of GnRH agonist. Two women required blood transfusion, and one required uterine tamponade with Foley catheter balloon in the first 48 hours to control heavy bleeding (Canadian Task Force Classification II-2). Results All 20 AVMs resolved within 1–3 months of treatment. Of 16 women who attempted pregnancy, all (100%), including two who had uterine artery embolization (one after hysteroscopic septoplasty), conceived spontaneously with 18 live births. Two women are using contraception (one taking an oral contraceptive, one using a levonorgestrel intrauterine system), and one 40-year-old is not using contraception. One woman had hysteroscopic endometrial ablation followed by vaginal hysterectomy for AUB at 1 and 2 years later. Conclusion A GnRH agonist in combination with transient aromatase inhibitor and tranexamic acid is an effective management strategy to treat and maintain reproduction in women with AVMs associated with AUB.

Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant inherited Rare Disease that causes a systemic anomalous vascular overgrowth. The approach and follow-up of these patients should be from multidisciplinary units. Its diagnosis is carried out according to Curaçao clinical Criteria. Telangiectasia in the nasal mucosa cause recurrent epistaxis, the main symptom of HHT and difficult to control. The three types of hepatic shunting, hepatic artery to hepatic vein, hepatic artery to portal vein or to portal vein to hepatic vein, can cause high-output heart failure, portal hypertension or porto-systemic encephalopathy, respectively. These types of vascular involvement can be established using computerised tomography. Pulmonary arteriovenous fistula should be screened for all HHT patients by contrast echocardiography. The main objective is to review the management of epistaxis, liver and lung involvement of the adult patient with HHT.

The objective of this study was to investigate the factors associated with the complication rate and treatment outcomes of arteriovenous malformations (AVMs) during a 20-year period.This was a retrospective study of 306 patients (135 men, 171 women; mean age, 30.8 years) with body and extremity AVMs who were treated between 1996 and 2017. A total of 913 sessions of endovascular treatment were performed. Patients were divided into two decades of the study period to compare complications and clinical results. Group 1 comprised 107 patients treated in the first decade of the study period, and group 2 comprised 199 patients treated in the last decade. AVMs were classified according to the angiographic findings. Complication rates, number of treatment sessions, and treatment results were compared between the two groups.Minor complication (group 1, 20.1%; group 2, 18.5%) and major complication (group 1, 3.1%; group 2, 4.1%) rates were similar between groups (P = .79). The mean number of treatment sessions in group 1 and group 2 was 4.2 and 2.3, respectively, indicating a 45% reduction in treatment sessions (P .0001). The treatment failure rate decreased from 9.3% in group 1 to 1.5% in group 2 (P = .04). The clinical success rate was 54.2% in group 1 and 64.3% in group 2 (P = .10).With an accumulation of AVM treatment experience, the number of treatment sessions and the rate of treatment failures were significantly reduced.

Objective Spinal arteriovenous malformations (SAVMs) and intramedullary spinal cord cavernous malformations (ISCCMs) have a very low incidence of disease. The purpose of this study was to compare the differences in electrophysiologic monitoring in these 2 surgeries. Methods The study included 109 patients (SAVMs, n = 55; ISCCMs, n = 54) recruited from November 2012 to January 2016. All patients underwent electrophysiologic monitoring during the entire operation, including somatosensory-evoked potentials, motor-evoked potentials, and electromyography. We used an amplitude reduction of >80% as warning criterion for motor-evoked potentials and an amplitude reduction of more than 50% and latency prolongation of more than 10% as warning criteria for somatosensory-evoked potentials. Results In our study, the sensitivity and specificity of intraoperative monitoring during SAVM surgery were 77.3% and 87.1%, respectively. The sensitivity and specificity of intraoperative monitoring during ISCCM surgery were 68.8% and 83.3%, respectively. We found that 21 patients with SAVM showed permanent changes, 17 had immediate postoperative impairment, 8 recovered before discharge, and 5 showed neurologic deficits at long-term follow-up. Of the 17 patients with ISCCMs showing permanent changes, 11 had immediate postoperative impairment, 5 recovered before discharge, and 2 had long-term residual neurologic deficits. Conclusions Electrophysiological monitoring provides effective guidance during operation on spinal vascular malformations. Electrophysiologic monitoring revealed that surgical resection of SAVMs resulted in more permanent changes and postoperative dysfunction when compared with ISCCMs. The incidence of both false-positive and -negative results suggests that electrophysiologic monitoring cannot fully predict the complete function of the patients.

Study Objective To evaluate fertility, pregnancy, and clinical outcomes after uterine arteriovenous malformation (UAVM) management. Design Single-center retrospective study (Canadian Task Force classification III). Setting One referral center. Patients Twenty-two patients with a UAVM diagnosed by magnetic resonance angiography or computed tomography angiography and managed by expectant management (EM) or uterine arterial embolization (UAE) during the study period were included. Interventions Nine of 22 patients underwent first-intention EM and 13 first-line UAE. Three of 9 EM patients (33.3%) required emergency second-intention UAE for nonresolution of the UAVM and severe genital bleeding. Measurements and Main Results To analyze fertility according to management approach, we defined 2 groups: EM only group (n = 6) and UAE group (n = 16; women who underwent first- or second-intention UAE). Overall, the median age was 29 years (range, 17–43). The mean follow-up after UAVM management was 39 months (range, 1–116). The success rate of the UAE procedure was 87.5% (14/16). Eight of 12 women (66.7%) who wished to conceive became pregnant: 2 of 5 (33.3%) in the EM group and 6 of 7 (85.7%) in the UAE group (p = 1). Overall, 11 patients (50%) conceived: 3 in the EM group (50%) and 8 in the UAE group (50%) (p = .9). The live birth rate was 36.4% (8/22) for the whole population. There were no miscarriages or ectopic pregnancies. The 8 women (72.7%) wishing to conceive who became pregnant all delivered: 1 by cesarean section and 7 by vaginal delivery. Median duration of pregnancy was 39 weeks and 5 days. The remaining 3 women (i.e., women who conceived but did not wish to become pregnant) had therapeutic abortions. One complication was experienced in the EM group (small for gestational age newborn) and none in the UAE group (p = .3). Limitations include retrospective design and small sample size. Conclusion UAE for UAVM is an effective and safe technique. It does not impair fertility, and obstetric prognosis is good.

To describe the effectiveness and safety of a sclerotherapy protocol with 5% ethanolamine oleate (EO) at 0.1 mL/3 mm for oral vascular anomalies (OVAs). Our hypothesis is that EO applied at a concentration of 5% may decrease the number of sessions necessary for clinical healing.We describe a cohort of 15 consecutive patients. OVAs20 mm were included. Clinical data of the OVAs were collected. Descriptive and bivariate statistical analyses were performed.Fifteen of the 19 OVAs were varicosities and the lower lip was the most affected site (n = 7). The median size was 6 mm, and one session was required in 89.5% of cases for clinical healing within 28 days. The pain/burning score was low (2) for most lesions (63.1%) and the degree of satisfaction was high (8) for all OVAs. The number of applications, final volume of drug and time to resolution differed significantly according to the size of the anomaly.The protocol with 5% EO was shown to be effective and safe to treat OVAs20 mm, and with a decrease in the number of sessions, volume and time to resolution, without complications and with high patient satisfaction.

Vascular endothelial growth factors regulate vascular and lymphatic growth. Dysregulation of VEGF signaling is connected to many pathological states, including hemangiomas, arteriovenous malformations and placental abnormalities. In heart, VEGF gene transfer induces myocardial angiogenesis. Besides vascular and lymphatic endothelial cells, VEGFs affect multiple other cell types. Understanding VEGF biology and its paracrine signaling properties will offer new targets for novel treatments of several diseases.

Objective: Uterine arteriovenous malformation (AVM) is an abnormal and nonfunctional communication between uterine arteries and veins, currently managed by uterine artery embolization (UAE). Pulmonary embolism (PE) is the most severe and life-threatening complication of this procedure. Case report: We report a case of 27 year-old woman with heavy vaginal bleeding and abdominal pain caused by AVM. UAE was performed uneventfully, but 2 h after the procedure the first attack of pulmonary embolism occurred, treated by anticoagulation therapy. Second attack happened on the third postinterventional day. Considering vaginal bleeding, continued extracorporeal membrane oxygenation (ECMO), and suspicion of embolic particles arising from uterus, a subtotal hysterectomy was done. Patient was discharged two weeks following surgery, after complete recovery. Conclusion: Although AVM is managed by UAE, clinicians must be aware of complications. To avoid PE, we suggest only large sized microspheres for carefully selected patients. Keywords: Uterine artery embolization, Uterine arteriovenous malformation, Pulmonary embolism

Benign adrenal vascular tumors, cysts, and pseudocysts are a heterogeneous group of relatively uncommon entities that may pose diagnostic challenges radiologically and pathologically. However, there are only a few small cases series systematically characterizing the clinicopathological features of these lesions. We identified 55 cases of benign adrenal vascular tumors, cysts, and pseudocysts (23 pseudocysts, 17 hemangiomas, 8 lymphangiomas, 6 angiomatous endothelial cysts, and 1 arteriovenous malformation) from a multi-institutional urologic pathology database between 2000 and 2017 and retrospectively analyzed their clinicopathological features. We found that these lesions have a female predominance and most are right sided. These lesions may occur simultaneously with other adrenal tumors associated with hormonal hypersecretion. A substantial portion of pseudocysts were semisolid or solid with no fluid collection, mimicking a solid adrenal tumor and resulting in adrenalectomy. In addition, a small proportion of benign vascular lesions may have coexisting epithelial tumors, requiring extensive specimen sampling and thorough microscopic examination.

The purpose of this study was to evaluate the diagnostic performance of ultra-short echo time magnetic resonance imaging (UTE MRI) in the assessment of pulmonary arteriovenous malformation (PAVM).Eighteen consecutive patients (mean [± standard deviation] age, 48.6 ± 16.8 years) with 46 untreated PAVMs who underwent and thin-section computed tomography (CT) and UTE MRI with a 1.5-Tesla and 3-Tesla unit were retrospectively assessed. Two radiologists evaluated the diagnostic capabilities of UTE MRI for the detection and classification of PAVMs with reference to CT. Sensitivity, specificity, and kappa statistics were calculated with reference to CT. We also compared the differences in PAVM measurements between CT and MRI.The sensitivity and specificity of UTE-MRI for the detection of PAVMs were 89.1% and 100%, respectively, for reader 1 and 87.0% and 100%, respectively, for reader 2. In the classification of PAVMs, inter-modality agreement in reader 1 and 2 were both substantial (κ = 0.78 and 0.69, respectively). The measurements of the PAVM feeding artery and sac on CT and MRI were strongly correlated in both readers 1 and 2 (RThis study indicates that UTE MRI is a feasible and promising modality for noninvasive assessment of PAVMs.