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Background: Spinal arteriovenous malformations (AVM) are rare lesions. They may present with intramedullary hemorrhage or edema, often inducing severe neurological deficits. Active treatment of spinal AVMs is challenging even for experienced neurosurgeons., Method: Anticipation of anatomy and AVM angiocharacteristics from preoperative imaging is key for successful treatment. Information gathered from MRI and DSA has to be then matched to intraoperative findings. This is a prerequisite for reasonably safe and structured lesion removal., Conclusion: We provide a structured approach for surgical treatment of spinal AVMs, supplemented by high-resolution video and imaging material., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Isolated lesions of the corpus callosum are rare and may represent permanent but also transient responses to various pathology termed "reversible splenial lesion syndrome" (RESLES) when in light of relevant clinical presentation. We present the first case of the RESLES after elective surgery for distant arteriovenous malformation (AVM), followed by a slight speech disturbance and MRI verified small, oval, well-circumscribed area of apparent cytotoxic edema in the center of the corpus callosum splenium, which completely resolved within 15 days. Surgery for AVM is followed by the complex adaptation to a new vascular pattern, RESLES might develop, and should be suspected., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Early diagnosis of basilar artery occlusion (BAO) based only on clinical findings is challenging. We present a fully recovered case of BAO caused by pulmonary arteriovenous malformation (PAVM) that was diagnosed early using a protocol for CT angiography (CTA) and promptly treated with endovascular therapy (EVT). A woman in her 50s complained of vertigo with normal level of consciousness (LOC). On arrival, her LOC decreased to a Grass Coma Scale score of 12, and we performed a CT chest-cerebral angiography protocol. Head CTA showed BAO, and an intravenous tissue plasminogen activator was administered, followed by EVT. Chest contrast-enhanced CT showed PAVM in segment 10 of the left lung, which was treated with coil embolisation. For patients with a complaint of vertigo, BAO should be suspected, even if they have an initially normal LOC. A CT chest-cerebral angiography protocol is useful for prompt diagnosis and treatment of BAO and can reveal undetermined causes., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

This review aimed to provide an overview of pulmonary arteriovenous malformations, including the major clinical and radiological presentations, investigation, and treatment algorithm of the condition. The primary etiology of pulmonary arteriovenous malformations is hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, with mutations in the ENG gene on chromosome 9 (HHT type 1) or in the ACVRL1/ALK1 complex (HHT type 2). Epistaxis should always be evaluated when repeated, when associated with anemia, and in some cases of hypoxemia. In the investigation, contrast echocardiography and chest CT are essential for evaluating this condition. Embolization is the best treatment choice, especially for correction in cases of hypoxemia or to avoid systemic infections. Finally, disease management was addressed in special conditions such as pregnancy. CT follow-up should be performed every 3-5 years, depending on the size of the afferent and efferent vessels, and antibiotic prophylactic care should always be oriented. Ultimately, knowledge of the disease by health professionals is a crucial point for the early diagnosis of these patients in clinical practice, which can potentially modify the natural course of the disease.

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, is a rare disorder with a case prevalence as high as one in 5000, causing arteriovenous malformations in multiple organ systems. HHT is familial with autosomal dominant inheritance, with genetic testing allowing confirmation of the diagnosis in asymptomatic kindreds. Common clinical manifestations are epistaxis and intestinal lesions causing anemia and requiring transfusions. Pulmonary vascular malformations predispose to ischemic stroke and brain abscess and may cause dyspnea and cardiac failure. Brain vascular malformations can cause hemorrhagic stroke and seizures. Rarely, liver arteriovenous malformations can cause hepatic failure. A form of HHT can cause juvenile polyposis syndrome and colon cancer. Specialists in multiple fields may be called to care for one or more aspects of HHT, but few are familiar with evidence-based guidelines for HHT management or see a sufficient number of patients to gain experience with the unique characteristics of the disease. Primary care physicians and specialists are often unaware of the important manifestations of HHT in multiple systems and the thresholds for their screening and appropriate management. To improve familiarity, experience, and coordinated multisystem care for patients with HHT, the Cure HHT Foundation, which advocates for patients and families with this disease, has accredited 29 centers in North America with designated specialists for the evaluation and care of patients with HHT. Team assembly and current screening and management protocols are described as a model for evidence-based, multidisciplinary care in this disease.

Background: Vascular malformations are rare diseases that are best managed in multidisciplinary vascular anomaly centers (VAC). So far, there are few published data on the logistic structure, patient allotment, and internal workflow in an interdisciplinary VAC., Purpose: To demonstrate the referral structure and patient allotment in a European VAC., Material and Methods: A retrospective cohort study was conducted. All patients treated in the VAC since its establishment in April 2014 until June 2020 were identified. A total of 461 patients were included in this study., Results: Most often, a venous malformation was diagnosed (49.9%), followed by arteriovenous malformations (8.7%) and syndromal vascular malformations (8.2%). Only 45.1% of referral diagnoses were correct. Of referrals, 35.1% were internal on-campus references, 28.0% were external references through physicians in private practice, and 19.7% came via external cooperating hospitals. Of the patients, 17.1% were self-admissions without a referral., Conclusion: The most important referring clinics are internal medicine, pediatric, and vascular surgery. A substantial proportion of referrals came from private practices. Recruiting these cooperation partners should be considered a high priority when establishing a new VAC. The rate of misdiagnosis is high, and more education of fellow clinicians is urgently needed. Therefore, patients with vascular malformations should be referred to and treated in dedicated centers for vascular anomalies.

Mandibular arteriovenous malformations (AVMs) are high flow vascular malformations that can cause pain, hypertrophy, deformity, malocclusion, jaw asymmetry, bone destruction, tooth loss, and severe bleeding [1]. Although general principles apply, the rarity of mandibular AVMs limits definitive agreement on the best course of treatment. Current treatment options include embolization, sclerotherapy, surgical resection, or some combination of techniques [2]. [3]. An alternative multidisciplinary technique of embolization with mandibular-sparing resection is presented. This technique aims to mitigate bleeding with effective removal of the AVM, while preserving mandibular form, function, dentition, and occlusion., Competing Interests: Declarations of competing interest None., (Copyright © 2023. Published by Elsevier B.V.)

Rationale: An arteriovenous malformation (AVM) is an abnormal tangle of blood vessels that connects the arteries and the veins. Because normal capillary bed is partially or completely absented in the AVM, the blood passes quickly from the arteries to the veins, which disrupts normal blood flow and oxygen supply to the surrounding tissues. This is called "steal phenomenon," and in the inferior mesenteric artery (IMA) territory, this may lead to abdominal pain, gastrointestinal bleeding, portal hypertension, and even ischemic colitis., Patient Concerns: A 67-year-old man presented to our emergency department because of left side abdominal pain., Diagnoses: The abdominal computed tomography with contrast enhancement revealed a cluster of abnormal vascular lesions abutting the IMA with early opacification of the left colonic marginal vein. In addition, poor enhancement of segmental colonic wall was found from proximal descending colon to middle rectum. The diagnosis of AVM of the IMA and ischemic colitis was made., Interventions: The patient underwent left hemicolectomy as well as the AVM resection., Outcomes: He was discharged uneventfully after the surgery without complications., Lessons: IMA AVM carries the risk of ischemic colitis. computed tomography scan is helpful not only to the diagnosis of AVMs but also to exclude other lesions as well. Treatment options include endovascular embolization, surgical intervention, and a combination of both. Due to the complexity of this disease, treatment requires a case-specific multidisciplinary approach and a coordination of medical, radiological, and surgical staffs., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Rationale: Hepatic arteriovenous malformations (HAVMs) are a rare disorder reported in association with hereditary hemorrhagic telangiectasia (HHT), known as Rendu-Osler-Weber syndrome. HAVMs are usually detected in adulthood., Patient Concerns: A 29-year-old pregnant woman underwent a routine prenatal examination at 37 weeks of pregnancy., Diagnosis and Interventions: There were fetal liver anomalies detected by prenatal ultrasonography and were managed. Furthermore, a hepatic mass was detected and was subsequently analyzed by fetal magnetic resonance imaging. There were no typical imaging findings in this case which was once misdiagnosed as a hepatoblastoma., Outcomes: Considering the massive hepatic lesion, labor induction was performed on a pregnant woman to avoid adverse maternal and fetal outcomes. Histopathological examination confirmed the diagnosis of HAVMs. Lesions detected by imaging were determined to be hemorrhagic and necrotic., Lessons: Prenatal hepatic hemorrhage and necrosis due to an arteriovenous malformation are rare. The authors describe their observations and results., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Gastrointestinal bleeding that originates in the small intestine is often difficult to diagnose. Bleeding from a small intestinal arteriovenous malformation (AVM) is rare, with congenital AVMs more commonly located in the rectum or sigmoid. There is a relative paucity of cases reported in the literature. In the gastrointestinal tract, it can cause acute and chronic bleeding, which can be fatal. Although the incidence of small bowel AVMs is quite low, such lesions can be identified as the bleeding source in patients with obscure gastrointestinal bleeding (OGIB) harbouring severe, transfusion-dependent anaemia. It can be exceedingly difficult to localise and diagnose gastrointestinal tract bleeding, particularly in cases of occult small bowel AVMs. CT angiography and capsule endoscopy can help to establish the diagnosis. Laparoscopy is an appropriate and beneficial treatment modality for small bowel resection. The authors present the case of a primigravida woman in her late 20s diagnosed with a symptomatic transfusion-dependent anaemia during her pregnancy. She developed OGIB and despite no history of chronic liver disease became encephalopathic. Due to her physical deterioration and uncertain diagnosis, her caesarean section was performed at 36+6 weeks to expedite investigations and treatment. She was diagnosed with a jejunal AVM and underwent coiled embolisation of her superior mesenteric artery. She became haemodynamically unstable and underwent a laparotomy and small bowel resection. A full non-invasive liver screen was negative, however, her MRI liver described multiple focal nodular hyperplasia (FNH) lesions raising the possibility of FNH syndrome in the context of a previous AVM malformation. A prompt stepwise, multimodality diagnostic approach is required to prevent patient morbidity and mortality., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Embolotherapy is the primary treatment for pulmonary arterio-venous malformations (PAVMs). Approximately, 2-25% of PAVMs demonstrate persistence following embolization. Early identification and treatment of persistent PAVMs are critical to mitigating life threatening complications. The presence of prior embolic devices and complex angioarchitecture of persistent PAVMs pose technical challenges during repeat embolotherapy. In this article, we review patterns of persistence, factors affecting the persistence, endovascular treatment techniques, and outcomes., (Copyright © 2022 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

Background: Pulmonary arteriovenous malformations (PAVMs) are direct connections between the pulmonary artery and the pulmonary vein, mostly associated with hereditary hemorrhagic telangiectasia (HHT). PAVMs can lead to severe neurologic complications such as stroke and brain abscess. The risk of complications decreases after embolization. Therefore, screening for PAVMs using transthoracic contrast echocardiography (TTCE) is recommended, including a rescreening interval of 5 years., Research Question: Is extension of the interval for rescreening patients without a pulmonary right-to-left shunt (RLS) of up to 10 years appropriate?, Study Design and Methods: Adult patients with HHT with 5- or 10-year follow-up TTCE, or both, were included. Patients who underwent PAVM embolization in the past or at baseline were excluded. The RLS grades and presence of a treatable PAVM were compared with baseline., Results: In total, 387 patients (median age, 45 years [interquartile range, 33-54 years]; 56% women) involving 5- and 10-year follow-up data in 363 and 166 patients, respectively, were included. None of the patients (n = 148) without a pulmonary RLS at baseline demonstrated a treatable PAVM after 5 and 10 years. Of the patients with a pulmonary RLS at baseline, 20 patients (9%) and three patients (3%) demonstrated a treatable PAVM at the 5- and 10-year follow-up, respectively. In most patients, the RLS grade remained stable over time., Interpretation: On the basis of the results of this retrospective study, we believe that the rescreening interval for patients with HHT without a pulmonary RLS at initial screening may be extended to 10 years. Those with a pulmonary RLS should be rescreened every 5 years because treatable PAVMs can evolve., (Copyright © 2022 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

BACKGROUND Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant genetic disease associated with arteriovenous malformations involving diverse organs. Neurological complications from brain abscesses (BA) secondary to pulmonary arteriovenous malformations (PAVMs) is a serious and recognized, albeit infrequent, medical problem. We report the case of a 37-year-old man with familial HHT and PAVMs who presented with seizures as a manifestation of BA. CASE REPORT A 37-year-old man was admitted for first tonic-clonic seizures. He had a history of recurrent epistaxis and recurrent gastrointestinal bleeds treated with fulguration and oral iron therapy. A diagnosis of HHT was made because the patient met 3 of 4 Curaçao criteria. Physical examination revealed hypoxemia without dyspnea. A chest X-ray detected nodular pulmonary lesions in both lower lobes. Cranial computed tomography (CT) revealed 3 space-occupying lesions. Antiepileptics and dexamethasone were started. Cranial magnetic resonance and positron emission tomography suggested that lesions were BA. Thoracoabdominal CT with contrast revealed several bilateral PAVMs. Blood cultures were repeatedly negative. With the presumptive diagnosis of septic-embolic BA, empirical antibiotic therapy was started for 8 weeks. Neurological symptoms resolved and malformations >2 cm were selectively embolized. A genetic study revealed exon5 mutations in the ENG gene. CONCLUSIONS This report highlights the association between PAVMs in a patient with HHT and development of BA. Clinicians should be aware of this association so that diagnosis and treatment can be provided as fast as possible to ensure the best outcome for the patient. Embolization was performed as preventive treatment, and a genetic study was conducted as it is potentially useful for primary prevention in the patient's offspring.

Pathogenic variants in RASA1 are typically associated with a clinical condition called "capillary malformation-arteriovenous malformation" (CM-AVM) syndrome, an autosomal dominant genetic disease characterized by a broad phenotypic variability, even within families. In CM-AVM syndrome, multifocal capillary and arteriovenous malformations are mainly localized in the central nervous system, spine and skin. Although CM-AVM syndrome has been widely described in the literature, only 21 cases with prenatal onset of clinical features have been reported thus far. Here, we report four pediatric cases of molecularly confirmed CM-AVM syndrome which manifested during the prenatal period. Polyhydramnios, non-immune hydrops fetalis and chylothorax are only a few possible aspects of this condition, but a correct interpretation of these prenatal signs is essential due to the possible fatal consequences of unrecognized encephalic and thoracoabdominal deep vascular malformations in newborns and in family members carrying the same RASA1 variant.

Mandibular arteriovenous malformations are rare congenital malformations that require multidisciplinary care. Implant-supported rehabilitation of significant bone defect after embolization and resection is poorly described in the literature. We present the case of a 24-year-old patient with a right-sided mandibular arteriovenous malformation diagnosed after massive hemorrhage and treated by embolization and resection surgery. Implant rehabilitation was carried out 9 years later with a prior bone graft through iliac extraction and 3 short implants. Implant survival rate and patient satisfaction were evaluated at 3 years postplacement. Arteriovenous malformations treatments frequently result in bone defects that are difficult to reconstruct because of probable unstable vascularization due to embolization. The presence of osteosynthesis material and artifacts at the radiological level complicates implant planning due to the lack of visualization of the inferior alveolar nerve or artery and necessitates the placement of low-height implants. Osteointegration in contact with embolization products should be monitored. The creation of a case series could be of interest in order to better understand implant treatment for patients with a history of arteriovenous malformations.

A 33-year-old pregnant woman presented with six months of right-sided proptosis. Neuroimaging revealed a right orbital arteriovenous malformation arising from the second segment of the ophthalmic artery. As she was 9 weeks pregnant, the decision was made to monitor her closely. Over the following six months, her proptosis progressed, accompanied by decreased visual acuity, afferent pupillary defect, and red desaturation concerning for compressive optic neuropathy. After planned c-section, she underwent embolization with n-butyl cyanoacrylate. Upon awakening after embolization, she had no light perception vision from her right eye and was found to have ophthalmic artery obstruction. She ultimately developed a blind painful right eye and underwent enucleation with histopathology demonstrating glue in the central retinal artery, posterior ciliary arteries, and choroid. This case highlights ophthalmic artery occlusion as a rare complication of orbital arteriovenous malformation embolization and demonstrates correlating histopathological findings, which have not previously been reported.

Background: Transcatheter embolization is a commonly used minimally invasive technique in the treatment of pulmonary arteriovenous malformation (PAVM) with a high probability of post-operative recurrence, and some recurrent cases of PAVM cannot be treated via pulmonary arterial re-embolization. Here, we report the first case, to our knowledge, a 55-year-old female undergoing percutaneous direct puncture embolization for recurrent PAVM with good short-term efficacy., Case Presentation: The patient was a 55-year-old female presenting to the emergency department of our hospital with acute exacerbation of chest tightness and shortness of breath for 2 hours. The patient was diagnosed with PAVM and undergone embolization half a year ago. DSA-guided embolization was performed through percutaneous puncture approach. After embolization with four spring coils, the blood flow to the PAVMs was stopped and the surgery completed. The patient reported improvement of chest tightness and shortness of breath and was discharged from the hospital 3 days later., Conclusion: Our case was unique as a portion of the PAVMs was closely attached to the chest wall and PVP was slightly increased, which made percutaneous puncture embolization possible. Our case can be helpful in the treatment of recurrent PAVM in patients who cannot undergo re-embolization through pulmonary artery.

Background: To evaluate the efficacy and safety of transarterial bleomycin sclerotherapy of early-stage facial arteriovenous malformation (AVM)., Methods: A retrospective review was performed of patients who underwent bleomycin sclerotherapy for early-stage AVM (Schobinger stage I or II) in a single-referral vascular anomalies center. Bleomycin was slowly infused transarterially with flow control techniques to prolong the effects of bleomycin. Procedure details, AVM characteristics, and previous treatments were reviewed. Initial therapeutic outcomes were determined by 5 categories using both radiological and clinical findings in a 6-month follow-up. Further follow-up outcomes were reviewed to evaluate the long-term efficacy and safety of the treatment. Procedure-related complications were also analyzed., Results: Nineteen patients (mean age 22.4 ± 14.0 years, 14 females) with 31 sessions of sclerotherapies were enrolled. All AVMs were Cho-Do classification type III (type IIIa [n = 13], type IIIb [n = 2], and type IIIa+b [n = 4]). Patients received a mean of 1.6 (range, 1-4) sessions of treatment. The mean cumulative bleomycin dose was 23,600 IU ± 14,500 (range, 8000 - 60,000 IU). The results showed that 14 patients (74%) were responsive to transarterial bleomycin sclerotherapy, including complete response (n = 3), marked improvement (n = 1), and partial improvement (n = 10). The remaining 5 (26%) showed no response. During a mean follow-up of 32.6 months, 5 (26%) showed slight progression compared with 6-month outcomes and 14 (74%) were stable. There were only 2 minor complications [hyperpigmentation (n = 1) and cellulitis (n = 1)]., Conclusions: Transarterial bleomycin sclerotherapy using flow control techniques can be a safe and feasible alternative treatment option for facial early-stage AVM., Competing Interests: Declaration of Competing Interest None., (Copyright © 2022 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

There is a lot of ambiguity in the usage of correct terminology in the description of vascular malformations and tumors. Hemangioma and arteriovenous malformation (AVM) are the most commonly used terms and are the mostly incorrectly used as well! The aim of this review article was to lay out the correct nomenclature and describe the correct usage for the physicians and radiologists involved in diagnosing and managing these lesions. We describe the various classification systems which have been devised to define the multiple entities included under vascular anomalies. The latest classification system that should be adhered to is per the International Society for the Study of Vascular Anomalies, approved at the 20th ISSVA Workshop held in Melbourne in April 2014, last revised in May 2018. The main features of the latest revision have been highlighted. This classification, however, does not list the diagnostic clinico-radiological features for each entity. In addition, guidelines regarding the appropriate use of available imaging modalities are lacking in the literature. We, hereby, aim to address these pertinent issues in this review article.

We performed robot-assisted laparoscopic radical prostatectomy (RARP) without transcatheter arterial embolization (TAE) for a 72-year-old male patient with prostate cancer and pelvic arteriovenous malformation (AVM). Though lymphatic dissection was made contralateral to the AVM, the operation time (robotic: 2h 40 min, and total: 3h 2 min) was not long. Moreover, the blood loss amount of 250 ml was less than those in the past reports of preoperative TAE. Robotic surgery, a dissection of an abnormal arterial branch from the internal iliac artery before the division of the bladder neck, bunching of the deep dorsal vein complex, and resection of the vascular pedicle connecting with AVM in the final step of prostatectomy, contributed to the safe operation. Moreover, the surgical margin was negative in the pathological report,and the prostate specific-antigen was 0.006 ng/ml 3months following the operation. In addition, CT revealed the same size of AVM and no postoperative complication. It has been demonstrated that in the absence of TAE for pelvic AVM, RARP for prostate cancer is safe and effectively controls cancer.

Background: Double balloon enteroscopy remains a resource and time-intensive procedure that is not available in many endoscopy units., Aims: We aimed to identify variables impacting the speed and completion of double balloon enteroscopy., Methods: We retrospectively reviewed 550 patients. Using a mean time and distance for both the antegrade and retrograde approach, we determined the procedure speed and assessed factors that influenced it. In addition, we assessed the factors that contributed to a complete double balloon enteroscopy., Results: A total of 386 antegrade and 164 retrograde double balloon enteroscopies were performed. Greater than 10 AVMs requiring treatment was a negative predictor (AOR 0.25, CI 0.11-0.51, p < 0.001), whereas age greater than 60 years (AOR 2.66, CI 1.18-6.65, p = 0.025) was a positive predictor of a fast antegrade enteroscopy. For retrograde, prior abdominal surgery was the only factor that trended to significance (AOR 0.38, CI 0.14-0.99, p = 0.052). A total of 120 combined procedures were performed. Female gender (AOR 2.62, CI 1.16-6.24, p = 0.02), history of prior abdominal surgery (AOR 0.31, CI 0.13-0.70, p = 0.006) and Boston bowel pre-preparation score of greater than 6 (AOR 4.50, CI 1.59-14.30, p = 0.006) were the only significant predictors of a complete procedure., Conclusion: By applying double balloon enteroscopy speed, a novel method of measuring procedure efficiency, we were able to more reliably identify the factors that will negatively impact the speed and success of the procedure., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Seven consecutive female patients with pathologically confirmed arteriovenous malformation (AVM) with intravenous leiomyomatosis (IVL) of the uterus (age range, 32-61 years; mean age, 43 years) treated between 2005 and 2021 from a single institution were reviewed. Computed tomography (CT) findings of congenital pelvic AVM of 10 female patients were compared with those of AVM with IVL. Characteristic CT findings of AVM with IVL revealed a focal soft tissue mass inside a dilated venous structure of the AVM. Multiple sessions of transvenous coil embolization of the AVM with or without the injection of ethanol were performed. After complete (6/7, 86%) or partial (1/7, 14%) embolization of the AVM, complete surgical resection of the IVL and embolized AVM mass was performed in 4 patients. Patients with lung metastasis or residual embolized AVM masses are under follow-up with antiestrogen hormonal therapy., (Copyright © 2022 SIR. Published by Elsevier Inc. All rights reserved.)

BACKGROUND. Endovascular embolization of pulmonary arteriovenous malformations (PAVMs) was historically performed with embolic coils. The Amplatzer Vascular Plug device (AVP) was introduced for this purpose in 2007 and the Micro Vascular Plug device (MVP) in 2013. OBJECTIVE. The purpose of this study was to compare coils, AVPs, and MVPs in terms of risk of persistence after PAVM embolization by use of propensity score weighting to account for biases in device selection. METHODS. This retrospective study included 112 patients (78 women and girls, 34 men and boys; mean age, 45 years) who underwent embolization of 393 PAVMs with a single device type (coil, MVP, or AVP) from January 2003 to January 2020. Persistence was defined as less than 70% reduction in PAVM sac size or contrast enhancement of the sac on follow-up pulmonary CTA. A Cox proportional hazards regression model was used to assess associations between embolic device selection and PAVM persistence. Inverse propensity score weighting was used to account for differences in embolic device selection based on patient and PAVM characteristics. RESULTS. The median postembolization follow-up period was 1.5 years (IQR, 0.3-5.6 years). Persistence was found in 10% (41/393) of PAVMs, including 16% (34/207) of those treated with coils, 8% (7/88) of those treated with AVPs, and 0% (0/98) of those treated with MVPs. Variables associated with embolization device ( p < .25) were age, sex, pediatric versus adult status, smoking status, PAVM complexity, PAVM laterality, number of feeding arteries, and feeding artery diameter. The Cox regression model incorporated inverse propensity score weighting to account for the differences between treatment groups in these variables and incorporated feeding artery diameter because of imbalance remaining after weighting. With coils as the referent, MVPs had a hazard ratio for persistence of less than 0.01 (95% CI, < 0.01 to < 0.01; p < .001), and AVPs had a hazard ratio of 0.37 (95% CI, 0.16-0.90; p = .03). CONCLUSION. The risk of persistence after PAVM embolization was significantly lower for MVPs alone than for coils or AVPs alone. In addition, the risk of persistence was lower for AVPs than for coils. CLINICAL IMPACT. The findings support the clinical use of MVPs as the preferred device for PAVM embolization over coils and polytetrafluoroethylene-covered plugs.

Background and Importance: Spinal vascular malformations (sVMs) are relatively uncommon, accounting for 5% to 10% of all spinal cord lesions. Spetzler and Kim developed a paradigm to classify sVMs based on a variety of characteristics into 1 of 6 types, including a subcategory for exclusively epidural sVMs. There is a paucity of literature focused on this category, specifically sources describing the clinical manifestation and management of these lesions., Clinical Presentation: We report 2 cases of purely epidural spinal arteriovenous fistula, with an emphasis on the radiographic features and combined endovascular and microsurgical treatment. We report 2 patients known to have epidural spinal arteriovenous fistula who underwent both embolization and surgical resection between May 2019 and August 2020 at our institution. Data collected included demographic, clinical, and operative course, including age, sex, medical history, presenting symptoms, and preoperative and postoperative imaging. Both of these patients were managed with a combination of an endovascular approach for embolization of feeding arterial source and surgical exploration/resection. In both cases, no residual vascular malformation was identified, and the patients went on to be symptom free after 6 weeks., Conclusion: This report describes the use of a combination of endovascular and surgical approaches to achieve maximal benefit for 2 patients. These cases reinforce the value of a staged multimodal treatment approach in achieving good functional outcomes for patients with these rare and challenging entities., (Copyright © Congress of Neurological Surgeons 2022. All rights reserved.)