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Persistent trigeminal artery or persistent primitive trigeminal artery (PTA) is the most common carotid-basilar anastomosis in both cadaveric and live patient studies, followed by persistent hypoglossal and persistent otic arteries. Approximately 0.2% of all angiographies reported this finding.We present the case of a 21-year-old male who arrived at the emergency department with tonic‒clonic seizures. After performing diagnostic contrast magnetic resonance imaging and digital subtraction angiography, the patient was diagnosed with a right occipital arteriovenous malformations (AVM) fed by the right calcarine artery associated with an ipsilateral PTA. After considering surgical and endovascular treatment options, the patient was selected for watchful waiting. We included a literature review of the PTA, the results of a PubMed search regarding the combined presence of these findings, and a brief discussion providing insight into the implications for treatment.Although several studies have linked PTA to different vascular pathologies, such as cerebral aneurysms, the association between PTA and AVMs remains scarce. This case, along with the literature review, shows that further research is needed to characterize the relationship between these findings.

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

Deep cerebral venous thrombosis is an uncommon condition, which usually produces headache, altered consciousness, and ocular movement abnormality. Parkinsonism occasionally occurs when there is basal ganglia involvement. We report a case of a 78-year-old man who presented with a rapidly progressive parkinsonism with poor response to dopaminergic therapy. The patient had bilateral and symmetrical hypokinesia, rigidity, and marked gait impairment with festination. Brain MRI showed bilateral thalamic hyperintensity on T2-weighted and FLAIR sequences, with right thalamic and intraventricular hemorrhage due to straight sinus thrombosis. Angiography revealed an arteriovenous malformation in the quadrigeminal cistern with afferent supply from the posterior cerebral arteries, as well as partial thrombosis of the vein of Galen and half of the straight sinus. No predisposing factor for thrombosis was found. Given the location and size of the malformation, and the substantial amount of thalamic and intraventricular hemorrhage, conservative management was decided, with slow but progressive gait improvement. The presence of deep cerebral venous thrombosis should be suspected in cases of rapidly progressive parkinsonism with cognitive decline. As in this case, thrombosis may be secondary to a deep arteriovenous malformation, a very rare occurrence that may require specific therapy.

Background The cure of an eloquent brain arteriovenous malformation (BAVM) and multiple intracranial aneurysms with preservation of neurological function and the minimal procedures is challenging. Method A 53-year-old male was admitted to treat a left frontal language-area BAVM and concomitant five bilateral intracranial aneurysms. After repairing the ruptured right middle cerebral artery (MCA) bifurcation aneurysms and the other two unruptured ones, at the second-stage multimodality-guided awake hybrid operation, we successfully obliterated the left frontal BAVM and two other left MCA aneurysms. Conclusion The multimodality-guided awake hybrid operation may be a promising technique to treat complicated cerebrovascular disease. Supplementary Information The online version contains supplementary material available at 10.1007/s00701-021-05093-7.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant condition that is linked to a myriad of neurologic complications arising from vascular malformations of the brain, spinal cord, and lungs. Our case describes a previously healthy 3-year-old male who presented to hospital with fever of unknown origin and was found to have a brain abscess stemming from a pulmonary arteriovenous malformation (PAVM). This etiology was identified after a period of diagnostic delay; the medical team was suspicious for a proximal embolic source due to the presence of multiple tiny infarcts seen on MRI of the brain, but transthoracic echocardiogram and head and neck angiogram were unremarkable. Fortunately, an enhanced CT of the chest was performed, identifying a moderately sized PAVM. PAVMs are associated with intracranial abscesses due to shunting and loss of the normal filtering effects of the lung capillary bed. Impaired pulmonary filtration can permit paradoxical thromboemboli and septic microemboli to enter systemic circulation, predisposing patients with PAVMs to cerebral abscess and ischemic stroke. Screening for PAVMs with contrast-enhanced echocardiogram or enhanced CT of the chest may be considered in patients with cryptogenic brain abscess or recurrent embolic stroke of unknown origin. PAVMs are often associated with hereditary hemorrhagic telangiectasia (HHT). As many features of HHT have delayed clinical manifestation, genetic testing for HHT should be considered in all people with PAVM, even in the absence of other clinical features. In our case, genetic testing returned positive, confirming a new diagnosis of HHT type 1.

Cobb Syndrome (Spinal Arteriovenous Metameric Syndrome 1-31 (SAMS 1-31)) is a rare, non-hereditary disorder. Approximately 100 cases of CS have been described to date. The actual incidence may be much higher since only symptomatic patients were documented. In particular, post mortem studies suggest a possibly higher incidence of this syndrome. The main clinical manifestations of this syndrome include skin stains of vascular nature on the torso, in combination with spinal vascular malformations localized in one and the same metameric or spinal segment. A rare diagnosis of this syndrome in patients over 18 is probably related to the fact that the disease may be asymptomatic throughout a long period of time, while patients may tend to disregard the skin lesions. As a result, most publications on this pathology are based on separate case reports. : Significant variability of clinical manifestations as well as prolonged progress of the disease often cause errors in diagnosis. What follows is a case report of a young patient with Cobb Syndrome, who was admitted to a regional vascular centre with a misdiagnosis of stroke. : 20 patients of young age (from 20 to 35 years old), with a diagnosis of stroke, who were admitted to a University Clinic (of the Russian National Research Medical University Named After Pirogov N.I., Moscow). Among this group of patients, a patient with Cobb syndrome was identified. Patient P., of 22 years, presented with acute, intensive cervical spinal pain, predominantly on the right, numbness and weakness in the arms and legs. About 3 weeks before admission to the hospital, the patient had ARVI with a fever of up to 37.5°C: two weeks before the onset of symptoms, he had undergone extirpation of 2 teeth, for which reason he spent over 2 hours in a forced position with his head thrown back (prolonged overextension in the cervical spine). Multiple skin angiomas on the chest spreading to the shoulder and scapula region. Tetraparesis up to 4 points: tetraparesis in hands with low muscle tone, low reflexes, tetraparesis in legs with high muscle tone, high reflexes, foot clonus when causing Achilles reflexes, tremor in the extremities and no plantar reflex pathology were detected, sensitivity disorders in the hands “the high gloves” and no pelvic disorders were detected. Given the presence and exacerbation of neurological symptoms and cutaneous angiomas, MRI with a contrast agent of the cervical spine was recommended. MR-image of an advanced arteriovenous malformation (AVM) of the cervical spinal cord with signs of gliosis and spinal cord oedema at the C2-C7 level. Endovascular embolization of the AVM in the cervical spinal cord was performed. The treatment led to the complete reversal of neurological symptoms. In the presence of skin lesions, the diagnosis of CS does not present particular difficulties, so in children and young patients with skin angiomatosis, it is advisable to conduct a comprehensive examination using selective spinal angiography or MR angiography to exclude arteriovenous malformations in the spinal cord.

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.

Sciatic nerve symptomatology may arise from both intra- and extra-neural pathology, at any point along descent from the sacral plexus to its bifurcation. The potential aetiology is broad, ranging from degenerative spinal disease to muscle, bony and vascular pathology. We present an extremely unusual case of position and exercise-induced nerve compression secondary to arteriovenous malformation and review the potential extraspinal causes, many of which may be ameliorated by surgical excision or decompression. We further discuss the usefulness of diagnostic imaging, specific clinical tests and histopathological tools that may aid in management.

Background The aim of the present study is to evaluate the short-term efficacy and feasibility of radiofrequency ablation in the treatment of complex diffuse arteriovenous (AV) malformations. Methods The data of 18 patients (8 male and 10 female) with complex AV malformations treated between December 2014 and June 2019 were analyzed retrospectively. The lesion area was 10 × 7 cm ~ 28 × 30 cm. Under duplex ultrasound guidance, the site with the most abundant blood flow signals in the lesion was percutaneously punctured with the radiofrequency ablation needle (electrode). The impedance automatic adjustment mode was adopted, and ablation was monitored usingduplex ultrasoundduring the entire process. Results Of the included patients, 1 had a high fever after two rounds of treatment, 2 had transient hemoglobinuria, and 1 had tissue necrosis in the original ruptured tumor area as well as a penetrating defect in the cheek, which was repaired with a pedicled trapezius myocutaneous flap. In 9 patients who experienced bleeding, the bleeding stopped after one round of treatment. During the follow-up period of 1–5 years, there were 0 grade I (poor) cases, 0 grade II (medium) cases, 7 grade III (good) cases, and 11 grade IV (excellent) cases. Conclusion The “high power and continuous” radiofrequency ablation technique conducted under real-time duplex ultrasoundmonitoring can completely destroy the deep core lesions of AV malformations and effectively control life-threatening massive hemorrhage; it is an effective alternative treatment method for complex diffuse AV malformations in which interventional embolization, sclerotherapy, and surgery are ineffective.

Background The life-threatening haemorrhagic complications of pulmonary arteriovenous malformations (PAVMs) are extremely rare, and only described in isolated cases. This study was designed to comprehensively investigate management of ruptured PAVMs. Methods We retrospectively assessed clinical and imaging data of ruptured PAVMs to summarize incidence, clinical characteristics, and outcomes following embolisation between January 2008 and January 2021. Results Eighteen of 406 (4.4%) patients with PAVMs developed haemorrhagic complications. Twelve of 18 patients were clinically diagnosed with hereditary haemorrhagic telangiectasia (HHT). Haemorrhagic complications occurred with no clear trigger in all cases. Eight of 18 patients (44.4%) were initially misdiagnosed or had undergone early ineffective treatment. 28 lesions were detected, with 89.3% of them located in peripheral lung. Computed tomography angiography (CTA) showed indirect signs to indicate ruptured PAVMs in all cases. Lower haemoglobin concentrations were associated with the diameter of afferent arteries in the ruptured lesions. Successful embolotherapy was achieved in all cases. After embolotherapy, arterial oxygen saturation improved and bleeding was controlled (P Conclusions Life threatening haemorrhagic complications of PAVMs are rare, they usually occur without a trigger and can be easily misdiagnosed. HHT and larger size of afferent arteries are major risk factors of these complications. CTA is a useful tool for diagnosis and therapeutic guidance for ruptured PAVMs. Embolotherapy is an effective therapy for this life-threatening complication.

To summarize the clinical experience of sandwich-shaped resection and cheiloplasty in the treatment of macrocheilia secondary to arteriovenous malformation, and to observe its clinical efficacy and complications. The clinical data of 27 patients with macrocheilia secondary to arteriovenous malformation who received surgical treatment from January 2018 to October 2020 in Linyi Cancer Hospital were retrospectively analyzed. There were 14 males and 13 females. The median age was 4.5 years (ranged from 2 to 57 years). There were 18 cases of upper lip and 9 cases of lower lip. All patients had received interventional embolization sclerotherapy for 5 to 10 times before surgery with unsatisfied effect. There was no obvious improvement in the appearance,whereas 3 cases progressed presenting as abnormal enlargement. Sandwich-shaped resection and cheiloplasty was performed in all the patients. Histopathological examinations confirmed arteriovenous malformations in all 27 cases. Twenty-six patients received one course of operation and one received two courses. Partial mucosal necrosis occurred in 1 case and healed after dressing change, while the rest healed by first intention. Local recurrence occurred in 2 cases within 6 months after operation. A four-level standard was used for long-term evaluation, and 24 cases got grade Ⅲ (good), three cases got grade Ⅱ (moderate) after 1 to 3 years of follow-up. Sandwich-shaped resection and cheiloplasty can achieve good long-term efficacy with excellent appearance and function. Due to its advantages of hidden incision and three-dimensional resection and reservation, it is worthy of further clinical promotion.总结“三明治”式唇病变切除成形术治疗动静脉畸形巨唇症的临床经验，观察其临床疗效和并发症。回顾性分析2018年1月至2020年10月在临沂市肿瘤医院接受手术治疗的27例动静脉畸形巨唇症患者的临床资料，其中男14例，女13例；中位年龄4.5岁（2~57岁）；上唇18例，下唇9例。所有患者在手术前均接受过（外院或本院）5~10次的介入栓塞硬化治疗，巨唇外形改善均不明显，3例病变进展增大。本组病例均采用“三明治”式唇病变切除成形术治疗。共行“三明治”式唇病变切除成形术28例次（1例2次手术）。27例次患者一期切口愈合，1例部分黏膜坏死，后经换药后愈合。有2例分别于术后半年内局部复发。随访1~3年，远期疗效评价Ⅲ级（好）24例，Ⅱ级（中）3例，Ⅰ级（差）0例。“三明治”式唇病变切除成形术切口隐蔽，立体式切除病变彻底，能最大限度保留三维结构，术后外形和功能良好，远期疗效好，建议临床推广该手术方式。.

Objective. We aimed to investigate the risk factors associated with hemorrhage and clarify the relation of homocysteine (Hcy) with brain arteriovenous malformations (bAVMs). Method. We retrospectively reviewed bAVM patients from Beijing Tiantan Hospital between January 2019 and December 2019. Clinical and laboratory variables were analyzed in enrolled patients with bAVMs. Potential predictors associated with hemorrhage were evaluated by logistic regression analysis. Results. A total of 143 bAVM patients were identified in the study, including 69 unruptured and 74 ruptured cases. Patients with hemorrhage were less likely to have hyperhomocysteinemia ( P = 0.023 ). Logistic regression analysis showed that increased maximum diameter of bAVM lesions (odds ratio (OR) 0.634, 95% confidence intervals (CI) 0.479-0.839; P = 0.001 ) and serum Hcy level (OR 0.956, 95% CI 0.920-0.993; P = 0.021 ) were associated with lower risk of hemorrhage in bAVMs. Conclusion. The present study provided evidence regarding the association between serum Hcy and hemorrhage in patients with bAVMs. Higher Hcy level was correlated with a lower risk of rupture. Detection of factors for subsequent hemorrhage is necessary to develop therapeutic strategies for bAVMs preferably.

Objective Head and neck are the predilection sites of arteriovenous malformations (AVMs). Although embolization is the first-line treatment for AVMs, complete surgical removal of the lesion still has its value due to the best outcome with low recurrence rate. Here, the authors made a retrospective analysis on the surgical treatment of AVMs in the head and neck. Methods From January 2006 to December 2019, a total of 18 patients with AVMs in the head and neck were enrolled in this study, including 10 males. The Schobinger clinical staging, Yakes' angioarchitecture type, and surgical treatment were analyzed. The follow-up data were collected. Then, individual treatment strategies were summarized. Results According to Schobinger clinical classification system, 6 patients were at stage I, 7 patients at stage II, and 5 patients at stage III. According to Yakes' AVM classification system, 3 Type I, 4 Type II, 5 Type III, and 3 Type IV were confirmed. 3 patients cannot be confirmed due to lacking of arteriographic data. Surgical treatments included simple surgical excision (8 patients), dilator therapy (6 patients), and skin grafting after surgical excision (4 patients). In the follow-up period, 2 patients had recurrence and accepted operation again. All patients were satisfied with the appearance. Conclusions Individual surgical treatment based on the clinical stage and angioarchitecture type can achieve satisfactory results in AVMs in the head and neck.

Background Vascular endothelial cells (ECs) are subject to continuous shear stress due to blood circulation. Mechanical stress due to high shear flow can also cause arteriovenous malformation (AVM) when ECs respond hyper-sensitively to shear flow. This study was conducted to test the hypothesis that angiogenesis could be promoted in response to mechanical stress via regulation of pro-angiogenic factors in AVM cells. Methods ECs were extracted from the tissue samples from six AVM patients and six normal patients. Shear stress at 7 dynes/cm2 were applied for 24 h. Before and after application of shear stress to each group, RT-PCR was performed to access the expression levels of angiopoietin2(AGP2), aquaporin1(AQP1) and TGFβR1. Immunofluorescences was also performed to evaluate the level of protein expressions. Results In both normal and AVM tissues, AGP2 and TGFβR1 under the shear stress showed increased expression in the ECs compared to the non-sheared samples. When AVMs and normal arterial vasculature were compared, the expression levels of both AGP2 and TGFβR1 in AVMs were higher when compared to normal arterial vasculature with or without shear stress. Immunofluorescence-based protein analysis also confirmed shear-induced AGP2 and TGFβR1 in both samples of normal and AVM patients. Conclusions AVMs exhibited higher sensitivity to shear stress by producing higher expressions of some marked genes and proteins that regulate the endothelial functions upon exposure to shear stress. While the physiological mechanism for AVMs remain elusive, our study shows the plausibility of physical stress imposed by the shearing flow can cause the occurrence of AVMs.

A 57-year-old man was admitted to our hospital because of frequent hematochezia. Colonoscopy exhibited a submucosal tumor-like lesion in the lower rectum. Abdominal contrast-enhanced computed tomography showed a rectal arteriovenous malformation (AVM) on the right side wall of the lower rectum. The feeder was the superior rectal artery, with early venous return. Embolization of the draining vein and feeding artery of the AVM with N-butyl-2-cyanoacrylate under balloon occlusion was planned. Angiography of the superior rectal artery showed the nidus in the rectum with early venous return of contrast material. The portal vein was punctured percutaneously under ultrasound guidance, and a balloon catheter advanced to the distal part of the superior rectal vein. Venography under balloon occlusion showed the outflow vein and nidus. Transvenous and transarterial nidus embolization with N-butyl-2-cyanoacrylate under balloon occlusion was then performed. Since the embolization, there have been no further episodes of bleeding. There is no established treatment for AVMs. Successful treatment requires targeting and eradication of the nidus. We successfully performed embolization therapy for a rectal AVM via a retrograde transvenous approach. This technique may be suitable for completely eradicating the nidus without the risk of embolism.

Background Resection of tumors adjacent to motor pathways carries risks of both postoperative motor deficit and incomplete resection. Our aim was to assess usefulness and limitations of a multimodal strategy that combines intraoperative ultrasound (iUS) guided resection with intraoperative neurophysiology. Methodology This is a prospective study of 25 patients with brain lesions adjacent to motor areas who underwent intracranial surgery with assistance of the iUS guidance system and intraoperative neurophysiological monitoring and mapping. Pathologies treated included 19 gliomas, 3 metastases, 1 anaplastic meningioma, 1 arteriovenous malformation (AVM), and 1 ependymoma. The iUS-guided lesion removal accuracy and the extent of resection were estimated and compared with a 30-day postoperative brain MRI. The results were assessed considering the extent of resection related to 6-month motor function outcome. Results iUS was accurate in checking the extent of resection in 17 patients, whereas in 8 cases the decline of the iUS images quality did not allow a valuable assessment. Positive mapping was obtained in 16 patients. Gross total resection was achieved in 16 patients. In five of nine cases with subtotal resection, surgery was stopped because a functional area was reached. In four patients, tumor removal was limited due to the difficulty of identifying neoplastic tissue. Motor function worsening was transient in six patients and permanent in two. Conclusions The integrated use of intraoperative neuromonitoring to identify motor areas and iUS to identify tumor–tissue interface could help increase the rate of radical resection respecting the eloquent areas.

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

Objective Pulmonary arteriovenous malformation (PAVM) is a rare pulmonary disease. Although most patients with PAVMs are asymptomatic, cerebral complications associated with PAVMs are often fatal. This study aimed to evaluate the risk factors for cerebral complications in patients with PAVMs. Methods We retrospectively reviewed the medical charts of patients with PAVMs between 2003 and 2021 at two tertiary referral hospitals and one secondary hospital. Results Fifty-five patients diagnosed with PAVMs were enrolled in this study. Most patients were female (89.1%), and the median age was 53 years. Thirty patients (54.5%) had incidentally detected PAVMs without symptoms. Twenty-four patients (43.7%) with PAVMs were treated with embolotherapy or surgery. Thirteen patients (23.6%) had cerebral complications. There was no significant difference in the development of cerebral complications according to treatment; however, older age (≥ 65 years) was associated with the development of new cerebral complications in untreated patients with PAVMs (odds ratio, 17.09; 95% confidence interval, 1.16–250.31; P = 0.038). Conclusion Older age (≥ 65 years) was a risk factor for the development of cerebral complications in patients with PAVMs; therefore, treatment should be considered in older patients with PAVMs.

OBJECTIVE The goal of this study was to describe clinical and neuroradiological features of arteriovenous malformations of the filum terminale (FT AVMs) and to present the authors’ diagnostic and therapeutic management in this rare disease. METHODS The presented cases were retrieved from a retrospectively collected database of all spinal vascular malformations treated between June 1992 and December 2021 at the Rheinisch-Westfälische Technische Hochschule (RWTH) University Hospital Aachen. Pretreatment and follow-up clinical and neuroradiological data were analyzed for this study. RESULTS Data in 15 patients with FT AVM with a mean age of 60 years were included, with an overall incidence of FT AVM of 19% among all spinal AVMs in our cohort. Twelve of 15 (80%) patients were men. Nonspecific but typical clinical and MR findings of thoracolumbar congestive myelopathy were found in all patients. Spinal MR angiography, performed in 10 patients, identified in all cases the arterialized FT vein as well as a lumbar/lumbosacral location of an AV shunt. Digital subtraction angiography (DSA) showed an arterial supply solely via the FT artery in 12/15 (80%) patients and via an additional feeder from the lumbosacral region in the other 3/15 (20%) patients. All patients were treated surgically. During 1-year follow-up, 2 patients presented with recurrent FT AVM due to further arterial supply from the lumbosacral region, and were treated surgically. Neurological status was improved in all patients within the 1-year follow-up, with marginal further changes during long-term follow-up. CONCLUSIONS Congestive myelopathy is the major pathological mechanism of symptoms in these patients, with no evidence for intradural bleeding. Missing the presence of possible multiple arterial supply of FT AVM during DSA may result in misdiagnosis and/or insufficient treatment. Due to the frequently prolonged course of FT artery, resection of the FT AVM may be a favorable treatment modality in comparison with endovascular treatment. Follow-up examinations are obligatory within the first 3 years after treatment, and further MR angiography and DSA examinations are indicated if congestive myelopathy persists.

This study presents the case of a 52-year-old male suffering from extensive melena. A detailed examination using lower gastrointestinal endoscopy revealed a polyp at the lower rectum; subsequently, endoscopic mucosal resection(EMR)was performed. No other lesion for the melena was observed. Histopathologic findings of the EMR confirmed adenocarcinoma in adenoma, indicating the need for additional surgery. Preoperative contrast-enhanced CT revealed an arteriovenous malformation( AVM)of about 5 cm in the rectal wall at the anal side of the lower rectal cancer. Extensive melena was inferred to be caused by AVM; hence, we performed laparoscopic low anterior resection combined with accessible excision of the AVM and blocked the superior rectal artery, acting as an inflow vessel. Postoperative contrast-enhanced CT confirmed the disappearance of AVM. Thus, we experienced a case in which the block of the inflow vessel, according to the treatment theory of embolism therapy without complete resection of the AVM, enabled the treatment of AVM. Therefore, this case could become a reference for the treatment of lower rectal AVM cases in the future.

A 61-year-old male patient with known kidney dysfunction who underwent a non-contrast thorax CT and was then referred to our department for metabolic assessment of solitary pulmonary nodule (SPN). The patient underwent dynamic FDG PET/CT to differentiate benign from malignant SPN. This case illustrates the usefulness of dynamic FDG PET/CT imaging when assessing SPN especially in patients with suspicion for pulmonary arterio-venous malformation accompanying renal failure or contrast allergy.

Arteriovenous malformation (AVM) in the pancreas rarely causes acute pancreatitis. However, even when it does cause pancreatitis, the pathogenesis is unclear. A 61-year-old man was admitted to our hospital for acute pancreatitis. The findings of computed abdominal tomography, magnetic resonance imaging, and endoscopic ultrasonography revealed pancreatic AVM and hematoma in the tail of the pancreas. These lesions were suspected to be associated with pancreatitis. Although endoscopic retrograde pancreatography could not confirm hemosuccus pancreaticus, distal pancreatectomy was performed because of repeated pancreatitis. The histopathological findings of the resected specimen revealed rupture of the AVM vessels into the main pancreatic ducts. Finally, we considered that intermittent bleeding due to AVM rupture and hematoma formation in the main pancreatic duct caused the repeated pancreatitis.

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.

Background When a flow-related aneurysm originates from an anterior inferior cerebellar artery (AICA) supplying the cerebellar arteriovenous malformation (AVM), the management becomes very complicated. Endovascular treatment (EVT) was an option, but no consensus has been achieved. Methods and materials: A retrospective investigation was performed for patients with flow-related aneurysm originating from an AICA supplying the cerebellar AVM. Results Ten patients, harboring 13 aneurysms, were identified. Of the 5 a1 aneurysms, 2 underwent stent assisted coiling, 2 underwent parent artery occlusion (PAO), and 1 was intact. Of the 8 a2 aneurysms, 3 underwent coiling with preservation of the AICA, 3 underwent PAO with Onyx, 1 underwent PAO with coils, and 1 was intact. Seven patients underwent partial embolization of the cerebellar AVM, 3 were intact. One patient died 6 hours postoperatively for cerebellar AVM rebleeding. During a follow-up from 6 months to 6 years, 9 patients had favorable recovery. Conclusion For the flow-related aneurysm originating from an AICA supplying the cerebellar AVM, the EVT depends on the specific circumstances. When the aneurysm is located at the a1 segment, coiling of the aneurysm with preservation of the parent AICA should be performed. PAO is the last resort. When the a2 aneurysm is proximal to the internal auditory artery, coiling of the aneurysm with preservation of the AICA is preferred. When the aneurysm is distal to the internal auditory artery, PAO can be safely performed.

Undiagnosed significant hydrocephalus is an uncommon finding at forensic autopsy as many cases present in life with complex neurological symptoms. We present a case of a 46-year-old man with no neurological deficits or history of head trauma that was incidentally found to have a massive hydrocephalus at autopsy. This was found to be associated with an unruptured arteriovenous malformation completely confined to the choroid plexus as well as complete agenesis of the corpus callosum. The arteriovenous malformation was found to form a calcified obstruction at the foramen of Monro analogous to a mass lesion, such as a colloid cyst of the third ventricle. The association of this malformation and agenesis of the corpus callosum has never been described. Histologic examination of the brain confirmed significant loss of white matter tracts and thinning of the cortical ribbon due to pressure atrophy of the ependymal lining without significant gliosis, cortical dysplasia, or evidence of other developmental malformations. Autopsy is a vital tool in the evaluation of such rare cases, enhances epidemiologic data, and increases the understanding of these pathophysiological associations.

Aims Transthoracic contrast echocardiography (TTCE) is the recommended screening tool to detect pulmonary right-to-left shunt (RLS) caused by pulmonary arteriovenous malformations (PAVMs). We assessed a novel method to quantify the RLS using the change in echo density (ED) following contrast injection. Methods and results An analysis of 437 consecutive patients [58% female, 47 years, interquartile range (IQR) 33–60] who underwent TTCE for the detection of a pulmonary RLS. Using ImageJ (National Institutes of Health), the change in ED was measured for each patient. This method was strongly correlated (Spearman’s ρ = 0.89; P Conclusions The use of ED quantification for pulmonary RLS is promising; resulting in a substantial decrease in the number of chest CT scans needed. However, this method and the threshold should be validated in an independent study population.

Gastrointestinal bleeding (GIB) is a common cause of morbidity among patients supported by left ventricular assist devices (LVADs). The aim of this study was to identify if pre-LVAD right ventricular (RV) dysfunction is associated with risk of GIB after LVAD implantation. Of 398 patients implanted with LVADs between July 2008 and July 2016, 130 (33%) developed GIB at a median of 2.6 months following LVAD implantation. Arteriovenous malformations (AVMs) were found in 42 (34%) GIB patients. Patients with GIB were older and more likely to have hypertension, diabetes, and ischemic cardiomyopathy. On pre-LVAD echocardiography, GIB patients had increased RV diastolic dimension (4.7 ± 0.8 vs. 4.4 ± 0.9 cm, p = 0.02), a higher rate of greater than mild tricuspid valve (TV) regurgitation (73 [60%] vs. 120 [47%], p = 0.006), and underwent TV repair more often (38 [30%] vs. 43 [16%], p = 0.0006) during LVAD implantation. After multivariable adjustment, preoperative greater than mild RV enlargement (hazard ratio [HR] 2.32, 95% CI 1.12-5.03; p = 0.03), TV regurgitation (HR 1.83, CI 1.02-3.44; p = 0.01), and TV repair (HR 3.76, confidence interval [CI] 1.02-4.44; p = 0.01) remained associated with risk of GIB. This finding was driven by the AVM-GIB subgroup. Preoperative RV enlargement and TV regurgitation are associated with post-LVAD AVM-related GIB.

A 54-year-old man was admitted to our hospital with dyspnea and heart failure. Contrast-enhanced computed tomography showed a giant pelvic arteriovenous malformation (AVM) fed by the left internal iliac artery (IIA), right IIA, and inferior mesenteric artery. (IMA). The AVM was treated with selective embolization via the left IIA. Time-resolved three-dimensional phase-contrast magnetic resonance imaging (4D-flow MRI) visualized a gradual flow reduction in the left IIA, whereas the flow in the IMA and right IIA increased relatively. After four sessions, the patient experienced symptom relief and the blood level of N-terminal prohormone brain natriuretic peptide decreased. To the best of our knowledge, we present the first reported use of 4D-flow MRI to quantitatively assess flow reduction in the case of pelvic AVM after embolization.

OBJECTIVE. The purpose of this study was to evaluate the CT angiography (CTA) findings of pulmonary arteriovenous malformation (PAVMs) in patients with hereditary hemorrhagic telangiectasia and to correlate these findings with those of graded contrast-enhanced transthoracic echocardiography (CE-TTE). MATERIALS AND METHODS. A retrospective review was conducted of PAVMs visualized at CTA of patients with abnormal CE-TTE findings (3-point scale). Location, distribution, size, number, volume, grade, and relative attenuation (attenuation of PAVM divided by attenuation of aorta) of PAVMs were recorded. PAVMs were graded as follows on conventional and maximum-intensity-projection (MIP) images: 0, nodule, unlikely PAVM; 1, ground-glass opacity (GGO); 2, GGO with increased vascular network; 3, GGO or nodule with single vessel; 4, GGO or nodule with two or more vessels; 5, GGO or nodule with afferent and larger efferent vessels; 6, mature arteriovenous malformation. Correlation between PAVM grade and relative attenuation and between CTA variables and CE-TTE grades was assessed. RESULTS. Forty patients (median age, 14.9 years; range, 0.6-27.9 years) had 117 PAVMs at CTA: 107 peripheral, eight central, and two both peripheral and central. None of the PAVMs was diffuse. Median size and volume were 0.4 cm (range, 0.1-4.4 cm) and 0.031 mL (range, 0.0009-10.019 mL). At CTA, seven PAVMs were grade 1, five grade 2, 28 grade 3, 62 grade 4, two grade 5, and 13 grade 6. MIP images showed 39 of 117 PAVMs were higher grade. Statistically significant correlation was found between relative attenuation and PAVM grade (p < 0.001, r = 0.58) in 40 patients and between all CTA variables and CE-TTE (p < 0.05, strongest correlation with highest grades [p < 0.0001, r = 0.81]) in 32 patients. CONCLUSION. In children and young adults with hereditary hemorrhagic telangiectasia, grade 4 PAVMs were most common. Higher-grade PAVMs more often have right-to-left shunts.

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.