Giant Arteriovenous Malformation Research Articles

Cerebral Proliferative Angiopathy as the Cause of Symptomatic Epilepsy in a Young Adult Male: A First Case Report from Indonesia

Background: Cerebral proliferative angiopathy (CPA) is a rare and distinct vascular malformation that was once considered a subset of cerebral arteriovenous malformation (AVM). Due to its relatively benign course with no distinctive clinical feature, CPA may often be overlooked and misdiagnosed with other diseases. We report a case of CPA as the underlying cause of symptomatic epilepsy. Case: A 31-year-old male presented to the outpatient clinic with a history of focal to bilateral tonic-clonic seizure for 2 years. Following conservative management with an oral antiepileptic agent, the seizure frequency significantly decreased from once daily to once or twice monthly. The patient was lost to follow-up; however, he was incidentally referred back to our clinic two years later for further evaluation. A head Magnetic Resonance Imaging and Magnetic Resonance Angiography revealed a suspicion of giant AVM in the left hemisphere. Cerebral digital subtraction angiography (DSA) was performed and revealed a CPA in the left frontal area. The patient was managed conservatively and during the 6-month follow-up period, the patient did not have any seizures. Discussion: In young adults, seizures may be caused by an underlying vascular abnormality. Cerebral DSA remained the gold standard for distinguishing various etiologies of vascular malformation, including CPA. Conservative treatment using oral antiepileptic agents was effective in controlling the seizure frequency in CPA. However, a complete diagnostic evaluation is still warranted to determine the most appropriate treatment, revealing some peculiar and unexpected etiologies in the process.

Brain abscess caused by escherichia coli following embolization of a giant arteriovenous malformation. Clinical case

Embolization is one of the four approaches to the treatment of arteriovenous malformations. Typical complications of endovascular interventions include intraoperative or postoperative hemorrhage, ischemic stroke, perforation or dissection of a vessel, forced retention of a microcatheter in vessels due to polymerization of embolic material. Infectious complications after endovascular interventions and brain abscesses caused by Escherichia coli have been reported quite rarely. Although abscess formation after embolization is extremely rare, it is a serious condition that requires immediate treatment. This is a case of a unique clinical picture of a spontaneous brain abscess caused by Escherichia coli on the background of a giant arteriovenous malformation in an adult man without a typical route of infection and immunosuppression. This possibility should be taken into account in the treatment of arteriovenous malformation of the brain. A 37-year-old patient complained of convulsive attacks, increased body temperature, impaired speech, and right-sided hemiparesis. A brain abscess developed 1 month after the third stage of embolization of a giant arteriovenous brain malformation. Cultures of the contents of the abscess showed a positive result for Escherichia coli. During the long course of treatment, relapses of the abscess were registered several times, with each of them a complex approach to solving the problem was used ‒ a combination of surgical intervention and antibiotic therapy. Choosing the most effective method of treatment makes it possible to prevent or reduce the likelihood of complications and improve the patient's quality of life. Long and repeated procedures, partial exclusion of the malformation, repeated passages with microinstrumentation, use of a significant amount of substance for embolization can cause the development of inflammation, thrombus formation in the vessels of arteriovenous malformations and increase the risk of developing purulent-infectious complications after endovascular neurosurgical interventions. Consideration of the given clinical case will contribute to deepening the understanding of this pathology.

Comprehensive Management of a Giant Venous Malformation of the Lip: Vascular Embolization Followed by Surgical Resection and Reconstruction.

Venous malformations often manifest in early childhood and do not spontaneously resolve. Most vein malformations of the lips are typically treated at a young age, with giant arteriovenous malformations being particularly rare. Herein, we introduce the case of a 47-year-old man who presented to our department complaining of a progressive mass on his lower lip. Clinical examination revealed a mass measuring 10 cm × 8 cm × 4 cm in size, characterized by a soft texture and smooth edges. Despite a series of sclerotherapy interventions, the lesion remained unresponsive. Consequently, we performed a preoperative embolization of the malformed vessel using digital angiography, followed by extensive resection of the lesion and repair of the defect using an adjacent flap. The postoperative period was uneventful, and no local recurrence was observed during a 4-year follow-up period. Therefore, we recommend preoperative angioembolization as a valuable approach for addressing large lower lip deformities to enable extensive surgical resection and robust therapeutic outcomes.

An Arteriovenous Malformation of the External Ear in the Pediatric Population: A Case Report

Arteriovenous malformation (AVM) results from errors in vascular development during embryogenesis; absent capillary beds lead to shunting directly from the arterial to venous circulation. Although it is common in the head and neck region, Arteriovenous malformations (AVM) of the external ear are relatively uncommon lesions. They typically present during childhood. We report a case of giant AVM of the right pinna in 8 years child, which are very rarely seen. We discussing the definition, clinical findings, diagnostic approaches of arteriovenous malformations.

A complex approach to the treatment of arteriovenous IV-V degree malformations according to Spetzler‒Martin scale. Clinical case

Four therapeutic approaches have been developed for the treatment of arteriovenous malformations (AVMs): microsurgery, radiosurgery, embolization, and conservative treatment. The lack of consensus regarding the choice of treatment method and the different specializations of the doctor who are onvolved to the patient with AVM often determine the wrong treatment strategy. We performed a prospective analysis of the results of AVM of the IV degree according to Spetzler‒Martin scale, 4–5 points on the Buffalo scale complex treatment, based on clinical and tomographic data after the use of partial endovascular embolization and radical microsurgical removal of the AVM node assisted by cell saving technology. According to cerebral angiography 4 months follow-up there is no visible AVM vessels, the malformation was completely resected.Treatment of large and giant AVMs (IV and V degrees according to the Spetzler‒Martin scale) requires tailored surgical treatment approache using endovascular, microsurgical and radiosurgical techniques, but in many cases without achieving a radical result. The use of cell saver technology is necessary to reduce the risks of intraoperative complications associated with blood loss during microsurgical intervention, and enables radical removal of the AVM and recovery of the patient. The role of cell saver technology is crucial in vascular microsurgical interventions, which are often accompanied by a significant volume of blood loss, ensuring rapid autologous hemotransfusion and restoration of circulating blood volume. Endovascular embolization is a necessary step to reduce the risks of intraoperative complications during microsurgical intervention, which, together with the use of cell saver technology, makes it possible to achieve radical AVM removal and patient recovery.

Analysis of results determination of level of vascular endothelial growth factor, receptor for vascular endothelial growth factor and big endotelin-1 in structure of arteriovenous malformations

Objective ‒ to assess the diagnostic significance of angiogenesis factors based on the study of the level of angiogenesis factors (vascular endothelial growth factor (VEGF-A), specific receptor for vascular endothelial growth factor (VEGFR-1), endothelial inflammation factor (Big Endothelin-1)) in arterial and venous blood of patients with arteriovenous malformations (AVM) and healthy individuals without AVM. Materials and methods. In 2019–2021 a study according to the data of enzyme-linked immunosorbent assay the level of VEGF-A, VEGFR-1 and Big Endothelin-1 in the plasma of arterial and venous blood of 50 (19 (38.0 %) men and 31 (62.0 % women)) with AVM of different localization was carried out. The age of patients ranged from 3 to 51 years, the mean age ‒ (27.79 ± 2.19) years. The control group consisted of 35 healthy individuals (20 men and 15 women) without AVM. Patients with AVMs were divided into two groups: with small and medium-sized AVM in the stage of compensation (n=32) and with large and giant AVM in the stage of subcompensation or decompensation (n=18). Results. A significant (p < 0.05) difference was proved between the concentration of VEGF-A, VEGFR-1, Big Endothelin-1 in the plasma of arterial and venous blood of patients with AVM. This fact proves that the processes of pathological angiogenesis occur precisely in the structure of AVM. The value of VEGF-A, VEGFR-1 and BE-1 in patients with small and medium AVM was higher than in healthy individuals. The VEGF-A level was significantly (p < 0.05) in 2.4 times higher than in the control group, the VEGFR-1 level ‒ in 1.4 times, and the ET-1 level ‒ in 1.5 times. The value of VEGF-A, VEGFR-1 and BE-1 level in patients with large and giant AVM was significantly (p < 0.05) higher than in healthy individuals: VEGF-A ‒ in 7.2 times, VEGFR-1 ‒ in 2.36 times, and BE-1 ‒ in 1.7 times. Conclusions. The data obtained make it possible to statistically reliably state that the processes of pathological angiogenesis occur directly in the structure of AVM. A statistically significant dependence of the level of VEGF-A, VEGFR-1 and BE-1 in the blood plasma of patients on the size of the AVM and the stage of its compensation was revealed.

Preoperative Percutaneous Glue Embolisation of Giant Chest Wall Arteriovenous Malformation

Peripheral arteriovenous malformations (AVMs) are uncommon compared with more common intracranial AVMs, with chest wall AVMs being further uncommon. There are multiple treatment options which include observation, endovascular embolisation, surgical resection or combination. Giant AVMs usually have multiple feeders and are difficult to treat completely by surgery or embolisation alone. Therefore, preoperative embolisation is done to achieve bloodless surgical field and decrease blood loss during surgery. We present a hybrid approach to treat giant chest wall AVM where both Interventional Radiology and Surgical teams worked in tandem and achieved complete cure.

Giant complicated glosso-cervical arterio venous malformation managed with external carotid artery ligation and serial injection sclerotherapy: a case report and review of literature

Giant arterio venous malformation (AVM) of the tongue and floor of mouth are rare. They become life threatening when ignored by the patients. Treatment protocols are not well established. This report describes the treatment of a 35 years old female who presented to us with complains of swelling of tongue, floor of mouth and left sub mandibular region since 15 years causing dysphagia, and oral bleed since one day. Magnetic resonance Angiography revealed a giant AVM at the above site with dilated left external carotid artery (ECA) and left lingual artery. Patient underwent ECA ligation under general anaesthesia and serial injection sclerotherapy at the local site. After 3 months and 6 cycles of sclerotherapy, the patient had a satisfactory outcome. A detailed report with review of literature is presented.

A rare case of treatment of Giant paravertebral arteriovenous malformation combined with idiopathic scoliosis

The paper describes a case of successful treatment of a 32-year-old female patient with idiopathic scoliosis combined with giant paravertebral arteriovenous malformation in the lower thoracic region, causing the T11 vertebral body destruction, circular epidural compression, and myelopathy. The treatment was performed using 3D printing technology and hybrid surgery. The follow-up period after the first surgery was 26 months. The case report is accompanied by a literature review that analyzes world experience in the treatment of spinal arteriovenous malformations.

A Giant Arteriovenous Malformation Involving Tongue, Neck and Face

Abstract not availableUniversity Heart Journal Vol. 13, No. 1, January 2017; 26-28

Glomangiomyoma of the neck in a child in Nepal: a rare case report and literature review

BackgroundGlomangiomyoma is a rare histological variant of glomus tumour. Clinically, it mimicks as a haemangioma and is challenging to diagnose. Its occurrence in the neck of a child has not been previously described.Case presentationA 3 year old girl presented with the complaints of painless progressive neck swelling in the right side for one and half year. Sonography, computed tomography (CT), magnetic resonance imaging (MRI), CT neck angiography and fine needle aspiration cytology (FNAC) were suggestive of vacular malformation i.e. giant haemangioma or arteriovenous malformation. The mass was removed in toto under general anaesthesia without postoperative complications. The histopathology confirmed it to be glomangiomyoma with haemangiopericytoma like features.ConclusionIt’s an extremely rare variant of glomus tumour and may be the first report of a glomangiomyoma in the neck of a child. Despite a rare entity, it should be borne in mind during differential diagnosis.

Complex Cerebrovascular Presentation in Central Nepal with Therapeutic Challenge

Effective management of complex vascular malformation remains one of the more challenging problems faced in most of the developing countries where modern therapeutic resources are deficient. Giant arteriovenous malformations and other vascular malformation like dural arteriovenous malformations are often associated with higher morbidity and mortality rates. These lesions are considered as untreatable or inoperable. Endovascular therapy is an expanding science for managing complex vascular malformations in Nepal that has many constraints. This outset will have positive impact in the country that has to deal with moderate resources to provide wide therapeutic benefit. Here we present our preliminary experience about the use of ethylene vinyl alcohol (Onyx- 18) to embolize dural arteriovenous fistula and use of n-butyl cyanoacrylate to embolize giant supratentorial arteriovenous malformation.Nepal Journal of Neuroscience 12:73-80, 2015

Treatment of a giant arteriovenous malformation associated with intracranial aneurysm rupture during pregnancy: A case report.

Arteriovenous malformations (AVMs) associated with aneurysm have rarely been reported in the literature. The present study reports the case of a 21-year-old pregnant female patient who presented with a subarachnoid hemorrhage and an intracranial hematoma located in the anterior end of the corpus callosum. Furthermore, an anterior cerebral aneurysm and an AVM were identified by digital subtraction angiography and magnetic resonance angiography. The aneurysm was clipped and the AVM was successfully removed by microsurgery. The diagnosis of AVM associated with an aneurysm was confirmed via intraoperative and postoperative pathological examinations. By performing a review of the current literature, issues and surgical considerations associated with AVM associated with aneurysm were analyzed.

Three-Dimensional Printed Modeling of an Arteriovenous Malformation Including Blood Flow

Arteriovenous malformations (AVMs) represent a complex pathologic entity in terms of their associated angioarchitecture and blood flow dynamics. Using existing imaging data, we generated a patient's giant AVM to scale. A series of 3-dimensional (3D) models were generated and blood flow dynamics were represented. Faculty and resident surveys were positive regarding the technology. This report represents a novel application of 3D printing in neurosurgery and a means to model dynamic blood flow in 3 dimensions. The 3D printed models may improve on our ability to plan for and treat complex vascular pathologies.

Late presentation of missed aorta-caval fistula as arteriovenous malformation following lumbar disc surgery

Arteriovenous malformation in the abdomen following excision of lumbar intervertebral disc is the nightmare of the spinal surgeons [ 1 Keskin M. Serin K.R. Genc F.A. Aksoy M. Yanar F. Kurtoglu M. Iatrogenic major vascular injury during lumbar discectomy: report of three cases. Turk Neurosurg. 2013; 23: 385-388 PubMed Google Scholar , 2 Gentile V. Musco B. Della Giovampaola C. Lippolis P.V. Seccia M. [Hemorrhagic shock caused by iatrogenic aorto-cava injury. Case report]. Ann Ital Chir. 2007; 78: 251-255 PubMed Google Scholar , 3 Duz B. Kaplan M. Gunay C. Ustunsoz B. Ugurel M.S. Iliocaval arteriovenous fistula following lumbar disc surgery: endovascular treatment with a Stent-graft. Turk Neurosurg. 2008; 18: 245-248 PubMed Google Scholar ].

Long-term Outcomes of Patients With Giant Intracranial Arteriovenous Malformations.

Giant intracranial arteriovenous malformations (AVMs) are rare cerebrovascular lesions that pose management challenges. To further clarify outcomes in patients with giant cerebral AVMs managed with conservative or interventional therapies. We performed a retrospective review of all patients diagnosed with AVMs evaluated at our institution from 1990 to 2013. Patients with a single intracranial AVM >6 cm were included. Patients were divided into 2 groups: conservative management or intervention (microsurgery, radiosurgery, or embolization). Functional outcome was assessed with the modified Rankin Scale (mRS) and compared between the 2 groups. A total of 55 patients with giant AVMs were included, and 35 patients (63.6%) had clinical follow-up with a mean of 11.8 years. Spetzler-Martin grades were as follows: grade III, n = 2 (3.6%); grade IV, n = 15 (27.3%); and grade V, n = 38 (69.1%). Twenty-four patients (43.6%) were conservatively managed. The patients in the conservatively managed group had larger AVMs (P < .05) with more frequent involvement of the temporal lobe (P = .02). Five patients (26.3%) in the conservatively managed group and 5 (31.3%) in the intervention group experienced hemorrhage during follow-up, translating to an annualized risk of 2.7% and 4.1%, respectively. No significant difference in risk of first subsequent hemorrhage was observed (P = .78). Despite comparable mRS scores at presentation, we observed a trend toward better outcomes (mRS < 2) in patients undergoing conservative management (P = .06) compared with the intervention group at last follow-up. This study suggests that interventions for giant AVMs should be considered cautiously because hemorrhagic risk is similar regardless of management strategy and functional outcome is likely to be same or better in the conservatively managed population. AVM, arteriovenous malformationmRS, modified Rankin Scale.

Embolization with Gamma Knife Radiosurgery of Giant Intracranial Arteriovenous Malformations.

Giant arteriovenous malformations (i.e., those greater than 6 cm maximum diameter or volume > 33 cc) are difficult to treat and often carry higher treatment morbidity and mortality rates. In our study, we reviewed the angiographic results and clinical outcomes for 11 patients with giant arteriovenous malformations who were treated between 1994 and 2012. The patients selected included 9 males (82%) and 2 females (18%). Their presenting symptoms were hemorrhage (n=2; 18%), seizure (n=7; 64%), and headache (n=2; 12%). Nine patients were Spetzler-Martin Grade III, 2 were Spetzler-Martin Grade IV. The mean arteriovenous malformation volume was 41 cc (33-52 cc). The mean age of the patients was 45.1 years (24-57 years) and the mean radiation dose delivered to the margin of the nidus was 14.2 Gy. Ten patients received pre-Gamma Knife radiosurgery embolization and Gamma Knife radiosurgery, 1 patient received pre-Gamma Knife radiosurgery embolization and Gamma Knife radiosurgery twice and the interval between Gamma Knife radiosurgeries was 3 months. The complete obliteration rate following Gamma Knife radiosurgery was 36%, subtotal obliteration ( > 70% decreased size of nidus) was 36%, and partial obliteration was 28%. One patient experienced a small hemorrhage after embolization. Combined embolization and Gamma Knife radiosurgery showed successful obliteration of the arteriovenous malformation nidus. The use of embolization to initially reduce nidus size followed by Gamma Knife radiosurgery improves the treatment results. Repeated Gamma Knife radiosurgery should be a treatment option when there is a small nidus remnant.

Dev Pulmoner Arteryovenöz Malformasyon

Pulmonary arteriovenous malformation is a rare clinical entity that can lead to emergency clinical situations such as bleeding, pneumothoraks and the long-term chronic hypoxic problems due to right-to-left shunt. Although pulmonary arteriovenous malformation is usually combined with hereditary hemorrhagic telangiectasia, it rarely seen as sporadic. Twenty years-old female patient had consolidated areas in the right lung on chest X-ray. The multiple, inter-related vascular masses with early arterial intense contrast enhancement which the largest of them approximately 25x20 mm in size were observed in computed tomography. There were not a similar disease in family and epistaxis complaint, in patient’s medical history. A case of giant pulmonary arteriovenous malformation is presented with radiological findings and the literature data.

A giant pulmonary arteriovenous malformation without hereditary hemorrhagic telangiectasia: successful interventional embolization using a PDA occluder

A giant pulmonary arteriovenous malformation without hereditary hemorrhagic telangiectasia: successful interventional embolization using a PDA occluder

Total resection in a giant left frontal arteriovenous malformation, grade V Spetzler-Martin - Case report

Abstract Background: Giant arteriovenous malformations (AVMs) are congenital lesions, with nidus sizing more than 6 cm. According to Spetzler-Martin scale, grade V AVMs have a nidus larger than 6 cm in diameter, profound venous drainage and are located in eloquent areas. Method: We report a case of a 39 years old woman, with giant left frontal AVM, grade V Spetzler-Martin, who was admitted for generalized seizures, with onset 32 years ago, refractory to full dose antiepileptic polytherapy, which severely impaired the patient’s quality of life. Results: The patient underwent surgery and we performed total resection of the AVM. We emphasize on surgical technique, intraoperative difficulties and outcome. Conclusions: Surgery is the therapy of choice in AVMs, because it provides cure of the lesion, and is the only treatment capable of preventing hemorrhage and controlling seizures. Management in grade V AVMs is challenging, because of their large size, multiple dilated arterial feeders from anterior and posterior circulation and external carotid arteries, high blood flow, vascular steel from the surrounding brain, enlarged draining veins, profound venous drainage and location in eloquent area. Giant AVMs with high flow nidus, causing a great degree of vascular steel in the surrounding brain, with hypoperfusion of normal parenchyma may develop early normal perfusion pressure breakthrough. Total resection in grade V AVMs can be performed with minimal transient morbidity and favorable outcome. Total resection permits control of intractable seizures with reduced dose of antiepileptic therapy.