Rare Intracranial Vascular Malformation Research Articles

A Rare Case of Pial Arteriovenous Fistula in an Infant – A Case Report

Introduction: Pial arteriovenous fistula (PAVF) is a rare intracranial vascular malformation with diverse clinical presentations. Early diagnosis and intervention are crucial due to its poor natural history. Endovascular treatment has emerged as a preferred option with promising results. Case Report: We report a case of an 8-month-old infant who presented with recurrent focal seizures and acute intraparenchymal hematoma. Further investigation with magnetic resonance angiography and digital subtraction angiography confirmed the presence of a PAVF. Results: Endovascular embolization successfully obliterated the PAVF with no postprocedural complications. Clinical follow-up demonstrated a favorable outcome. Conclusion: This case highlights the significance of early detection through suitable imaging modalities and timely intervention in preventing life-threatening complications associated with PAVFs. Endovascular embolization offers an effective and safe treatment option for pediatric PAVFs, leading to positive outcomes in this rare entity.

Dural Fistula of the Superior Cerebellar Artery: A Case Report

Dural arteriovenous fistulas are rare intracranial vascular malformations with a propensity for hemorrhage. The Cognard classification system is the most widespread classification system wherein type IIB through V must be promptly treated to avoid the risk of hemorrhage. The case presented herein reports a 65 -year-old male presenting with vague non-hemorrhagic neurologic deficits found to have a Cognard type I right dural superior cerebellar arteriovenous fistula. Although quite obvious in retrospect, a DAVF can be missed even by an astute radiologist. This should be considered a “never miss” diagnosis as it carries a risk of intracranial hemorrhage and death.

Role of Stereotactic Radiosurgery in the Management of Dural AV Fistula.

Dural arteriovenous fistulas (DAVFs) are a relatively rare intracranial vascular malformation. The various treatment options for DAVFs include observation, compression therapy, endovascular therapy, radiosurgery, or surgery. A combination of these therapies may also be used. The treatment choice for DAVFs depends on the type of fistula, the severity of symptoms, DAVF angioarchitecture, and the efficacy and safety of treatments. The use of stereotactic radiosurgery (SRS) in DAVFs started in the late 1970s. There is a delay before the fistula gets obliterated after SRS and there is a risk of hemorrhage from the fistula till the fistula gets obliterated. Initial reports described the role of SRS in small DAVFs without severe symptoms, which were inaccessible by endovascular or surgical measures or in combination with embolization in larger DAVFs. SRS can be appropriate for indirect cavernous sinus DAVF fistulas (Barrow type B, C, and D). Borden types II and III and Cognard types IIb-V DAVFs have a high risk of hemorrhage and are traditionally considered less favorable to be treated with SRS as immediate treatment is required to decrease the risk of hemorrhage. However, recently SRS has been tried in these high-grade DAVF as a monotherapy. Factors that have a positive impact on the obliteration rates of DAVF following SRS are the location of DAVF with the cavernous sinus DAVF having much better obliteration rates than DAVF located at other locations, Borden Type I or Cognard Types III or IV DAVFs, absence of CVD, hemorrhage at the time of initial presentation, and target volume lesser than 1.5 mL.

Arterial approach in vein of Galen aneurysmal malformation: Coils and EVOH

BackgroundThe vein of Galen aneurysmatic malformation VAGM, is a rare intracranial vascular malformation, that represents approximately 1 % of neurovascular malformations and 20–30 % of all pediatric vascular malformations, although it has high rates of morbidity, it can be treated with an endovascular approach, with better results. Material and methodsWe made a retrospective, monocentric study with a total of 4 patients with a diagnostic vein of Galen malformation over a period of 9 years, reviewing medical charts, and diagnostic imaging studies, using the Bicêtre score to decide treatment, and using new techniques, combining materials in one or multiple sessions. ResultsComplete occlusion was achieved in all cases, almost all cases (75 %) had a better clinical presentation from the first session with just one embolization session, in 50 % of patients (2/4) both with a mural feeders type 2 and 1, respectively. The case with the lowest score 8/21, died after the first embolization session due to multiorgan dysfunction syndrome. ConclusionAn endovascular arterial approach combining EVOH, and coils is considered the best technique to treat vein of Galen aneurysmal malformation, because it has better control of the embolization, adding security and precision in the right hands, while also enhancing the cost-effectiveness of the procedure.

Vein of Galen Aneurysmal Malformation with Anomalous Right Superior Vena Cava to the Left Atrium Leading to Atypical Clinical and Echocardiographic Findings.

Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which typically presents in a critically ill newborn with intractable heart failure, severe pulmonary hypertension, and right heart dilation. We report two unusual cases of neonates with VGAM and anomalous connection of right superior vena cava to the left atrium. Both neonates were diagnosed with VGAM in utero and were clinically stable after birth with dilation of the left atrium and left ventricle and no evidence of pulmonary hypertension. One case with hydrocephalus underwent transcatheter embolization at 1week of age. The other case without hydrocephalus underwent elective transcatheter embolization at 4months. We postulate that the presence of a right superior vena cava to the left atrium provides a physiological advantage and counters the left-to-right shunt from the arteriovenous malformation. This provides insight to a potential treatment strategy to improve outcomes in patients with severe heart failure and pulmonary hypertension secondary to VGAM.

Endovascular Management of Intracranial Pial Arteriovenous Fistula with Giant Venous Varix: Case Report and Review of Literature.

Abstract: Background: Intracranial pial arteriovenous fistulas (AVFs) are rare intracranial vascular malformation and their treatment is challenging because of high-flow pial supply of the lesion. We report a case of intracranial pial AVF with associated giant venous varices managed successfully by embolization. Case Description: A 25-year-old male presented with sudden onset headache and altered sensorium. Computed tomographic scan was suggestive of frontal lobar hematoma that had dissected into the third ventricle with associated intraventricular hemorrhage. Magnetic resonance imaging revealed presence of large vascular channels with the presence of multiple flow voids in bilateral frontal location. Digital subtraction angiography revealed pial fistula with arterial supply from hypertrophied left anterior cerebral artery. Treatment was done using combined coil and onyx embolization after careful analysis of angioarchitecture of the lesion using three-dimensional rotational angiogram cross-sectional reconstructions. Postprocedure angiogram revealed closure of fistula and giant varices. Conclusion: Pial AVFs with giant venous varix in adults are rare vascular malformation and understanding the angioarchitecture with exact identification of fistulous point is important to plan for surgical/endovascular management strategies. Combined strategy of embolization using coils and onyx is a safe option in such cases with giant varices.

Dural Arteriovenous Fistulas - Regarding Two Clinical Cases

Dural arteriovenous fistulas (AVF) are rare intracranial vascular malformations, consisting of communications between dural venous arteries and sinuses, meningeal veins, cortical veins, or combinations thereof. In most cases the etiology is unknown. The clinical presentation is determined by the location and mainly by the drainage pattern. The recognition of this rare clinical entity is essential for an early diagnosis and intervention, to avoid the potentially serious complications of the disease, such as, intracranial hypertension, vascular dementia, hemorrhage, among others.

Pial arteriovenous fistula: A clinical and neuro-interventional experience of outcomes in a rare entity

Purpose:Pial arteriovenous fistulae (PAVF) are rare intracranial vascular malformations, predominantly seen in children and distinct from arteriovenous malformations and dural arteriovenous fistulae. PAVF often leads to high morbidity and mortality. The aim of our study was to describe the clinical features and endovascular management of PAVF at various intracranial locations; to analyze the use of liquid embolic agents and coils alone or in combination in the treatment of PAVF and to analyze the outcome of embolization.Materials and Methods:Retrospective review of diagnostic angiography and neurointerventional database of our institution identified a cohort of 15 patients with non-galenic PAVF from 2008 to 2014 out of 6750 patients. Fourteen patients were treated endovascularly with coils and liquid embolic materials in combination or alone. Patients were followed up for evaluation of prognosis.Results:Age of the patients ranged from 3 to 37 years. Most patients were male and most common presentation was headache followed by seizure. Most common location of fistula was frontal lobe. The most common type was single artery single hole fistula with venous varix. Satisfactory obliteration was seen in all cases. One patient developed intraparenchymal hematoma on the first post procedural day and outcome was poor.Conclusions:PAVF are rare intracranial vascular malformations which can effectively be managed endovascularly with liquid embolic, coils alone, or in combination. Complete occlusion of the fistula can be achieved in most cases in a single sitting with a reasonable morbidity related to the procedure, compared with the natural history of this disease.

De Novo Formation of Pial Arteriovenous Fistulas: Systematic Review of Acquired Lesions and Their Clinical Differences Compared with Primary Lesions

Acquired pial arteriovenous fistula (pAVF) is an extremely rare intracranial vascular malformation, with few case reports in the English literature. This study presents a thorough review and analysis of all acquired pAVF cases from the literature in addition to an illustrated case. We report a case with de novo development of intracranial pAVF after craniotomy. A medical literature database search between 1975 and 2018, including the Medline, Ovid, and PubMed databases, was performed to identify all reports with possible acquired lesions. Differences between these acquired lesions and previously reported primary lesions were evaluated. A total of 8 patients with de novo formation of acquired pAVF were included in this series. Most of these pAVFs were fed and drained via a similar arteriovenous pattern, from distal/cortical branches of the middle cerebral artery (6/8, 75%) to the superficial middle cerebral vein (6/8, 75%). Compared with a previously reported primary pAVF series, acquired pAVF tended to be asymptomatic (P < 0.0001) and found essentially in adults (P= 0.0061). Fewer venous varices (P= 0.0049) and associated intracranial mass effect (P= 0.0189) were found in the cases of acquired pAVF. All 4 reported acquired pAVFs that were treated microsurgically resulted in complete angiographic obliteration (4/4, 100%). The overall outcome was good or stable even with observation only (7/8, 87.5%). Acquired pAVF is highly correlated with sentinel neurosurgical procedures or venous occlusion events. These lesions should be regarded as a different disease entity from primary pAVF because of the relatively low-flow shunting and benign clinical course.

Sonographic Diagnosis of Fetal Adrenal Hemorrhage Complicating a Vein of Galen Aneurysmal Malformation.

The vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which causes end-organ ischemia or venous congestion due to heart failure. Adrenal hemorrhage associated with VGAM has not been reported in the literature. We present the imaging findings of a fetal VGAM with adrenal hemorrhage. A 26 year-old primigravida woman whose fetus with VGAM and mild cardiomegaly was scanned in the 34th week. On fetal ultrasound, a hyperechoic, well-circumscribed mass in the left suprarenal region was shown. Fetal and postnatal magnetic resonance imaging confirmed the diagnosis of adrenal hemorrhage. The baby died after delivery. Adrenal hemorrhage can complicate VGAM in fetuses with severe heart failure. Evaluation of the adrenal gland in affected cases may contribute to the prenatal counseling, and postnatal management.

Surgical management of a pial arteriovenous fistula with giant varix in an infant

A seven-month-old infant presented with a generalized seizure. The radiological evaluation revealed a large arteriovenous fistula in the left sylvian region. The fistula connected the left middle cerebral artery and the vein of Trolard. A giant varix was present at the venous end. The child underwent craniotomy, complete disconnection of the fistula and excision of the varix. Except for recurrent seizures, which were eventually controlled on anticonvulsants, the child's neurological development has been good on long-term follow-up of three years. Pial arteriovenous fistulae are rare intracranial vascular malformations. Though significant operative risks exist, they can be successfully managed surgically with good long-term prognosis.