Surgical flow disconnection of cerebral pial dual-channel arteriovenous fistula with a large varix: the role of anti-platelet agent or anti-coagulation therapy

Introduction

Intracranial pial arteriovenous fistula (AVF) is a rare cerebrovascular lesion, and the literature is sparse. Most authors insist that simple disconnection of arteriovenous shunting is enough to treat intracral single-channel pial AVF in most cases, either by microsurgery or endovascular embolization without resection of entire vascular malformation.

Discussion

We report an insidious onset of peri-lesional edema formation after abrupt disconnection of AVF shunting with surgical arterial ligation. Treatment entailed anti-platelet and anti-coagulation agent to slow down thrombus formation in intracranial dual-channel pial AVF. We suggest that slowing down thrombus formation in the large varix with anti-platelet and/or anti-coagulation agents seems to be necessary in case of abrupt disconnection of shunting in intracranial single- or dual-channel pial AVF to prevent postoperative cerebral edema or infarction.     

Endovascular Therapy of a Craniocervical Pial AVF Fed by the Anterior Spinal Artery

Though pial arteriovenous fistulae (PAVF) are an uncommon cerebrovascular disorder, their presentation with subarachnoid hemorrhage (SAH) is not rare. PAVF near the craniocervical junction are rare and may have a worse outcome. These fistulae are often fed from either the carotid and/or the vertebrobasilar systems, but are rarely fed by the anterior spinal artery. We report the case of a young man presenting with SAH. Cerebral angiography revealed 2 AVF, a symptomatic PAVF located at the craniocervical junction and fed from the anterior spinal artery and incidental dural AVF (DAVF) originate from the left occipital and middle meningeal arteries. These fistulae were treated with different endovascular techniques, including Onyx and platinum coil embolization into the feeding arteries of the DAVF and PAVF, respectively.     

A Case of Pial Arteriovenous Fistula with Giant Venous Aneurysm and Multiple Varices Treated with Coil Embolization

Intracranial pial arteriovenous fistulas (AVFs) are rare vascular lesions of the brain. These lesions consist of one or more arterial connection to a single venous channel without true intervening nidus. A 24-year-old woman visited to our hospital because of headache, vomiting, dizziness and memory disturbance that persisted for three days. She complained several times of drop attack because of sudden weakness on both leg. Cerebral angiograms demonstrated a giant venous aneurysm on right frontal lobe beyond the genu of corpus callosum, multiple varices on both frontal lobes fed by azygos anterior cerebral artery, and markedly dilated draining vein into superior sagittal sinus, suggesting single channel pial AVF with multiple varices. Transarterial coil embolization of giant aneurysm and fistulous portion resulted in complete disappearance of pial AVF without complication.     

Total endovascular occlusion of a giant direct arteriovenous fistula in the posterior fossa in a case of Rendu-Osler-Weber disease

We report a case of giant single-hole direct arteriovenous fistula (AVF) located in the posterior fossa of a child with Rendu-Osler-Weber disease (ROW). There is neither a clinical nor an angioarchitectural difference between AVFs associated with ROW and sporadic AVFs, but ROW should be suspected in children with multifocal pial AVFs. Transarterial embolization was performed to obliterate the AVF in a unique procedure using simultaneous bifemoral catheterization. At follow up 4 years later, the clinical examination was normal. Control MR scans and angiography showed the total occlusion of the fistula, permanent thrombosis of the venous pouch, and disappearance of the abnormal venous drainage. In this case, the endovascular procedure was available and we estimated that it represented a lesser risk than the surgical approach (risk of hemorrhage and risk of thrombosis involving the feeding arteries and the brain stem venous drainage). Furthermore, it avoids craniotomy and reduces the duration of hospitalization.     

Delayed Dural Arteriovenous Fistula after Microvascular Decompression for Hemifacial Spasm

Dural arteriovenous fistula (AVF) is very rare, acquired lesion that may present with intracranial hemorrhage or neurological deficits. The etiology is not completely understood but dural AVF often has been associated with thrombosis of the involved dural sinuses. To our knowledge, this is the first well documented intracranial hemorrhage case caused by dural AVF following microvascular decompression for hemifacial spasm. A 49-year-old male patient had left microvascular decompression of anterior inferior cerebellar artery via retrosigmoid suboccipital craniotomy. The patient was in good condition without any residual spasm or surgery-related complications. However, after 10 months, he suffered sudden onset of amnesia and dysarthria. Computed tomography and magnetic resonance imaging revealed the presence of dural AVF around the left transverse-sigmoid sinus. The dural AVF was treated with Onyx® (ev3) embolization. At the one-year follow up visit, there were no evidence of recurrence and morbidity related to dural AVF and its treatment. This case confirms that the acquired etiology of dural AVF may be associated with retrosigmoid suboccipital craniotomy for hemifacial spasm, even though it is an extremely consequence of this procedure.     

A case of intracranial arteriovenous fistula in an infant with neurofibromatosis type 1

INTRODUCTION: Reported cases of arteriovenous fistula (AVF) with neurofibromatosis type1 (NF1) are rare. CASE REPORT: In this paper we report the first case of intracranial AVF in an NF1 infant who developed heart failure. Endovascular treatment using coils successfully obliterated the AVF. The mechanism underlying the AVF in this case was believed to be a congenital mesenchymal abnormality of the intracranial vessels. DISCUSSION: The mechanism underlying the development of heart failure in this case is also discussed.     

三维影像融合在软脑膜动静脉瘘诊断与治疗中的应用价值

目的 探讨三维影像融合在软脑膜动静脉瘘（pAVF）诊断与治疗中的应用价值。方法 回顾性分析三家医院2014年9月到2015年2月收治的5例pAVF患者的临床资料。患者三维影像在Siemens Artis Zee Biplane双大平板DSA机的System Syngo X-WP三维后处理工作站上应用融合软件自动融合。分析3D-DSA双血管融合、CT/MR影像融合、3D-DSA/Dyna-CT影像融合,用于协助诊断与实施血管内治疗。从供血动脉、瘘口形态和位置、引流静脉及动静脉瘘与相邻脑结构的空间关系这4个方面来评价三维影像融合对诊断与治疗的应用价值。结果 3D-DSA双血管、MR/CT及3D-DSA/Dyna-CT等三维影像融合,可以把3D-DSA提供的血管系统信息与MR影像或Dyna-CT影像信息通过后处理融合在一张图像上,实现同步可视,将病变的双重血供和病变与脑组织、颅神经及颅骨的解剖关系显示清楚。5例患者的3D-DSA双血管、MR/CT或3D-DSA/Dyna-CT影像融合比未融合资料在对上述4个方面的评估上更具有价值,对诊断和手术更有意义。结论 三维融合影像更有助于对pAVF进行术前评估和设计手术计划,特别是有助于术前更好地理解病变的解剖特点和对栓塞材料的选择。     

A retromedullary arteriovenous fistula associated with the Klippel-Trenaunay-Weber syndrome

Summary An intraspinal vascular malformation associated with the Klippel-Trenaunay-Weber (KTW) syndrome initially was thought to be intramedullary on angiographic findings. Postmortem examination revealed an entirely posterior extramedullary arteriovenous fistula (AVF) fed by the anterior spinal artery. The association of the KTW syndrome with a so-called intramedullary AVF has been described in the literature without any pathologic confirmation (11 cases).Our case emphasizes the difficulty of determining the exact morphology and location of spinal AVF on arteriography. The association of the KTW syndrome with a retromedullary AVF can be explained on a developmental basis.     

Two consecutive dural arteriovenous fistulae in a child: a case report of successful treatment with gamma knife radiosurgery

Introduction The occurrence of dural arteriovenous fistulae in children is quite rare. Endovascular embolization is typically the first line treatment. In general, Gamma Knife radiosurgery is used as adjuvant treatment and seldom performed as the first line treatment in children. Discussion We report a case of a 27-month-old girl who presented with an initial dural arteriovenous fistula (AVF) located at anterior base of the left middle cranial fossa. She subsequently developed another dural AVF over the left transverse-sigmoid sinus region 2 years later. Conclusion Both fistulae were successfully obliterated with Gamma Knife radiosurgery.     

Conus perimedullary arteriovenous fistula in a child: unusual angio-architectural features and pathophysiology

INTRODUCTION: The perimedullary arteriovenous fistulas are located on the pial surface and are usually supplied by spinal medullary arteries, that is, either by the anterior or posterior spinal arteries, with no intervening nidus between the feeder arteries and the venous drainage. The clinical findings are, more commonly, caused by progressive radiculomedullary ischemic processes secondary to steal vascular mechanism. As the vascular supply to the spinal cord and to the arteriovenous fistulas (AVF) is not shared with one another, the vascular steal phenomenon cannot be implicated in this case's physiopathology. Most probably, the mass effect caused by the giant venous dilatation was the pathophysiological mechanism involved in this lesion CASE REPORT: The authors describe the case of a 6-year-old girl with an intradural ventral arteriovenous fistula, with a giant venous dilatation, fed directly by L2 and L3 radiculomedullary arteries at the conus medullaris. There was no arterial supply to the fistula from the anterior or posterior spinal arteries. Selective spinal angiography showed an arteriovenous fistula supplied directly by two radiculomedullary arteries, with a large draining vein caudally. Interposing the arterial and venous vessels was a giant venous aneurysmal dilatation located ventral to the conus medullaris and extending from L3 to T6. The patient was successfully treated by a surgical approach through a laminotomy from L3 to T11. CONCLUSION: The type IV-C spinal arteriovenous malformations or perimedullary AVFs are rare lesions predominately described at the conus medullaris with various types of angio-architecture and controversial treatment.     

Venous congestive myelopathy of the cervical spinal cord: An autopsy case showing a rapidly progressive clinical course

We report a rapidly progressive myelopathy in a 74‐year‐old Japanese man who was admitted to our hospital with a 4‐month history of progressive gait disturbance and died of pneumonia followed by respiratory failure on the 22nd day of admission. During the course of his illness, magnetic resonance imaging (MRI) revealed intramedullary lesions with edematous swelling from the medulla oblongata to the spinal cord at the level of the fourth vertebra. After administration of contrast medium, the ventral portion of the lesion was mildly and irregularly enhanced and a dilated vessel was recognized along the ventral surface of the upper cervical cord. At autopsy, ischemic changes were observed in the upper‐to‐middle cervical cord segments, with so‐called arterialized veins in the subarachnoid space. No neoplastic lesions were found within or outside the brain and spinal cord. These pathological findings were essentially those of venous congestive myelopathy (VCM) associated with dural arteriovenous fistulae (AVF), formerly known as Foix–Alajouanine syndrome. VCM associated with dural AVF, which is now considered to be treatable in the early stages, is rare found in the cervical spinal cord. The present autopsy case, with MRI findings, provides further information that might be useful for recognition and diagnosis.     

Vein of Galen malformation combined with atrial septal defect in a neonate

An arteriovenous fistula (AVF) is an abnormal connection between an artery and a vein, whereby the interconnecting capillary network is missing. Such a malformation frequently occurs in the deep midline regions of the brain, and the subsequent increased flow into the draining vein of Galen substantially dilates in an aneurysmal manner. Congenital forms of the aneurysmal dilatation of the vein of Galen (AVG) often lead to death in the neonatal period, predominantly due to cardiac failure caused by the increased venous inflow as a consequence of the intracerebral arteriovenous shunting. In the presented case a male baby suffered from a rare combination of a cerebral AVF and an atrial septal defect (ASD). He was born at week 38 of pregnancy and subsequently developed tachydyspnoe. Ultrasound (US) and CT scans revealed a large bilateral AVF with dilated basal venous sinuses, hydrocephalus and brain atrophy. In the heart, severe right ventricular hypertrophy, patent ductus arteriosus and an ASD were detectable by US. Neurosurgical consultation rejected the possibility of an operative treatment due to size and localization of the lesion and the existing irreversible brain damage. The child died because of cardiac failure 6 days after birth. Autopsy examination in the brain demonstrated a large conglomerate of dilated blood vessels predominantly in the midline and left occipital lobe, edema and hydrocephalus. In the heart, the ASD detected by US proved to be an ostium secundum-type lesion. Histologically, the conglomerate of vessels revealed features of an AVF and matched the characteristics of AVG. Consequences of chronic ischemic brain injury were also present, with ferruginated neurons suggesting intrauterine damage caused by a congenital AVF. Based on data in the literature, we assume that the left-to-right shunt due to increased venous influx into the heart caused not only cardiomegaly, but may have also interfered with the normal development of the atrial septum leading to an ASD, contributing to the rapid progression of the cardiac failure.     

Spinal intradural arteriovenous fistula with unusual presentation: case report and literature review

Object  The authors present their experience with a Cervical Spinal intradural arteriovenous fistula (AVF) with unusual clinical presentation in a pediatric patient and review of the international literature on this condition. Methods  A 5-year-old girl was found by her parents in bed screaming and talking inappropriately. Later on, she calmed down and complained of occipital, back and neck pain. She was admitted to the CHEO-ICU for work up and observation. The admission head CT scan showed evidence of SAH. Brain MRI showed blood in the third and fourth ventricles. MRA showed a C2 vascular lesion consistent with spinal cord AVF. Conventional angiogram was done which confirmed the AVF fed by a posterior medullary artery originating from the left vertebral artery. An attempt to embolize the AVF with glue was made but proved to be unsuccessful. On December 4, 2002, direct surgery on the AVF was performed, through a midline posterior approach (C2–C3 laminectomies) and microsurgical technique a complete excision of the intradural extramedullary AVF was achieved. The postoperative course was uneventful and the patient remained neurologically intact. Post-operative angiography confirmed complete excision of the AVF. Conclusion  The rarity of a pediatric spinal AVF presenting with spontaneous Intracranial SAH, makes this case very peculiar. This is the first reported pediatric case of cervical cord AVF with this clinical presentation found in the literature.     

Venous congestive myelopathy: Three autopsy cases showing a variety of clinicopathologic features

We describe three patients with progressive myelopathy, in whom autopsy revealed spinal cord pathology compatible with that of venous congestive myelopathy (VCM) associated with dural arteriovenous fistula (AVF), formerly known as angiodysgenetic necrotizing myelopathy (Foix–Alajournine syndrome). In these three patients, common symptoms were gait disturbance and sensory disturbance of the extremities, and these symptoms slowly worsened. The clinical diagnoses varied and included spinal cord intramedullary tumor, cervical spondylosis and multiple sclerosis. At autopsy, all the patients showed enlarged, tortuous venous vessels on the dorsal surfaces of the spinal cord at the affected levels. In the affected spinal cord parenchyma, necrotic lesions manifested by various degrees of neuronal loss and gliosis, with increased numbers of hyalinized vessels, were evident. The presence or absence of associated spinal dural AVF could not be identified histopathologically. Even with the help of modern neurological examination methods, early and accurate clinical diagnosis of VCM is sometimes difficult. When encountering patients with progressive myelopathy, VCM, although recognized as rare, should be considered as an important differential diagnosis.     

Acquired pial arteriovenous fistula following cerebral vein thrombosis.

BACKGROUND: We report a unique case of an acquired pial arteriovenous fistula occurring after an asymptomatic thrombosis of a superficial cerebral vein. CASE DESCRIPTION: A cerebral angiogram performed in a 51-year-old man with subarachnoid hemorrhage revealed a 10-mm ruptured anterior communicating artery aneurysm and a thrombosed left superficial middle cerebral vein. Coil embolization of the anterior communicating aneurysm was performed. Follow-up angiography 18 months later revealed a new, asymptomatic, pial arteriovenous fistula between the previously thrombosed left superficial middle cerebral vein and a small sylvian branch of the left middle cerebral artery. CONCLUSIONS: This case provides evidence that pial arteriovenous fistulas may develop as acquired lesions and furthermore may rarely follow cerebral vein thrombosis. Several cases of dural arteriovenous fistulas, as well as a single case of a mixed pial-dural arteriovenous fistula, occurring after dural sinus thrombosis have been reported previously. However, to our knowledge, this is the first report of an acquired pial arteriovenous fistula following a cerebral vein thrombosis.     

A case of cervical myelopathy due to dural arteriovenous fistula at the craniocervical junction]

A 70-year-old woman noted paresthesia ascending from both legs to her thighs 27 months previously. She also suffered from urinary urgency and incontinence. Thereafter, weakness in both legs developed and gradually became worse. At the time of admission, a neurological examination revealed diffuse atrophy and mild spasticity in all four extremities, bilateral mild weakness in both upper extremities, and severe weakness in both lower extremities. Her superficial sensation was moderately impaired below the Th 3 level on her right side, and below the Th 4 level on her left side along with a mildly decreased sense of vibration in her left leg. Marked hyperreflexia in all four extremities and bilateral pathological reflexes were also observed. Pollakisurea, urinary incontinence and constipation were also present. Cervical MRI showed a swelling of the spinal cord at the C3 to C7 levels. Inside the spinal cord, low signal intensity lesions were seen on the T1-weighted MRI, and high signal intensity lesions were observed on the T2-weighted MRI, and the rim of the cervical cord was also enhanced by gadolinium-DTPA. MR angiography revealed enlarged and tortuous vessels at the craniocervical junction, thus suggesting the presence of a dural arteriovenous fistula (AVF). Vertebral arteriography demonstrated abnormal vessels at the spinomedullary junction supplied by the right vertebral artery, which drained into the anterior and posterior spinal veins. After surgically treating the dural AVF, the swelling of the spinal cord, the abnormal signals on MRI, and the clinical symptoms all markedly improved. Although most of the spinal dural AVF were located at the thoracic and lumbar levels, the present case was considered to be a very rare case of dural AVF, since it was located at the craniocervical junction and thus led to the development of cervical myelopathy.     

Posterior Cerebral Artery Aneurysm Re-Rupture Following Revascularization for Moyamoya Disease

ObjectivesAneurysms associated with hemorrhagic moyamoya disease (MMD) are reported to stabilize or recede following revascularization.Case report/resultsA 29 year-old male with no past medical history presented obtunded with diffuse intraventricular hemorrhage and vascular imaging demonstrating bilateral MMD without any associated aneurysms. He underwent a delayed right-sided STA-MCA bypass and pial synangiosis, and was subsequently discharged on hospital day 24 with a modified Rankin Scale score (mRS) of 2. He returned eleven days later from a rehabilitation facility with recurrent IVH. A saccular 5 mm right P4 segment posterior cerebral artery aneurysm was seen on a diagnostic angiogram and embolized with Onyx glue.ConclusionsDistal posterior circulation artery aneurysmal rupture is a rare cause of hemorrhagic MMD. This case demonstrates the capacity of these aneurysms to re-rupture following revascularization and underscores the importance of treating the aneurysms directly.     

Animal Verbal Fluency and Design Fluency in school-aged children: effects of age, sex, and mean level of parental education, and regression-based normative data

The Animal Verbal Fluency (AVF) and Design Fluency (DF) structured and unstructured test versions were administered to N?=?294 healthy native Dutch-speaking children who were aged between 6.56 and 15.85 years. The AVF and DF structured test scores increased linearly as a function of age, whilst the relation between age and the DF unstructured test score was curvilinear (i.e., the improvement in test scores was much more pronounced for younger children than for older children). A higher mean level of parental education was associated with significantly higher AVF and DF structured test scores. Sex was not associated with any of the outcomes. Demographically corrected norms for the AVF and DF tests were established, and an automatic scoring program was provided.     

Spinal dural arteriovenous fistulae: exacerbation after myelography with metrizamide

Metrizamide dorsal myelography was performed in two patients with minor to moderate sensorimotor paraparesis. Direct and indirect myelographic signs of spinal arteriovenous aneurysm were seen and spinal cord angiography showed thoracic dural arteriovenous fistulae (AVF) in both cases. Within 24 hours following myelography, clear neurological worsening occurred, associated with cephalalgia, nausea and transient diplopia in one case, leading to paraplegia in a few days. Paraplegia was complete six months after surgery in one case, and had resolved after embolization of fistula in the other patient. The mechanism of neurological worsening may include: substraction of cerebrospinal fluid, sitting position during and after myelography and local increase of metrizamide concentration secondary to impaired resorption caused by the fistula. Water-soluble myelography is of invaluable aid in the diagnosis of dural AVF and must be followed by early angiography, thus allowing prompt therapeutic embolization.