Surgical treatment of pial cerebellar arteriovenous fistulas with aneurysm of the main feeding artery

doi:10.1016/j.surneu.2005.04.005

Surgical Neurology

Volume 65, Issue 1, January 2006, Pages 90-94

https://doi.org/10.1016/j.surneu.2005.04.005 Get rights and content

Abstract

Background

Intracranial pial arteriovenous fistulas (pAVFs) are rare vascular lesions only recently considered distinct from arteriovenous malformations. Conservative management was associated with a high mortality rate. The abnormality of the lesion arises from its high-flow nature. The authors present a case of a cerebellar pAVF associated with aneurysms of the main feeding artery that was surgically treated.

Case Description

A 54-year-old woman was referred to us after an attack of severe headache and vomiting. Computerized tomography scan visualized a hematoma of the fourth ventricle. Digital subtraction angiography (DSA) revealed a cerebellar pAVF mainly fed by the right posteroinferior cerebellar artery (PICA) and with drainage into one of the superior cerebellar veins. The PICA presented a saccular aneurysm on its fourth segment and a fusiform dilatation on its third segment. The patient underwent surgical operation via a midline suboccipital craniotomy. The fusiform dilatation was wrapped and the saccular aneurysm was clipped. The most fistulous connections were closed. The draining vein at the level of torcular herophili was closed by application of a clip. Control DSA demonstrated exclusion of both the aneurysms and the fistula from the circulation. At 1-year follow-up, the patient was neurologically intact.

Conclusions

The association of a cerebellar pAVF and an aneurysm is rare. Surgical treatment of multiple-channel arteriovenous fistulas by closure of venous varix should guarantee the exclusion from the circulation and avoid recruitment of new arterial connections and recurrence. Attention must be paid to normal venous channels draining into the varix.

Introduction

Intracranial pAVFs are rare vascular lesions that have only recently been acknowledged as distinct from AVMs. They consist of a single or, more frequently, multiple arterial connections to a single venous channel without any intervening nidus of vessel or capillaries. They may be localized anywhere in the brain but display a preference for the supratentorial regions [17]. Pial arteriovenous fistulas can be acquired traumatically or iatrogenically or may be congenital. Their natural evolution is unfavorable; conservative management of pAVFs fistulas has been associated with mortality in 63% of patients [25]. Owing to the absence of a nidus, closure of the shunt by either endovascular or surgical technique represents a satisfactory therapeutic procedure [18].

We present the case of a 54-year-old woman with a pial cerebellar fistula of the left hemisphere, which was fed by the right PICA and the right superior cerebellar artery, and was associated with a small fusiform dilatation of the third segment as well as an aneurysm of the fourth segment of the PICA. We were unable to find any similar cases described by either English- or French-speaking authors. The surgical treatment of this case is described in the light of other possible therapeutic options.

Section snippets

Case report

A 54-year-old woman presented with abrupt onset of severe posterior headache and vomiting. Neurological examination showed disorientation, impaired attentiveness, and cerebellar ataxia. Computerized tomography (CT) scan documented the presence of a hematoma in the fourth ventricle, with mild dilatation of the supratentorial ventricular system. Angiography revealed a pAVF fed by a branch of the fourth segment of the PICA and branches of the superior cerebellar artery (Fig. 1A). There was also a

Discussion

Intracranial pAVF has only recently been distinguished from AVMs [18]. They consist of one or more arterial connections to a single venous channel without any intervening nidus of vessels or capillaries. They are rare vascular lesions. In fact, in a series of 320 AVMs reported by Halbach et al [14], single-channel arteriovenous fistulas accounted for 1.6% of all lesions. In the surgical series of 419 brain AVMs by Yasargil [35], pAVF accounted for 2.4% of the total.

Intracranial pAVFs differ

Conclusions

Only recently, pAVFs have been recognized as a pathological entity distinct from AVMs. They are characterized by the absence of a nidus and their high-flow nature. Because their prognosis appears very poor, endovascular or surgical treatment is recommended.

References (35)

K. Kikuchi et al.
Vascular malformations of the brain in hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease)
Surg Neurol
(1994)
P. Albert et al.
A study on the venous drainage of 150 cerebral arteriovenous malformations as related to hemorrhagic risks and size of the lesion
Acta Neurochir (Wien)
(1990)
G.M. Almeida et al.
Hemispheric arteriovenous fistulae with giant venous dilation
Childs Nerv Syst
(1990)
J.L. Antunes et al.
Giant hemispheric arteriovenous fistula in an infant
Surg Neurol
(1977)
N. Aoki et al.
Intracranial arteriovenous fistula manifesting as progressive neurologic deterioration in an infant: case report
Neurosurgery
(1991)
S.L. Barnwell et al.
Intracerebral arteriovenous fistulas associated with intraparenchymal varix in childhood: case reports
Neurosurgery
(1990)
H. Batjer et al.
Arteriovenous malformations of the posterior fossa: clinical presentation, diagnostic evaluation and surgical treatment
Neurosurg Rev
(1986)
A. Beltramello
Endovascular treatment of cerebrovascular pathology
B.R. Bendok et al.
Resection of a large arteriovenous fistula of the brain using low-flow deep hypothermic cardiopulmonary bypass: technical case report
Neurosurgery
(1999)
R. Carrillo et al.
Giant aneurysm arising from a single arteriovenous fistula in a child
J Neurosurg
(1984)

P. Coubes et al.

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

Childs Nerv Syst

(1996)

C.G. Drake

Cerebral arteriovenous malformations: considerations for and experience with surgical treatment in 166 cases

Clin Neurosurg

(1979)

R. Garcia-Monaco et al.

Congestive cardiac manifestations from cerebrocranial arteriovenous shunts: endovascular management in 30 children

Childs Nerv Syst

(1991)

C.A. Giller et al.

Interdisciplinary evaluation of cerebral hemodynamics in the treatment of arteriovenous fistulae associated with giant varices

Neurosurgery

(1994)

V.V. Halbach et al.

Transarterial occlusion of solitary intracerebral arteriovenous fistulas

AJNR Am J Neuroradiol

(1989)

A.L. Halliday et al.

Intracranial vertebral arteriovenous fistula: case report

J Neurosurg

(1993)

M. Hermier et al.

Endovascular treatment of an infantile nongalenic cerebral arteriovenous fistula with cyanoacrylate

Childs Nerv Syst

(1995)

Cited by (22)

A Systematic Review of Non-Galenic Pial Arteriovenous Fistulas
2023, World Neurosurgery
Citation Excerpt :
The systematic search of the literature disclosed 3280 candidate articles, of which 105 were retrieved for full-text evaluation. Ultimately, 86 articles met the eligibility criteria and were included in this review (Supplemental Table 1).3,6,7,9-91 Details of the study selection are illustrated in Figure 1.
Non-galenic pial arteriovenous fistulas (NGPAVFs) are rare cerebrovascular pathologies accounting for only 1.6%–4.8% of all brain vascular malformations. We performed a comprehensive review of NGPAVF cases reported in the literature to further characterize their clinical patterns of presentation, angiographic features, management, clinical outcomes, and complications.
We searched PubMed, Google Scholar, and Embase from each database's earliest records to April 2022 for all relevant English language articles. A total of 3280 articles were screened to identify those that met prespecified inclusion criteria. Differences in clinical outcomes between children (≤18 years old) and adults (>18 years old) and those articles in which NGPAVFs were associated with the presence of a varix or a hemorrhage were statistically examined.
A total of 242 patients in 86 articles were included. The mean patient age was 18.51 ± 18.80 years. The male-to-female ratio was 1.44:1. Headache was the most common initial presentation (42.6%) in the study cohort. Hemorrhage occurred at a significantly higher frequency in adults (P = 0.004), whereas more children presented with congestive heart failure (P < 0.001). Surgical, endovascular, and combination therapy led to comparable rates of complete NGPAVF obliteration (86.8%, 85.2%, and 88.5%, respectively). Fifty-nine patients (24.4%) experienced a complication, ranging from minor neurological deficit to severe hemorrhage. The mortality rate for the overall cohort was 3.3%, and all deceased patients had a varix associated with their fistulas.
To our knowledge, we report the largest literature review describing the clinical course and characteristics of NGPAVFs. All treatment approaches resulted in favorable obliteration rates and overall patient outcomes.
Pial-Dural Intracranial Arteriovenous Fistula with Flow-Associated Aneurysmal Rupture—Case Report with Review of Literature and Proposal on the Mechanism of Hemorrhage and Treatment Options
2017, World Neurosurgery
Citation Excerpt :
Articles not available in English or articles that could not be retrieved were excluded. The literature review of adults with PAVF yielded 51 (24 men and 27 women) identifiable cases (Table 1).7,10,12,15-45 Of 51 patients, 20 (39%) presented with hemorrhage; the remaining 31 patients presented with neurologic symptoms commonly secondary to the mass effect from the venous varix or other vascular abnormality.
Vascular anomalies in the form of dural arteriovenous fistulas (DAVFs), arteriovenous malformations, and aneurysms are well described in the literature. Pial arteriovenous fistulas (PAVFs) are described to a lesser extent in the literature. When these anomalies are combined, diagnosis and treatment become complex.
A 55-year-old man presented with PAVF/DAVF with a ruptured flow-related aneurysm in the distal left posterior inferior cerebellar artery, which required surgical clipping and disconnection. We performed a PubMed search of all identifiable cases of PAVFs in adults. We then identified which cases had components of DAVFs and flow-related aneurysms. We identified 51 PAVF cases and identified which patients presented with hemorrhage and the treatment modalities of all cases. Of 51 cases identified, 4 were a DAVF/FRA, and 5 were a PAVF/DAVF. One case of PAVF/DAVF/FRA was identified.
The exact mechanism of PAVF/DAVF formation is unknown. Hemorrhage predilection of PAVF/DAVF is more difficult to predict than a sole fistula, likely secondary to the complex flow dynamics. To our knowledge, this literature review is the largest review to date regarding PAVFs in adult patients and can provide insight into decision making when evaluating treatment options.
Endovascular treatment of cerebral aneurysms associated with arteriovenous malformations
2012, European Journal of Radiology
In univariate survival analysis, coexisting aneurysms was associated with a significantly increased risk of hemorrhage in AVMs. We report here on our clinical experience with AVMs associated with arterial aneurysms that were managed by endovascular treatment.
To determine the incidence of associated aneurysms, the authors reviewed 366 consecutive patients with AVMs managed between 1999 and 2009. In 86 (23.5%) of these 366 patients, 55 intranidal aneurysms and 40 proximal aneurysms were observed. Targeted endovascular treatment with coils, n-butylcyanoacrylate(NBCA) and Onyx was performed for patients in this series, using a standard protocol. We reviewed the treatment plans, radiological findings and clinical courses of 86 patients suffering AVM associated with aneurysm.
Ninety-five aneurysms in 86 patients with AVMs were enrolled in this study. Hemorrhage was the most frequent presenting symptom (69 patients, 80.2%). Bleeding was caused by an AVM nidus in 44 cases, aneurysm rupture in 20 and an undetermined origin in 5. Four patients were treated for associated aneurysm with coils followed by AVM embolization and 82 patients were treated with NBCA or Onyx embolization. There were total of 3 complications (3.5%) clinically significant complications in this series. Excellent or good outcomes (Glasgow Outcome Scale ≥ 4) were observed in 63 (73.3%) patients at discharge. Neurological deficits (Glasgow Outcome Scale 1–4) were 16.6% at discharge.
Endovascular treatment can be adequately used for cerebral aneurysms associated with AVMs as an adjunct to microsurgery and radiosurgery.
Cerebral arteriovenous malformations associated with flow-related and circle of willis aneurysms
2011, World Neurosurgery
Citation Excerpt :
Willis circle aneurysms have a greater rate of a subarachnoid hemorrhage. Flow-related aneurysms may develop as a result of increased intravascular shearing stresses secondary to the increased blood flow of AVMs acting on the vascular wall proximal to AVMs (6, 13, 14). This hypothesis is supported by a report in which the authors demonstrated that some extranidal aneurysms exhibited marked shrinkage during the course of endovascular AVM treatment (20).
To evaluate the characteristics of brain arteriovenous malformations (AVMs) with coexisting flow-related and Willis circle aneurysms.
The 302 consecutive retrospectively reviewed patients from the Beijing Tiantan Hospital were analyzed in this study. The presence of cerebral aneurysms was confirmed by pretreatment selective and superselective angiography. Univariate and multivariate analyses were performed for patient age, sex, history of rupture, associated aneurysms, AVM size, and deep and superficial venous drainage.
Of the 302 patients, 41 (13.6%) had AVMs associated with intranidal aneurysms, and 33 (10.9%) had AVMs associated with extranidal aneurysms. Of the 33 patients, 24 (72.7%) had a flow-related and 9 (27.3%) had a Willis circle aneurysm. Flow-related and Willis circle aneurysms correlated positively with intracranial hemorrhage (P = 0.003), patient age (P = 0.003), and infratentorial AVMs (P = 0.040) in multiple univariate analysis.
Flow-related and Willis circle aneurysms coexisting with cerebral AVMs frequently are associated with initial hemorrhage presentation, patient age, and infratentorial AVM location.
Characteristics of arteriovenous malformations associated with cerebral aneurysms
2011, World Neurosurgery
Citation Excerpt :
Cerebral AVM detection rate is 1.3/100,000 every year (5). Estimates for the frequency of coexisting aneurysms in patients with brain arteriovenous malformations (BAVMs) have ranged from 4% to 17% in most series (17-20). However, a greater proportion of BAVMs has been noted to have aneurysms when superselective angiography is performed (13, 22).
To analyze the characteristics of brain arteriovenous malformations (AVMs) associated with cerebral aneurysms.
A total of 302 consecutive, retrospectively reviewed patients from the Beijing Tiantan Hospital were analyzed in this study. The presence of cerebral aneurysm was confirmed by pretherapeutic selective and superselective angiography. Clinical presentation was categorized as intracranial hemorrhage or nonhemorrhagic presentation. Univariate and multivariate statistical models were applied to test the effect of age, sex, AVM size and location, and venous drainage pattern.
Of the 302 patients, 74 (24.5%) had AVMs associated with cerebral aneurysms. AVMs coexisting with aneurysms were significantly associated with hemorrhage at initial presentation (2.27; 95% confidence interval 1.31-3.95; P = 0.003). Infratentorial AVM location was significantly associated with AVMs coexisting with aneurysms (2.31; 95% confidence interval 1.02-5.24; P = 0.040). Deep or superficial venous drainage, female sex, or AVM size were not significantly associated with AVMs coexisting with aneurysms. The regression model showed significant effect was found for initial presentation with hemorrhage (P = 0.003), age (P = 0.003), and infratentorial AVM location (P = 0.040).
Our findings suggest that AVMs associated with cerebral aneurysms are frequently associated with initial hemorrhage presentation and infratentorial AVM location.
Endovascular Management of Hemorrhagic Stroke
2022, Biomedicines

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AneurysmSurgical treatment of pial cerebellar arteriovenous fistulas with aneurysm of the main feeding artery

Abstract

Background

Case Description

Conclusions

Introduction

Section snippets

Case report

Discussion

Conclusions

Surg Neurol

A study on the venous drainage of 150 cerebral arteriovenous malformations as related to hemorrhagic risks and size of the lesion

Acta Neurochir (Wien)

Hemispheric arteriovenous fistulae with giant venous dilation

Childs Nerv Syst

Giant hemispheric arteriovenous fistula in an infant

Surg Neurol

Intracranial arteriovenous fistula manifesting as progressive neurologic deterioration in an infant: case report

Neurosurgery

Intracerebral arteriovenous fistulas associated with intraparenchymal varix in childhood: case reports

Neurosurgery

Arteriovenous malformations of the posterior fossa: clinical presentation, diagnostic evaluation and surgical treatment

Neurosurg Rev

Endovascular treatment of cerebrovascular pathology

Resection of a large arteriovenous fistula of the brain using low-flow deep hypothermic cardiopulmonary bypass: technical case report

Neurosurgery

Giant aneurysm arising from a single arteriovenous fistula in a child

J Neurosurg

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

Childs Nerv Syst

Cerebral arteriovenous malformations: considerations for and experience with surgical treatment in 166 cases

Clin Neurosurg

Congestive cardiac manifestations from cerebrocranial arteriovenous shunts: endovascular management in 30 children

Childs Nerv Syst

Interdisciplinary evaluation of cerebral hemodynamics in the treatment of arteriovenous fistulae associated with giant varices

Neurosurgery

Transarterial occlusion of solitary intracerebral arteriovenous fistulas

AJNR Am J Neuroradiol

Intracranial vertebral arteriovenous fistula: case report

J Neurosurg

Endovascular treatment of an infantile nongalenic cerebral arteriovenous fistula with cyanoacrylate

Childs Nerv Syst

Aneurysm
Surgical treatment of pial cerebellar arteriovenous fistulas with aneurysm of the main feeding artery