Intracranial dural arterio-venous fistula presenting with progressive myelopathy

Background Intracranial dural arterio-venous fistulas are pathological anastomoses between arteries and veins located within dural sheets and whose clinical manifestations depend on location and hemodynamic features. They can sometimes display perimedullary venous drainage (Cognard type V fistulas—CVFs) and present as a progressive myelopathy. Our review aims at describing CVFs’ variety of clinical presentation, investigating a possible association between diagnostic delay and outcome and assessing whether there is a correlation between clinical and/or radiological signs and clinical outcomes. Methods We conducted a systematic search on Pubmed, looking for articles describing patients with CVFs complicated with myelopathy. Results A total of 72 articles for an overall of 100 patients were selected. The mean age was 56.20 ± 14.07, 72% of patients were man, and 58% received an initial misdiagnosis. CVFs showed a progressive onset in 65% of cases, beginning with motor symptoms in 79% of cases. As for the MRI, 81% presented spinal flow voids. The median time from symptoms’ onset to diagnosis was 5 months with longer delays for patients experiencing worse outcomes. Finally, 67.1% of patients showed poor outcomes while the remaining 32.9% obtained a partial-to-full recovery. Conclusions We confirmed CVFs’ broad clinical spectrum of presentation and found that the outcome is not associated with the severity of the clinical picture at onset, but it has a negative correlation with the length of diagnostic delay. We furthermore underlined the importance of cervico-dorsal perimedullary T1/T2 flow voids as a reliable MRI parameter to orient the diagnosis and distinguish CVFs from most of their mimics.

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Watch brain circulation in unexplained progressive myelopathy: a review of Cognard type V arterio-venous fistulas

Grado

Manfredi

Brugnera

et al. 2023

Neurol Sci

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Unilateral contrast-enhancing pontomedullary lesion due to an intracranial dural arteriovenous fistula with perimedullary spinal venous drainage: the exception that proves the rule

Roelz

Velthoven²,

Reinacher

et al. 2015

JNS

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Dural arteriovenous fistulas (DAVFs) are abnormal communications between dural arteries and dural venous sinuses, meningeal veins, or cortical veins. They account for 10%-15% of intracranial arteriovenous malformations.12,33 Grading of DAVF is based on the venous drainage pattern according to the classification schemes of Borden et al. and Cognard et al. 4,8 Venous cortical or perimedullary drainage and dilation are the hallmarks of high-grade DAVF and strongly correlate with increased risk of intracranial hemorrhage and nonhemorrhagic neurological deficit. The exact etiology of DAVFs is unknown. They are predominantly idiopathic, though they may also be congenital lesions or found in association with dural venous sinus thrombosis, trauma, previous craniotomy, and tumors. While spinal DAVF is an established cause of vascular myelopathy, intracranial DAVFs with perimedullary spinal venous drainage (Cognard Type V) are rare disorders and often represent a diagnostic and therapeutic challenge. They have been mainly described in case reports or small series that reported a typical clinical and radiological presentation of progressive myelopathy, central cord edema, and engorged perimedullary veins.Here, we report a case of an intracranial DAVF with exclusive perimedullary spinal venous drainage that presented initially with a very unusual MRI appearance suggestive of a right lateral brainstem neoplasm. After delayed diagnosis, the DAVF was completely occluded by transarterial embolization and the patient's symptoms gradually improved.abbreviatioNs DAVF = dural arteriovenous fistula; DSA = digital subtraction angiography; MRA = MR angiography. A large spectrum of possible diagnoses must be taken into consideration when a contrast-enhancing lesion of the pontomedullary region is found on MRI. Among these diagnoses are neoplastic, inflammatory, and infectious, as well as vascular pathologies. The authors report a rare case of an intracranial dural arteriovenous fistula (DAVF) with perimedullary spinal venous drainage (Cognard Type V) that initially presented as a unilateral contrast-enhancing pontomedullary lesion mimicking a brainstem neoplasm in a 76-year-old man. Following occlusion of the DAVF by transarterial embolization that resulted in clinical and radiological improvement, the fistula recurred 10 months later and was finally cured by a combined endovascular and surgical approach that resulted in complete occlusion. Clinical symptoms and MRI findings gradually improved following this treatment. A literature review on the MRI findings of Cognard Type V DAVF was performed. Centrally located medullary or pontomedullary edema represents the typical imaging finding, while unilateral edema as seen in the authors' patient is exceptionally rare. The hallmark imaging finding suggestive of DAVF consisting of perimedullary engorged vessels may not always be present or may only be very subtly visible. Therefore, the authors suggest performing contrast-enhanced MR angiography or even digital subtraction angiography in the ...

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Intracranial Dural Arteriovenous Fistulas With Brainstem Engorgement: An Under-Recognized Entity in Diagnosis and Treatment

Hou

et al. 2020

Front. Neurol.

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Background: In rare circumstances, patients with intracranial (dural arteriovenous fistulas) DAVFs could be complicated with brainstem engorgement, which might lead to delayed or false diagnosis and subsequent improper management. Methods: On July 2th, 2019, a systematic search was conducted in the PubMed database for patients with intracranial DAVFs complicated with brainstem engorgement. Results: Sixty-eight articles reporting of 86 patients were included for final analysis. The patients were aged from 20 to 76 years (57.10 ± 12.90, n = 82). The female to male ratio was 0.68 (35:51). Thirty-three (40.2%, 33/82) patients were initially misdiagnosed as other diseases. The specific location distributions were cranio-cervical junction, cavernous sinus, superior petrosal sinus, transverse and/or sigmoid sinus, tentorium, and other sites in 27 (32.5%), 11 (13.2%), 9 (10.8%), 10 (12.0%), 21 (25.3%), and 5 (6.0%) patients, respectively. The Cognard classification of DAVFs were II, III, IV, and V in 9 (10.7%, 9/84), 1 (1.2%, 1/84), 1 (1.2%, 1/84), and 73 (86.9%, 73/84) patients. Eighteen (22%, 18/82) patients were demonstrated to have stenosis or occlusion of the draining system distal to the fistula points. The mean follow-up period was 7.86 (n = 74, range 0-60 months) months. Fifty-four (70.1%, 54/77) patients experienced a good recovery according to the mRS score. Conclusions: Intracranial DAVFs complicated with brainstem engorgement are rare entities. Initial misdiagnosis and delayed definite diagnosis are common in the past three decades. The treatment outcome is still unsatisfactory at present. Early awareness of this rare entity and efficiently utilizing the up to date investigations are of utmost importance.

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Intracranial dural arterio-venous fistula presenting with progressive myelopathy

Cited by 4 publications

References 4 publications

Watch brain circulation in unexplained progressive myelopathy: a review of Cognard type V arterio-venous fistulas

Watch brain circulation in unexplained progressive myelopathy: a review of Cognard type V arterio-venous fistulas

Unilateral contrast-enhancing pontomedullary lesion due to an intracranial dural arteriovenous fistula with perimedullary spinal venous drainage: the exception that proves the rule

Intracranial Dural Arteriovenous Fistulas With Brainstem Engorgement: An Under-Recognized Entity in Diagnosis and Treatment

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