Dural Arteriovenous Fistulas: A Characteristic Pattern of Edema and Enhancement of the Medulla on MRI

Copelan, Alexander; Krishnan, Anant; Marin, Horia; Silbergleit, Richard

doi:10.3174/ajnr.a5460

Cited by 11 publications

(6 citation statements)

References 13 publications

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“…While conventional angiography is still to be considered the gold standard for definite diagnosis of CVFs, MRI can strongly aid the diagnosis and dramatically shorten the time to diagnosis, especially when MRA sequences or contrast studies are carried out. Abnormal vascular flow voids, which are tortuous and dilated veins generally found on the dorsal or ventral surface of the spinal cord, were eventually found in 81.6% of patients, even when they were not reported initially [ 20 , 21 ]. A high index of clinical suspicion is then required to carefully evaluate MRI images looking for flow voids so to reduce the interval to diagnosis and, accordingly, achieve a better outcome.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2023

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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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Section: Discussionmentioning

confidence: 99%

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

et al. 2023

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“…In T2-weighted MRI, DAVF-related cord oedema is hyperintense, longitudinally extensive, centromedullary and poorly delineated [ 14 ]. Cognard type V DAVF have been reported to cause a characteristic pattern of central brainstem oedema with sparing of the periphery as well as internal linear segments in a tigroid pattern [ 15 ]. Asymmetric brainstem involvement, as seen in case 2, has also been reported [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

“…A non-enhancing cord segment amidst the enhancement (‘the missing piece sign’) due to inconsistent venous drainage, may be particularly suggestive of spinal DAVF over ATM [ 18 ]. Cognard type V DAVF have been reported to cause intense central or patchy brainstem enhancement, which may be mistaken for brainstem glioma [ 12 , 15 ].…”

Section: Discussionmentioning

confidence: 99%

A case series of intracranial dural arteriovenous fistulae mimicking cervical myelitis: a diagnosis not to be missed

et al. 2021

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Objective To describe the diagnostic features of intracranial dural arteriovenous fistulae (DAVF) presenting with cervical cord or brainstem swelling. Methods Retrospective case note and neuroimaging review of patients with angiographically confirmed DAVF diagnosed during January 2015–June 2020 at a tertiary neuroscience centre (Walton Centre NHS Foundation Trust, Liverpool, UK). Results Six intracranial DAVF causing cervical cord or brainstem oedema (all males aged 60–69 years) and 27 spinal DAVF (88% thoracolumbar) were detected over a 5.5-year period. Significantly more patients with intracranial DAVF received steroids for presumed inflammatory myelitis than those with spinal DAVF (5/6 vs 1/27, p = 0.0001, Fisher’s exact test). Several factors misled the treating clinicians: atypical rostral location of cord oedema (6/6); acute clinical deterioration (4/6); absence (3/6) or failure to recognise (3/6) subtle dilated perimedullary veins on MRI; intramedullary gadolinium enhancement (2/6); and elevated CSF protein (4/5). Acute deterioration followed steroid treatment in 4/5 patients. The following features may suggest DAVF rather than myelitis: older male patients (6/6), symptomatic progression over 4 or more weeks (6/6) and acellular CSF (5/5). Conclusion Intracranial DAVF are uncommon but often misdiagnosed and treated as myelitis, which can cause life-threatening deterioration. Neurologists must recognise suggestive features and consider angiography, especially in older male patients. Dilated perimedullary veins are an important clue to underlying DAVF, but may be invisible or easily missed on routine MRI sequences.

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“…Congestion with hemorrhage and ischemia secondary to intracranial dAVF is a very rare presentation, with fewer than 40 cases reported to date. 5,7 Choice of treatment-endovascular or neurosurgical-depends on the specific vascular anatomy of the dAVF. If these approaches are unavailable or unsuccessful, stereotactic radiosurgery may be an option.…”

Section: Discussionmentioning

confidence: 99%

Clinical Reasoning: A 63-year-old man with gastroenteritis progressing to stupor and quadriparesis

2020

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A 63-year-old man with a history of chronic right-sided headaches and intermittent nausea presented to the emergency department with worsening headache, nausea, and nonstop vomiting for 12 hours. Headache history dated back to the patient's early 20s. Headaches were exclusively right-sided, throbbing, associated with right eye and neck pain, photophobia and osmophobia, but without autonomic features. They occurred several times per week. He underwent multiple dental procedures, chiropractic manipulations, and C2-3 discectomy, but these interventions did not provide lasting relief, and he relied on nonsteroidal anti-inflammatory drugs to control the pain. Four years prior to his current hospital admission, he started to experience lightheadedness on getting up that resolved with lying down. Two years prior, he noticed problems with balance and had several falls. Six months prior, he had profound nausea for a week and lightheadedness that prevented him from getting out of bed. The symptoms abated on their own, but he would wake up daily with mild nausea and lightheadedness ever since. His medical history was otherwise only significant for asthma, hypertension, and obstructive sleep apnea. On admission, the patient was diagnosed with gastroenteritis and treated with fluids and antiemetics. The vomiting stopped, but the headache continued, and he developed new neurologic signs over several days: diplopia on left gaze, weakness progressing to quadriparesis, and increasing unresponsiveness (family described him as being "completely out of it-comatose"). Questions for consideration: 1. What is the localization of these signs and symptoms? 2. What diagnostic tests would you perform now? GO TO SECTION 2

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Dural Arteriovenous Fistulas: A Characteristic Pattern of Edema and Enhancement of the Medulla on MRI

Cited by 11 publications

References 13 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

A case series of intracranial dural arteriovenous fistulae mimicking cervical myelitis: a diagnosis not to be missed

Clinical Reasoning: A 63-year-old man with gastroenteritis progressing to stupor and quadriparesis

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