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Central nervous system (CNS) dural arteriovenous fistulas (DAVF) have been reported in PTEN‐related hamartoma tumor syndrome (PHTS). However, PHTS‐associated DAVF remain an underexplored field of the PHTS clinical landscape. Here, we studied cases with a PTEN pathogenic variant identified between 2007 and 2020 in our laboratory (n = 58), and for whom brain imaging was available. Two patients had DAVF (2/58, 3.4%), both presenting at advanced stages: a 34‐year‐old man with a left lateral sinus DAVF at immediate risk of hemorrhage, and a 21‐year‐old woman with acute intracranial hypertension due to a torcular DAVF. Interestingly, not all patients had 3D TOF/MRA, the optimal sequences to detect DAVF. Early diagnosis of DAVF can be lifesaving, and is easier to treat compared to developed, proliferative, or complex lesions. As a result, one should consider brain MRI with 3D TOF/MRA in PHTS patients at genetic diagnosis, with subsequent surveillance on a case‐by‐case basis. [ABSTRACT FROM AUTHOR]

Background: Cognitive decline is a non‐hemorrhagic, major complication of intracranial dural arteriovenous fistula (DAVF), thought to be primarily related to venous hypertension. However, imaging features to predict cognitive decline are scanty in the literature. Purpose To evaluate functional connectivity (FC) changes of resting‐state networks (RSNs) in DAVF before and after treatment and its relation to cognitive impairment. Study Type: Prospective. Subjects: DAVF subjects were screened for inclusion. Pre‐embolization (N = 33, mean age 45.9 years, 29 males), 1 month post‐embolization (N = 20, mean age 42.7 years, 19 males), and healthy controls (HC, N = 33, mean age 45.09 years, 27 males). Field Strength/Sequence: 3.0 T, resting‐state functional magnetic resonance imaging (MRI), three‐dimensional (3D) T1, T2 fast spin echo (FSE), diffusion weighted imaging (DWI), susceptibility weighted imaging (SWI), fluid‐attenuated inversion recovery, and time of flight. Assessment Data quality assessment was performed. FC analysis was done using group independent component analysis (ICA) and seed to voxel analysis. Neuropsychology (NP) scores of patients were compared with HC and correlated with FC changes. Statistical Tests: Voxel‐wise parametric T‐statistics for F‐test was executed in FC analysis (p‐FDR corrected <0.05). NP scores between DAVF group and HC group were compared using one‐way analysis of variance with post hoc Bonferroni correction (P < 0.05). Results: Both RSNs analysis methods showed reduced FC at the precuneus‐posterior cingulate cortex (PC‐PCC) of default mode network (DMN), anterior cingulate cortex (ACC) of the salience network (SN), and possible compensatory increased connectivity at the frontoparietal (FPN) and dorsal attention (DAN) networks. DAVF with low NP scores showed reduced FC at DMN and SN and minimal to absent connectivity at FPN and DAN. At post‐embolization 1‐month follow‐up, improvement in FC at PC‐PCC of DMN and ACC of SN were noted. Data Conclusion: RS‐fMRI in DAVF displayed FC changes that may be related to cognitive decline and its subsequent reversibility after treatment. FC changes at DMN, SN, FPN, and DAN were linked to cognitive decline and the corresponding NP scores. Level of Evidence: 2 Technical Efficacy: Stage 2 [ABSTRACT FROM AUTHOR]

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Purpose To develop a novel dynamic 3D noncontrast magnetic resonance angiography (MRA) technique that combines dynamic pseudo-continuous arterial spin labeling (dynamic PCASL), accelerated 3D radial sampling (VIPR), and time-of-arrival (TOA) mapping to provide quantitative assessment of arterial flow. Materials and Methods Digital simulations were performed to investigate the effects of acquisition scheme and sequence parameters on image quality and TOA mapping fidelity. Five patients with vascular malformations (arteriovenous malformation [AVM] = 3, dural arteriovenous fistula [DAVF] = 2) were scanned and the images were compared to digital subtraction angiography (DSA) for the ability to identify the arterial supply, AVM location, nidus size, and venous drainage. Results Digital simulations demonstrated reduced image artifacts and improved TOA accuracy using radial acquisition over Cartesian. TOA mapping accuracy is more sensitive to sampling window length than time spacing. Dynamic PCASL MRA depicted seven of eight arterial pedicles, and accurately measured the AVM nidus size when the nidus was compact. The venous drainage in the AVM patients was not consistently visualized. Conclusion Dynamic 3D PCASL-VIPR with TOA mapping is able to acquire both high temporal and spatial resolution inflow dynamics that could improve diagnosis of high-flow intracranial vascular diseases. J. Magn. Reson. Imaging 2014;39:1320-1326. © 2013 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

BSTRACT [ABSTRACT FROM AUTHOR]

ABSTRACT The clinical features, imaging and angiographic findings of thirty four patients with cranial dural arteriovenous malformations and fistulae are presented in four groups. Group 1 - Seven patients with anterior cavernous malformations, predominant superior ophthalmic vein drainage, and symptoms and signs of carotico-cavernous fistula. Group 2 - Twelve patients with malformations of the superior petrosal, transverse and sigmoid sinus regions, presenting predominantly with bruit. Group 3 - Seven patients with malformations of the basal sinuses and prominent cortical venous drainage, presenting with intracranial haemorrhage, headache and impaired cortical function. Intracranial haemorrhage never occured in the absence of cortical venous drainage. Group 4 - Eight patients with infrequent manifestations. Group 1 and 2 patients are readily recognized and diagnosed. Group 3 and 4 patients are often misdiagnosed. Treatment modalities comprised embolisation therapy, surgical excision, and carotid compression. Twenty patients were treated by one or more of these modalities with a successful outcome in thirteen patients. Group 1 patients are the most amenable to trans-arterial embolisation. Carotid compression as the sole modality of treatment was successful in four patients. Unless the fistula is successfully closed, Group 3 patients and patients who present with cervical or thoracic myelopathy carry a grave prognosis. There is need for greater radiologist awareness of Group 3 and the rarer presentations, particularly myelopathy. With the exception of one patient, the morphological features of our cases are consistent with the now-accepted view that these lesions are acquired arterio-venous fistulae and not congential malformations. [ABSTRACT FROM AUTHOR]