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Cranial Dural Arteriovenous Fistula

2018; Lippincott Williams & Wilkins; Volume: 49; Issue: 12 Linguagem: Romeno

10.1161/strokeaha.118.022508

1524-4628

Patrick Chen, Michael M Chen, Marin A. McDonald, Kevin McGehrin, Jeffrey A. Steinberg, Jason Handwerker, Jamie Nicole LaBuzetta,

Meningioma and schwannoma management

HomeStrokeVol. 49, No. 12Cranial Dural Arteriovenous Fistula Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessReview ArticlePDF/EPUBCranial Dural Arteriovenous FistulaA Treatable Mimic Patrick M. Chen, MD, Michael M. Chen, MD, Marin McDonald, MD, PhD, Kevin McGehrin, MD, Jeffrey Steinberg, MD, Jason Handwerker, MD and Jamie Nicole LaBuzetta, MD Patrick M. ChenPatrick M. Chen Correspondence to Patrick Ming Chen, MD, University of California, San Diego Medical Center 200 W Arbor Dr. MC 8465, San Diego, CA 92103. Email E-mail Address: [email protected] From the Department of Neurosciences (P.M.C., M.M.C., K.M., J.N.L.), University of California, San Diego. , Michael M. ChenMichael M. Chen From the Department of Neurosciences (P.M.C., M.M.C., K.M., J.N.L.), University of California, San Diego. , Marin McDonaldMarin McDonald Department of Radiology (M.M., J.H.), University of California, San Diego. , Kevin McGehrinKevin McGehrin From the Department of Neurosciences (P.M.C., M.M.C., K.M., J.N.L.), University of California, San Diego. , Jeffrey SteinbergJeffrey Steinberg Department of Neurosurgery (J.S.), University of California, San Diego. , Jason HandwerkerJason Handwerker Department of Radiology (M.M., J.H.), University of California, San Diego. and Jamie Nicole LaBuzettaJamie Nicole LaBuzetta From the Department of Neurosciences (P.M.C., M.M.C., K.M., J.N.L.), University of California, San Diego. Originally published27 Sep 2018https://doi.org/10.1161/STROKEAHA.118.022508Stroke. 2018;49:e332–e334Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: September 27, 2018: Ahead of Print A 25-year-old previously healthy black female presented to the intensive care unit with acute-onset distal paresthesias and rapid, complete paralysis of her lower extremities, distal upper extremity weakness, bulbar findings, and dyspnea. One month prior she had sustained a severe kick to her posterior head and neck with persistent vomiting and photophobia that resolved before the onset of her presenting symptoms. Her admission exam was notable for transient ptosis, a weak cough and gag, complete paresis of her lower extremities, and distal greater than proximal weakness of her upper extremities. She had 4 mm reactive bilateral pupils, 3+ upper extremity reflexes with bilateral Hoffman reflex, intact normal lower extremity reflexes, no objective sensory deficit or sensory level, and had no bowel or bladder symptoms. Magnetic resonance imaging (MRI) brain and cervical spine revealed expansile T2 weighted imaging/fluid attenuation inversion recovery intramedullary signal abnormality in her lower pons/upper medulla extending to C2/C3 without significant internal contrast enhancement. After multiple negative blood and cerebrospinal fluid studies, she underwent treatment with various immunomodulating therapies for a presumed brainstem encephalitis and cervical myelitis without significant improvement on clinical examination.Repeat cervical spine MRI performed for clinical reevaluation showed markedly progressive T2 weighted imaging/fluid attenuation inversion recovery signal hyperintensity centered at the cervicomedullary junction which now extended to the level of C5/6 and was associated with patchy internal parenchymal enhancement (Figure 1). At this time, conspicuous flow voids along the dorsal pial surface of the upper cervical spine were noted, concerning for an underlying dural arteriovenous fistula (dAVF; Figure 1A and 1B). Diagnostic cerebral angiography revealed a dAVF supplied by a posterior meningeal branch of the right vertebral artery (Figure 2A and 2B). An early draining vein was evident within the suboccipital midline dura, ultimately draining into an anterior spinal vein, compatible with a Cognard type V dAVF. Microcatheterization of the posterior meningeal branch supplying the fistula was performed followed by liquid embolization with no residual fistula evident at the conclusion of the procedure (Figure 2C). Repeat MRI at follow-up showed improved edema, and she has shown slow clinical improvements in swallowing and ventilator weaning.Download figureDownload PowerPointFigure 1. Follow-up cervical spine magnetic resonance imaging. Sagittal (A) and axial (B) T2-weighted images demonstrated progressive expansile edema extending from the pontomedullary junction inferiorly to the level of C6 (A, arrows). At this time, subtle flow voids were noted along the pial surfaces of the dorsal brainstem and lower cervical spinal cord (arrowheads in A and B), raising suspicion for an underlying craniocervical dural arteriovenous fistula. In addition, sagittal (C) and axial (D) T1-weighted fat-saturated postcontrast images demonstrated patchy contrast enhancement within the regions of edema in the pontomedullary parenchyma and, to a lesser extent, the cervical cord parenchyma (C, arrows), with preservation of the underlying normal brain stem architecture (best seen in D, arrow).Download figureDownload PowerPointFigure 2. Diagnostic cerebral angiography. A, On sagittal oblique images injecting from the right vertebral artery, an abnormal fistulous connection was observed between a dural branch of the right vertebral artery (arrow) and an early draining vein (arrowheads) with (B) eventual drainage into an anterior anterior spinal vein (frontal delayed image, arrow), indicating a Cognard type V craniocervical dural arteriovenous fistula (dAVF). C, After liquid embolization, the early draining vein is no longer seen (dashed circle) and no residual dAVF is observed.DiscussionIntracranial dAVFs are anomalous interconnections between dural venous sinuses or cortical veins and dural arteries without a capillary nidus. Although the absolute incidence is unknown, dAVFs account for 15% of intracranial vascular lesions; 6% of these are supratentorial, and 35% are infratentorial lesions.1Although the pathophysiology of these lesions remains unknown, dAVFs in adults largely seem to be an acquired disease, consisting of multiple feeders from localized portions of the meninges supplied by external/internal carotid and vertebral arteries. Previous craniotomy, trauma, thrombosis, and occult thrombotic diseases (eg, Protein C/S deficiency, Factor V Leiden) have been implicated as predisposing factors. It is suspected that progressive dural venous sinus stenosis or thrombosis with secondary neoangiogenesis may underlie the pathogenesis of dAVF formation.2Clinically, cranial dAVFs have a heterogenous presentation which largely depends on the location of the fistula and its downstream effects. Cavernous sinus dAVFs often present with pulsatile tinnitus, cranial nerve palsies, and proptosis.1 Deep vein draining fistulas commonly present with signs of intracranial hypertension, including papilledema, hydrocephalus, and seizures. Brainstem dAVFs—as in this case—are exceedingly rare, have a variable onset, and present with cranial nerve palsies and myelopathy. An awareness of the insidious nature of dAVF in the posterior fossa is necessary to avoid treatment delay. Moreover, the clinical presentation is greatly influenced by associated edema from venous congestion along the distribution of drainage.The diagnosis of a dAVF requires an extensive clinical, neurological, and radiographic evaluation. Neurological examination should include funduscopic evaluation and auscultation for ocular bruits. Radiographically, computed tomography head noncontrast yield is often low unless it provides evidence of secondary hemorrhage and edema from an advanced dAVF. dAVF should be suspected with atypical hemorrhage locations. MRI T2 weighted imaging sequences can show the hallmarks of dAVF: large arterialized veins presenting as flow voids and edema from retrograde leptomeningeal venous drainage. Suspicious flow voids warrant further vessel imaging. Magnetic resonance angiography and computed tomography angiography have relatively low sensitivities of 15.4 % and 50%, respectively.1 However, the pairing of classic clinical symptoms and utilization of newer dynamic magnetic resonance angiography can increase sensitivity of radiographic modalities.1,2 Ultimately, catheter-based cerebral angiography (eg, diagnostic cerebral angiography) is the gold standard for diagnosis and provides the highest spatial resolution for fistula architecture, size, location, reflux, and treatment planning.2The most common classification schemes for dAVFs are the Borden and Cognard systems (Table). The Borden classification is simplest and is determined by fistula site and the presence of cortical venous drainage (CVD). The Cognard classification is based on the presence of CVD, the direction of blood flow (antegrade or retrograde), and the venous outflow architecture.3 Multiple studies have shown that the presence of CVD portends a more aggressive clinical course, and this distinction is used to differentiate benign (Borden I, Cognard I and IIa) and aggressive lesions (Borden type II and III and Cognard Type IIb–V).1,2 The dAVF in our case is best characterized as a Cognard type V dAVF, which by definition exclusively involves drainage of brain stem and spinal veins.3Table. Simplified and Collated Classification Schemes for dAVFCognard ClassificationVenous DrainageDrainage CharacteristicsCVDClinical CourseBorden ClassificationIDural sinusAntegradeNoBenignIIIaDural sinusAt least partially retrogradeNoBenignIIbDural sinus and cerebral veinsAntegrade into sinus; retrograde into cortical veinsYesAggressiveIIIIa+bDural sinus and cerebral veinsRetrogradeYesAggressiveIIICerebral veinsRetrogradeYesAggressiveIIIIVCerebral veins with venous ectasiaRetrogradeYesAggressiveVSpinal perimedullary veins……Aggressive— progressive myelopathyThe Cognard system incorporates venous drainage site, drainage characteristics, and presence/absence of CVD, whereas the Borden system is largely predicated on morphological characteristics of the venous drainage. Here these 2 systems are overlaid, principally based on venous drainage and clinical course. CVD indicates cortical venous drainage; and dAVF dural arteriovenous fistula.Our case demonstrates the diversity of clinical presentations and difficulty in diagnosing these dAVFs, especially infratentorially. Often, dAVFs clinically and radiographically mimic inflammatory, infectious, and infiltrative causes. Pontomedullary edema and contrast enhancement were seen in this case, but these are uncommon in the already rare Cognard type V dAVF. Given that a type V dAVF is predominantly from the anterior spinal vein, flow voids are difficult to visualize and often missed.4 Only 37% of Cognard V dAVFs will show flow voids, with sensitivity increasing to 76% when contrast MRI is utilized.5 Of note, our imaging shows edema sparing the internal structure of the brain stem; a cohort study recently described this as a highly characteristic radiographic hallmark of Cognard V variants.4,5Treatment of dAVFs depends on clinical course and the risks and benefits of the intervention. Lower-grade dAVFs are associated with low rates of hemorrhage and mortality—both 0% in dAVF without CVD.6 The presence of clinical symptoms (eg, tinnitus, ocular symptoms) increases hemorrhage and mortality rates to 1.4% and 0 %, respectively.6 Accordingly, Borden I and Cognard I and IIa dAVFs are typically managed conservatively with medical management and surveillance for new clinical symptoms.1,2,6However, in higher grade lesions—those with CVD—the annual rate of hemorrhage and mortality are 8% and 10%, respectively.1,6 As such, these cases warrant treatment targeting obliteration of the fistula. Endovascular therapy using either a transarterial or transvenous approach has become the first line intervention in the last 2 decades because of the development of advanced liquid embolizates, particulates, and coils. Multiple small cohorts show first-time complete embolization rates ranging from 60% to 87%.1 Surgery is reserved for failed endovascular therapy or when endovascular access is difficult to achieve safely. There is also preliminary data to support the adjunctive role of stereotactic radiation surgery.1In summary, cranial dAVFs are rare, heterogenous (both clinically and radiographically), and particularly difficult to diagnose infratentorially. Early recognition and treatment is paramount in management of this potentially reversible condition.TAKE HOME POINTSDural arteriovenous fistulas are a heterogeneous collection of arteriovenous shunts without a capillary nidus. The presence of cortical venous drainage portends a more aggressive clinical course.Prominent flow voids from arterialized veins are the classic magnetic resonance imaging hallmark of this condition, but occult dural arteriovenous fistula can often be missed. Diagnostic cerebral angiography is the gold standard for diagnosis, and endovascular intervention is the first line treatment for aggressive lesions.Cognard type V dural arteriovenous fistulas are defined by brain stem and perimedullary venous drainage. They can present with edema and contrast enhancement and should be considered in the differential diagnosis of patients with brainstem and myelopathy symptoms.DisclosuresNone.FootnotesCorrespondence to Patrick Ming Chen, MD, University of California, San Diego Medical Center 200 W Arbor Dr. MC 8465, San Diego, CA 92103. Email [email protected]eduReferences1. Gandhi D, Chen J, Pearl M, Huang J, Gemmete JJ, Kathuria S. Intracranial dural arteriovenous fistulas: classification, imaging findings, and treatment.AJNR Am J Neuroradiol. 2012; 33:1007–1013. doi: 10.3174/ajnr.A2798CrossrefMedlineGoogle Scholar2. Reynolds MR, Lanzino G, Zipfel GJ. Intracranial dural arteriovenous fistulae.Stroke. 2017; 48:1424–1431. doi: 10.1161/STROKEAHA.116.012784LinkGoogle Scholar3. Cognard C, Gobin YP, Pierot L, Bailly AL, Houdart E, Casasco A, et al. Cerebral dural arteriovenous fistulas: clinical and angiographic correlation with a revised classification of venous drainage.Radiology. 1995; 194:671–680. doi: 10.1148/radiology.194.3.7862961CrossrefMedlineGoogle Scholar4. Copelan AZ, Krishnan A, Marin H, Silbergleit . Dural arteriovenous fistulas: a characteristic pattern of edema and enhancement of the medulla on MRI [published online December 17, 2017].AJNR. http://www.ajnr.org/content/early/2017/12/07/ajnr.A5460. Accessed August 29, 2018.Google Scholar5. Roelz R, Van Velthoven V, Reinacher P, Coenen VA, Mader I, Urbach H, et al. Unilateral contrast-enhancing pontomedullary lesion due to an intracranial dural arteriovenous fistula with perimedullary spinal venous drainage: the exception that proves the rule.J Neurosurg. 2015; 123:1534–1539. doi: 10.3171/2014.11.JNS142278CrossrefMedlineGoogle Scholar6. Zipfel GJ, Shah MN, Refai D, Dacey RG, Derdeyn CP. Cranial dural arteriovenous fistulas: modification of angiographic classification scales based on new natural history data.Neurosurg Focus. 2009; 26:E14. doi: 10.3171/2009.2.FOCUS0928CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Foo S, Swaminathan S and Krings T (2021) Dilated MMA sign in cDAVF and other arterial feeders on 3D TOF MRA, The Neuroradiology Journal, 10.1177/19714009211041530, 35:3, (290-299), Online publication date: 1-Jun-2022. Lu X, Fang X, Huang Y, Zhou P, Wang Z, Brinjikji W and Chen G (2021) Cerebral Revascularization for the Management of Symptomatic Pure Arterial Malformations, Frontiers in Neurology, 10.3389/fneur.2021.755312, 12 Fesslova V, Colli A, Boito S, Fabietti I, Triulzi F and Persico N (2021) Dural Sinus Arteriovenous Malformation in the Fetus. Case Report and Discussion of the Literature, Diagnostics, 10.3390/diagnostics11091651, 11:9, (1651) Hashimoto R, Otsuka Y, Yoneda Y, Shiomi Y, Sekiya H, Morimoto T, Yamada K and Kageyama Y (2021) Cranio-cervical junction dural arteriovenous fistula mimicking neuromyelitis optica spectrum disorders presenting with intractable hiccups: a case report難治性吃逆で発症し視神経脊髄炎スペクトラム障害と鑑別を要した頭蓋頸椎移行部硬膜動静脈瘻の1例, Japanese Journal of Stroke, 10.3995/jstroke.10792, 43:2, (132-136), . Chen P, Olson S and Handwerker J (2020) Bithalamic Lesions, Stroke, 51:12, (e355-e358), Online publication date: 1-Dec-2020. Patel P, Talbot C, Meybodi A, Al-Mufti F, Sun H, Khandelwal P, Nanda A, Roychowdhury S and Gupta G (2020) Cognard Grade IV Tentorial Dural Arteriovenous Fistula Presenting as Trigeminal Neuralgia: Endovascular Management, World Neurosurgery, 10.1016/j.wneu.2020.08.119, 144, (184), Online publication date: 1-Dec-2020. Hou K, Li G, Qu L, Liu H, Xu K and Yu J (2020) Intracranial Dural Arteriovenous Fistulas With Brainstem Engorgement: An Under-Recognized Entity in Diagnosis and Treatment, Frontiers in Neurology, 10.3389/fneur.2020.526550, 11 December 2018Vol 49, Issue 12 Advertisement Article InformationMetrics © 2018 American Heart Association, Inc.https://doi.org/10.1161/STROKEAHA.118.022508PMID: 30571436 Manuscript receivedJune 15, 2018Manuscript acceptedAugust 29, 2018Originally publishedSeptember 27, 2018Manuscript revisedAugust 7, 2018 Keywordslower extremityarteriovenous fistulamagnetic resonance imagingangiographystrokePDF download Advertisement SubjectsCerebrovascular Disease/StrokeCerebrovascular MalformationsCerebrovascular ProceduresMagnetic Resonance Imaging (MRI)