Borden type I intracranial dural arteriovenous fistula

Borden type I intracranial dural arteriovenous fistula

Type I dural arteriovenous fistulas are supplied by a meningeal artery or arteries and drain into a meningeal vein or dural venous sinus. The flow within the draining vein or venous sinus is anterograde.

Equivalent to Cognard type I and IIa, with a favorable natural history 1) 2).

Type Ia – simple dural arteriovenous fistulas have a single meningeal arterial supply

Type Ib – more complex arteriovenous fistulas are supplied by multiple meningeal arteries The distinction between Types Ia and Ib is somewhat specious as there is a rich system of meningeal arterial collaterals. Type I dural fistulas are often asymptomatic, do not have a high risk of bleeding and do not necessarily need to be treated

A small number of Type I DAVFs will convert to more aggressive DAVFs with CVD over time. This conversion to a higher-grade DAVF is typically heralded by a change in patient symptoms. Follow-up vascular imaging is important, particularly in the setting of recurrent or new symptoms. 3).

A comparative meta-analysis was completed to evaluate the outcomes of intervention versus observation of Borden type I intracranial dural arteriovenous fistula. Outcome measures included: grade progression, worsening symptoms, death due to dural arteriovenous fistula, permanent complications other than death, functional independence (mRS 0-2), and rate of death combined with permanent complication, were evaluated. Risk differences (RD) were determined using a random effects model.

Three comparative studies combined with the authors’ institutional experience resulted in a total of 469 patients, with 279 patients who underwent intervention and 190 who were observed. There was no significant difference in dAVF grade progression between the intervention and observation arms, 1.8% vs. 0.7%, respectively (RD: 0.01, 95% CI: -0.02 to 0.04, P = 0.49), or in symptom progression occurring in 31/279 (11.1%) intervention patients and 11/190 (5.8%) observation patients (RD: 0.03, CI: -0.02 to 0.09, P = 0.28). There was also no significant difference in functional independence on follow-up. However, there was a significantly higher risk of dAVF-related death, permanent complications from either intervention or dAVF-related ICH or stroke in the intervention group (11/279, 3.9%) compared to the observation group (0/190, 0%) (RD: 0.04, CI: 0.1 to 0.06, P = 0.007).

CoIntervention of Borden Type I dAVF results in a higher risk of death or permanent complication, which should be strongly considered when deciding on the management of these lesions 4).

From April 2013 to March 2016, consecutive patients with DAVF were screened at 13 study institutions. We collected data on baseline characteristics, clinical symptoms, angiography, and neuroimaging. Patients with Borden type I DAVF received conservative care while palliative intervention was considered when the neurological symptoms were intolerable, and were followed at 6, 12, 24, and 36 months after inclusion.

Results: During the study period, 110 patients with intracranial DAVF were screened and 28 patients with Borden type I DAVF were prospectively followed. None of the patients had conversion to higher type of Borden classification or intracranial hemorrhage during follow-up. Five patients showed spontaneous improvement or disappearance of neurological symptoms (5/28, 17.9%), and 5 patients showed a spontaneous decrease or disappearance of shunt flow on imaging during follow-up (5/28, 17.9%). Stenosis or occlusion of the draining sinuses on initial angiography was significantly associated with shunt flow reduction during follow-up (80.0% vs 21.7%, p = 0.02).

Conclusion: In this 3-year prospective study, patients with Borden type I DAVF showed benign clinical course; none of these patients experienced conversion to higher type of Borden classification or intracranial hemorrhage. The restrictive changes of the draining sinuses at initial diagnosis might be an imaging biomarker for future shunt flow reduction 5)


Davies MA, TerBrugge K, Willinsky R, Coyne T, Saleh J, Wallace MC. The validity of classification for the clinical presentation of intracranial dural arteriovenous fistulas. J Neurosurg. 1996 Nov;85(5):830-7. doi: 10.3171/jns.1996.85.5.0830. PMID: 8893721.

Strom RG, Botros JA, Refai D, Moran CJ, Cross DT 3rd, Chicoine MR, Grubb RL Jr, Rich KM, Dacey RG Jr, Derdeyn CP, Zipfel GJ. Cranial dural arteriovenous fistulae: asymptomatic cortical venous drainage portends less aggressive clinical course. Neurosurgery. 2009 Feb;64(2):241-7; discussion 247-8. doi: 10.1227/01.NEU.0000338066.30665.B2. PMID: 19190453.

Shah MN, Botros JA, Pilgram TK, Moran CJ, Cross DT 3rd, Chicoine MR, Rich KM, Dacey RG Jr, Derdeyn CP, Zipfel GJ. Borden-Shucart Type I dural arteriovenous fistulas: clinical course including risk of conversion to higher-grade fistulas. J Neurosurg. 2012 Sep;117(3):539-45. doi: 10.3171/2012.5.JNS111257. Epub 2012 Jun 22. PMID: 22725983.

Schartz D, Rahmani R, Gunthri A, Kohli GS, Akkipeddi SMK, Ellens NR, Romiyo P, Kessler A, Bhalla T, Mattingly TK, Bender MT. Observation versus intervention for Borden type I intracranial dural arteriovenous fistula: A pooled analysis of 469 patients. Interv Neuroradiol. 2022 Sep 13:15910199221127070. doi: 10.1177/15910199221127070. Epub ahead of print. PMID: 36113111.

Nishi H, Ikeda H, Ishii A, Kikuchi T, Nakahara I, Ohta T, Sakai N, Imamura H, Takahashi JC, Satow T, Okada T, Miyamoto S. A multicenter prospective registry of Borden type I dural arteriovenous fistula: results of a 3-year follow-up study. Neuroradiology. 2022 Apr;64(4):795-805. doi: 10.1007/s00234-021-02752-5. Epub 2021 Oct 10. PMID: 34628528; PMCID: PMC8907088.

Dural arteriovenous fistula

Dural arteriovenous fistula

Dural arteriovenous fistulas (DAVFs) are pathologic vascular connections that shunt dural arterial flow directly to dural venous drainage.

DAVFs comprise 10–15% of all intracranial AVMs 1). 61–66% occur in females, and patients are usually in their 40 s or 50 s. They occur rarely in children, and when they do they tend to be complex, bilateral dural sinus malformations 2)

Dural arteriovenous fistulas can occur at any dural sinus but are found most frequently at the cavernous or transverse sinus.

Intracranial dural arteriovenous fistula.

Spinal dural arteriovenous fistula.

The etiology and pathophysiology of DAVFs is not fully understood. Several hypotheses for development of DAVF and classifications for predicting risk of hemorrhage and neurological deficit have been proposed to help clinical decision making according to its natural history 3).

Radical treatment is to obliterate the draining veins in any treatment modalities including endovascular treatment or surgical treatment. Radiosurgery is the last choice. Transvenous embolization plays the main role in the DAVF of the cavernous sinus and anterior condylar confluence. Transarterial embolization with Onyx has dramatically improved the obliteration rate of the transverse-sigmoid, superior sagittal sinuses, and other non-sinus lesions. Transarterial NBCA injection is still the gold standard in the endovascular treatment of the spinal dural and epidural AVFs. Understanding of the functional microvascular anatomy is mandatory, especially in the transarterial liquid injection (Onyx and NBCA). Surgical treatment in the DAVF of the anterior cranial base, craniocervical junction, tentorial region, and spine is a safe and radical treatment. Postoperative follow-up is necessary from the viewpoint of chronological and spacial multi-occurrence of this disease 4).


Arnautovic KI, Krisht AF. Transverse-Sigmoid Sinus Dural Arteriovenous Malformations. Contemp Neurosurg. 2000; 21:1–6

Ashour R, Aziz-Sultan MA, Soltanolkotabi M, et al. Safety and efficacy of onyx embolization for pediatric cranial and spinal vascular lesions and tumors. Neurosurgery. 2012; 71:773–784

Sim SY. Pathophysiology and classification of intracranial and spinal duraAVF. J Cerebrovasc Endovasc Neurosurg. 2022 Apr 21. doi: 10.7461/jcen.2022.E2021.04.001. Epub ahead of print. PMID: 35443276.

Kuwayama N. Management of Dural Arteriovenous Fistulas. Adv Tech Stand Neurosurg. 2022;44:251-264. doi: 10.1007/978-3-030-87649-4_14. PMID: 35107684.

Intracranial dural arteriovenous fistula clinical features

Intracranial dural arteriovenous fistula clinical features

Clinical features of DAVF vary depending on their location, arterial supply, degree of arteriovenousshunting, and most importantly, their venous drainage pattern 1) 2) 3) 4)

DAVF lacking cortical vein drainage (CVD) may be asymptomatic, or present with symptoms related to increased dural sinus blood flow, such as pulsatile tinnitus, the latter particularly common for transverse sinus and sigmoid sinuses lesions.

Generalized central nervous system symptoms that may be related to venous hypertension or cerebrospinal fluid malabsorption, while resulting cranial nerve palsy, are often because of an arterial steal phenomenon or occasionally mass effect from an enlarged arterial feeder.

In addition, cavernous sinus dural arteriovenous fistula may present with orbital symptoms, including chemosisproptosisophthalmoplegia, and decreased visual acuity.

DAVF with CVD typically have more aggressive clinical presentations, including the sudden onset of severe headacheseizures, nonhemorrhagic neurological deficit (NHND), and intracranial hemorrhage, including intraparenchymal, subarachnoid, and subdural hematoma.

In a meta-analysis, Lasjaunias et al 5) reviewed 195 cases of DAVF and found that focal neurological deficits were related to the presence of associated cortical venous drainage (CVD) and venous congestion in the affected vascular territory. Less common aggressive presentations include brain stem or cerebellar dysfunction secondary to venous congestion, parkinsonism-like symptoms, extra-axial hemorrhage in the cervical spine, as well as cervical and upper thoracic myelopathy.

DAVF with extensive arteriovenous shunting, particularly in the setting of dural sinus thrombosis, can result in impaired venous drainage from the brain and the global venous hypertension. This can lead to cerebral edema, encephalopathy, and cognitive decline 6).

Pulsatile tinnitus is the most common presenting symptom of a DAVF. Cortical venous drainage with resultant venous hypertension can produce intracranial hypertension, and this is the most common cause of morbidity and mortality and thus the strongest indication for Intracranial dural arteriovenous fistula treatment.

DAVFs may also cause global cerebral edema or hydrocephalus due to poor cerebral venous drainage or by impairing the function of the arachnoid granulations, respectively. Other DAVF symptoms/signs include headaches, seizures, cranial nerve palsies, and orbital venous congestion.

Leptomeningeal venous drainage can lead to venous hypertension and intracranial hemorrhage.

The majority of patients presented with non-aggressive symptoms. 18% presented with intracranial hemorrhage: all the hemorrhages occurred in high-grade DAVFs 7).

see Dural arteriovenous fistula presenting as an acute subdural hemorrhage.

Only 4 cases of DAVF causing syncope have been reported, all in combination with other neurological symptoms. In comparison, they report a unique case of DAVF presenting solely with recurrent syncope, a previously undocumented finding in the literature. The case adds to other reports of nonspecific DAVF presentations and highlights the importance of considering this etiology 8).


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.A2798.

Sarma D, ter Brugge K.Management of intracranial dural arteriovenous shunts in adults.Eur J Radiol. 2003; 46:206–220.

Houser OW, Campbell JK, Campbell RJ, Sundt TMArteriovenous malformation affecting the transverse dural venous sinus–an acquired lesion.Mayo Clin Proc. 1979; 54:651–661.
4) , 5)

Lasjaunias P, Chiu M, ter Brugge K, Tolia A, Hurth M, Bernstein M.Neurological manifestations of intracranial dural arteriovenous malformations.J Neurosurg. 1986; 64:724–730. doi: 10.3171/jns.1986.64.5.0724.

Miller TR, Gandhi D. Intracranial Dural Arteriovenous Fistulae: Clinical Presentation and Management Strategies. Stroke. 2015 Jul;46(7):2017-25. doi: 10.1161/STROKEAHA.115.008228. Epub 2015 May 21. PMID: 25999384.

Signorelli, F. et al. Diagnosis and management of dural arteriovenous fistulas: A 10 years single-center experience Clinical Neurology and Neurosurgery , Volume 128 , 123 – 129

Sheinberg DL, Luther E, Chen S, McCarthy D, Starke RM. Recurrent Syncope Caused by a Dural Arteriovenous Fistula: A Case Report and Review of the Literature. Neurologist. 2021 Mar 4;26(2):62-65. doi: 10.1097/NRL.0000000000000322. PMID: 33646991.

Lumbosacral dural arteriovenous fistula

Lumbosacral dural arteriovenous fistula


The most common neurologic findings at the time of admission were paraparesis (85%), sphincter dysfunction (70%), and sensory disturbances (20%).

Clinical symptoms caused by deep lumbosacral spinal dural arteriovenous fistulas are comparable with those of spinal dural arteriovenous fistulas at other locations 1).


Spinal dural arteriovenous fistulas located in the deep lumbosacral region are rare and the most difficult to diagnose among spinal dural arteriovenous fistulas located elsewhere in the spinal dura. Specific clinical and radiologic features of these fistulas are still inadequately reported.

Medullary congestion in association with an enlargement of the filum vein or other lumbar radicular veins is a characteristic finding in these patients. Spinal time-resolved contrast-enhanced dynamic MRA facilitates the detection of the drainage vein and helps to localize deep lumbosacral-located fistulas with a high sensitivity before DSA. Definite detection of these fistulas remains challenging and requires sufficient visualization of the fistula-supplying arteries and draining veins by conventional spinal angiography.

Medullary T2 hyperintensity and contrast enhancement were present in most cases. The filum vein and/or lumbar veins were dilated in 19/20 (95%) patients. Time-resolved contrast-enhanced dynamic MRA indicated a spinal dural arteriovenous fistula at or below the L5 vertebral level in 7/8 (88%) patients who received time-resolved contrast-enhanced dynamic MRA before DSA. A bilateral arterial supply of the fistula was detected via DSA in 5 (25%) patients 2).


Patients with deep lumbosacral dural arteriovenous fistula had a higher risk of early recurrence compared to patients with thoracolumbar SDAVF, with a considerable percentage of late functional deterioration. Thus strict clinical and radiologic long-term follow-up examinations are recommended in those patients 3).

Case series

Jablawi et al. retrospectively evaluated all data of patients with spinal dural arteriovenous fistulas treated and/or diagnosed in RWTH Aachen University Hospital, and Paracelsus Kliniken, OsnabrückGermany, between 1990 and 2017. Twenty patients with deep lumbosacral spinal dural arteriovenous fistulas were included in this study.

They retrospectively analyzed our radiological and medical records for patients presenting with SDAVF between 1990 and 2018 at the University Hospital Aachen. We identified twenty patients with a lsDAVF. All patients were treated surgically. One patient died of pulmonary embolism three months after treatment and was excluded from our outcome analysis. Clinical data at the time of admission, discharge, one year after discharge and at the last follow-up were evaluated according to the modified Aminoff-Logue disability score (AL-score) for this analysis.

The mean age was 65 ± 7 years (median, 67; range, 53-78), sixteen patients (84 %) were male. After surgery, four patients developed a recurrent fistula in the same shunt zone and were re-treated microsurgically. Follow-up data one year after treatment was available in 15 patients. No relevant changes in AL-score were observed within this period. For the long-term follow-up analysis, data of 13 patients were available; 38.5 % of patients developed late functional deterioration.

In this cohort, patients with deep lumbosacral dural arteriovenous fistula had a higher risk of early recurrence compared to patients with thoracolumbar SDAVF, with a considerable percentage of late functional deterioration. Thus strict clinical and radiologic long-term follow-up examinations are recommended in those patients 4).

Rosi et al. describe a case series of five patients presenting with a conus medullaris AVS associated with a lower lumbar or sacral DAVF.

Three of the patients were <30 years old at presentation. In four of these five cases the intradural scAVS drained caudally, engorging the epidural plexus in the same location as the sDAVF. In only one case, who presented with thrombosis of the drainage of the main compartment of a conus medullaris pial AVF, was the location of the DAVF opposite to the location of the residual drainage.

They discuss the pathophysiological link between scAVS and sDAVF on the basis of the rarity of the DAVF, the uncommon association between scAVS and sDAVF, the presence of sDAVF in young patients, and the venous hypertension created by the venous drainage towards the sacral area responsible for angiogenesis creating the dural shunt 5).

Twenty-five consecutive patients with 16 thoracic dural arteriovenous fistula and 9 lumbosacral DAVFs were included (mean age, 63.9 years; 20 men). All patients presented with progressive myelopathy. Preoperative and postoperative neurologic deficits were compared between thoracic and lumbosacral DAVF groups. Using magnetic resonance imaging, the extent of T2 high-intensity areas and signal flow voids were documented. Follow-up after surgical interventions ranged from 6 to 96 months (mean, 38.1 months).

Preoperatively, patients suffering lumbosacral DAVF tended to be more severely disabled compared with thoracic DAVF patients. Lumbosacral DAVF patients exhibited diminished patellar (P = 0.04) and Achilles tendon reflexes (P < 0.01), while most thoracic DAVF patients exhibited hyperreflexia. In magnetic resonance imaging, signal flow voids around the spinal cord were evident in only 4 of 9 lumbosacral DAVF patients (P = 0.012). Rather, a serpentine signal flow void of the filum terminale was a hallmark of lumbosacral DAVFs to distinguish them from thoracic DAVFs. In the lumbosacral DAVF group, postoperative improvements were significantly better in micturition function (P = 0.02).

In lumbosacral DAVF, postoperative micturition function recovery was superior to thoracic DAVF. Intradural lumbar signal flow void is indicative of lumbosacral DAVF. For appropriate management, it is important to recognize these differences between lumbosacral and thoracic DAVF 6).

Case reports

A 65-year-old man presented with a 4-year history of progressive sensory, motor, and sphincter dysfunction. Spinal magnetic resonance imaging and digital subtraction angiography showed 2 spinal dural arteriovenous fistulas (fed by the right L2 lumbar artery and the right lateral sacral artery, respectively) and 1 perimedullary arteriovenous fistula (fed by the filum terminale artery from the left L2 lumbar artery [i.e., filum terminale arteriovenous fistulas]. A hybrid technique was used to perform embolization of the right L2 spinal dural arteriovenous fistula and microsurgery of the L5 level filum terminale vein. The patient was asymptomatic 1 year later.

Multifocal spinal vascular malformations may coexist in 1 case, and standardized spinal digital subtraction angiography, including the bilateral internal iliac arteries and median sacral artery, should be performed to avoid a missed diagnosis. The concomitant phenomenon indicates that venous hypertension may be a risk factor for the development of arteriovenous fistulas. Hybrid techniques are effective in treatment of multifocal and complex spinal AVMs 7).

Seven cases of adult spinal vascular malformations presenting in conjunction with spinal dysraphism have been reported in the literature. Two of these involved male patients with a combined dural arteriovenous fistula (DAVF) and lipomyelomeningocele. The authors present the third case of a patient with an extraspinal DAVF and associated lipomyelomeningocele in a lumbosacral location. A 58-year-old woman with rapid decline in bilateral motor function 10 years after a prior L4-5 laminectomy and cord detethering for diagnosed tethered cord underwent magnetic resonance imaging showing evidence of persistent cord tethering and a lipomyelomeningocele. Diagnostic spinal angiogram showed a DAVF with arterial feeders from bilateral sacral and the right internal iliac arteries. The patient underwent Onyx embolization of both feeding right and left lateral sacral arteries. At 6-month follow-up, MRI revealed decreased flow voids and new collateralized supply to the DAVF. The patient underwent successful lipomyelomeningocele exploration, resection, AV fistula ligation, and cord detethering. This report discusses management of this patient as well as the importance of endovascular embolization followed by microsurgery for the treatment of cases with combined vascular and dysraphic anomalies 8).


1) , 2) , 3) , 4)

Jablawi F, Nikoubashman O, Dafotakis M, Schubert GA, Hans FJ, Mull M. Treatment strategy and long-term outcome in patients with deep lumbosacral arteriovenous fistulas. A single center analysis in nineteen patients. Clin Neurol Neurosurg. 2019 Nov 11;188:105596. doi: 10.1016/j.clineuro.2019.105596. [Epub ahead of print] PubMed PMID: 31739154.

Rosi A, Consoli A, Condette-Auliac S, Coskun O, Di Maria F, Rodesch G. Concomitant conus medullaris arteriovenous shunts and sacral dural arteriovenous fistulas: pathophysiological links related to the venous drainage of the lesions in a series of five cases. J Neurointerv Surg. 2018 Jun;10(6):586-592. doi: 10.1136/neurintsurg-2017-013505. Epub 2018 Jan 19. PubMed PMID: 29352055.

Endo T, Kajitani T, Inoue T, Sato K, Niizuma K, Endo H, Matsumoto Y, Tominaga T. Clinical Characteristics of Lumbosacral Spinal Dural Arteriovenous Fistula (DAVF)-Comparison with Thoracic DAVF. World Neurosurg. 2018 Feb;110:e383-e388. doi: 10.1016/j.wneu.2017.11.002. Epub 2017 Nov 10. PubMed PMID: 29133002.

Li J, Li G, Bian L, Hong T, Yu J, Zhang H, Ling F. Concomitant Lumbosacral Perimedullary Arteriovenous Fistula and Spinal Dural Arteriovenous Fistula. World Neurosurg. 2017 Sep;105:1041.e7-1041.e14. doi: 10.1016/j.wneu.2017.06.149. Epub 2017 Jul 4. PubMed PMID: 28684369.

Krisht KM, Karsy M, Ray WZ, Dailey AT. Extraspinal type I dural arteriovenous fistula with a lumbosacral lipomyelomeningocele: a case report and review of the literature. Case Rep Neurol Med. 2015;2015:526321. doi: 10.1155/2015/526321. Epub 2015 Apr 8. PubMed PMID: 25949837; PubMed Central PMCID: PMC4407406.

Sylvian fissure meningioma without dural attachment

Sylvian fissure meningioma without dural attachment

Sylvian fissure meningiomas (SFMs) represent a rare subgroup of nondural-based tumors arising from the meningothelial cells within the arachnoid of the Sylvian fissure.

In 1938 Harvey Williams Cushing and Louise Eisenhardt reported two cases of this type of tumor named as “Deep Sylvian Meningiomas” 1).


The reported adult SFMs patients are young (mean age of 34.95 ± 3.35 years; 95% CI [27.93–41.97]) with a M:F ratio of 1.22 (11/9) and in the pediatric population (mean age is 5.71 ± 1.61 years; 95% CI [1.76-9.66]; the M:F ratio is 2:1 (4/2 and 1 unknown). When comparing grade I and grade II lesions, there is no significant differences in terms of mean age (grade I: 26.87 ± 3.90 years; vs grade II 24.33 ± 7.01 years; t-test p > 0.05), gender (grade I M:F ratio – 1.2 [12/10] versus grade II M:F ratio – 5 [5/1)]), clinical presentation (seizures is the most common presentation in both groups – grade 1 – 74% (17/23) and grade II – 67% (4/6)] and extent of resection (total resection in grade 1 – 65% [1/23] and total resection in grade II – 50% [3/6].

Only six atypical WHO grade II SFMs have been previously described 2).

Clinical features

They usually manifest with seizures and display the same radiological features of meningiomas in other locations.

Differential diagnosis

SFMs are rare entities and it is important to differentiate them from the sphenoid wing meningiomas. These are attached to the dura overlying the sphenoid wings, are usually associated with hyperostosis and they displace the MCA backwards as they grow, while the SFMs do not have dural attachment, do not produce hyperostosis and grow inbetween the MCA branches 3).


Although the absence of dural attachment makes these tumors suitable for a complete resection, their anatomical relationships with the middle cerebral artery branches may impair its achievement.

In the case of recurrent meningioma, surgical resection and adjuvant radiation therapy could be effective for long-term control of the tumor. 4).

Case reports

Hong et al., presented a histologically regressed relapsed meningioma, which spontaneously regressed after subtotal resection. In the case of recurrent meningioma, surgical resection and adjuvant radiation therapy could be effective for long-term control of the tumor. 5).

Donovan and Thavapalan, report two additional cases of sylvian fissure meningioma without dural attachment and one case of perisylvian meningioangiomatosis in the medial temporal lobe. All three patients presented with complex partial seizures, but the diagnosis was delayed in each case because the symptoms were misinterpreted to be behavioral rather than epileptic. The seizures were eventually confirmed with electroencephalogram, and subsequent imaging showed enhancing masses within the sylvian fissure region that were at least partially calcified in all three cases. Each patient underwent craniotomy. In the first case, gross total resection was achieved, and in the second case, a small residual portion of tumor was densely calcified and adherent to the middle cerebral artery branches. Both of these were World Health Organization (WHO) grade I meningiomas. The third patient underwent biopsy and limited resection of meningioangiomatosis. No dural attachments were noted in any of the tumors, but one of the meningiomas was intraparenchymal in location, surrounding the sylvian fissure in both the frontal and temporal lobes, which has been described in only a small number of these cases previously. The patients underwent pre- and postsurgical neuropsychiatric testing and did not experience any significant cognitive deficits. At 10-year follow-up, the patient who had gross total resection of the tumor has had no recurrence and is seizure-free without anticonvulsant medications. The incompletely resected intraparenchymal meningioma in the second patient recurred after 5 years, however, and at repeat surgery was found to have transformed to a WHO grade II tumor. Radiation therapy was delivered and the tumor has been stable for 2 years, but the patient continues to have occasional seizures despite medication. The patient with meningioangiomatosis has had no further growth and has excellent control of seizures but remains on medication. 6).

A heterogeneous contrast-enhanced mass in the right sylvian fissure of a 10-year-old boy with a 3-year history of epilepsy was identified via magnetic resonance imaging. The patient underwent partial surgical resection because the tumor was hard and contained numerous perforators arising from the right middle cerebral artery. The tumor was histologically diagnosed as sclerosing meningioma. Twelve months after surgery, the patient was asymptomatic and did not require any additional therapies. This case is the first report of a sclerosing meningioma arising in the deep sylvian fissure 7).

Aras et al., reported staged surgery for sylvian fissure meningiomas without dural attachment in two cases 8).

In 2013 a Deep Sylvian Meningioma in a 43-Year-Old Man 9).

Ma et al., reported a case of sylvian fissure atypical meningioma with a 20-year history. The tumor was excised subtotally, thereafter a postoperative radiation therapy was done. The patient had a favorable outcome during the two-year follow-up 10).

Miyahara et al., reported a 34-year-old female with an 8-year history of temporal lobe epilepsy. Magnetic resonance imaging showed a multilobular, well-demarcated and homogeneous tumorous lesion of 5 cm in diameter deep in the left sylvian fissure. Intraoperative findings revealed that the tumor was mainly in the left insular region without dural attachment and strongly adhered to the left middle cerebral artery and its perforators. The histopathological diagnosis was transitional meningioma without malignancy 11).

Cecchi et al., described an atypical sylvian fissure meningioma in a 23-year-old male with a brief history of headache and mild hemiparesis 12).

A 6-year-old boy presented with seizures. Computed tomography and magnetic resonance imaging showed a large enhancing mass in the left temporo-parietal region.

He underwent left temporo-parietal craniotomy and total excision of the lesion. At surgery, there was no dural attachment, and the tumor was mainly in the posterior part of left sylvian fissure. The biopsy was reported as WHO grade I meningioma.

At 4-year follow-up, he was asymptomatic, and there was no tumor recurrence. 13).

Brain CT scan performed on a 73-year-old woman on admission for non-specific symptoms revealed. a heterodense temporoparietal mass which was demonstrated on carotid angiography as being fed by the middle cerebral artery. Preoperatively, a glioma was considered as being most probable because of its radiological features. The mass, which at surgery was found to be located in the sylvian fissure, was histologically confirmed to be a meningotheliomatous meningioma with fibroblastic component 14).

In 2005 McIver et al., first reported case of a chordoid meningioma without dural attachment arising in the sylvian fissure.

The patient presented with a generalized seizure. A heterogeneously enhancing right frontotemporal mass was identified on magnetic resonance imaging of the brain.

The patient underwent a failed stereotactic biopsy attempt elsewhere. The tumor was ultimately resected using standard microsurgical techniques.15).

A 35-year-old male. The patient visited the hospital because of a 10-year history of simple partial seizures. Magnetic resonance imaging revealed a 3.5-cm, well-circumscribed, homogenously enhanced, circular mass without dural attachments in the left insular region. The tumor was not stained on angiogram. The tumor was located in the extra-axial space of the sylvian fissure without any dural attachment, and was strongly attached to the middle cerebral artery. The tumor was excised, and a histological diagnosis of a transitional meningioma without a malignancy was made 16).

In 2002 a Pediatric sylvian fissure meningioma 17).

A one-year-eight-month old child who experienced the onset of a convulsive seizure. He had no neurological deficit and no developmental disorders. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a large left temporal tumor which was well enhanced and without dural attachment. Angiography revealed a slight tumor stain in the left Sylvian fissure supplied by branches of the internal carotid artery. Total removal of the tumor was performed, and they found that the tumor had no dural attachment, but was strongly attached to the M2 segment of the left middle cerebral artery. Pathological examinations revealed it to be a fibrous meningioma without malignancy 18).

Cooper et al., reported in 1997 a case in a 4-year-old child 19).

A 62-year-old woman was admitted because of one year history of temporal lobe epilepsy. She had no neurological deficit except for EEG abnormality. CT scans showed a small calcified mass in the left temporal lobe adjacent to the sylvian fissure with no enhancement by contrast medium. The mass was low-intense in both T1- and T2-weighted MR images. The T1-weighted image after the infusion of gadolinium revealed enhancement of the middle cerebral artery adjacent to the mass, similar to dural tail sign. Left external carotid angiography did not show any tumor stain nor the dilatation of the middle meningeal artery. Left internal carotid angiography disclosed enlarged middle cerebral artery without tumor stain. A left frontotemporal craniotomy was performed and the mass was totally removed. The tumor was located deep in sylvian fissure without any connection to the dura or ventricular system, which was firmly adherent to the middle cerebral artery. The histological examination of the surgical specimen revealed a psammomatous meningioma MR findings in deep sylvian meningioma was described 20).

Mori et al., reported a 12-year-old boy who has been suffering from severe headache for a month. Neurological examination was normal. CT scan and MR images showed a well-enhanced 7 cm mass lesion with small cysts, located in the left sylvian fissure. Peritumoral edema was slight and the midline structures were minimally shifted in spite of its large size. A fronto-temporal craniotomy was made and the tumor was grossly totally removed. The tumor had no dural attachment and existed in the left sylvian fissure, involving the middle cerebral artery and its branches. The histological diagnosis was transitional meningioma without malignancy. The postoperative course was uneventful except for transient mild left oculomotor palsy for several days. He is doing well now one year after the surgery and follow-up MR images showed no recurrence. Intracranial meningioma is rare in children. According to the literature, meningioma in children is slightly more frequent in males. There is a higher incidence of lack of dural attachment and cystic tumors than in adults. Deep sylvian meningioma without dural attachment is also very rare. Including our case, 13 cases of deep sylvian meningioma were reported in the literature. Four of them were under 20 years old. We report this case in detail with other cases reported previously. 21).

Chiocca et al., reported a deep sylvian fissure meningioma without dural attachments in the right hemisphere of an adult patient. The patient initially presented with simple partial seizures. Magnetic resonance imaging revealed a contrast-enhancing circular mass in the superior aspect of the insular region, deep to the inferior parietal lobule. Surgical exploration confirmed the absence of dural attachments. Microscopically, the tumor was found to be a sparsely cellular meningioma with an extensive collagenous matrix 22).

Graziani et al., in 1992 reported a case 23).

Cho et al., published a 2-year-old boy with a deep sylvian meningioma 24).

Silbergeld et al., a Sylvian fissure meningioma in a 4-year-old female 25).

In 1986 a 34-year-old Japanese woman, who had experienced several episodes of fainting attacks since 19 years old, was admitted to our hospital on March 22, 1983. Her plain skull roentgenogram showed abnormal calcification in her left fronto-temporal region. CT scan demonstrated clear-marginal high density mass in the left sylvian fissure which was homogeneously enhanced after administration of contrast medium. Left carotid angiogram showed intrasylvian mass with small tumor stain in late arterial phase, but external carotid artery had no concern with this tumor. On March 30, left fronto-temporal craniotomy was performed. The tumor was located in the extra-axial space of the sylvian fissure without any attachment to the dura mater or to the choroid plexus of the ventricles. This hard tumor, 70 grams in its weight, was successfully removed. It was histologically diagnosed fibroblastic meningioma. The patient was discharged without any neurological deficits. Twenty-four cases of meningiomas not attached to the dura mater or to choroid plexus could be reviewed from the literature. This type of meningioma is occasionally called “deep sylvian meningioma”, but in some reports the tumors developed far from sylvian fissure. And even in the cases in which the tumors were reported to be located in the sylvian fissure, macroscopic space where tumors developed was various. The clinical features, diagnosis, and surgical management of this tumor were also discussed in the report 26).

Okamoto et al., published a 35-year-old woman who was precisely diagnosed preoperatively with the aid of computed tomography and stereoscopic cerebral angiography. On reviewing the literature, it appears to be the first case that has been accurately diagnosed preoperatively and successfully treated by a total excision without serious complication 27).

Tsuchida et al., in 1981 published the only one of deep sylvian meningioma in the whole series of 181 intracranial meningiomas and probably the twentieth case reported so far in the literature. 28).

Saito et al., reported a case of 31-year-old female who had episodes of fainting attack. She had no significant neurological deficit but had EEG abnormality. Carotid angiography showed a tumor stain, ca. 1.7 cm in diameter, near the right insula. CT scan also revealed a high density area at the same site. At the time of operation, a small tumor located deeply in the right sylvian fissure was found out and successfully removed. Histologically, this tumor was diagnosed as a meningioma having some typical psammomatous features 29).

Mori et al., reported a case of “deep sylvian meningioma” 30).

Barcia-Goyanes and Calvo-Garra described a case in 1953 31),



Cushing H, Eisenhardt L: Meningiomas: Their Classification, Regional Behavior, Life History, and Surgical End Results. Springfield, Charles C. Thomas, 1938.

Brogna C, Lavrador JP, Patel S, Ribas EC, Aizpurua M, Vergani F, Ashkan K, Bhangoo R. Grade II Sylvian fissure meningiomas without dural attachment: case report and review of the literature. CNS Oncol. 2018 Dec 1;7(4):CNS20. doi: 10.2217/cns-2018-0004. Epub 2018 Oct 2. PubMed PMID: 30277091; PubMed Central PMCID: PMC6331700.

Cecchi PC, Campello M, Rizzo P, Mair K, Schwarz A. Atypical meningioma of the sylvian fissure. J Clin Neurosci. 2009 Sep;16(9):1234-9. doi: 10.1016/j.jocn.2008.10.027. Epub 2009 Jun 3. PubMed PMID: 19497747.
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Hong S, Usami K, Hirokawa D, Ogiwara H. Pediatric meningiomas: a report of 5 cases and review of literature. Childs Nerv Syst. 2019 Apr 18. doi: 10.1007/s00381-019-04142-y. [Epub ahead of print] PubMed PMID: 31001646.

Donovan DJ, Thavapalan V. Pediatric Meningeal Tumors of the Sylvian Fissure Region without Dural Attachment: A Series of Three Patients and Review of the Literature. Surg J (N Y). 2016 May 26;2(2):e31-e36. doi: 10.1055/s-0036-1584166. eCollection 2016 Apr. Review. PubMed PMID: 28824987; PubMed Central PMCID: PMC5553465.

Fukushima S, Narita Y, Yonezawa M, Ohno M, Arita H, Miyakita Y, Ichimura K, Yoshida A, Shibui S. Short communication: sclerosing meningioma in the deep sylvian fissure. Brain Tumor Pathol. 2014 Oct;31(4):289-92. doi: 10.1007/s10014-013-0167-8. Epub 2013 Oct 19. PubMed PMID: 24141724.

Aras Y, Akcakaya MO, Aydoseli A, Izgi N. Staged surgery for sylvian fissure meningiomas without dural attachment: report of two cases. Clin Neurol Neurosurg. 2013 Aug;115(8):1527-9. doi: 10.1016/j.clineuro.2012.12.010. Epub 2013 Jan 3. PubMed PMID: 23290420.

Kim JY, Lee EJ, Chang HW, et al. Deep Sylvian meningioma in a 43-year-old man: a case report. J. Korean Soc. Magn. Reson. Med. 2013;17(4):308–311

Ma L, Xiao SY, Zhang YK. Atypical meningioma of sylvian fissure with a 20-year history: a rare case report. Neurol Sci. 2012 Feb;33(1):143-5. doi: 10.1007/s10072-011-0637-x. Epub 2011 May 27. PubMed PMID: 21617950.

Miyahara K, Ichikawa T, Yagishita S, Mukaihara S, Okada T, Kaku S, Tanino S, Uriu Y, Fujitsu K, Niino H. [Deep sylvian meningioma without dural attachment: a case report]. No Shinkei Geka. 2011 Nov;39(11):1067-72. Review. Japanese. PubMed PMID: 22036818.

Cecchi PC, Campello M, Rizzo P, Mair K, Schwarz A. Atypical meningioma of the sylvian fissure. J Clin Neurosci. 2009 Sep;16(9):1234-9. doi: 10.1016/j.jocn.2008.10.027. Epub 2009 Jun 3. PubMed PMID: 19497747.

Samson Sujit Kumar G, Rajshekhar V. Deep sylvian meningioma: a case report and review of literature. Childs Nerv Syst. 2009 Jan;25(1):129-32. doi: 10.1007/s00381-008-0686-5. Epub 2008 Aug 19. Review. PubMed PMID: 18712398.

Eghwrudjakpor PO, Mori K. Sylvian cleft meningioma: surgical approach and postoperative morbidity. Niger J Med. 2006 Oct-Dec;15(4):437-40. PubMed PMID: 17111734.

McIver JI, Scheithauer BW, Atkinson JL. Deep Sylvian fissure chordoid meningioma: case report. Neurosurgery. 2005 Nov;57(5):E1064; discussion E1064. PubMed PMID: 16284544.

Chang JH, Kim JA, Chang JW, Park YG, Kim TS. Sylvian meningioma without dural attachment in an adult. J Neurooncol. 2005 Aug;74(1):43-5. PubMed PMID: 16078106.

Kaplan SS, Ojemann JG, Park TS. Pediatric sylvian fissure meningioma. Pediatr Neurosurg. 2002 May;36(5):275-6. PubMed PMID: 12053048.

Mitsuyama T, Kasuya H, Kubo O, Hirasawa K, Hori T. [Left Sylvian fissure meningioma in a one-year-eight-month old child]. No Shinkei Geka. 2000 May;28(5):459-64. Review. Japanese. PubMed PMID: 10806631.

Cooper JR, Marshman LA, Smith CM, Powell T. Case report: Sylvian fissure meningioma without dural attachment in a 4-year-old child. Clin Radiol. 1997 Nov;52(11):874-6. PubMed PMID: 9392468.

Matsumoto S, Yamamoto T, Ban S, Sato S, Shingu T, Yoshida S, Tokuno T, Nakazawa K, Saiwai S, Shirane H. [A case of deep sylvian meningioma presenting temporal lobe epilepsy]. No To Shinkei. 1995 May;47(5):503-8. Japanese. PubMed PMID: 7786628.

Mori Y, Shibuya M, Sugita K, Nagasaka T. [Deep sylvian meningioma: a case report of a child]. No Shinkei Geka. 1994 Dec;22(12):1147-51. Review. Japanese. PubMed PMID: 7845511.

Chiocca EA, Boviatsis EJ, Westmark RM, Short MP, Richardson EP, Zervas NT. Deep sylvian fissure meningioma without dural attachment in an adult: case report. Neurosurgery. 1994 Nov;35(5):944-6; discussion 946. Review. PubMed PMID: 7838346.

Graziani N, Donnet A, Vincentelli F, Dechambenoit G, Grisoli F. [Deep sylvian meningioma. Apropos of a case. Review of the literature]. Neurochirurgie. 1992;38(3):179-82. Review. French. PubMed PMID: 1461337.

Cho BK, Wang KC, Chang KH, Chi JG. Deep sylvian meningioma in a child. Childs Nerv Syst. 1990 Jun;6(4):228-30. PubMed PMID: 2383879.

Silbergeld D, Berger M, Griffin B. Sylvian fissure meningioma in a child: case report and review of the literature. Pediatr Neurosci. 1988;14(1):50-3. PubMed PMID: 3217285.

Hirao M, Oka N, Hirashima Y, Horie Y, Takaku A. [Deep sylvian meningioma: case report and review of the literature]. No Shinkei Geka. 1986 Nov;14(12):1471-8. Japanese. PubMed PMID: 3808209.

Okamoto S, Handa H, Yamashita J, Tokuriki Y. Deep Sylvian meningiomas. Surg Neurol. 1985 Mar;23(3):303-8. PubMed PMID: 3975816.

Tsuchida T, Ito J, Sekiguchi K, Honda H, Ueki K. [A case of deep sylvian meningioma with intracerebral hematoma (author’s transl)]. No Shinkei Geka. 1981;9(3):395-400. Japanese. PubMed PMID: 7242824.

Saito A, Mizuno Y, Adachi Y, Itoh T. [Deep Sylvian psammomeningioma, report of a case (author’s transl)]. No To Shinkei. 1979 Jan;31(1):79-83. Japanese. PubMed PMID: 426936.

Mori S, Ishihara H, Sogabe T, Kodama Y, Hibino H. [A case of deep sylvian meningioma (author’s transl)]. No Shinkei Geka. 1977 Apr;5(4):385-92. Japanese. PubMed PMID: 558541.

Barcia-Goyanes JJ, Calvo-Garra W. Meningiomas without dural attachment. Acta Neurochir. 1953;3:241–247.

Cognard Type V Dural Arteriovenous Fistula

Cognard Type V Dural Arteriovenous Fistula

Cognard classification Type V of Dural Arteriovenous Fistula (dAVF), is a rare subtype of cranial dAVF, characterized by congestion of the perimedullary venous system with drainage into a spinal vein. Dysfunction of the medulla and spinal cord caused by venous hypertension is the most probable cause of the neurological symptoms in such cases 1).


Frequently located around the foramen magnum most commonly fed by dural branches of the vertebral artery and external carotid artery. Detection requires meticulous angiography to define the angioarchitecture of the fistula, which in turn defines the treatment modality.

Roelz et al., suggested performing contrast-enhanced MR angiography or even digital subtraction angiography in the presence of an unclear edematous brainstem lesion before scheduling stereotactic biopsy 2).

Anterior venous drainage is dominant. Because the anterior spinal veins are located subpially, flow voids are less prominent on sagittal T2-weighted MRI. This may lead to difficulties in diagnosing. Evaluation with MR angiography may compensate for these limitations 3).


They present with progressive myelopathy in 50% of cases and may lead to devastating spinal cord pathology and brainstem dysfunctions if left untreated 4).

Because of this typical features aggressive treatments should be considered.

The optimal approach for each case should involve discussions among a multidisciplinary team of interventional neuroradiologists, neurosurgeons, neurologists, and radiation oncologists. Careful assessment of a patient’s clinical presentation, current status (age, medical condition, comorbidities), and type of lesion (location, classification, and angiographic features) should be conducted before embarking on any treatment 5).

To eliminate venous congestion of the spinal cord, various approaches including surgical interruption of the spinal draining vein or transarterial embolization with cyanoacrylate have been reported. The introduction of nonadhesive Onyx has changed the treatment of dAVF, although little is known about the clinical usefulness of this type of fistula 6).

The endovascular technique is the gold standard treatment in dural arteriovenous fistulas. Due to the limited number of series published it is difficult to create rigid guidelines in terms of the best endovascular treatment approach. Treatment must be tailored to each particular case, but it is important to keep in mind that the possibility of treating a type V dAVF by the transvenous approach should not be discarded. In selected cases the transvenous approach may be helpful to increase the chance of success in the endovascular treatment of type V dAVF 7).

Case reports

Sattur et al., presented the case of a 63 yr old woman, with prolonged, severe and progressive lower extremity myelopathy for 6 yr, with T2-weighted signal changes in the thoracic spinal cord on magnetic resonance imaging (MRI). Cervical MRI was unremarkable. Following computed tomography (CT), angiography of the spinal canal that revealed tortuous and dilated veins on the thoracic spinal cord surface, catheter angiography was performed. This demonstrated a fistula in relation to a dural branch of left vertebral artery with a characteristic single draining vein coursing caudally to the thoracic level, with delayed outflow suggestive of venous hypertension. Given the fact that the dural feeder was tortuous and relatively small, thus, precluding distal microcatheter access, and with presence of a single accessible draining vein, microsurgical treatment was preferred. Following a midline suboccipital craniotomy in prone position, a limited vertical dural opening was performed. Careful microsurgical arachnoid dissection revealed the arterialized draining vein with the aid of Indocyanine green videoangiography. The draining vein was clipped, coagulated, and disconnected. Postoperative recovery was uneventful and the patient is undergoing rehabilitation therapy. Follow-up angiographyshowed complete elimination of the fistula 8).

Jermakowicz et al., published the first known case of a pediatric. A 14-year-old girl presented with a 3-week history of slowly progressive unilateral leg weakness that quickly progressed to bilateral leg paralysis, sphincter dysfunction, and complete sensory loss the day of her presentation. MRI revealed an extensive T2 signal change in the cervical spine and tortuous perimedullary veins along the entire length of the cord. An emergency cranial angiogram showed a Type V dAVF fed by the posterior meningeal artery with drainage into the perimedullary veins of the cervical spine. The fistula was not amenable to embolization because vascular access was difficult; therefore, the patient underwent urgent suboccipital craniotomy and ligation of the arterialized venous drainage from the fistula. The patient’s clinical course immediately reversed; she had a complete recovery over the course of a year, and she remains asymptomatic at the 2-year follow-up. This report adds to a growing body of evidence that describes the diverse and unpredictable nature of Type V dAVFs and highlights the need to obtain a cranial angiogram in pediatric patients with unexplained myelopathy and cervical cord T2 signal change on MRI 9).

Tanaka et al., described a case of the Cognard type V dAVF, draining into the spinal vein through the occipital sinus (OS) successfully treated by transarterial double catheter injection of Onyx. They used the alternating injections from 2 microcatheters until the Onyx reached the OS and reflowed into feeders adequately. This technique contributed to the elimination of the remaining afferent flow in an early stage of Onyx injection and achieved enough penetration into the draining vein 10).

Roelz et al., 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, they suggest performing contrast-enhanced MR angiography or even digital subtraction angiography in the presence of an unclear edematous brainstem lesion before scheduling stereotactic biopsy 11).

Aixut Lorenzo et al., described a patient in whom the first arterial treatment failed to achieve occlusion of the fistulous point with the glue. Clinical symptoms improved due to the diminished flow at the fistula after the first embolization but as soon as collateral arteries were recruited by the fistula, spinal cord venous drainage impairment led to symptoms recurrence. Transvenous access allowed them to close the fistula completely in one only session with a complete disappearance of the pathologically inverted perimedullary venous flow 12).



Li J, Ezura M, Takahashi A, Yoshimoto T. Intracranial dural arteriovenous fistula with venous reflux to the brainstem and spinal cord mimicking brainstem infarction–case report. Neurol Med Chir (Tokyo). 2004 Jan;44(1):24-8. PubMed PMID: 14959933.
2) , 11)

Roelz R, Van Velthoven V, Reinacher P, Coenen VA, Mader I, Urbach H, Meckel S. 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 Dec;123(6):1534-9. doi: 10.3171/2014.11.JNS142278. Epub 2015 Jun 5. PubMed PMID: 26047415.

Haryu S, Endo T, Sato K, Inoue T, Takahashi A, Tominaga T. Cognard type V intracranial dural arteriovenous shunt: case reports and literature review with special consideration of the pattern of spinal venous drainage. Neurosurgery. 2014 Jan;74(1):E135-42; discussion E142. doi: 10.1227/NEU.0000000000000069. Review. PubMed PMID: 23839515.

Zyck S, Gould GC. Fistula, Dural Arteriovenous. 2019 Jan 13. StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-. Available from PubMed PMID: 30335307.

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 Jun;33(6):1007-13. doi: 10.3174/ajnr.A2798. Epub 2012 Jan 12. Review. PubMed PMID: 22241393.
6) , 10)

Tanaka J, Fujita A, Maeyama M, Kohta M, Hosoda K, Kohmura E. Cognard Type V Dural Arteriovenous Fistula Involving the Occipital Sinus. J Stroke Cerebrovasc Dis. 2017 Apr;26(4):e62-e63. doi: 10.1016/j.jstrokecerebrovasdis.2017.01.004. Epub 2017 Feb 1. PubMed PMID: 28161148.
7) , 12)

Aixut Lorenzo S, Tomasello Weitz A, Blasco Andaluz J, Sanroman Manzanera L, Macho Fernández JM. Transvenous approach to intracranial dural arteriovenous fistula (Cognard v): a treatment option. A case report. Interv Neuroradiol. 2011 Mar;17(1):108-14. Epub 2011 Apr 29. Erratum in: Interv Neuroradiol. 2012 Mar;18(1):114. Aiuxut Lorenzo, S [corrected to Aixut Lorenzo, S]. PubMed PMID: 21561567; PubMed Central PMCID: PMC3278034.

Sattur MG, Abi-Aad KR, Richards AE, Chong BW, Welz ME, Tian F, Bendok BR. Microsurgical Treatment of Foramen Magnum Cognard Type V Dural Arteriovenous Fistula: 2-Dimensional Operative Video. Oper Neurosurg (Hagerstown). 2019 Mar 28. pii: opz030. doi: 10.1093/ons/opz030. [Epub ahead of print] PubMed PMID: 30919907.

Jermakowicz WJ, Weil AG, Vlasenko A, Bhatia S, Niazi TN. Cognard Type V intracranial dural arteriovenous fistula presenting in a pediatric patient with rapid, progressive myelopathy. J Neurosurg Pediatr. 2017 Aug;20(2):158-163. doi: 10.3171/2017.3.PEDS16363. Epub 2017 May 19. PubMed PMID: 28524790.

Asymptomatic cervical dural arteriovenous fistula

A report of an asymptomatic cervical dural arteriovenous fistula in a patient with Lhermitte-Duclos disease was published in 2006. Almubarak et al. from Riyadh, presented in 2018 the second case of Lhermitte-Duclos disease associated with an asymptomatic spinal cervical AVF in a 17-year-old young woman with literature review of central nervous system vascular lesions in Lhermitte-Duclos disease 1).1) Almubarak AO, Haq AU, Alzahrani I, Shail EA. Lhermitte-Duclos Disease with Cervical Arteriovenous Fistula. J Neurol Surg A Cent Eur Neurosurg. 2018 Dec 5. doi: 10.1055/s-0038-1670636. [Epub ahead of print] PubMed PMID: 30517962.

Update: Dural ectasia

Dural ectasia is widening or ballooning of the dural sac surrounding the spinal cord. This usually occurs in the lumbosacral region, as this is where the cerebrospinal fluid pressure is greatest, but the spinal canal can be affected in any plane.

Case courtesy of Dr Franco Ruales, From the case rID: 16114
Most common symptoms include low back painheadaches, weaknessnumbness above and below the involved limb, leg pain, and sometimes there can be rectal and genital pain. Bowel and bladder dysfunction, urinary retention or even incontinence may occur.
The symptoms are usually exacerbated by upright posture and often but not always relieved by lying down. However, in many patients it is asymptomatic.
It is common in Marfan syndrome, occurring in 63–92% of people with the syndrome. Dural ectasia may also occur in Ehlers-Danlos Syndrome, neurofibromatosis type I, ankylosing spondylitis, and trauma.
A “classic” picture of dural ectasia in the Marfan patient may consist of low back pain, headache, proximal leg pain, weakness and numbness above and below the knee, and genital/rectal pain. Symptoms, when present, are typically moderate to severe, occur several times per week (often daily), are commonly exacerbated by upright posture, and are not always relieved by recumbency 1).

Radiographic features

Dural ectasia is dilation of the dural sac. Anteroposterior diameter of the thecal sac at the S1 level greater than that of the thecal sac at the L4 level ref required.

Plain radiograph

Posterior vertebral scalloping may be an indirect indicator 2) 3). However, this is not specific, as it is seen in a significant percentage of the normal population and is also associated with several other conditions.


Increase in the AP diameter of the dural sac, usually in the lumbar region.

Differential diagnosis


Among 1519 patients with spinal space-occupying lesions, 66 patients demonstrated spinal dura mater pathologies. Neuroradiological and surgical features were reviewed and clinical data analyzed.
Saccular dural diverticula (type I, n = 28) caused by defects of both dural layers, dissections between dural layers (type II, n = 29) due to defects of the inner layer, and dural ectasias (type III, n = 9) related to structural changes of the dura were distinguished. For all types, symptoms consisted of local pain followed by signs of radiculopathy or myelopathy, while one patient with dural ectasia presented a low-pressure syndrome and 10 patients with dural dissections additional spinal cord herniation. Type I and type II pathologies required occlusion of their dural defects via extradural (type I) or intradural (type II) approaches. For type III pathologies of the dural sac no surgery was recommended. Favorable results were obtained in all 14 patients with type I and 13 of 15 patients with type II pathologies undergoing surgery.
The majority of dural pathologies involving nerve root sleeves remain asymptomatic, while those of the dural sac commonly lead to pain and neurological symptoms. Saccular dural diverticula (type I) and dissections between dural layers (type II) pathologies were treated with good long-term results occluding their dural defects, while dural ectasias (type III) were managed conservatively 4).


Dural ectasia is one of the likely causes of incomplete or failed spinal anaesthesia. Its association with diseases like Marfans syndrome, neurofibromatosis, osteogenesis imperfecta, vertebral fracture, postopertative adhesions, trauma etc., is often overlooked as a reason for inadequate spinal anaesthesia. Greater than normal volume of cerebrospinal fluid in the lumber theca in dural ectasia is postulated to restrict the spread of intrathecally injected Local anaesthetic 5).

Foran JR, Pyeritz RE, Dietz HC, Sponseller PD. Characterization of the symptoms associated with dural ectasia in the Marfan patient. Am J Med Genet A. 2005 Apr 1;134A(1):58-65. PubMed PMID: 15690402.

Habermann CR, Weiss F, Schoder V et-al. MR evaluation of dural ectasia in Marfan syndrome: reassessment of the established criteria in children, adolescents, and young adults. Radiology. 2005;234 (2): 535-41. doi:10.1148/radiol.2342031497

Wakely SL. The posterior vertebral scalloping sign. Radiology. 2006;239 (2): 607-9. doi:10.1148/radiol.2392040224

Klekamp J. A New Classification for Pathologies of Spinal Meninges, Part 1: Dural Cysts, Dissections, and Ectasias. Neurosurgery. 2017 Mar 17. doi: 10.1093/neuros/nyx049. [Epub ahead of print] PubMed PMID: 28327939.

Gupta N, Gupta V, Kumar A, Kumar G. Dural ectasia. Indian Journal of Anaesthesia. 2014;58(2):199-201. doi:10.4103/0019-5049.130829.