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Table of Contents    
Year : 2022  |  Volume : 70  |  Issue : 8  |  Page : 330-334

Spinal Dural Arteriovenous Fistula in a Young Male Associated with Craniospinal Leptomeningeal Spread of a Treated High-Grade Glioma

1 Department of Neuroendovascular and Interventional Radiology, Apollo Speciality Hospital, Jayanagar, Bangalore, Karnataka, India
2 Department of Neurology, Apollo Speciality Hospital, Jayanagar, Bangalore, Karnataka, India
3 Department of Neurosurgery, Institute of Neurosciences, Apollo Speciality Hospital, Jayanagar, Bangalore, Karnataka, India

Date of Submission10-Jun-2020
Date of Decision15-Jul-2020
Date of Acceptance19-Jul-2020
Date of Web Publication11-Nov-2022

Correspondence Address:
Chinmay P Nagesh
Department of Neuroendovascular and Interventional Radiology, Apollo Speciality Hospital, #2, 14th Cross, 3rd Block Jayanagar, Bangalore - 560 011, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.360935

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 » Abstract 

Spinal dural arteriovenous fistulae (SDAVF) are most commonly idiopathic in origin but may occasionally be seen secondary to surgery, trauma, or inflammation. We report a case of 27-year-old male who came with features of a myelopathy. He was found to have an SDAVF associated with leptomeningeal spread (LMS) of a previously treated high-grade cerebral glioma. Hemorrhagic presentation of gliomas, as in this case, is due to upregulation of vascular endothelial growth factor, which has also been postulated to play a role in the development of SDAVFs. This may suggest a possible mechanism of induction of secondary SDAVFs associated with such tumors. While the coexistence of intracranial neoplasms with vascular malformations has been reported previously, this is the first case report of LMS of a high-grade glioma associated with an SDAVF.

Keywords: Dural arteriovenous fistula, glioma, glioblastoma, leptomeningeal spread, spinal vascular malformation
Key Message: We describe a hitherto unreported novel association of spinal dural arteriovenous fistula secondary to spinal leptomeningeal spread of a treated intracranial high-grade glioma. We discuss atypical clinicoradiological features and possible mechanisms of such an association.

How to cite this article:
Nagesh CP, Satischandra P, Krishna K N, Joshi G, Devaraj R, Herur A. Spinal Dural Arteriovenous Fistula in a Young Male Associated with Craniospinal Leptomeningeal Spread of a Treated High-Grade Glioma. Neurol India 2022;70, Suppl S2:330-4

How to cite this URL:
Nagesh CP, Satischandra P, Krishna K N, Joshi G, Devaraj R, Herur A. Spinal Dural Arteriovenous Fistula in a Young Male Associated with Craniospinal Leptomeningeal Spread of a Treated High-Grade Glioma. Neurol India [serial online] 2022 [cited 2022 Dec 3];70, Suppl S2:330-4. Available from: https://www.neurologyindia.com/text.asp?2022/70/8/330/360935

Spinal dural arteriovenous fistulae (SDAVF) are acquired lesions of indeterminate etiology. Uncommonly, they may be seen after a prior insult such as trauma, infections, or surgery.[1],[12] With intracranial neoplasms, there are rare case reports of coexistent arteriovenous malformations and a solitary reported case of a glioblastoma-associated dural arteriovenous fistula.[2] Leptomeningeal spread (LMS), in particular to the spine, is an uncommon occurrence with primary intracranial neoplasms and occurs in less than 4% of cases.[3] Hitherto, a vascular shunt or malformation has never been reported in conjunction with this entity. This report details the first description of spinal LMS of a high-grade glioma (HGG) with a concomitant spinal dural arteriovenous fistula in a young male and explores possible mechanisms of such an occurrence.

 » Case History Top

A 27-year-old male, presented with a 2-week history of intermittent, gradually worsening headaches associated with vomiting. Apart from generalized weakness and drowsiness, he also had moderate diffuse back pain and had developed gradual onset of bilateral lower limb weakness for a week with urinary retention for a day. He was a known case of left temporal grade 3 anaplastic astrocytoma, status post complete surgical resection and adjuvant chemotherapy and cranial radiotherapy 18 months prior. He had been asymptomatic in the intervening period.

At examination, he was found to be conscious but drowsy with meningeal signs. There were no cranial nerve palsies. His lower limb had normal tone with power grade 3/5 and normal upper limb power. Tendon reflexes were brisk with flexor plantar response. Clinical possibility of craniospinal arachnoiditis (possibly tuberculous) versus carcinomatosis was made.

Based on clinical diagnosis, he underwent a contrast-enhanced craniospinal MRI. Spinal MRI [Figure 1] revealed long segment cord T2 hyperintensity in the upper thoracic segments (C7 to T4 vertebral levels) with multiple posterior predominant perimedullary flow voids. Also seen was ill-defined moderately enhancing T2 hyperintense, T1 isointense intradural perimedullary soft tissue most prominent in the upper thoracic spine. A TRICKS MR angiography was also performed, which revealed arteriovenous shunting into the dilated perimedullary veins. Brain MRI revealed no evidence of residual or recurrent lesion at the operative site but showed diffuse leptomeningeal enhancement and exudates in the basal and posterior fossa cisterns [Figure 2]. A preliminary diagnosis of LMS or spinal arachnoiditis with a spinal vascular malformation was made.
Figure 1: Initial hemorrhagic presentation of left temporal grade 3 anaplastic astrocytoma on CT (arrow in a and b). Follow-up MRI 18 months later reveals no residual or recurrent lesion in the operative bed (FLAIR images c and d). Postcontrast T1-weighted images (e-g) show diffuse leptomeningeal enhancement in the basal and posterior fossa cisterns

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Figure 2: Spine MRI at current presentation shows perimedullary flow voids (black arrow in a) with hyperintense cord edema (white arrow in a) on T2-weighted images. Pre- and postcontrast T1 fat-saturated images (b and c, respectively) show avid homogeneous enhancement of the T1 isointense perimedullary soft tissue of concern for leptomeningeal spread

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He underwent a diagnostic craniospinal subtraction angiography [Figure 3] and 3d rotational angiography [Figure 4], which revealed arteriovenous shunting at the level of the left T6-7 and right T7-8 neural foramina fed by radiculomeningeal branches of the left T6 and right T7 intercostal arteries respectively. Venous egress was seen to the T12 vertebral level inferiorly and superiorly into the posterior fossa with dilatation of the pontomesencephalic vein. Intracranial vasculature was unremarkable.
Figure 3: Sequential images of angiographic runs of the left T6 (a-e) and right T7 (f-k) intercostal arteries (partial digital subtraction – landscaped for bony landmarks) show shunting located at the neural foramina at T6-7 and T7-8 disc levels (white arrows in a and f). Prominent perimedullary veins are seen first draining in a caudal direction (arrowhead in k) and then draining cranially (arrowhead in e)

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Figure 4: Three-dimensional rotational angiography of the right T7 intercostal artery showing the extensive perimedullary veins (volume rendering – a), foraminal location of shunt (arrow in b), and posterior perimedullary location of the draining veins (c) – all typical features of an SDAVF

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Lumbar puncture revealed grossly elevated protein of 676 mg/dl, leucocyte count of 26 cells/mL with glucose of 139 mg/dl. Cytology was negative for acid fast bacilli and malignant cells. Routine blood parameters were otherwise normal. In view of CSF cytology being nondiagnostic and progressive clinical course, the patient underwent a meningocortical biopsy of the right sylvian region. The spinal lesion was not separately biopsied in view of higher surgical risk. Histopathology revealed dense infiltration of the pia-arachnoid by anaplastic fibrillary glial cells that were GFAP positive and having a MIB-1 index of 20% consistent with LMS of WHO grade IV glioblastoma [Figure 5]. Owing to poor prognosis, palliative treatment was suggested to the patient. The patient progressively deteriorated with increasing drowsiness as well as lower limb weakness, and he passed away a month later.
Figure 5: Figures (a-f) depict the initial tumor; figures (g-i) depict leptomeningeal biopsy at second presentation. Initial histopathology revealed anaplastic astrocytoma (WHO grade III) with neoplastic astrocytes with nuclear atypia and mitoses (a). Tumor cells are diffusely positive for GFAP (b), negative for IDH1R132H (c), show lost ATRX immunopositivity (d) and moderate MIB-1 labeling (e). DNA sequencing chromatogram shows absent mutations in IDH 1 and 2 (wild type) (f). Rebiopsied tumor shows leptomeningeal spread of pleomorphic tumor cells with features of glioblastoma (WHO grade IV) (g and h) with very high MIB-1 labeling (i)

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 » Discussion Top

The coexistence of vascular malformations with a neoplasm is an uncommon combination. While gliomas coexisting with AVMs, developmental venous anomalies and cavernous malformations have been reported previously, to the best of our knowledge, only one case of a glioblastoma with an intracranial DAVF is known in the literature.[2],[13],[14] Our case is the first reported description of an SDAVF coexisting with and possibly induced by LMS of a high-grade glioma.

SDAVFs are acquired disorders with a propensity to present in elderly men usually in the fifth to seventh decades.[1] They tend to progress insidiously over a variable time period resulting in congestive myelopathy secondary to a combination of chronic hypoxia and venous hypertension. Diagnosis is often delayed, usually 11–18 months after symptom onset.[4],[5],[15] They are usually idiopathic, but may rarely be secondary to prior trauma, inflammation, or surgery.[6]

LMS of high-grade gliomas is rare, occurring in less than 4% of cases. Approximately 10% of cases have LMS at initial diagnosis with the majority being diagnosed at a median of 16.4 months after initial diagnosis of the intracranial primary. Prognosis with LMS is dependent on histology of the primary neoplasm but is uniformly poor for high-grade neoplasms averaging 2–5 months after diagnosis.[3],[16]

In our patient, the initial presentation of the temporal anaplastic astrocytoma was that of an intraparenchymal hematoma. Possible mechanisms of intratumoral hemorrhage may be fragile neovasculature, tumoral invasion of pre-existing vasculature, or even presence of intratumoral arteriovenous fistulae.[7],[17],[18] These point to a role of upregulation of vascular endothelial growth factor (VEGF) within these tumors.[8] Enhanced expression of VEGF has also been cited in the development of DAVFs. Further, the grade of the neoplasm was also seen to increase from grade 3 anaplastic astrocytoma to grade 4 glioblastoma by the time LMS was detected, a phenomenon reported prior and suggestive of an aggressive variant.[3]

It is thus possible that the HGG in our patient had a propensity for tumoral neoangiogenesis and may imply a possible mechanism of generation of this secondary SDAVF with extensive LMS. Many points exist that the SDAVF may have been consequential to, rather than coincidental with the glioma and LMS. The age of the patient is atypical for a primary idiopathic SDAVF. The onset of symptoms suggestive of a myelopathy was only for a few weeks suggesting a relatively rapid progression that is uncommon in a primary SDAVF in the absence of an inciting factor such as lumbar puncture, spinal surgery or steroid administration.[9],[10],[11] This may point to an acceleration of development and progression of shunting of the SDAVF. Multisite SDAVFs are also an uncommon occurrence pointing to induction by the extensive LMS. Thus, our case provides an interesting insight into the possible mechanisms of SDAVF etiopathogenesis and highlights a rare association of HGG-related LMS with SDAVF.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 » References Top

Krings T, Geibprasert S. Spinal dural arteriovenous fistulas. AJNR Am J Neuroradiol 2009;30:639-48.  Back to cited text no. 1
Yuan X, Gao M, Zhao H, Niu J, Bai Y, Guo L. Glioblastoma multiforme in association with a dural arteriovenous fistula. Neurol India 2016;64(Suppl):S121-6.  Back to cited text no. 2
Andersen BM, Miranda C, Hatzoglou V, DeAngelis LM, Miller AM. Leptomeningeal metastases in glioma: The Memorial Sloan Kettering Cancer Center experience. Neurology 2019;92:e2483-91.  Back to cited text no. 3
Fugate JE, Lanzino G, Rabinstein AA. Clinical presentation and prognostic factors of spinal dural arteriovenous fistulas: An overview. Neurosurg Focus 2012;32:E17.  Back to cited text no. 4
Jellema K, Canta LR, Tijssen CC, van Rooij WJ, Koudstaal PJ, van Gijn J. Spinal dural arteriovenous fistulas: Clinical features in 80 patients. J Neurol Neurosurg Psychiatry 2003;74:1438-40.  Back to cited text no. 5
Nishimura Y, Natsume A, Ginsberg HJ. Spinal dural arteriovenous fistula associated with L-4 isthmic spondylolisthesis. J Neurosurg Spine 2014;20:670-4.  Back to cited text no. 6
Choi G, Park DH, Kang SH, Chung YG. Glioma mimicking a hypertensive intracerebral hemorrhage. J Korean Neurosurg Soc 2013;54:125-7.  Back to cited text no. 7
Lugano R, Ramachandran M, Dimberg A. Tumor angiogenesis: Causes, consequences, challenges and opportunities. Cell Mol Life Sci 2020;77:1745-70.  Back to cited text no. 8
Noh T, Chandra R, Kim J, Lee I. A case of symptomatic spinal dural arteriovenous fistula after high-volume lumbar puncture. Surg Neurol Int 2017;8:164.  Back to cited text no. 9
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Stevens EA, Powers AK, Morris PP, Wilson JA. Occult dural arteriovenous fistula causing rapidly progressive conus medullaris syndrome and paraplegia after lumbar microdiscectomy. Spine J 2009;9:e8-12.  Back to cited text no. 11
Niu X, Zhou X, Wang C, Li J. A Spinal Dural Arteriovenous Fistula Mimicking Spinal Arteriovenous Malformation. Neurol India 2021;69:204-205.  Back to cited text no. 12
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