Intracranial dural arteriovenous fistula showing diffuse MR enhancement of the spinal cord: Case report and review of the literature

doi:10.1016/S0090-3019(02)00861-3

Surgical Neurology

Volume 58, Issues 3–4, September–October 2002, Pages 251-257

https://doi.org/10.1016/S0090-3019(02)00861-3 Get rights and content

Abstract

BACKGROUND

Cervical myelopathy resulting from intracranial dural arteriovenous fistula (AVF) is uncommon. Knowledge of the magnetic resonance imaging (MRI) appearance of such lesions is important because many patients with myelopathy are initially examined by MRI, and an incorrect diagnosis could result in delayed or improper treatment. We describe a rare case of myelopathy due to an intracranial dural AVF showing diffuse enhancement of the cervical spinal cord.

CASE DESCRIPTION

A 64-year-old male presented with progressive myelopathy and respiratory insufficiency. Cerebral angiography disclosed an AVF at the craniocervical junction draining intrathecally into the spinal medullary veins. MRI revealed dilated perimedullary vessels around the craniocervical junction, as well as spinal cord swelling with high signal intensity changes. Diffuse intense enhancement of the cervical spinal cord was also seen on postcontrast images. Embolization via the afferent artery was successfully performed, and the fistula was then microsurgically obliterated via a lateral suboccipital approach. This procedure arrested a rapidly progressive myelopathy.

CONCLUSION

Intracranial dural AVF showing diffuse enhancement of the spinal cord is extremely rare, and this enhancement effect may indicate pathologic changes of the spinal cord. MRI only is not useful for diagnosis, but also for demonstrating pathologic changes and predicting the outcomes of patients with intracranial dural AVF.

Section snippets

Case report

A 64-year-old male experienced transient weakness of the lower extremities; the symptoms disappeared spontaneously within 24 hours. Two weeks later, weakness in both legs developed and gradually became worse, and the patient visited a local hospital. Magnetic resonance imaging at that hospital demonstrated swelling of the cervical spinal cord with signal intensity changes, and the patient was referred to our hospital for further investigations and treatment.

Neurologic examination on admission

Clinical characteristics of AVFs at the craniocervical junction

Most spinal dural AVFs occur in the thoracolumbar region [13]. Dural AVFs at the craniocervical junction leading to myelopathy are uncommon because the dural fistulas are located intracranially and have secondary effects on the cervical cord. To our knowledge, only 31 cases, including ours, of intracranial dural AVF causing myelopathy have been reported 1, 2, 3, 4, 5, 6, 7, 8, 9, 11, 12, 13, 14, 15, 16, 17, 18, 19, 24. The patient ages ranged from 31 to 74 years (mean 56.4 years), with men

Acknowledgements

The authors wish to thank Drs Wataru Katayama, Noriyuki Kato, Yuji Matsumaru, and Akira Matsumura, of the Departments of Neurosurgery, Institute of Clinical Medicine, University of Tsukuba (Tsukuba, Japan), for their help in treating this patient.

References (24)

R. Willinsky et al.
The variable presentations of craniocervical and cervical dural arteriovenous malformations
Surg Neurol
(1990)
K. Yanaka et al.
Distal anterior choroidal artery aneurysm associated with an arteriovenous malformation. Intraoperative localization and treatment
Surg Neurol
(2000)
V. Bousson et al.
Intracranial dural fistula as a cause of diffuse MR enhancement of the cervical spinal cord
J Neurol Neurosurg Psychiatry
(1999)
P. Bret et al.
Dural arteriovenous fistula of the posterior fossa draining into the spinal medullary veins—an unusual cause of myelopathycase report
Neurosurgery
(1994)
L. Brunereau et al.
Intracranial dural arteriovenous fistulas with spinal venous drainagerelation between clinical presentation and angiographic findings
AJNR Am J Neuroradiol
(1996)
M. Collice et al.
Surgical interruption of leptomeningeal drainage as treatment for intracranial dural arteriovenous fistulas without dural sinus drainage
J Neurosurg
(1996)
C.E. Deopujari et al.
Cervical myelopathy caused by an intracranial dural arteriovenous fistula
Br J Neurosurg
(1995)
R.J. Ernst et al.
Cervical myelopathy associated with intracranial dural arteriovenous fistulaMR findings before and after treatment
AJNR Am J Neuroradiol
(1997)
E.H. Gaensler et al.
Arteriovenous fistulas of the cervicomedullary junction as a cause of myelopathyradiographic findings in two cases
AJNR Am J Neuroradiol
(1990)
Y.P. Gobin et al.
Endovascular treatment of intracranial dural arteriovenous fistulas with spinal perimedullary venous drainage
J Neurosurg
(1992)

S. Hahnel et al.

MR appearance of an intracranial dural arteriovenous fistula leading to cervical myelopathy

Neurology

(1998)

H. Kinouchi et al.

Dural arteriovenous shunts at the craniocervical junction

J Neurosurg

(1998)

Cited by (40)

Dural arteriovenous fistula causing progressive myelopathy: Limited drainage to the perimedullary veins of the cervical area alone
2022, Interdisciplinary Neurosurgery: Advanced Techniques and Case Management
Citation Excerpt :
In contrast to these spinal DAVFs, intracranial DAVFs with perimedullary drainage are rare. In a survey of previous literature, we found 39 reported cases (Table 1) [5,7,8,17]. A classification system for intracranial DAVFs according to their venous drainage was introduced in 1978 by Djindjian and Merland [3].
Intracranial dural arteriovenous fistulas (DAVFs) are uncommon cerebrovascular malformations that usually drain into a sinus or cortical veins. Depending on the pattern of the venous drainage, various clinical symptoms can be present, and most of them are ocular and/or intracranial symptoms, such as cranial nerve paralysis, intracerebral hemorrhage(ICH), subarachnoid hemorrhage (SAH), and intracranial hypertension. DAVFs that drain from a cortical to a perimedullary vein and show venous congestion that results in progressive myelopathy are extremely rare lesions. These symptoms are most commonly associated with an abnormal drainage pattern of Adamkiewicz's artery or when the venous drainage descends toward the thoracolumbar level. We report a case of DAVFs with drainage limited to the cerebellar and perimedullary veins of the cervical area causing progressive myelopathy.
Type V Dural Arteriovenous Fistula Supplied by the Artery of Wollschlaeger and Wollschlaeger Causing Cervical Myelopathy and Quadriparesis
2020, World Neurosurgery
Citation Excerpt :
Contrast-enhanced magnetic resonance angiography increased the perimedullary vein detection rate to 85%.21 In many cases, such as ours, one can observe pathologic enhancement in areas of altered signal after contrast administration.22,23 Furthermore, brainstem T2 signal abnormality in the setting of engorged perimedullary veins suggests a diagnosis of a cranial dAVF rather than a spinal dAVF.24
The artery of Wollschlaeger and Wollschlaeger is a tentorial branch of the superior cerebellar artery that is usually not visualized on conventional cerebral angiography, unless it is pathologically enlarged. It can be recruited as part of the blood supply to tentorial dural arteriovenous fistulas (AVFs), although this occurs infrequently.
Here we report the clinico-radiologic evaluation and treatment of a 48-year-old man referred to our institution for hitherto workup negative progressive, relapsing quadriparesis. This represents the first reported case of cervical myelopathy caused by venous congestion from a type V dural AVF supplied by the artery of Wollschlaeger and Wollschlaeger.
The anatomic discrepancy between the symptomatic spinal cord lesion and the etiologic intracranial fistula frequently results in delayed care in cases of myelopathy due to intracranial dural AVFs. Familiarity with these disorders and of their pathophysiologic mechanisms is important to avoid unnecessary diagnostic delays.
Brainstem Venous Congestion Caused by Perimedullary Drainage in Anterior Cranial Fossa Dural Arteriovenous Fistula
2019, World Neurosurgery
Myelopathy develops relatively rarely in intracranial dural arteriovenous fistula (DAVF); it has only been reported in posterior cranial fossa DAVF. Herein, we report the first, to our knowledge, case of anterior cranial fossa (ACF) DAVF with myelopathy.
A 75-year-old man presented with dizziness, nausea, and gait disturbance. T2-weighted magnetic resonance imaging revealed a hyperintense area in the left cerebellum and medulla; a flow void was also detected around the medulla. The patient was first diagnosed with spinal DAVF, but the shunt point was detected at the anterior ethmoidal artery, flowing from the olfactory vein to the basal vein of Rosenthal and anterior/posterior spinal veins. The shunt point was clipped during craniotomy, and neurologic symptoms improved.
Myelopathy because of intracranial DAVF potentially involves diagnostic pitfalls. Even in ACF DAVF, there is a possibility of myelopathy caused by perimedullary venous drainage.
Myelopathy Due to Intracranial Dural Arteriovenous Fistula with Perimedullary Venous Drainage: Clinical and Medicolegal Aspects in a Case of Diagnostic Pitfall
2019, World Neurosurgery
Intracranial dural arteriovenous fistula (DAVF) consists of a meshwork of arteriovenous shunts. Drainage of the fistula may be into a dural sinus or directly into cortical veins. Rarely, dural arteriovenous fistulas drain exclusively into perimedullary veins. Prompt diagnosis is important, as the clinical course is potentially life threatening.
A 67-year-old man presented with difficulties in walking and bladder retention. Magnetic resonance imaging showed central myelopathy, edema from C6 to T8, and dilated posterior medullary veins. Spinal angiography showed no abnormalities. The diagnostic impression was one of inflammatory demyelinating disease, and the patient was treated with corticosteroids. Three days later, the patient had become paraparetic. Brain magnetic resonance imaging showed abnormal vascular structures around the lower brainstem and cervical cord. Cerebral angiography showed a dural fistula at the skull base supplied by the neuromeningeal branch of the ascending pharyngeal artery and draining into the posterior medullary veins. Following neurosurgical referral, the draining vein was clipped just beyond the arterial feeders. At that time, the patient was wheelchair-bound and needed assistance in daily activities.
Early diagnosis of this rare vascular malformation is challenging; angiographic studies must include the cranial vasculature when spinal studies are normal or if the abnormality on magnetic resonance imaging is maximal in the upper spinal cord. In the case described, as only medullary angiography was performed, the intracranial dural arteriovenous fistula with perimedullary venous drainage was not diagnosed. Delayed diagnosis is likely to lead to severe neurologic consequences.
Dural Venous Sinus Connections to the Vertebral Venous Plexus
2019, Anatomy, Imaging and Surgery of the Intracranial Dural Venous Sinuses
Only vague statements regarding the cranial confluence of the vertebral venous system are found in the literature. Even studies dedicated to evaluating the spinal venous plexus and sinuses near the foramen magnum have not commented on the specific anatomy of the intracranial connections. As such connections are not only important for normal venous drainage of the cranium, they also have clinical and surgical significance. This chapter, therefore, reviews the anatomy and clinical application of the connections between the venous sinuses of the skull base and the vertebral venous plexuses.
The Long and Winding Road: Thoracic Myelopathy Associated With Occipitocervical Dural Arteriovenous Fistula
2017, World Neurosurgery
Spinal dural arteriovenous fistulas (DAVFs) have diverse presentations. Magnetic resonance imaging (MRI) reveals spinal cord swelling in only 45% to 74% of cases. We present an unusual case of a 57-year old man with a craniocervical junction DAVF in which the edema appeared in the thoracolumbar region, skipping the cervical spinal cord.
A case report and literature review from a tertiary referral center are provided.
In our patient, symptoms progressed over 3 months from low back and radicular pain to weakness, saddle anesthesia, and urinary retention. MRI showed T2 hyperintensity from T3-4 to the conus medullaris. The results from initial brain and spine angiograms were negative. Repeated angiography with the patient under anesthesia revealed a DAVF in the craniocervical junction composed of a cervical branch arising from the right vertebral artery and entering the nidus at the foramen magnum. A prominent intradural draining vein extended inferiorly along the posterior aspect of the cervical spinal cord. The patient was taken to the operating room, where the extradural vertebral artery was dissected and a suboccipital craniectomy was performed. After the dura was opened, the arterialized vein was visualized and clipped. Somatosensory evoked potentials and motor evoked potentials remained at baseline throughout the occlusion. The dura and fistula were removed en bloc, and the draining vein was confirmed to lose flow on indocyanine green. Postoperatively, the patient's symptoms completely resolved, and he experienced full strength in the lower extremities and perineal sensation. A review of 19 cases of DAVF at the craniocervical junction with cord edema shows that >90% involve the brainstem or cervical cord. Only 1 other patient had a similar “skip lesion,” in which the cord edema first appeared in the thoracic cord.
This rare presentation highlights the importance of diagnostic persistence when unexplained cord findings are seen on MRI. Repeated angiography and the inclusion of proximal vascular lesions may be considered in the evaluation of isolated thoracic edema.

View all citing articles on Scopus

View full text

VascularIntracranial dural arteriovenous fistula showing diffuse MR enhancement of the spinal cord: Case report and review of the literature

Abstract

Section snippets

Case report

Clinical characteristics of AVFs at the craniocervical junction

Acknowledgements

Surg Neurol

Surg Neurol

Intracranial dural fistula as a cause of diffuse MR enhancement of the cervical spinal cord

J Neurol Neurosurg Psychiatry

Dural arteriovenous fistula of the posterior fossa draining into the spinal medullary veins—an unusual cause of myelopathycase report

Neurosurgery

Intracranial dural arteriovenous fistulas with spinal venous drainagerelation between clinical presentation and angiographic findings

AJNR Am J Neuroradiol

Surgical interruption of leptomeningeal drainage as treatment for intracranial dural arteriovenous fistulas without dural sinus drainage

J Neurosurg

Cervical myelopathy caused by an intracranial dural arteriovenous fistula

Br J Neurosurg

Cervical myelopathy associated with intracranial dural arteriovenous fistulaMR findings before and after treatment

AJNR Am J Neuroradiol

Arteriovenous fistulas of the cervicomedullary junction as a cause of myelopathyradiographic findings in two cases

AJNR Am J Neuroradiol

Endovascular treatment of intracranial dural arteriovenous fistulas with spinal perimedullary venous drainage

J Neurosurg

MR appearance of an intracranial dural arteriovenous fistula leading to cervical myelopathy

Neurology

Dural arteriovenous shunts at the craniocervical junction

J Neurosurg

Vascular
Intracranial dural arteriovenous fistula showing diffuse MR enhancement of the spinal cord: Case report and review of the literature