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Table of Contents    
NEUROIMAGES
Year : 2016  |  Volume : 64  |  Issue : 6  |  Page : 1380-1381

Spinal dysraphism masking the presentation of spinal dural arteriovenous fistula: A serendipitous development or a congenital predisposition to an early-onset variant of spinal dural arteriovenous fistula?


1 Department of Neurosurgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Neuroradiology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication11-Nov-2016

Correspondence Address:
Dr. Sivashanmugam Dhandapani
Department of Neurosurgery, Post Graduate Institute of Medical Education and Research, Sector 12, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.193830

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How to cite this article:
Srinivasan A, Roy NK, Ghuman MS, Kumar A, Dhandapani S. Spinal dysraphism masking the presentation of spinal dural arteriovenous fistula: A serendipitous development or a congenital predisposition to an early-onset variant of spinal dural arteriovenous fistula?. Neurol India 2016;64:1380-1

How to cite this URL:
Srinivasan A, Roy NK, Ghuman MS, Kumar A, Dhandapani S. Spinal dysraphism masking the presentation of spinal dural arteriovenous fistula: A serendipitous development or a congenital predisposition to an early-onset variant of spinal dural arteriovenous fistula?. Neurol India [serial online] 2016 [cited 2018 Nov 20];64:1380-1. Available from: http://www.neurologyindia.com/text.asp?2016/64/6/1380/193830


Spinal vascular malformations are rare and can be divided into two categories: congenital lesions, consisting of spinal arteriovenousmal formations (AVMs) and cavernomas, and acquired lesions, mainly spinal dural arteriovenous fistula (SDAVF) presenting in the elderly.[1] In this article, we present a young adult who had spinal dysraphism on magnetic resonance imaging (MRI), masking the presence of an early-onset variant of SDAVF, and discuss the clinical significance of its presence.

A 21-year-old male patient with a history of a swelling in the lower back since birth presented with a history of acute onset, rapidly progressive, severe weakness in the lower limbs for 2 months associated with urinary retention. On examination, the patient had flaccid paraplegia with absence of deep tendon reflexes in bilateral lower limbs and reduced perianal sensations. MRI lumbosacral spine [Figure 1] revealed L3–L5 spina bifida associated with low lying tethered cord with a lipomatous component. Prominent corkscrew vessels were seen suggestive of a dural AVF [Figure 1] and [Figure 2]. Subsequently, the patient underwent spinal angiography [Figure 2], which revealed a dural AVF at the dorsolumbar region. The patient underwent embolization of the AVF. However, the tethered cord was not surgically detethered. At an 18-month follow-up, power improved in the lower limbs and the patient was able to walk with support.
Figure 1: Dorsolumbar magnetic resonance imaging sagittal T2-weighted (a) and sagittal contrast-enhanced T1-weighted fat saturated images show the defect (arrow) in the posterior elements at the L3 vertebral level, posterior herniation of the neural placode till the subcutaneous tissues, and a prominent ventral subarachnoid space consistent with lumbar meningomyelocele. Multiple serpentine flow voids (braces in A) are seen more pronounced along the dorsal perimedullary intradural space showing enhancing on postcontrast image (brace in B) representing engorged perimedullary draining veins

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Figure 2: Digital subtraction angiogram images anterior-posterior projection (a) taken after cannulation of right L3 lumbar artery show the dural arteriovenuos fistula (short thick arrow) with a prominent ascending perimedullary venous channel (thin arrows). Post-N-butyl cyanoacrylate embolization check angiogram (b) shows complete embolization of the fistula and feeding branch with no residual opacification of the arteriovenous fistula

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Spinal AVMs are classified into four categories: Dural AVFs (Type I), intramedullary AVMs with or without extramedullary extension (Types II and III), and perimedullaryAVFs (Type IV).[1] Dural AVFs are considered to be acquired in origin, usually secondary to trauma or surgery. They typically present with insidious onset of paraparesis or sphincteric dysfunction and occasionally with acute dysfunction. Low-back or radicular pain often precedes the onset of a gradually progressive myelopathy.[1] Spinal dysraphism is due to congenital defect of neurulation or gastrulation,[2] which typically presents with swelling since birth, low-back pain, some degree of atrophy of the leg muscles, or foot deformity with trophic ulcers.[3]

There are only six cases of spinal dysraphism and co-existing SDAVF reported in literature.[4] Acute onset myelopathy in the form of Foix–Alajouanine syndrome is noted only in our case. Long-standing tethering might have been associated with venous hypertension or chronic hypoxia (aberrant neoangiogenesis) predisposing to the formation of SDAVF. However, in view of an early age at onset, an early-onset variant of SDAVF due to a congenital etiology also may be considered with shared embryological origin with spinal dysraphism.

Our patient had presented with rapidly progressive paraparesis and bladder dysfunction. These symptoms could have been attributed to either the tethered cord or chronic venous hypertension from the SDAVF.[1] In our patient, the relatively acute presentation hinted towards SDAVF as a more plausible cause for the neurological deficit.

This association of early-onset variant of SDAVF with spinal dysraphism is a rare occurrence, which can be missed easily. Thus, a proper history, clinical examination and radiological work up can help in guiding the correct treatment.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Dhandapani S, Gupta A, Singh J, Sharma BS, Mahapatra AK, Mehta VS. Spinal dural arterio-venous fistula: Clinico-radiological profile and outcome following surgical occlusion in an Indian neurosurgical center. Neurol India 2013;61:406-10.  Back to cited text no. 1
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2.
Dhandapani S, Mehta VS, Sharma BS. “Horseshoe cord terminus” sans filum around a bone spur: A rare composite of faulty gastrulation with agenesis of secondary neurulation: Case report. J Neurosurg Pediatr 2013;12:411-3.  Back to cited text no. 2
    
3.
Dhandapani S, Srinivasan A. Contiguous triple spinal dysraphism associated with Chiari malformation Type II and hydrocephalus: An embryological conundrum between the unified theory of Pang and the unified theory of McLone. J Neurosurg Pediatr. 2016;17:103-6.  Back to cited text no. 3
    
4.
Mavani SB, Nadkarni TD. Tethered cord due to caudal lipomeningocele associated with a lumbar dural arteriovenous fistula. J Neurosurg Spine 2014;21:489-93.  Back to cited text no. 4
    


    Figures

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[Pubmed] | [DOI]



 

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