- Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India
Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India
DOI:10.4103/2152-7806.76280Copyright: © 2011 Kumar A This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Kumar A, Deopujari CE, Mhatre M. Misdiagnosis in a case of non-compressive myelopathy due to a lumbar spinal intradural fistula supplied by the Artery of Adamkiewicz. Surg Neurol Int 29-Jan-2011;2:12
How to cite this URL: Kumar A, Deopujari CE, Mhatre M. Misdiagnosis in a case of non-compressive myelopathy due to a lumbar spinal intradural fistula supplied by the Artery of Adamkiewicz. Surg Neurol Int 29-Jan-2011;2:12. Available from: http://sni.wpengine.com/surgicalint_articles/misdiagnosis-in-a-case-of-non-compressive-myelopathy-due-to-a-lumbar-spinal-intradural-fistula-supplied-by-the-artery-of-adamkiewicz/
Background:Spinal vascular malformations comprise a rare but significant group of spinal disorders where clinching the diagnosis early is absolutely necessary since the morbidity increases as the time goes by. These malformations present mainly with symptoms of myelopathy with a gradually worsening course and thus early diagnosis and intervention may revert the symptoms to some extent. Owing to ignorance, sometimes the diagnosis may be missed or delayed and this delay can make a significant difference in the final outcome.
Case Description:A 44-year-old male presented to us with an 8-month history of gradually worsening difficulty in walking and lower limb paraesthesias along with recent bladder complaints. Earlier, the imaging had revealed prolapsed lumbar disc and he had undergone L4-5 micro-discectomy few months back. As his symptoms worsened further, he developed paraparesis and then a more detailed analysis revealed a missed spinal arterio-venous fistula at L4-5 level causing congestive myelopathy. He was re-operated and the fistula was disconnected which led to an improvement months after surgery.
Conclusion:Thus, to differentiate between compressive and non-compressive myelopathy and detailed investigation of the latter to identify the actual cause remains imperative. Misdiagnosis leading to a wrong surgery caused further deterioration which could have been avoided by careful analysis of imaging. Open surgery remains the preferred treatment for the fistulas supplied by the artery of Adamkiewicz.
Keywords: Myelopathy, spinal vascular malformation, venous hypertension
Spinal vascular malformations are a rare group of disorders which require a very high index of suspicion for diagnosis especially in early stages where imaging is not too helpful usually. But, subtle findings are always present to clinch the diagnosis at the earliest. Awareness about these relatively rare vascular disorders is required for a good clinical outcome so that diagnostic angiography is ordered immediately in all suspicious cases and nothing remains undiagnosed. This becomes important as the degree of neurological recovery depends mainly on the pre-operative neurological status. We report a case of a 44-year-old male with a spinal intradural arterio-venous fistula (AVF) who underwent micro-discectomy due to an erroneous diagnosis and further deteriorated clinically till the actual cause was identified and treated. The history, examination, imaging and operative details are discussed along with relevant review of literature.
A 44-year-old male presented to us with an 8-month history of chronic backache and lower limb paraesthesias. Initially, only symptomatic treatment was given which did not help him and he developed bladder complaints in the form of urgency and poor flow. Magnetic resonance imaging (MRI) of spine was done and multiple disc bulges were identified in the lumbar region, the maximum being at L4-5 level. He was diagnosed to have a cauda equina syndrome and hence, L4-5 micro-discectomy was done. Clean intermittent self catheterization (CISC) was advised for bladder care and active physiotherapy was started. He continued to deteriorate and developed difficulty in walking with increasing stiffness and weakness in legs. He was admitted at our institute and a thorough examination was performed. He had an overall grade 3 spastic paraparesis with poor left foot dorsiflexion (grade 1). The reflexes were depressed and plantars were upgoing bilaterally. The anal tone and the peri-anal sensations were reduced. We reviewed the previous MRI and detected T2 hyperintense signal in the dorsolumbar cord which was missed at the first surgery [
These peri-medullary intradural fistulae account for 20% of all spinal vascular lesions and are seen in younger patients. Spetzler has classified the spinal vascular pathologies in the most apt manner although various other classifications by Wyburn-Mason, Pia and Vogelsang, Rosenblum and Borden have been described in the past.[
High index of suspicion is required to diagnose this potentially curable spinal vascular malformation as delay or wrong diagnosis can prove detrimental for the patient. These peri-medullary fistulas fed by the anterior spinal artery are best managed by surgical disconnection as this gives long-lasting cure with minimal risk. The intraoperative confirmation of the obliteration should always be done with angiography. A good outcome can be achieved if the surgery is performed at the right time by a vigilant neurosurgeon.
1. Aminoff MJ, Barnard RO, Logue V. The pathophysiology of spinal vascular malformations. J Neurol Sci. 1974. 23: 255-63
2. Berenstein A, Lasjaunias P, TerBrugge K.editors. Spinal arteriovenous malformation of the spinal cord. Surgical neuroangiography. Berlin: Springer; 2004. 2: 737-847
3. Djindjian R, Vinken PJ, Bruyn GW.editors. Neurological examination of spinal cord angiomas. Handbook of clinical neurology. Elsevier: Amsterdam; 1972. p. 631-43
4. Jellema K, Tijssen CC, van Gijn J. Spinal dural arteriovenous fistulas: A congestive myelopathy that initially mimics a peripheral nerve disorder. Brain. 2006. 129: 3150-64
5. Porras M, Juvela S. Spinal cord and spinal dural arteriovenous fistulas and intradural calcification. Clin Neuroradiol. 2000. 10: 161-5
6. Rodesch G, Pongpech S, Alvarez H, Zerah M, Hurth M, Sebire G. Spinal cord arteriovenous malformations in a pediatric population/children below 15 years of age/the place of endovascular management. Interv Neuroradiol. 1995. 1: 29-42
7. Spetzler RF, Detwiler PW, Riina HA, Porter RW. Modified classification of spinal cord vascular lesions. J Neurosurg. 2002. 96: 145-56