- Department of Neurosurgery, Institute of Clinical Medicine, University of Tsukuba, Ibaraki, Japan
Department of Neurosurgery, Institute of Clinical Medicine, University of Tsukuba, Ibaraki, Japan
DOI:10.4103/2152-7806.99922Copyright: © 2012 Matsubara T. 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: Matsubara T, Akutsu H, Watanabe S, Nakai K, Ayuzawa S, Matsumura A. Histologically proven venous congestive myelopathy without concurrent vascular malformation: Case reports and review of the literature. Surg Neurol Int 21-Aug-2012;3:87
How to cite this URL: Matsubara T, Akutsu H, Watanabe S, Nakai K, Ayuzawa S, Matsumura A. Histologically proven venous congestive myelopathy without concurrent vascular malformation: Case reports and review of the literature. Surg Neurol Int 21-Aug-2012;3:87. Available from: http://sni.wpengine.com/surgicalint_articles/histologically-proven-venous-congestive-myelopathy-without-concurrent-vascular-malformation-case-reports-and-review-of-the-literature/
Background:Venous congestive myelopathy is a progressive myelopathy that is generally caused by a spinal dural arteriovenous fistula.
Case Description:We report a patient with histologically confirmed venous congestive myelopathy without concurrent vascular malformations in radiological and intraoperative findings.
Conclusions:The definitive underlying etiology of this congestive myelopathy was unclear. However, this case report highlights the possibility of venous congestive myelopathy with etiology other than a dural arteriovenous fistula. Further, a systematic and elaborate examination should be undertaken to explore the underlying pathology whenever this type of spinal parenchymal lesion is detected.
Keywords: Foix-Alajouanine syndrome, Spinal cord tumor, Spinal dural arteriovenous fistula, Venous congestive myelopathy
Venous congestive myelopathy, generally known as Foix-Alajouanine syndrome, is clinically characterized by a progressive motor and sensory deterioration. The pathological finding of this condition is described as a necrosis in the spinal cord.[
There have been few reports of venous congestive myelopathy without concurrent dAVF or other vascular malformations.[
We report a patient with histologically confirmed venous congestive myelopathy without concurrent vascular malformation in radiological and intraoperative findings. Several possible mechanisms other than occult vascular malformation are discussed.
A 68-year-old man with no significant past medical history had suffered from paresthesia in his right lower leg. The area of paresthesia slowly extended rostrally up to the umbilicus in 6 months. At 5 months after the onset, he could not walk without cane and at 6 months, vesicorectal dysfunction appeared. On physical examination upon admission at 9 months after the onset, a sensory disturbance below the level of T12, impaired vesicorectal function, and spastic gait were observed. Manual muscle testing showed no weakness in the lower extremities, but he could not walk without cane due to the spasticity and impaired deep sense in lower legs. The deep tendon reflex was promoted in the lower extremities. Blood cell and chemistry examinations revealed no abnormalities. Cerebrospinal fluid test was normal (cell 2/3, protein 21 mg/dl, glucose 65 mg/dl).
Magnetic resonance imaging (MRI) showed an intramedullary T2 hyperintensity and swelling of the spinal cord extending from the level of T5 to T7 [
Preoperative magnetic resonance images of the thoracic spine. Sagittal (a) and axial (b) T2-weighted images show intramedullary hyperintensity and swelling of the spinal cord extending from the level of T5 to T7. Sagittal (c) and axial (d) T1-weighted MR images show faint and partial Gadolinium enhancement. Findings of flow void or dilated pial venous plexus are not observed. The spinal cord is rotated approximately 45° to the right in the axial section (e)
Based on his progressing deterioration and radiological findings, the differential diagnosis included demyelinization disease, transverse myelitis, sarcoidosis, Lyme's disease, spinal cord infarction, and spinal cord tumor. The patient was first admitted to the neurology department and completely examined. He had no history or finding of being bitten by a tick. Vitamin B12 and angiotensin converting enzyme was normal. HIV, syphilis, and hepatitis were negative. Immunoglobulin was also within normal level. The ophthalmological examination showed no abnormal finding. Chest computed tomography (CT) scan showed no bilateral hilar lymphadenopathy. A trial of steroid-pulse therapy (i.v. methylprednisolone 1000 mg for 3 days) resulted in no clinical or radiological improvement. Based on the no efficacy of steroid-pulse therapy in addition to the slowly progressing clinical course transverse myelitis or demyelinization disease was less likely suggested. It was thus suspected that the patient had a spinal cord tumor, and he was referred to our department for histological diagnosis.
We performed a midline myelotomy following T5 to T8 laminectomy. Because the spinal cord was rotated, it was difficult to find its midline. Thus we used dorsal column mapping at the midline myelotomy. A biopsy specimen was obtained at the T7 level on the enhanced area of the MRI. No dilated or tortuous vessels suggestive of vascular malformation were observed on the surface of the spinal cord or the dural sac [
The biopsy specimen showed an increased number of small hyalinized vessels, gliosis, vascular thrombosis, and hemosiderin deposition. Neither tumor cells nor signs of active inflammation were observed [
Photomicrographs of a biopsy specimen showing an increased number of small hyalinized vessels (a, b), gliosis (b), vascular thrombosis (c), and hemosiderin deposition (d). Neither tumor cells nor signs of active inflammation are observed. EVG stain, ×100 (a); H and E, ×200 (b, c) and 400 (d)
One week-postoperative MRI showed a marked decrease of the T2 hyperintensity area, spinal cord swelling and lesion enhancement [
DAVF has been reported to cause neurologic dysfunction on the basis of venous hypertension. Direct communication between arteries and veins increases venous pressure, which causes an increase in intramedullary arterial vasodilation in order to maintain perfusion pressure. These changes then result in the increased tissue pressure, possibly associated with progressive exhaustion of the autoregulatory capacity.[
Pathological studies of congestive myelopathy associated with dAVF have shown marked white matter hypocellularity and small vessels with thickened and hyalinized walls. Extensive vascular sclerosis and gliosis of the white matter is demonstrated with disorganization of the white matter, patchy myelin loss, and concomitant axonal loss.[
There have been several reports of histologically confirmed venous congestive myelopathy or Foix-Alajouanine syndrome without concurrent dAVF [
However, we can state the absence even of occult dAVF with high probability for the following reasons. First, the preoperative MRI failed to show flow void around the spinal cord. Although we did not perform a spinal angiography, the intraoperative findings showed no abnormal, tortuous, or dilated vessels. Second, both his symptoms and MRI findings showed marked improvement just after the biopsy, despite the fact that he underwent no surgical intervention for a vascular malformation.
In the reported cases, the symptoms of 3 of the 14 patients, for whom descriptions of the clinical course were available, improved after biopsy like our patient, although the reasons were unknown. In our case, judging from the rapid radiological improvement after the biopsy, we can state that the decompression procedure together with the biopsy might have played a role in his improvement irrespective of the cause. Furthermore, the mechanical compression or distortion of a perimedullary vein could have caused congestion in our case.
A recent report described a patient who developed venous congestive myelopathy associated with a non compressive herniated cervical intervertebral disc at the same level.[
In our case, rotation of the spinal cord or other unclear underlying etiology could have diminished venous return from the spinal cord and caused the venous congestive myelopathy. Although this mechanism of venous congestive myelopathy is different from that of cases with a dAVF, the pathophysiologic sequelae may be similar in both types of venous congestion.
We reported a case of histologically confirmed venous congestive myelopathy without concurrent vascular malformation. The definitive underlying etiology of this congestive myelopathy is still unclear. However, venous congestive myelopathy can be caused by a dAVF as well as by other etiologies, and thus a systematic and elaborate examination should be undertaken to explore the underlying pathology whenever this type of spinal parenchymal lesion is detected.
1. Aminoff MJ, Barnard RO, Logue V. The pathophysiology of spinal vascular malformations. J Neurol Sci. 1974. 23: 255-63
2. Bhatt N, Bhatt N. Foix-Alajouanine syndrome: A case report. Eur J Neurol. 2007. 14: e4-5
3. Criscuolo GR, Oldfield EH, Doppman JL. Reversible acute and subacute myelopathy in patients with dural arteriovenous fistulas. Foix-Alajouanine syndrome reconsidered. J Neurosurg. 1989. 70: 354-9
4. Girolami UD, Frosch MP, Tator CH, Graham DI, Lantos PL.editors. Diseases of the spinal cord and vertebral column. Greenfield's neuropathology. London: Arnold Publishers; 2002. p. 1071-4
5. Hurst RW, Kenyon LC, Lavi E, Raps EC, Marcotte P. Spinal dural arteriovenous fistula: The pathology of venous hypertensive myelopathy. Neurology. 1995. 45: 1309-13
6. Jellema K, Tijssen CC, van Gijn J. Spinal dural arteriovenous fistulas: A congestive myelopathy that initially mimics a peripheral nerve disorder. Brain. 2006. 129: 3156-64
7. Kalamangalam GP, Bhattacharya J, Teasdale E, Thomas M. Myelopathy from intracranial dural arteriovenous fistula. J Neurol Neurosurg Psychiatry. 2002. 72: 816-8
8. Kataoka H, Miyamoto S, Nagata I, Ueba T, Hashimoto N. Venous congestion is a major cause of neurological deterioration in spinal arteriovenous malformations. Neurosurgery. 2001. 48: 1224-9
9. Kendall BE, Logue V. Spinal epidural angiomatous malformations draining into intrathecal veins. Neuroradiology. 1977. 13: 181-9
10. Kimura A, Tan CF, Wakida K, Saio M, Hozumi I, Inuzuka T. Venous congestive myelopathy of the cervical spinal cord: An autopsy case showing a rapidly progressive clinical course. Neuropathology. 2007. 27: 284-9
11. Koeppen AH, Barron KD, Cox JF. Foix-Alajouanine syndrome. Acta Neuropathol. 1974. 29: 187197-
12. Krishnan C, Malik JM, Kerr DA. Venous hypertensive myelopathy as a potential mimic of transverse myelitis. Spinal Cord. 2002. 42: 261-4
13. Matsuo K, Kakita A, Ishizu N, Endo K, Watanabe Y, Morita T. Venous congestive myelopathy: Three autopsy cases showing a variety of clinicopathologic features. Neuropathology. 2008. 28: 303-8
14. Mirich DR, Kucharczyk W, Keller MA, Deck J. Subacute necrotizing myelopathy: MR imaging in four pathologically proved cases. AJNR Am J Neuroradiol. 1991. 12: 1077-83
15. Montine TJ, O’Keane JC, Eskin TA, Giangaspero F, Gray L, Burger PC. Vascular malformations presenting as spinal cord neoplasms: Case report. Neurosurgery. 1995. 36: 194-7
16. Oldfield EH, Di Chiro G, Quindlen EA, Rieth KG, Doppman JL. Successful treatment of a group of spinal cord arteriovenous malformations by interruption of dural fistula. J Neurosurg. 1983. 59: 1019-30
17. Perkash I, Punj V, Ota DT, Lane B, Skirboll SL. Intracranial dural arteriovenous fistula causing a myelopathy. Spinal Cord. 2002. 40: 438-42
18. Rodriguez FJ, Crum BA, Krauss WE, Scheithauer BW, Giannini C. Venous congestive myelopathy: A mimic of neoplasia. Mod Pathol. 2005. 18: 710-8
19. Schwartz TH, Chang Y, Stein BM. Unusual intramedullary vascular lesion: Report of two cases. Neurosurgery. 1997. 40: 1295-301
20. Smith AJ, McCreery DB, Bloedel JR, Chou SN. Hyperemia, CO2 responsiveness, and autoregulation in the white matter following experimental spinal cord injury. J Neurosurg. 1978. 48: 239-51
21. Terwey B, Besinger U, Schuck M, Vahldiek G, Kuhn F, Kuhn M. MR imaging related to p.m. findings in angiodysgenetic myelomalacia. A case report. Neurosurg Rev. 1993. 16: 323-6
22. Tsutsumi S, Abe Y, Yasumoto Y, Ito M. Lumbar congestive myelopathy mimicking neoplasia without concurrent vascular malformation. Neurol Med Chir (Tokyo). 2009. 49: 316-9
23. Wrobel CJ, Oldfield EH, Di Chiro G, Tarlov EC, Baker RA, Doppman JL. Myelopathy due to intracranial dural arteriovenous fistulas draining intrathecally into spinal medullary veins. Report of three cases. J Neurosurg. 1988. 69: 934-9