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Foix-Alajouanine Syndrome Treatment & Management

  • Author: Cheryl Ann Palmer, MD; Chief Editor: Helmi L Lutsep, MD  more...
 
Updated: Jun 08, 2016
 

Approach Considerations

The choice of treatment for Foix-Alajouanine syndrome is either endovascular embolization or surgical ligation of the fistula[5] ; in some cases, both modalities are used.[7]

Successful embolization of the vascular malformation can halt progression of the disease and may result in clinical improvement. Vascular embolization procedures are required in patients in whom surgery is contraindicated.

Many authors consider direct surgical obliteration of spinal dural arteriovenous (AV) fistulas to be the criterion standard of management, since the surgery reportedly provides better disability scores and lower recurrence rates than do embolization procedures.[7, 8] Moreover, surgical management is required if lesions are not amenable to endovascular treatment or if such therapy has failed.[7]

Consultations

An interventional neuroradiologist with expertise in vascular embolization may be able to offer initial, noninvasive therapy.

Neurosurgical consultation is recommended unless the medical condition of the patient precludes the possibility of surgical intervention.

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Follow-up

Inpatient care

Perform a daily postoperative neurologic examination to document improvement or deterioration. Address proper bladder and bowel care.

Outpatient care

Further outpatient care in a rehabilitation center may be required. Proper bladder care may be needed, and an indwelling catheter may be required for several months.

Perform follow-up angiographic studies if symptoms recur. Angiography at 2- to 3-month intervals is recommended for patients who have not made a full recovery.

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Activity

Activity restrictions depend entirely on the patient's neurologic condition.

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Embolization

Endovascular embolization is the least invasive means of therapy and should be attempted if an experienced interventional radiologist is available. The success of endovascular treatment is believed to be highly dependent on complete occlusion of the proximal radiculomedullary draining vein and on the site of the fistula itself.[7]

Embolization with a liquid polymer, such as isobutyl 2-cyanoacrylate (IBCA) or n -butyl 2-cyanoacrylate (NBCA), is considered preferential to embolization with particles, such as those made from polyvinyl alcohol (PVA), because particle use is associated with a higher recurrence rate.[5] Occlusion performed with liquid polymers is successful in 70-90% of cases.[5, 7]

If arterial feeders of fistulas are discovered by imaging studies to involve tributaries of the anterior spinal artery, embolization is not possible due to the risk of spinal cord ischemia and/or infarction.[5]

A novel embolization technique utilizing two microcatheters to selectively place the embolic agent has been developed and tested on a limited number of patients in South Korea.[9] This procedure may have a lower recanalization rate than conventional methods, though does not appear to be widely available at this time for treatment of arteriovenous fistulas.

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Surgical Ligation

Surgical intervention consists of an intradural interruption of the vein draining the fistula. This procedure reduces venous flow (thus diminishing congestion and venous hypertension) and prevents subsequent edema. These measures improve cord perfusion.

A meta-analysis of patients who underwent spinal dural AV fistula operations showed that almost 98% of the surgical procedures were technically successful.[5] The use of intraoperative micro-Doppler evaluation was helpful in one study in increasing the safety of the surgical procedure and in minimizing surgical exposure.[10]

Complications in the form of rapid loss of neurologic function after surgery (weakness or loss of bowel and bladder control) have been observed. These complications are attributed to lack of preoperative identification of the disease process and incorrect recognition of the site of the lesion.

Perform surgical treatment as soon as possible, since the longer the duration of venous hypertension, the greater the magnitude of irreversible neurologic impairment. Prognosis for total recovery is poor if treatment is not rendered early, before neurologic deterioration occurs.

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Contributor Information and Disclosures
Author

Cheryl Ann Palmer, MD Professor of Pathology, Director of Pathology Residency Program, Director of Neuropathology, ARUP Laboratories, University of Utah School of Medicine

Cheryl Ann Palmer, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuropathologists, Society for Neuro-Oncology, International Society of Neuropathology

Disclosure: Nothing to disclose.

Coauthor(s)

Meghan J Driscoll, MD Resident Physician, Department of Pathology, University of Utah School of Medicine

Meghan J Driscoll, MD is a member of the following medical societies: College of American Pathologists, Academy of Clinical Laboratory Physicians and Scientists, Society for Pediatric Pathology, Wyoming Public Health Association

Disclosure: Nothing to disclose.

Chief Editor

Helmi L Lutsep, MD Professor and Vice Chair, Department of Neurology, Oregon Health and Science University School of Medicine; Associate Director, OHSU Stroke Center

Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology, American Stroke Association

Disclosure: Medscape Neurology Editorial Advisory Board for: Stroke Adjudication Committee, CREST2.

Acknowledgements

Howard S Kirshner, MD Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice Chairman, Department of Neurology, Vanderbilt University School of Medicine; Director, Vanderbilt Stroke Center; Program Director, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Staff, Department of Neurology, Nashville Veterans Affairs Medical Center

Howard S Kirshner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neurorehabilitation, National Stroke Association, Phi Beta Kappa, and Tennessee Medical Association

Disclosure: BMS/Sanofi Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

Richard M Zweifler, MD Chief of Neurology, Sentara Healthcare, Norfolk, VA; Professor of Neurology, Eastern Virginia Medical School, Norfolk, VA

Richard M Zweifler, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, American Medical Association, American Stroke Association, Royal Society of Medicine, and Stroke Council of the American Heart Association

Disclosure: Nothing to disclose.

References
  1. Krishnan P, Banerjee TK, Saha M. Congestive myelopathy (Foix-Alajouanine Syndrome) due to intradural arteriovenous fistula of the filum terminale fed by anterior spinal artery: Case report and review of literature. Ann Indian Acad Neurol. 2013 Jul. 16(3):432-6. [Medline]. [Full Text].

  2. Mishra R, Kaw R. Foix-Alajouanine syndrome: an uncommon cause of myelopathy from an anatomic variant circulation. South Med J. 2005 May. 98(5):567-9. [Medline].

  3. Foix CH, Alajouanine T. La myelite necrotique subaigue. Rev Neurol. 1926. 46:1-42.

  4. Rodriguez FJ, Crum BA, Krauss WE, Scheithauer BW, Giannini C. Venous congestive myelopathy: a mimic of neoplasia. Mod Pathol. 2005 May. 18(5):710-8. [Medline].

  5. Jellema K, Tijssen CC, van Gijn J. Spinal dural arteriovenous fistulas: a congestive myelopathy that initially mimics a peripheral nerve disorder. Brain. 2006 Dec. 129:3150-64. [Medline].

  6. Iovtchev I, Hiller N, Ofran Y, Schwartz I, Cohen J, Rubin SA, et al. Late diagnosis of spinal dural arteriovenous fistulas resulting in severe lower-extremity weakness: a case series. Spine J. 2013 Oct 2. [Medline].

  7. Sivakumar W, Zada G, Yashar P, Giannotta SL, Teitelbaum G, Larsen DW. Endovascular management of spinal dural arteriovenous fistulas. A review. Neurosurg Focus. 2009 May. 26(5):E15. [Medline].

  8. Andres RH, Barth A, Guzman R, et al. Endovascular and surgical treatment of spinal dural arteriovenous fistulas. Neuroradiology. 2008 Oct. 50(10):869-76. [Medline].

  9. Zhao LB, Shim JH, Lee DG, Suh DC. Two microcatheter technique for embolization of arteriovenous fistula with liquid embolic agent. Neurointervention. 2014 Feb. 9(1):32-8. [Medline].

  10. Hessler C, Regelsberger J, Grzyska U, Illies T, Zeumer H, Westphal M. Therapeutic clues in spinal dural arteriovenous fistulas - a 30 year experience of 156 cases. Cen Eur Neurosurg. 2010 Feb. 71(1):8-12. [Medline].

  11. Krause F. Chirurgie des Gehirns und Ruckenmarks nach eigenen Erfarungen. Berlin: Urban & Schwarzenberg; 1911.

  12. Thorn, A. Spinale durale arteriovenöse Fisteln. Der Radiologe. November 2001. 41:955-960.

 
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Gross photograph of the dorsal surface of the spinal cord showing dilated and tortuous vessels.
Photomicrograph of the cervical spinal cord region showing a thickened subarachnoid vein with a thrombotic occlusion (hematoxylin and eosin stain).
Photograph of the cervical spinal cord illustrating dilated, abundant subarachnoid veins (hematoxylin and eosin stain).
Photomicrograph of the cervical spinal cord region demonstrating several dilated, hyalinized intraparenchymal vessels (hematoxylin and eosin stain).
Photomicrograph of the cervical spinal cord depicting ischemic necrosis of the parenchyma (hematoxylin and eosin stain).
 
 
 
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