Foix-Alajouanine Syndrome Treatment & Management

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.

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.

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

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.

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.