gms | German Medical Science

Objective: Arteriovenous fistulae of the filum terminale (AVFFT) are rare lesions, and are therefore easily missed. AVFFT is characterized by an ascending myelopathy (Foix-Alajouanine syndrome). Edema, swelling and malfunction of the spinal cord are essentially due to a venous congestion caused by an acquired arteriovenous (AV) shunt. We report on 3 patients presenting with a Foix-Alajouanine syndrome related to an arteriovenous fistula of the filum terminale. Diagnostic issues of this disorder and the treatment strategies are discussed.

Method: Three patients with mean age of 52 years were referred to our institution with a predominantly asymmetrical paraplegia, progressive numbness of both legs and bladder dysfunction with urinary retention. The duration of symptoms varied between 4 and 24 months. Initial MRI studies showed an increased intramedullary T2 signal, swollen cord, and dilated perimedullary veins in all patients. Digital subtraction angiography showed an AV shunt supplied by the artery of the filum terminale.

Results: Surgery was performed in all three patients and resulted in an interruption of the AV shunt. There was no treatment-related complication in two patients. Venous congestion of the spinal cord improved on follow-up in all patients. Clinical improvement, however, was incomplete in all three.

Conclusions: The arterial supply and the location of the fistula of AVFFT are mostly well accessible for a microsurgical approach. After the interruption of the AV shunt, the majority of the patients will improve clinically. The functional outcome, however, depends on early diagnosis and treatment.