Congenital carotid to jugular arteriovenous fistulae

Congenital carotid to jugular arteriovenous communications are rare lesions presenting a challenging surgical problem. Including our case, five cases have been reported so far and all were female. The communications represent the persistence of embryonic arteriovenous channels and consequently they are usually multiple. Ligation of the external carotid artery or the use of multiple ligatures should be avoided as a recurrence usually ensues. In addition, both methods render secondary complete excision more difficult and hazardous to the patient. Primary complete resection is recommended as the procedure of choice.