Article
Craniocervical dural arteriovenous fistulas – a life-threatening diagnosis requiring a thorough treatment plan
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Published: | May 13, 2014 |
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Objective: Dural arteriovenous fistulas (DAVF) at the cranicervical junction present with specific characteristics of an incomplete transversal syndrome with/without lower cranial nerve deficits. Insidious onset of brainstem dysfunction may mislead in the diagnosis of craniocervical DAVF but may be life threatening due to venous congestion of the upper, pontine or pontomedullary brainstem.
Method: 11 patients have been retrospectively evaluated for their clinical history, treatment modality and potential pitfalls. All patients underwent superselective angiography in which the ascendent pharyngeal and the external occipital arteries were found to be the feeding arteries. Collateral feeders may originate from tentorial branches or the middel menigeal artery. The arteriovenous fistulous point was typically found around the jugular foramen.
Results: None of the lesions in our series had bled, therefore treatment could be planned on a regular workday involving all interdisciplinary capacities. Embolization with diluted, very liquid acrylic embolization material is worth an attempt although the feeding vessels are thin and the distance to the nidus is long. Nevertheless curative endovascular occlusion could be achieved in more than 50% of the cases. If embolization failed, intraluminal material allowed an exact anatomical localization of the fistulous point intraoperatively. Surgical exposure with lateral approaches of the cranicervical junction is mandatory in these cases to obliterate the fistulous point either by coagulation, clipping or transsection after coagulation. In our series 9 out 11 patients had a favourable outcome with improvement of their symptoms, one remained unchanged and one patient died due to secondary complications.
Conclusions: The prognosis of neurological deficits after obliteration of a csDAVF is rather unpredictable as surgical therapy as well as embolization result in an immediate change of local hemodynamics. All maneuvers eliminating DAVF of the craniocervical junction are associated with a certain risk to compromise brain stem perforaters. Palliative embolization and multi-step strategies may be advisable in these cases to reduce the fistulous shunting in a rather controlled way and to allow gradual adaptation of the hemodynamic flow in this highly sensitive area.