Background Dural arteriovenous fistulas (DAVFs) of the craniocervical junction (CCJ) represent
very rare arteriovenous shunts between dural arteries and veins of this area. The
classification of these fistulas is based on the draining vein or sinus. Hypoglossal
canal, jugular foramen, posterior condylar vein, and marginal sinus fistulas are the
commonest types. The clinical presentation of the patients with DAVFs of the CCJ depends
on their venous drainage pattern, and thus, the detailed knowledge of the complex
venous anatomy and its variations of the CCJ is very important in understanding the
pathophysiology, interpreting the clinical symptomatology and establishing their treatment
plan. Microsurgical disconnection of the fistulas site using skull base approaches
remains a very important consideration for the treatment of these pathologies.
Objective The purpose of this study is to present two representative cases of DAVFs of the
CCJ, analyzing their angiographic characteristics and their clinical symptomatology
and discussing the most appropriate treatment plan.
Methods Case 1: A 72-year-old man presented with right side intramedullary hemorrhage resulted
in lower cranial nerve dysfunction. Six vessels angiogram revealed a right side DAVF
supplied by right C1 and C3, branches, right ascending pharyngeal artery, and right
occipital artery and draining to prepontine vein and superior petrosal sinus. The
patient underwent two cycles of arterial embolization with total obliteration of the
shunt and his cranial nerve function complete recovered. The follow-up angiogram 8
months later revealed recanalization of the DAVF. Right far lateral craniotomy was
performed and disconnection of the DAVF was achieved. Early postoperative angiogram
confirmed complete disconnection of the shunt. Case 2: A 39-year-old woman presented with HH4 subarachnoid hemorrhage with blood localization
around the foramen magnum and inside the fourth ventricle. The angiography revealed
a left perimedullary DAVF feed by a branch of the left vertebral artery and draining
to a premedullary pial vein. The patient underwent a left far lateral craniotomy and
disconnection of the fistula. Intraoperative angiography revealed no residual shunt.
Postoperatively the clinical examination of the patient improved and returned at her
baseline status. Follow-up angiography 1 year after the procedure confirmed remained
occlusion of the shunt.
Results Skull base approaches and microsurgical disconnection remain very useful in the treatment
armamentarium of DAVFs of the CCJ.
Conclusion Microsurgical disconnection of DAVFs of the CCJ represents a very important tool
in our armamentarium for the treatment of these lesions. Understanding of their complex
angiographic characteristics and knowledge of the various skull base approaches in
the area of the CCJ are critical for the successful treatment of these pathologies.