Acquired pial and dural arteriovenous fistulae following superior sagittal sinus thrombosis in patients with protein S deficiency: a report of two cases

Abstract

Two patients with protein S deficiency with acquired multiple pial and dural arteriovenous fistulae (AVFs) following superior sagittal sinus (SSS) thrombosis are reported. Case 1 is a 38-year-old male with protein S deficiency who developed generalized seizure due to SSS thrombosis. Local fibrinolysis was achieved in the acute stage. His 10-month follow-up angiogram revealed an asymptomatic acquired dural AVF arising from the middle meningeal artery and the anterior cerebral artery with drainage to the thrombosed cortical vein in the right frontal lobe. Furthermore, his 2-year follow-up angiogram revealed a de novo pial AVF from the middle cerebral artery in the Sylvian fissure with drainage to the cortical vein initially thrombosed. However, this asymptomatic pial AVF caused bleeding in the ipsilateral cerebral hemisphere 12 years after onset, whereas the dural AVF spontaneously disappeared. Surgical disconnection was successfully performed to eliminate the source of hemorrhage. Case 2 is a 50-year-old male with a past history of SSS thrombosis with protein S deficiency who developed pulsatile tinnitus and generalized seizure. His angiogram showed a cortical dural AVF in the left parietal lobe and a sporadic dural AVF involving the right sigmoid sinus. The parietal lesion was eliminated by transarterial embolization followed by craniotomy. However, a de novo pial AVF emerged from the middle cerebral artery adjacent to the previously treated lesion. Of four cortical AVFs in two patients, thrombosis of cortical veins caused by protein S deficiency might play an important role in their formation. Long-term follow-up is required because this peculiar disorder has an unusual clinical course.

Fig. 1

Case 1 A: The patient's initial magnetic resonance imaging T₂-weighted image demonstrated a small hyperintensity abnormal lesion in the right frontal lobe (closed arrow). B: Lateral view of right carotid injection (venous phase) showing sinus occlusion in the middle of the superior sagittal sinus (open arrow). C, D: The same injection at 2 weeks after local fibrinolysis using urokinase demonstrating remaining occlusion of the superior sagittal sinus. However, no dural arteriovenous fistula was present at this point. Note that a cortical venous occlusion is indicated by the closed arrows (D).

Fig. 2

Case 1 Ten-month follow-up right carotid injection, anterior view (A), right oblique view (B), and lateral view (C, D) showing dural arteriovenous fistula (arrows) supplied by the middle meningeal artery (white dotted arrowhead), superior temporal artery and anterior cerebral artery (white arrowhead) with drainage to the Rolandic vein connecting to the superficial Sylvian vein (black arrowhead). Dotted arrows show faint contrast of thrombosed cortical veins (D). Note that the posterior parietal artery never participated in the shunt at this point.

Fig. 3

Case 1 Two-year follow-up angiogram after the superior sagittal sinus thrombosis (A–G): right internal carotid injection showing that, in addition to the fistula of the parietal cortex, another fistula emerged from the ipsilateral Sylvian fissure, arising from the M3–4 portion of the posterior parietal artery with drainage to the previously thrombosed cortical vein shown in Figs. 1D and 2D. Irregularity of the vessel was consistent with the residual thrombosis in the lumen of this vein (dotted arrows). This draining vein finally connected with the Rolandic vein (black arrowhead). The initial shunt from the anterior cerebral artery was barely recognized (A–C). However, the shunt of the initial arteriovenous (AV) fistula was considerably reduced, as the external carotid artery no longer contributed to this fistula (D). E–G: Anterior oblique view series of right internal carotid injection confirmed this new pial AV fistula arising from the middle cerebral artery. The fistulous point was indicated (long arrows) with drainage to the previously thrombosed cortical vein (dotted arrows) connecting to the Sylvian vein (black arrowheads).

Fig. 4

Case 1 Twelve years later, the patient was transferred to the local hospital when he had severe headache and motor dysfunction in his left arm and leg. His computed tomography scan demonstrated intracerebral hemorrhage in the right frontal lobe (A). The lateral view of the right carotid injection confirmed the lesion (B: black arrowhead), in which the de novo draining vein arising from the corner of the main draining vein was found (B, C: black arrows). The patient underwent surgery to eliminate the fistula at the acute stage. Postoperative angiogram confirmed no presence of the fistula (D).

Fig. 5

Angiographical scheme of Case 1. A: Anterior half of superior sagittal sinus and part of the cortical vein are thrombosed at the time of onset. B: Ten months after onset, a de novo dural AVF arose from the dural and pial arteries with drainage to the cortical vein. C: Two years after onset, another pial AVF occurred, while the initial dural AVF became a low-flow shunt. D: When the patient ultimately bled in the frontal lobe 12 years after the onset, the shunt flow of the pial AVF increased with the de novo draining vein arising from the main draining vein, while the dural AVF disappeared. AVF: arteriovenous fistula.

Fig. 6

Case 2: Preoperative left external carotid injection, anterior view (A, B) and lateral view (C, D) demonstrating dural arteriovenous (AV) fistula arising from the middle meningeal artery (white arrowhead) and superficial temporal artery with drainage to the possibly thrombosed Rolandic vein (black arrowhead) connecting with the superficial Sylvian vein. In addition, the second dural AV fistula (long black arrow) involving the sigmoid sinus with anterograde drainage coexsisted, as shown by the superselective occipital artery injection (E). A sinus thrombosis involving the superior sagittal sinus and the sigmoid sinus was found (B).

Fig. 7

Case 2 A, B: No arteriovenous (AV) fistula was found at the time of the preoperative left internal carotid injection. Note the corkscrew appearance of the cortical veins due to the superior sagittal sinus thrombosis at the venous phase (B). Angiogram after embolization and craniotomy (C–F). Anterior view of the left carotid artery injection revealing a de novo pial AV fistula supplied by the central artery (white arrows) (C). Lateral view of the left carotid injection showing that the new fistula with drainage (white arrows) was apparently distinct from the previous fistula, in a comparison with the location and shape of the draining cortical vein (D, E). The external carotid artery system was not attributed to this new fistula (F).

Fig. 8

Scheme of Case 2. A: Posterior half of superior sagittal sinus and cortical vein are thrombosed. Dural AVF supplied by external carotid arteries is present. B: Initial dural AVF is obliterated by transarterial embolization and surgical excision; however, de novo pial AVF emerges adjacent to the initial lesion. AVF: arteriovenous fistula.