2025 年 12 巻 p. 295-301
Dural arteriovenous fistula can present with cerebral venous sinus thrombosis and retrograde leptomeningeal venous drainage. Given the associated intracranial hemorrhage risk, immediate retrograde leptomeningeal venous drainage obliteration is desirable. Herein, we report a case of dural arteriovenous fistula with acute cerebral venous sinus thrombosis and intracranial hemorrhage that was successfully managed using sinus thrombectomy alone. A 76-year-old woman was admitted to our hospital with a sudden headache. Radiological assessments showed a subarachnoid hemorrhage in the left cerebellopontine cistern, Borden type III (Cognard type III) dural arteriovenous fistula with cerebral venous sinus thrombosis in the left transverse-sigmoid sinus, and retrograde leptomeningeal venous drainage in the superficial middle cerebral vein, the vein of Labbé, and the petrosal vein. To prevent re-bleeding, we performed an acute thrombectomy of the transverse-sigmoid sinus, aiming to obliterate retrograde leptomeningeal venous drainage. Following the procedure, the dural arteriovenous fistula was downgraded to Borden type I (Cognard type IIa). In this case, a simple thrombectomy alone prevented the re-bleeding of a dural arteriovenous fistula without direct fistula access. Understanding the relative contributions of the fistula and cerebral venous sinus thrombosis to retrograde leptomeningeal venous drainage in transverse-sigmoid sinus dural arteriovenous fistula is essential for refining the treatment strategy. Sinus thrombectomy is a tentative treatment, and this strategy is the emergency procedure to reduce re-bleeding risk. However, it may be a viable approach to prevent re-bleeding by improving venous outflow in dural arteriovenous fistula cases complicated by acute cerebral venous sinus thrombosis and intracranial hemorrhage.
Dural arteriovenous fistula (dAVF) is an intracranial vascular malformation with an incidence of 0.15 to 0.29 cases per 100,000 person-years,1) with transverse-sigmoid sinus (TSS) being the most common site of occurrence (33.4% of dAVF cases).2) TSS dAVF often presents retrograde leptomeningeal venous drainage (RLVD), significantly elevating the risk of intracranial hemorrhage (ICH) and requiring surgical intervention, due to a high early re-bleeding rate of 35% compared to 12% in all dAVF cases.2-5) The occurrence of RLVD is closely associated with cerebral venous sinus thrombosis (CVST) and enlargement of the fistula, both of which represent primary pathological mechanisms in the progression of TSS dAVF.1,5-10)
A precise diagnosis causing RLVD is crucial for assessing appropriate and effective surgical intervention in cases of TSS dAVF presenting with ICH.7,11) Treatment strategies such as transarterial embolization (TAE) and transvenous embolization (TVE) generally focus on obliterating dAVF fistulas.11) In contrast, treatment strategies focused on CVST are rarely applied, despite its association with RLVD. Herein, we present the first case of dAVF with CVST and ICH solely treated with sinus thrombectomy to prevent re-bleeding.
A 76-year-old woman had been undergoing annual magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) for an unruptured aneurysm in the right middle cerebral artery. The most recent follow-up MRA showed increased intensity in the left superior petrosal sinus and left TSS, indicating a possible dAVF (Fig. 1). Severe chronic kidney disease (Creatinine level 2.85 and estimated glomerular filtration rate [eGFR] 13.2 mL/min1.73 m2) had previously prevented the patient from undergoing detailed examinations, such as digital subtraction angiography (DSA). Four months after the last MRA, the patient was admitted to our hospital with a sudden headache. Despite the absence of consciousness or neurological deficits, computed tomography (CT) and MRI performed immediately after admission revealed a subarachnoid hemorrhage in the left cerebellopontine cistern and a CVST in the left transverse-sigmoid junction (Fig. 2A and B). MRA showed high-intensity signals similar to those observed before admission (Fig. 2C). DSA confirmed the presence of a left TSS Borden type III (Cognard type III) dAVF. The arterial supply comprised the left middle meningeal, occipital, and tentorial arteries. RLVD involved retrograde blood flow through the superior petrosal sinus to the sphenopetrosal vein, superficial middle cerebral vein and the vein of Labbé, and petrosal vein with occlusion of the left transverse and sigmoid sinuses (Fig. 2D-H). Additionally, thrombi appeared in the superior sagittal sinus during the examination (Fig. 2I), suggesting acute-phase CVST. Venous return was disrupted at the left TSS junction (Fig. 2J). At the end of the examination, the patient's consciousness deteriorated to a Glasgow Coma Scale (GCS) score of E3V4M6. Subsequent CT imaging 3 hrs after admission revealed enlargement of the hemorrhage into the ventricles (Fig. 2K and L). A diagnosis of TSS dAVF with CVST and progressing ICH was made, and an attempted acute sinus thrombectomy was performed for TSS to eliminate RLVD.
Imaging findings before admission.
A: Magnetic resonance angiography (MRA) examined 8 years ago before admission, showing no abnormal signals.
B: MRA examined one year before admission, showing high-intensity signals in the left superior petrosal vein and left transverse-sigmoid sinus (TSS) (arrows), suggesting dural arteriovenous fistula (dAVF).
C: MRA performed 4 months before admission, showing an increase in the high-intensity signals in the sinuses (arrows).
Imaging findings of intracranial hemorrhage and dural arteriovenous fistula.
A, B: Computed tomography (CT) and magnetic resonance imaging (MRI) reveal subarachnoid hemorrhage in the left cerebellopontine cistern (arrows) and cerebral venous sinus thrombosis (CVST) in the left transverse-sigmoid junction (arrowheads).
C: Magnetic resonance angiography (MRA) at admission, showing high-intensity signals before admission (arrows).
D, E, F: digital subtraction angiography (DSA) indicates a left transverse-sigmoid sinus (TSS) Borden type III (Cognard type III) dural arteriovenous fistula (dAVF) with left TSS occlusion.
D: Lateral view of the left external carotid angiography (arterial phase) shows arterial supply from the left middle meningeal artery petrosal branch (arrow), posterior convexity branch (arrowheads), and occipital artery transosseous branch (double arrows). Early venous drainage into the superior petrosal sinus is also observed (asterisk).
E: Lateral view of the left external carotid angiography (delayed arterial phase), demonstrating retrograde venous flow through the sphenopetrosal vein (arrow) to the superficial middle cerebral vein (double arrows) and vein of Labbé (arrowheads), with occlusion of the left transverse and sigmoid sinuses.
F, G: Anteroposterior and lateral view of the left external carotid angiography (venous phase), demonstrating petrosal vein dilation (arrows).
H: Lateral view of the left internal carotid angiography (arterial phase), with the left tentorial artery also contributing to the arterial supply (arrow).
I: Lateral view of the left internal carotid angiography (venous phase), showing thrombosis in the superior sagittal sinus (double arrows).
J: Anteroposterior view of the left internal carotid angiography (venous phase), showing that venous return was disrupted at the left TSS junction (arrow).
K, L: CT performed immediately after a decreased level of consciousness, indicating expansion and extension of the hemorrhage into the ventricles.
The procedure was carried out under general anesthesia. An 8-Fr FUBUKI guiding catheter (ASAHI INTECC, Seto, Aichi, Japan) was placed in the left internal jugular vein via a transfemoral approach. An AXS Vecta 71 (Stryker Neurovascular, Fremont, CA, USA) was guided into the occluded left sigmoid and transverse sinuses with a 0.035-inch guidewire to aspirate the thrombus (Fig. 3A and B). Red thrombi were removed from both sinuses (Fig. 3C), and DSA revealed venous flow restoration in the sinuses along with RLVD and petrosal vein dilation disappearance (Fig. 3D-G). Venous return was disrupted at the left TSS junction, indicating that the affected venous sinus was not used for normal venous return. Although remaining stenosis in the sigmoid sinus, the dAVF transformed into Borden type I (Cognard type IIa). The thrombus in the Superior sagittal sinus was not intervened, as it was irrelevant to the hemorrhage. The total procedure time, including the initial examination, was 146 min, with a total contrast agent volume of 63 mL. As the medical treatment for CVST, the patient received anticoagulant therapy following the sinus thrombectomy. As far as can be examined, the patient had no thrombogenesis tendency, and warfarin was used as an oral anticoagulant after heparin therapy with a target therapeutic value of Prothrombin Time-International Normalized Ratio 2 to 3. Follow-up DSA 2 weeks after admission maintained the obliteration of RLVD.
Sinus thrombectomy.
A: Lateral view of the angiogram during thrombectomy in the left sigmoid sinus. An 8-Fr FUBUKI guiding catheter (arrow) was placed in the left internal jugular vein with a transfemoral approach. The AXS Vecta 71 (arrowhead) was guided in the occluded left sigmoid sinus using a 0.035-inch guidewire to aspirate the thrombus.
B: Anteroposterior view of the angiogram obtained during thrombectomy of the left transverse sinus. The AXS Vecta 71 (arrowhead) was guided into the occluded left transverse sinus using a 0.035-inch guidewire (double arrows) to aspirate the thrombus.
C: Red thrombus aspirated from the AXS Vecta 71 in the left transverse-sigmoid sinus (TSS). Scale bar: 1 cm.
D: Lateral view of the external carotid angiography after aspiration of the left sigmoid sinus, showing antegrade flow restored in the left sigmoid sinus.
E: Lateral view of the external carotid angiography after aspiration of the left transverse sinus, showing retrograde flow in the left transverse sinus following thrombectomy.
F: Lateral view of the left external carotid angiography (arterial phase) after acute venous sinus revascularization. The retrograde leptomeningeal venous drainage (RLVD) and petrosal vein dilation disappeared after thrombectomy, with venous drainage now directed antegradely through the left sigmoid sinus.
G: Anteroposterior view of the left internal carotid angiography (venous phase) after acute venous sinus revascularization, showing that there is no venous flow in TSS and petrosal vein.
The eGFR did not deteriorate after the surgery (Creatinine level 2.84 and eGFR 13.1 mL/min1.73 m2), and the patient's consciousness improved to GCS E4V5M6. She was discharged on hospital day 32 and is currently under careful observation. The Ethical Review Board of the Osaka Neurological Institute (approval number: OR 02-3) approved this study. Since this was a retrospective study, informed consent was not required.
We performed an acute sinus thrombectomy on a patient with TSS dAVF and CVST, who presented with an ICH. The procedure effectively restored antegrade sinus revascularization, which alleviated RLVD by reopening the previously existing drainage pathway, thereby preventing re-bleeding.
Acute-phase CVST was identified as the primary cause of RLVD and ICH, based on the examinations and clinical course. In this case, Borden type I dAVF had been suspected on MRA before admission (Fig. 1). CT and MRI at admission showed a subarachnoid hemorrhage in the left cerebellopontine cistern and thrombosis in TSS for the first time (Fig. 2A and B). DSA also revealed thrombi in the superior sagittal sinus (Fig. 2I) and RLVD involving the superficial middle cerebral vein, the vein of Labbé, and the petrosal vein (Fig. 2E-G). CVST is known to occur near the fistula in the downstream venous drainage pathways,5) amplifying retrograde venous flow due to venous hypertension.10) These findings support the hypothesis that the presence of RLVD resulted from the disruption of the downstream venous drainage pathway, primarily due to progressive clot formation. The venous reflex in the left petrosal vein is also consistent with the location of ICH in the left cerebellopontine cistern.
Following the thrombectomy, DSA indicated stenosis distal to the shunt point toward the heart, potentially suggesting compartmentalization or sequestration in the lateral part of the left transverse sinus.12,13) Together, a TSS dAVF with impaired reflux in the distal part might complicated by CVST, resulting in RLVD and ultimately leading ICH.
Sinus thrombectomy effectively prevented re-bleeding by obliterating RLVD in this case. Generally, TVE and TAE have been applied for TSS dAVF with RLVD, targeting the obliteration of fistulas.14,15) Although these procedures require careful preoperative strategies of embolization technique and the number of treatment sessions, the cases presenting ICH sometimes make them challenging due to the urgency.11,15,16) Previous studies indicated TAE with Onyx for dAVF requiring 267 to 271 mins of procedure time and 238 mL of contrast agent.17,18) Therefore, a simple and fast intervention aimed at obliterating RLVD is preferred as a prevention for re-bleeding. In this case, the total procedure time was 146 min, with a total contrast agent volume of 63 mL. Sinus thrombectomy also required no special equipment, perioperative preparation, or specific surgeon, and also obliterated RLVD through reviving venous drainage pathway suffering from CVST.19-22) Balloon angioplasty or self-expanding stent placement also obliterates both RLVD and fistulas by alleviating sinus hypertension.8,23,24) However, these procedures require multiple devices and antiplatelet drugs.8,23,24) Sinus thrombectomy would be a simple and effective intervention for TSS dAVF presenting ICH, from the perspective of acute management aimed at obliterating RLVD and preventing re-bleeding.
It should also be noted that sinus thrombectomy is a tentative treatment, as it does not treat the fistula itself. Moreover, this strategy is the emergency procedure to reduce re-bleeding risk. The patient in this case received only anticoagulant therapy, and curative endovascular surgery was not performed because of concerns regarding worsening renal function. Given the risk of sinus re-occlusion, additional embolization to occlude the fistula, balloon angioplasty, or self-expanding stent placement to keep the sinus open may be considered in the future. Although the benefit of rapid improvement in venous reflux during the acute phase, it is crucial to acknowledge that sinus thrombectomy does not constitute a definitive treatment for TSS DAVF.
Determining whether the fistula or CVST plays a more significant role in RLVD is crucial for guiding the treatment strategy in TSS dAVF with ICH. In this case, we were able to perform effective acute surgical treatment against re-bleeding because CVST was identified as the primary cause of RLVD. Accurate diagnosis of the underlying cause of RLVD may provide critical insights into selecting effective re-bleeding prevention strategies in cases of TSS dAVF presenting with ICH (Fig. 4).
Treatment strategy.
Treatment strategy for transverse-sigmoid sinus (TSS) dural arteriovenous fistula (dAVF) with retrograde leptomeningeal venous drainage (RLVD), presenting with intracranial hemorrhage (ICH). Determining whether the fistula or intervening cerebral venous sinus thrombosis (CVST) plays a more significant role in RLVD is crucial for guiding treatment strategy in TSS dAVF presenting ICH. In this case, CVST (thick arrow) contributed more significantly to RLVD than fistula (dashed arrow). Transarterial embolization (TAE) and transvenous embolization (TVE) obliterate RLVD through the intervening fistula. Stent implantation and balloon angioplasty obliterate RLVD through intervening fistulas. Simple thrombectomy for CVST obliterates RLVD when CVST predominantly contributes to RLVD, as applied in the present case.
In conclusion, venous sinus thrombectomy may be a viable acute treatment option for TSS dAVF with CVST and ICH, as it helps to prevent re-bleeding by reducing RLVD.
We would like to thank Editage for English language editing.
The authors declare no conflict of interest.
