Cerebral venous sinus thrombosis (CVST) may develop a rare and chronic complications such as dural arteriovenous malformation (DAVM). Two-fifths of the patients with DAVM had been found to be associated with CVST in an earlier study involving 69 patients. DAVM may be completely asymptomatic or may present with mild symptoms to fatal cerebral hemorrhage. In the earlier case reports, DAVM after CVST presented with varied symptoms such as a headache, tinnitus, aphasia, seizure, irritability, hemiparesis, and depending on the location. Cases presenting with ophthalmic symptoms leading to the diagnosis of DAVM have been reported rarely.
This is a first case series reporting the cases of the transverse sinus (TS) thrombosis who after successful endovascular stenting showed the recurrence of papilledema and visual loss which on further evaluation led to the diagnosis of DAVM.
A 48-year-old male patient presented with the blurring of vision, with a headache, and tinnitus for the past 1 month. Best-corrected visual acuity (BCVA) was 6/6 both eyes. Fundus showed florid papilledema both eyes [Fig. 1a and b]. Visual fields showed the enlargement of a blind spot. Optical coherence tomography (OCT) showed marked thickening of retinal nerve fiber layer (RNFL). Magnetic resonance venography (MRV) revealed thrombosis of the right TS with partial thrombosis of the superior sagittal sinus. The patient was positive for antiphospholipid antibodies. After starting medical treatment (acetazolamide, furosemide, and glycerine) for raised intracranial pressure with anticoagulant, the papilledema improved minimally. After 2 months, vision deteriorated to 6/60 and 6/9 in the right and left eye, respectively, with persistent papilledema. Digital subtraction angiography (DSA) revealed chronic right TS thrombosis with narrowing of lumen [Fig. 2a] with >10 mmHg pressure gradient at the site of narrowing. He underwent balloon dilatation and stenting of right TS [Fig. 2b] with patent stent and normal flow in poststent angiogram [Fig. 2c]. Papilledema resolved with stable vision and [Fig. 1c and d] secondary optic atrophy in the right eye [Fig. 1c]. OCT showed the resolution of thickened RNFL.
On the recurrence of a headache, diminished vision and papilledema after 3 months, DSA showed the patent stent with DAVM near right TS [Fig. 2d]. On glue embolization of DAVM [Fig. 2e], papilledema resolved in both eyes. On the last follow-up of 5 years, patient is asymptomatic.
A 26-year-old male patient presented with transient obscuration of vision with tinnitus for 1 year and diminution of vision for 2 months. BCVA was 6/60 and 6/9 in the right and left eye, respectively. Examination revealed relative afferent pupillary defect, secondary optic atrophy in the right eye and full-blown papilledema left eye. Bruit was heard behind the right ear. MRV revealed chronic thrombosis with narrowing of the lumen of right TS. He was advised endovascular angioplasty of the sinus, but patient deferred that treatment. After 6 months, the patient presented with further decrease of vision with papilledema, DSA demonstrated right TS thrombosis with DAVM. Angioplasty and stenting of stenosed TS was performed, but no significant improvement in fundus was noticed. Hence, DAVM was embolized with coils and onyx. Postembolization of DAVM symptoms and papilledema improved with no further deterioration of vision. On follow-up of 3 years, stable vision with no evidence of papilledema was noticed.
A 48-year-old female patient presented with a headache, vomiting for 1 month, and diminution of vision for 1 year which deteriorated recently. On examination, BCVA of 6/36 right eye and 6/24 left eye with chronic papilledema was noted. MRV showed chronic right TS thrombosis with partial recanalization. DSA confirmed the MRV findings. She underwent balloon dilatation with stenting of right TS. Following that, vision improved (6/18 both eyes) with the resolution of papilledema. After 6 months, she had the recurrence of symptoms and papilledema. DSA showed a DAVM near right TS which resolved on glue embolization. On follow-up of 4 years, the patient is asymptomatic with the vision of 6/18 both eyes.
A 35-year old female patient who had undergone stenting for right TS thrombosis outside presented with right-sided headache, transient obscuration of vision and tinnitus for 4 months. On examination, BCVA of 6/18 both eyes with chronic papilledema was noticed. DSA indicated DAVM near left TS [Fig. 3a] with enlarged cortical veins with partially thrombosed sinuses [Fig. 3b] which was embolized with coils and glue [Fig. 3c]. After embolization, DAVM resolved [Fig. 3d]. The patient is asymptomatic with stable vision on follow-up of 5 years.
After a successful endovascular intervention of CVST, occasionally patient may develop DAVM. Houser et al. first reported the development of DAVM in patients with CVST. DAVMs are acquired vascular malformations formed between meningeal arteries and dural veins. The exact mechanism for the genesis of DAVM in patients with CVST is not known but likely to involve multiple factors. It has been proposed that DAVM arises from venous hypertension leading to low perfusion pressure and subsequent cerebral ischemia further leading to the release of angiogenic factors such as vascular endothelial growth factor with subsequent formation of abnormal connections between arteries and venous sinuses.
Patients with DAVM may present with ophthalmic features such as the blurring of vision, transient obscuration of vision, rapid fall of vision, and papilledema. In an earlier case report, Houser et al. reported a patient of DAVM presenting with blurred vision, headache, low back pain, and slurred speech after 3 years of TS thrombosis. Another case of DAVM presenting with ophthalmic features developing 1 year after treatment of CVST reported by Micieli et al. recently. Similarly, our cases of CVST presented with various ophthalmic features with symptoms of raised intracranial pressure which resolved after endovascular management. We reported the reappearance of papilledema associated with a headache and blurring of vision associated with raised intracranial pressure which pointed toward the new appearance of vascular thrombosis or a dural malformation such as DAVM which was confirmed on DSA.
OCT was an effective tool to document the RNFL thickening in papilledema and monitor the treatment response in the follow-up period similarly used by Micieli et al.
After successful management of CVST, continuous monitoring including ophthalmic evaluation is highly required as these patients may develop rare complications such as DAVM which may further lead to a visual and neurological deficit.
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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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