Development of Dural Arteriovenous Fistulas After Cerebral Venous Sinus Thrombosis

Central nervous system involvement from chronic lymphocytic leukemia (CLL) occurs infrequently, and manifestations include cognitive and cerebellar dysfunction and cranial nerve palsies. We report a 45-year-old man with CLL believed to be in clinical remission, who presented with vision loss and bilateral optic disc edema. His optic neuropathy due to CLL was proven by optic nerve sheath biopsy, and he experienced visual recovery after treatment with ibrutinib and intrathecal methotrexate.

Clinical Observation Development of Dural Arteriovenous Fistulas After Cerebral Venous Sinus Thrombosis Jonathan A. Micieli, MD, CM, Sheldon Derkatch, MD, Vitor M. Pereira, MD, Edward A. Margolin, MD Abstract: A 57-year-old man presented with papilledema due to partially recanalized dural sinus thrombosis and was treated with anticoagulation and acetazolamide. One year after treatment and resolution of his symptoms, he had an increase in his optic disc edema accompanied by headaches. Subsequent neuroimaging indicated development of arteriovenous fistulas resulting in cortical and deep venous reflux. Given the high risk of mortality from intracranial hemorrhage, the patient underwent urgent treatment with a liquid embolic agent, which resulted in the resolution of his optic disc edema. Our case is unique in that recurrence of bilateral optic disc edema led to discovery of the rare complication of dural arteriovenous fistulas developing after dural sinus thrombosis. Ongoing monitoring of patients after cerebral venous sinus thrombosis is, therefore, important. Journal of Neuro-Ophthalmology 2016;36:53-57 doi: 10.1097/WNO.0000000000000288 © 2015 by North American Neuro-Ophthalmology Society I ntracranial dural arteriovenous fistulas (dAVFs) are rare but potentially fatal vascular malformations. They account for 10%-15% of all intracranial arteriovenous lesions and are characterized by shunting of blood from meningeal and extracranial arteries directly into the dural sinuses or less commonly into meningeal or pial veins (1). Although congenital dAVFs have been reported, most dAVFs are acquired secondary to trauma, thrombophlebitis, surgery, or infection Department of Ophthalmology and Vision Science (JAM, EM), University of Toronto, Toronto, Ontario, Canada; Division of Neuroradiology (SD, VMP), Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada; and Division of Neurosurgery (VMP), Department of Surgery, Toronto Western Hospital, University Health Network, Toronto, Ontario, Canada. The authors report no conflicts of interest. Address correspondence to Edward A. Margolin, MD, Department of Ophthalmology and Vision Sciences, University of Toronto, 801 Eglinton West, Suite 301, Toronto, Ontario, M5N 1E3, Canada; E-mail: [e-mail] Micieli et al: J Neuro-Ophthalmol 2016; 36: 53-57 (2). These lesions may come to clinical attention as a result of intracranial hemorrhage (intraparenchymal, subarachnoid, subdural) or as a consequence of venous hypertension (3). The latter situation may manifest as a number of symptoms including those of raised intracranial pressure. We present a unique case of the development of dAVFs 1 year after treatment of cerebral venous sinus thrombosis (CVST). The recurrence of bilateral optic disc edema was a key finding that led to the discovery of these dAVFs, which put the patient at high risk for intracranial hemorrhage. CASE REPORT A 57-year-old man was referred for bilateral optic disc edema and decreased vision in the left eye for the past 3 months. In addition, he reported an 8-month history of worsening daily headaches, transient visual obscurations, and pulsatile tinnitus. His medical history was significant for right arm superficial venous thrombosis after corrective jaw surgery 2 years before presentation, which was treated with anticoagulation. Other than hypertension treated with amlodipine, he was healthy. There was no family history of hypercoagulable events. On examination, visual acuity was 20/30 in the right eye and 20/50 in the left eye. Pupillary examination was normal without a relative afferent pupillary defect, whereas ophthalmoscopy demonstrated severe bilateral optic disc edema with peripapillary hemorrhages and increased peripapillary retinal nerve fiber layer (RNFL) thickness measured by optical coherence tomography (OCT) (Fig. 1A). There was mild cystoid macular edema in the left eye. Automated perimetry (Humphrey 24-2) revealed an enlarged blind spot in each eye. The remainder of the neurological examination was normal. Magnetic resonance imaging with venography revealed irregularity of the right transverse, sigmoid, and superior sagittal sinuses (Fig. 2). The cord-like appearance of these 53 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation vessels was suggestive of old CVST that subsequently recanalized. Lumbar puncture in the left lateral decubitus position demonstrated an opening pressure of 38 cm H2O with normal cerebrospinal fluid cell count, protein, and glucose. The patient was started on subcutaneous enoxaparin 1 mg/kg twice a day and acetazolamide 2 g daily. Despite an extensive search, no underlying etiology was found for the CVST. Two weeks after beginning the treatment, transient visual obscurations, pulsatile tinnitus, and headaches had resolved. Oral rivaroxaban was substituted for enoxaparin and acetazolamide continued. Eight months later, visual acuity improved to 20/25 in the right eye and 20/30 in the left eye with diminished optic disc edema (Fig. 1B). One year later, the patient was noted to have worsening bilateral optic disc edema (Fig. 1C). On further questioning, he admitted to new frontal headaches, but visual function remained unchanged. A time-resolved magnetic resonance angiogram demonstrated prominent venous channels over the cerebrum, cerebellum, and brainstem (Fig. 3). There also was evidence of partial thrombosis in the superior sagittal sinus and right transverse sinus. These findings were consistent with long-standing partial thrombosis of the dural sinuses and rerouting of blood over the cerebral hemispheres and the cerebellum toward the skull base and toward the perimedullary venous system. There was a high suspicion of a superimposed dAVF causing increased venous pressure and placing the patient at high risk for future intracranial hemorrhage. A catheter angiogram confirmed old CVST with new superimposed dAVFs in the superior sagittal sinus, left sigmoid sinus, and right transverse sinus close to the torcula. Marked rerouting of blood was noted with reflux into the deep venous system (Fig. 4). The patient underwent embolization with a liquid embolic agent, onyx 18, until sufficient reflux into the arterial feeders was achieved to close the fistulas (Fig. 5). When he was seen in follow-up, 3 months later, his optic disc edema had resolved, suggesting the recurrent papilledema was secondary to the newly developed dAVFs. DISCUSSION FIG. 1. Spectral domain optical coherence tomography. A. At presentation, bilateral optic disc edema is present with thickening of the peripapillary retinal nerve fiber layer (RNFL). B. Eight months later, there is less optic disc edema. C. Twelve months after initial presentation, the optic disc edema has worsened with increased thickness of the RNFL. 54 Development of dAVF as a rare chronic complication of CVST was first reported by Houser et al (4). The mechanism is multifactorial and likely involves aberrant angiogenesis induced by tissue hypoxia and/or the opening of preexisting physiological shunts between meningeal arterial networks and dural venous sinuses from venous hypertension (5,6). Venous hypertension is thought to be a critical factor since sinus occlusion alone in the absence of venous hypertension does not result in the development of dAVFs in experimental animals (7). Our case highlights the importance of continued monitoring of patients with a history of CVST for signs of dAVF and increased intracranial pressure. A review of the literature disclosed 8 previous cases of dAVF after CVST Micieli et al: J Neuro-Ophthalmol 2016; 36: 53-57 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation FIG. 2. Magnetic resonance venogram. Maximum intensity coronal (A) and sagittal (B) projections demonstrate filling defects in the right transverse sinus (arrowhead) and superior sagittal sinus (arrows). The right transverse and sigmoid sinus defects are not conspicuous on the sagittal view due to superimposition of the left-sided sinuses. with serial angiography (8-15) (Table 1). In a case of right transverse sinus thrombosis reported by Houser et al (15), the presence of papilledema was noted during physical examination of a patient with fluctuating level of consciousness. A dAVF of the right transverse sinus was FIG. 3. Magnetic resonance angiography 1 year after initial presentation. Sagittal view reveals chronic occlusions of portions of the superior sagittal sinus (arrows). There is early filling of patent portions of the sagittal, transverse, sigmoid, and straight sinuses and presence of tortuous, dilated, cortical, and deep venous collaterals (arrowheads) with enlargement of branches of the external carotid artery (hatched arrow). Micieli et al: J Neuro-Ophthalmol 2016; 36: 53-57 detected 3 years later when the patient presented with severe headaches, slurred speech, back pain, and blurred vision. In our patient, OCT was helpful in detecting and following the course of papilledema. With recurrent increase FIG. 4. 3D volumetric rendering from catheter angiogram with selective injection of the right occipital artery (branch of the external carotid artery). There is contrast shunting into the right dural venous sinuses as well as cortical and deep cerebral veins plus additional sources of shunting from branches of the left external carotid artery into the transverse and superior sagittal sinuses (not shown). 55 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation in the peripapillary RNFL thickness, the patient underwent repeat neuroimaging with discovery of dAVFs (16). Importantly, dAVFs with cortical venous reflux, galenic drainage, and venous congestion are considered aggressive lesions with a high risk of intracranial hemorrhage (17). Annual mortality rates as high as 20% mandate urgent treatment of these lesions making familiarity with their development important for clinicians caring for these patients (18). FIG. 5. Frontal projection of skull radiograph shows the posttreatment appearance after endovascular embolization of shunts into the transverse sinuses, sigmoid sinuses, and torcular with a nonadhesive liquid agent, onyx 18. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: J. A. Micieli and E. Margolin; b. Acquisition of data: J. A. Micieli, S. Derkatch, V. M. Pereira, and E. Margolin; c. Analysis and interpretation of data: J. A. Micieli, S. Derkatch, V. M. Pereira, and E. Margolin. Category 2: a. Drafting the manuscript: J. A. Micieli, S. Derkatch, V. M. Pereira, and E. Margolin; b. Revising it for intellectual content: J. A. Micieli, S. Derkatch, V. M. Pereira, and E. Margolin. Category 3: a. Final approval of the completed manuscript: J. A. Micieli, S. Derkatch, V. M. Pereira, and E. Margolin. TABLE 1. Reported cases of arteriovenous fistula after CVST Authors Age Initial Clinical Symptoms of CVST Morales et al (8) 5 mo Lethargy Safavi-Abbasi et al (10) Nishio et al (9) 40 y Aphasia 57 y Ozawa et al (12) 39 y Touho et al (11) Pierot et al (13) 58 y 21 y 63 y Chaudhary et al (14) 56 y Houser et al (15) 54 y 52 y Present case (2015) 57 y CVST Location Time (mo) dAVF Developed after CVST 3 Location of dAVF Cortical venous Drainage Found on routine follow-up imaging Rt TS-sigmoid confluence No Bilateral pulsatile tinnitus Right hemiparesis TS confluence SSS No 29 Found on routine follow-up imaging Yes 63 Gait disturbance, dementia Headache, pulsatile tinnitus Found on routine follow-up imaging Subsequent head injury prompted imaging Pulsatile tinnitus Rt TS, parietal region Lt TS, straight sinus SSS Rt TS No Rt sigmoid No Lt TS No SSS, ICVs, TS, sigmoid, straight sinus Left TS 12 Headache and vomiting Headache, vomiting, hemiparesis Headache, aphasia Headache, seizure Headache, seizure Headache, dizziness SSS, Bt TS 17 SSS, Rt TS Lt TS, Straight sinus SSS Rt sigmoid 6 Limb weakness Headache, irritability Lt sigmoid 12 Rt TS 36 Headache, blurred vision SSS, Rt TS, Rt sigmoid 12 SSS Clinical Symptom of dAVF 108 11 Rt TS Headache, low back pain, blurred vision, slurred speech Increased optic disc SSS, Lt edema, headache sigmoid, torcular Yes Yes Yes Yes Yes Bt, bilateral; CVST, cerebral venous sinus thrombosis; dAVF, dural arteriovenous fistula; ICV, internal cerebral veins; Lt, left; Rt, right; SSS, superior sagittal sinus; TS, transverse sinus. 56 Micieli et al: J Neuro-Ophthalmol 2016; 36: 53-57 Copyright © North American Neuro-Ophthalmology Society. 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