Abstract 1122‐000107: Charles Bonnet Syndrome Secondary to Massive Dural Arteriovenous Fistula Disguised as Extensive Venous Sinus Thrombosis

Author(s):  
Ahmad A Ballout ◽  
Timothy G White ◽  
Athos Patsalides

Introduction : Charles Bonnet Syndrome is characterized by visual hallucinations that can occur following severe visual insult, rarely due to dural arteriovenous fistulas (DAVF) or cerebral venous sinus thrombosis (CVST). Prompt differentiation between DAVF and CVST is important since treatments may differ and inadequate treatment may result in blindness. We highlight a patient who presented with Charles Bonnet Syndrome initially misdiagnosed with CVST by MR venography and later correctly diagnosed with a massive DAVF with superimposed CVST by digital subtraction angiography and underwent DAVF embolization with complete resolution. Methods : Case Report. Results : A 78 year‐old man with hypertension and hyperlipidemia presented with three weeks of bilateral vision loss associated with formed hallucinations exacerbated by dark rooms. Neurological exam revealed decreased visual acuity of 20/400 and grade five papilledema bilaterally. Non‐Contrast (TOF) MR venogram revealed lack of flow in the superior sagittal sinus (SSS), straight sinus (SS) and deep venous system, and partial flow of the left transverse and sigmoid sinus and left jugular vein. MR brain without gadolinium was unremarkable. Cerebral angiography revealed a high‐grade DAVF predominantly supplied by the occipital branch of the left external carotid artery [Figure 1; A‐C], with retrograde flow into the left sigmoid, transverse, superior sagittal, and straight sinuses, as well as retrograde flow into the right vein of Trolard [Figure 1; A‐D]. The left distal sigmoid sinus and left jugular bulb were occluded. The left transverse and proximal left sigmoid venous sinuses were compartmentalized from non‐occlusive thrombus, while the SSS and bilateral transverse sinuses where patent [Figure 1; A, B]. Embolization using coils and onyx was performed with complete occlusion of the left transverse and sigmoid sinuses, the points of main drainage of the fistula, as there was no single trans arterial pedicle suitable for embolization. Postembolization angiography demonstrated a Cognard Grade 1 fistula with some residual fistulous shunting of the occipital artery to the torcula. Follow up angiogram at six weeks showed interval occlusion of the residual shunt. He had minimal improvement in his vision at three months of follow up. Conclusions : This case highlights a patient with Charles Bonnet Syndrome due to a high flow DAVF. The MR venogram failed to capture the DAVF since the retrograde flow was interpreted as thrombosis on MRV. DAVF and CVST have a complex cause‐effect relationship, since thrombosis may open up venous channels that can lead to a fistula and sluggish blood flow from a fistula may stimulate thrombus formation. Treatments between CVST and DAVF differ since high grade DAVF often require endovascular embolization and anticoagulation may increase the risk of intracerebral hemorrhage in a subset of patients. Digital subtraction angiography and/or contrast enhanced MRV should be considered in cases of suspected extensive thrombosis to help differentiate between thrombosis and DAVF.

2019 ◽  
Vol 7 (3) ◽  
Author(s):  
Afdi Arahim Putra ◽  
Eppy Buchori ◽  
Hilman Hilman ◽  
Lisda Amalia

Cerebral venous sinus thrombosis (CVST) is a cerebrovascular disease in the form of occlusion due to thrombus in the venous and cerebral sinuses. It rarely occurs and has varied clinical symptoms and radiological features and challenging to diagnose. D-dimer used as a diagnostic marker for cases of venous thromboembolism, with a sensitivity of around 90–92%. However, the specificity is not too high (70–73%) because it can also increase in other conditions. Digital subtraction angiography (DSA) is a gold standard examination to establish the diagnosis of CVST. The purpose of this study was to determine the relationship between the D-dimer level and CVST using DSA at Dr. Hasan Sadikin General Hospital in Bandung. This study used an observational analytic method with a case-control study design using retrospective data from medical records at Dr. Hasan Sadikin General Hospital in January 2017–August 2019. The research subjects divided into two groups, namely the high D-dimer levels and the normal/low D-dimer level. Forty people meet the inclusion criteria, ages averaging from 44.77±14.40 years, and consists of 9 male patients (22%) and 31 women patients (78%). For normal/low D-dimer levels 20 patients (50%) and high D-dimer levels 20 patients (50%). Statistical test results measuring D-dimer and CVST levels found a significant relationship (p<0.05). In conclusion, there is a relationship between D-dimer levels with CVST events that have been done by DSA. The higher the D-dimer level, the higher the suspicion of CVST. KADAR D-DIMER DENGAN KEJADIAN CEREBRAL VENOUS SINUS THROMBOSIS (CVST) MENGGUNAKAN DIGITAL SUBTRACTION ANGIOGRAPHY (DSA)Penyakit cerebral venous sinus thrombosis (CVST) merupakan penyakit serebrovaskular berupa oklusi akibat trombus di saluran vena dan sinus serebral yang jarang terjadi dengan gejala klinis dan gambaran radiologis yang bervariasi, serta sangat sulit untuk didiagnosis. D-dimer dapat dijadikan sebagai penanda diagnostik bagi kasus-kasus tromboembolisme vena dengan sensitivitas 90–92%, namun spesifisitasnya tidak terlalu tinggi (70–73%) karena dapat juga meningkat pada kondisi lain. Digital subtraction angiography (DSA) merupakan pemeriksaan baku emas untuk menegakkan diagnosis CVST. Tujuan penelitian ini mengetahui hubungan antara kadar D-dimer dan CVST menggunakan DSA di RSUP Dr. Hasan Sadikin Bandung. Penelitian ini merupakan observasional analitik dengan rancangan kasus kontrol menggunakan data retrospektif dari rekam medis di RSUP Dr. Hasan Sadikin Bandung pada bulan Januari 2017–Agustus 2019. Subjek penelitian dibagi menjadi 2 kelompok, yaitu kelompok D-dimer tinggi dan kelompok D-dimer normal/rendah. Hasil penelitian didapat 40 orang yang memenuhi kriteria inklusi, usia rerata 44,77±14,40 tahun yang terdiri atas pasien laki-laki 9 orang (22%) dan perempuan 31 orang (78%). Untuk kadar D-dimer kategori normal/rendah 20 orang (50%) dan tinggi 20 orang (50%). Hasil uji statistik mengukur kadar D-dimer dan CVST didapatkan hubungan yang bermakna (p<0.05). Simpulan, terdapat hubungan antara kadar D-dimer dan kejadian CVST yang telah dilakukan DSA. Semakin tinggi kadar D-dimer, semakin tinggi kecurigaan kejadian CVST.


Neurosurgery ◽  
2013 ◽  
Vol 72 (5) ◽  
pp. 730-738 ◽  
Author(s):  
Guangwen Li ◽  
Xianwei Zeng ◽  
Mohammed Hussain ◽  
Ran Meng ◽  
Yi Liu ◽  
...  

Abstract BACKGROUND: Although the majority of patients with cerebral venous sinus thrombosis (CVST) obtain an optimistic clinical outcome after heparin or warfarin treatment, there remains a subgroup of patients who do not respond to conventional anticoagulation treatment. These patients, especially younger people, as documented by hospital-based studies, have a high morbidity and mortality rate. OBJECTIVE: To verify the safety and efficacy of a dual mechanical thrombectomy with thrombolysis treatment modality option in patients with severe CVST. METHODS: Fifty-two patients diagnosed with CVST were enrolled and treated with mechanical thrombectomy combined with thrombolysis. Patients underwent urokinase 100 to 1500 × 103 IU intravenous sinus injection via a jugular catheter after confirming diagnoses of CVST by using either magnetic resonance imaging/magnetic resonance venography or digital subtract angiography. Information obtained on the patients included recanalization status of venous sinuses as evaluated by magnetic resonance venography or digital subtract angiography at admission, during operation, and at 3- and 6-month follow-up after treatment. RESULTS: The percentage of patients that showed complete and partial recanalization were 87% and 6%, respectively, after mechanical thrombectomy combined with thrombolysis treatment; 8% of the patients showed no recanalization. The modified Rankin Scale scores were 1.0 ± 0.9, 0.85 ± 0.63, and 0.37 ± 0.53 for discharge, and 3- and 6-month follow-up, respectively. A total of 6 patients died despite receiving aggressive treatment. No cases of relapse occurred after 3 to 6 months of follow-up. CONCLUSION: Thrombectomy combined with thrombolysis is a safe and valid treatment modality to use in severe CVST cases or in intractable patients that have shown no adequate response to antithrombotic drugs.


2020 ◽  
Vol 5 (4) ◽  
pp. 368-373
Author(s):  
Shen Li ◽  
Kai Liu ◽  
Yuan Gao ◽  
Lu Zhao ◽  
Rui Zhang ◽  
...  

ObjectiveTo evaluate the prognosis values of systemic immune–inflammation index (SII) in non-chronic cerebral venous sinus thrombosis (CVST).Methodspatients with CVST, admitted to the First Affiliated Hospital of Zhengzhou University, were retrospectively identified from January 2013 to December 2018. We selected patients in acute/subacute phase from database. Functional outcomes of patients were evaluated with the modified Rankin Scale (mRS)—mRS 3–6 as poor outcomes and mRS 6 as death. The overall survival time was defined as the date of onset to the date of death or last follow-up date. Survival analysis was described by the Kaplan-Meier curve and Cox regression analysis. Multivariate logistic regression analysis assessed the relationship between SII and poor functional outcome. The area under the Receiver Operating Curve curve (AUC) was estimated to evaluate the ability of SII in prediction.ResultsA total of 270 patients were included and their duration of follow-up was 22 months (6–66 months), of whom 31 patients had poor outcomes and 24 patients dead. Cox regression analysis showed that SII (HR=1.304, 95% CI: 1.101 to 1.703, p=0.001) was a predictor of death in non-chronic CVST. Patients with higher SII presented lower survival rates (p=0.003). The AUC of SII was 0.792 (95% CI: 0.695 to 0.888, p=0.040) with a sensitivity of 69.6% and specificity of 80.1%. Subgroups analysis demonstrated that SII was an important predictor of poor outcomes in male (OR=1.303, 95% CI: 1.102 to 1.501, p=0.011) and pregnancy/puerperium female (OR=1.407, 95% CI: 1.204 to 1.703, p=0.034).ConclusionsSII was a potential predictor in the poor prognosis of patients with acute/subacute CVST, especially in male and pregnancy/puerperium female.


2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Ali Alsafi ◽  
Amish Lakhani ◽  
Lalani Carlton Jones ◽  
Kyriakos Lobotesis

Purpose. Retrospectively evaluate the density of cerebral venous sinuses in nonenhanced head CTs (NCTs) and correlate these with the presence or absence of a cerebral venous sinus thrombus (CVST).Materials and Methods.Institutional review board approval was obtained and informed consent waived prior to commencing this retrospective study. Over a two-year period, all CT venograms (CTVs) performed at our institution were retrieved and the preceding/subsequent NCTs evaluated. Hounsfield Units (HUs) of thrombus when present as well as that of normal superior sagittal and sigmoid sinuses were measured. HU of thrombus was compared to that of normal vessels with and without standardisation to the average HU of the internal carotid arteries.Results. 299 CTVs were retrieved, 26 with a thrombus. Both raw and standardised HU measurements were significantly higher in CVST (p<0.0001) compared to normal vessels. Both raw and standardised HUs are good predictors of CVST. A HU of ≥67 and a standardised measurement of ≥1.5 are associated with high probability of CVST on NCT.Conclusion.Cerebral venous sinus HU measurements may help improve sensitivity and specificity of NCT for venous sinus thrombosis and avoid potentially unnecessary follow-up examinations.


2016 ◽  
Vol 9 (5) ◽  
pp. 512-515 ◽  
Author(s):  
Michael R Levitt ◽  
Felipe C Albuquerque ◽  
Bradley A Gross ◽  
Karam Moon ◽  
Ashutosh P Jadhav ◽  
...  

BackgroundVenous sinus stenting is an effective treatment for patients with idiopathic intracranial hypertension (IIH) and venous sinus stenosis.ObjectiveTo determine the usefulness of venous sinus stenting in the treatment of patients with symptomatic venous sinus stenosis without a diagnosis of IIH.MethodsWe performed a retrospective review of a prospective multicenter database of patients undergoing venous sinus stenting between January 2008 and February 2016. Patients with acute dural venous sinus thrombosis, arteriovenous fistula or arteriovenous malformation, or IIH were excluded. Clinical, radiological, and ophthalmological information was recorded.ResultsNine patients met the inclusion criteria and underwent venous sinus stenting for symptomatic dural venous sinus stenosis. Reasons for treatment included isolated unilateral pulsatile tinnitus (n=1), congenital hydrocephalus (n=2), unilateral pulsatile tinnitus following prior venous sinus thrombosis (n=1), acquired hydrocephalus following dural sinus thrombosis (n=2), meningitis (n=2) and tumor invasion into the dural venous sinus (n=1). Six patients underwent lumbar puncture or shunt tap, and all of these patients had elevated intracranial pressure. All stenoses were located in the transverse sinus, transverse–sigmoid junction and/or jugular bulb, and all were treated with self-expanding bare-metal stents. At follow-up, clinical symptoms had resolved in all but two patients, both of whom had congenital hydrocephalus and pre-existing shunts. There was no significant in-stent stenosis, and patients with ophthalmological follow-up demonstrated improvement of papilledema.ConclusionsDural venous sinus stenting may be an effective treatment for patients with symptomatic venous sinus stenosis without IIH in carefully selected cases, but may not be effective in resolving the symptoms of congenital hydrocephalus.


2015 ◽  
Vol 21 (6) ◽  
pp. 719-723 ◽  
Author(s):  
Hidemitsu Adachi ◽  
Yohei Mineharu ◽  
Tatsuya Ishikawa ◽  
Hirotoshi Imamura ◽  
Shiro Yamamoto ◽  
...  

Endovascular treatment for superior sagittal sinus (SSS) thrombosis is not always successful because of difficult access and long thrombus lesions. We report the first two cases of patients with acute cerebral venous sinus thrombosis at the SSS that was not recanalized by anticoagulation, mechanical thrombectomy, or thrombolysis, but was successfully treated by stent placement. Case 1 was a 37-year-old woman with bilateral subdural hematomas. Digital subtraction angiography showed obstruction of the sinus from the SSS to the right transverse sinus. Recanalization was achieved by selective thrombolysis using urokinase followed by balloon angioplasty, but re-occlusion occurred on the next day of treatment. Repeated endovascular treatment including balloon angioplasty, thrombus aspiration and thrombolysis using recombinant tissue plasminogen activator failed to achieve recanalization. We thus placed intracranial stents in the SSS, which did achieve recanalization. Case 2 was a 69-year-old woman with a small infarction in the left parietal lobe. Digital subtraction angiography showed sinus obliteration from the SSS to the bilateral transverse sinuses. Recanalization was not achieved by balloon angioplasty, thrombus aspiration and selective thrombolysis. We thus placed intracranial stents in the SSS, which did achieve recanalization. Postoperative course was uneventful in both cases and venous sinus patency was confirmed by venography >1.5 years after treatment. When conventional endovascular strategies have been unsuccessful, placement of intracranial stents, which can easily gain access to the distal part of the SSS as compared with carotid stents, may be a useful treatment option for the acute sinus thrombosis in this region.


2017 ◽  
Vol 23 (6) ◽  
pp. 656-660
Author(s):  
Yoshinari Osada ◽  
Hidenori Endo ◽  
Kenichi Sato ◽  
Yasushi Matsumoto ◽  
Toshiki Endo ◽  
...  

We report a case of a 39-year-old man presenting with a high-grade left parieto-occipital arteriovenous malformation (AVM) complicated by superior sagittal sinus (SSS) stenosis, seven years after the first presentation. Magnetic resonance imaging revealed a newly developed perilesional edema. Venous sinus stenosis acutely progressed to occlusion and induced multiple intracerebral hemorrhages. An emergent balloon venoplasty of the SSS successfully recanalized the thrombosed sinus. Further, multistage transarterial nidus embolization was performed followed by surgical resection, resulting in a complete eradication of the large AVM. The emergence of perilesional edema is a key radiological feature for the early recognition of a newly developed venous drainage route disturbance, which would result in hemorrhagic events. Venous sinus thrombosis is a rare cause of drainage route disturbance in cerebral AVMs. For such cases, the recanalization of venous drainage concomitant with flow reduction by performing transarterial embolization is effective in preventing further hemorrhage, which enables a safe performance of subsequent radical surgery.


Sign in / Sign up

Export Citation Format

Share Document