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The pathophysiology of benign intracranial hypertension-like presentation of cerebral venous thrombosis (a) arachnoid granulations mainly located in the superior sagittal and the transverse sinuses (b) thrombus occluding the arachnoid granulations and decreasing the cerebrospinal fluid reabsorption (c) formation of multiple collateral channels
Source publication
Visual impairment can complicate cerebral venous thrombosis (CVT). Here, we describe the various pathophysiological mechanisms and treatments available. A retrospective chart review of all patients treated for CVT in a large quaternary teaching hospital was done, and cases with visual impairment due to CVT were identified. The various mechanisms ca...
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Citations
... There is no clarity on what should be the best treatment strategy for saving the vision or the ideal timing for intervention. Often, the treatment may need to be tailored for a given patient [9] or a combination of interventions may be beneficial. Further research and studies are needed on this rather uncommon but dangerous complication of CVT. ...
... Visual evoked potentials were recorded in seven patients during admission, of which three had absent waveforms in both eyes (2,12,14, and 17), prolonged VEP latencies in both eyes in one patient (11), and prolonged in either eye in two patients (4,6). ...
... One patient had no headache at presentation (patient 18). Four patients had progressively worsened despite the best medical therapy and shunt surgery (3,4,15,18). Vision improved in eight patients after surgery. ...
... It can occur due to (1) optic nerve dysfunction secondary to raised ICT, (2) infarcts in the occipital cortex, (3) chronic CVT, leading to dural arterio-venous malformations, and (4) occipital infarcts causing uncal herniation and PCA infarcts. [4] The findings of elevated lumbar CSF pressure without pleocytosis and prolonged VEP in most of our cases indicate that optic nerve dysfunction due to raised ICT may be the leading cause of visual impairment in CVT patients. Chronic occlusion of cerebral venous sinuses (commonly superior sagittal and transverse sinus) causes congestion of cortical veins, which leads to raised ICT. ...
Background and Purpose
Cerebral venous thrombosis (CVT) presenting as vision loss is uncommon. Raised intracranial tension in CVT is proposed as one of the mechanisms (13.2%). There are still unknown underlying mechanisms to explain vision loss in CVT. The safety and outcome of the surgery (optic nerve sheath fenestration [ONSF] or theco-peritoneal shunt [TPS]) to reduce intracranial hypertension and prevent vision loss has not been studied.
Methods
A retrospective case record review of CVT patients with impending vision loss who underwent ONSF/TPS from 2007 to 2019 was performed from the stroke registry. All patients had formal neuro-ophthalmological evaluation and documentation of visual acuity, supplemented by visual field assessments by perimetry in a subset of patients. Safety and outcomes were assessed based on vision improvement and adverse effects after the surgery.
Results
Among approximately 1400 patients with CVT admitted in the stroke ward over 12 years, surgery for rescuing vision was done in 18. Among these, the males were 6, and the females were 12. The mean age of presentation was 24 (range 18–52 years). All of them had headaches and progressive blurring of vision with papilledema. The number of patients who underwent TPS was 13, ONSF was 1, and both were 4. In the TPS group (26 eyes), vision improved in 15 eyes (57.7%), remained status-quo in 8 eyes (30.7%), and worsened in 3 eyes (11.5%). Four patients underwent both surgeries; three eyes improved, two remained status quo, and three worsened. One patient underwent ONSF, and his vision remained status quo (no perception of light). Three patients (17.6%) of the TPS group had minor complications (low-pressure headache, subdural hygroma), and five (29.4%) had major complications like subdural hemorrhage, abdominal wound infection, and meningitis.
Conclusion and Implications
In patients with CVT, adequate vision monitoring is mandatory. Shunt surgeries (especially TPS) may help in stabilizing/improving vision in CVT patients with impending vision loss, despite adequate anti-edema measures (53.8% improved). Early diagnosis and precise decisions in referring for surgery are crucial.
... Apart from using systemic anticoagulants to attempt recanalization and drugs with carbonic anhydrase inhibitor activity to reduce the ICPs, surgical treatment for cerebral venous disease, including CVST and CVSS, also include (1) recanalization by local thrombolysis, stenting, or mechanical devices; (2) cerebrospinal fluid diversion procedures such as ventriculoperiotoneal shunting; and (3) specific treatment for conditions such as dural arteriovenous fistula (DAVF) occurring as a late complication (19)(20)(21). However, for visual impairment due to intracranial hypertension caused by CVT/CVSS, these methods do not provide rapid relief of optic nerve damage. ...
Objective
Visual impairment is the most common clinical feature of cerebral venous sinus occlusion or cerebral venous thrombosis-induced intracranial hypertension, which can result in optic atrophy, leading to irreversible vision loss, visual field defections, and finally, permanent blindness. Papilledema is a typical early pathophysiological alteration in visual impairment. Optic nerve sheath fenestration (ONSF) has become increasingly accepted as an option to prevent or halt progressive visual loss owing to its low risk and complications. The objective of this study is to review the latest research progress on ONSF for the treatment of visual impairment related to cerebral venous diseases.
Methods
Study were searched following PRISMA guidelines based on three electronic databases (Pubmed, Embase and Medline-Ovid). We used the following keywords and variations as keywords to identify studies: “optic nerve sheath fenestration, papilledema, cerebral venous diseases, cerebral venous stenosis, cerebral venous thrombosis, idiopathic intracranial hypertension”. The publication date of studies was restricted between 1,872.1.1 and 2,021.12.31. The application of ONSF in papilledema due to cerebral venous diseases is reviewed. Additionally, the common surgical approaches as well as advantages and disadvantages are also described graphically.
Results
With the improvement of specific details of the ONSF procedure and surgical instruments, complications of ONSF have reduced and its safety has been significantly improved, although the number of clinically investigated cases in the literature remains low.
Conclusion
We recommend that ONSF should be considered as an imperative alternative to reduce or delay the visual morbidity of cerebral venous diseases, although there is yet no consensus on the optimal surgical timing.
... Elevations in ONSD (3.0-5.9 mm) have been reported in patients with traumatic brain injury and idiopathic intracranial hypertension. An elevated ICP seems to be the common mechanism underpinning these conditions [7][8][9][10][11][12][13][14][15][16][17][18][19]. To our knowledge, this is the first study to evaluate the role of ONSD in patients with CVST. ...
... Comparison of measures of diagnostic accuracy[15][16][17]19] ...
Purpose:
To ascertain the role of optic nerve sheath diameter (ONSD) in the evaluation of cerebral venous sinus thrombosis (CVST).
Material and methods:
A retrospective study with convenient sampling was done during the period November 2018 to January 2020. Forty-one patients across all age groups with magnetic resonance venography (MRV) diagnosis of CVST and 82 gender-matched controls were included in the study. ONSD was measured on axial T2-weighted magnetic resonance images. CVST was identified on axial TOF-MRV including the stage, degree, and location. Intraparenchymal infarction and haemorrhage were also noted with their stage and size. The distribution of values for ONSD was tested for equality of variances using independent samples t-test. A cut-off value for ONSD was derived using ROC analysis.
Results:
The mean difference between ONSD of cases and controls was 0.84 mm (95% CI: 0.61-1.06) and was statistically significant at p < 0.001. Mean ONSD in cases was 5.33 ± 0.66, and in the control group it was 4.49 ± 0.31. The cut-off value for ONSD was taken as 4.57 mm, derived using the ROC analysis (AUC was 0.876 suggesting good diagnostic accuracy). ONSD showed a sensitivity of 87% and a negative predictive value of 89% in predicting CVST. However, we found no statistical significance between ONSD in patients with acute or chronic thrombosis or in those with neuroparenchymal complications.
Conclusions:
ONSD is a valid triage tool with high sensitivity and negative predictive value, and it can be used in the evaluation of CVST.
... Occipital arterial infarcts secondary to mass effect from the herniated large venous infarcts [83]. ...
Thromboembolic manifestations of the eye can vary from a trivial tributary retinal vein occlusion to a catastrophic cerebral venous sinus thrombosis. These conditions can be classified as pathologies directly affecting the eye or those causing secondary lesions due to systemic issues and can be managed accordingly. Also, recently the incidence of thrombotic phenomenon affecting multiple organs (with the eye being no exception) is estimated to be around 25% among patients hospitalized in the intensive care unit for COVID-19, even though anticoagulant treatment was administered prophylactically. In this chapter, the various pathophysiologies of the ocular thrombotic events are highlighted with a special focus on the COVID-19 induced thrombotic ocular complications. Ophthalmologists, sometimes being the first responder, have a vigilant role to play with a heightened awareness of these atypical extrapulmonary thrombotic ocular manifestations, which are not only vision-threatening; in certain instances, life-threatening too. This chapter summarizes the recent advances in ocular thrombotic diseases with focal points on the current recommendations in COVID-19 induced ocular thrombotic complications. The potential diagnostic and preventive actions such as the prophylactic role of anti-thrombotic therapy, baseline non-contrast chest computed tomography, as well as recommendations for patients with COVID-19 infection are discussed in detail.
... Visual disturbances may occur due to (1) raised ICP as a result of venous thrombosis (without venous infarcts, resulting in a benign intracranial hypertension-like presentation), (2) venous infarction involving the occipital cortex, (3) raised ICP following the development of a secondary dural arteriovenous (AV) fistula, and (4) arterial occipital infarcts due to posterior cerebral artery compression, secondary to herniation in large venous infarcts. 18 Visual disturbances were seen in two (16.66%) of our study subjects. ...
Stroke is a common neurological emergency. Almost 80% of strokes are due to arterial occlusion. Venous thrombosis comprises less than 1–2% of all strokes. Involvement of the deep cerebral venous system is still rare and accounts for about 10.9% of all cerebral venous thromboses (CVT). CVT diagnosis is often delayed or missed, because of its variable clinical manifestations. We retrospectively (2015–18) and prospectively (2018–20) reviewed all the cases of CVT in a tertiary care center in south India. Out of a total of 52 CVT cases, 12 were due to the involvement of deep cerebral venous system. Their clinical presentation, imaging characteristics, and outcomes were assessed. The most frequent presentation was headache followed by seizures. Hyperhomocysteinemia was the most common risk factor noted. Imaging characteristics were variable, and a high index of suspicion was required for early diagnosis. All patients had favorable outcome in our study, and except one, all were treated conservatively.
... The CVT has a wide range of clinical manifestations which may be similar to many neurological disorders. The symptoms vary from subacute headache with or without the signs of intracranial hypertension (e.g., nausea, vomiting, diplopia, and blurred vision) to focal sensory and motor deficits, aphasia, visual impairment, seizures, behavioral disorders, loss of consciousness, and even coma in case of bilateral deep vein thrombosis occurring in approximately 16% of the patients or significant hemorrhagic infarcts (28)(29)(30)(31)(32)(33)(34)(35). ...
... The diagnosis is typically based on clinical suspicion and confirmatory neuroimaging. Formerly, the reports of thrombosis were made using angiography; however, today CTV and MRV can be used as reliable alternatives to confirm the diagnosis (11,35,37,38). ...
Background and Aim: Cerebral venous thrombosis (CVT) is a relatively rare disorder of the cerebral venous system accounting for less than 1% of all strokes with a higher prevalence in Iran. Despite the considerable advances in recent years, the diagnosis and treatment of CVT is still challenging predominantly in the emergency setting. Therefore, the present study sought to provide an update on the diagnosis and treatment of CVT.
Materials and Methods: PubMed, Web of Science, Google Scholar, Scopus, Elsevier, and Iranian domestic scientific databases were searched within January 1, 2000, to July 1, 2020. In all the electronic databases, the keywords (in the title/abstract) of “cerebral vein thrombosis” OR “CVT” OR “Cerebral venous sinus thrombosis” OR “CVST” AND “risk factor”, “diagnosis”, “treatment”, and “prognosis” were searched. The unrelated articles, studies not concerning humans or pediatric population, and case reports were excluded from the study. In addition, the articles related to CVT in association with coronavirus disease 2019 were excluded.
Results: According to the findings of the present study, CVT can present with a multitude of signs and symptoms, making it difficult to distinguish from other neurological conditions. While the diagnosis is typically based on brain magnetic resonance imaging and magnetic resonance venography, there is evidence suggesting that the plain computed tomography markers with an attenuation value of > 60.4 Hounsfield unit (HU) and Hounsfield-hematocrit (H: H) ratio of > 1.42 are specific enough to make a definite diagnosis. In terms of therapeutic aspects, although warfarin is still the mainstay of treatment, there is increasing interest toward new oral anticoagulants with promising results in both thrombus recanalization and excellent functional recovery. Balloon angioplasty and mechanical thrombectomy might also be considered in severely progressive cases without response to conventional treatments. Even for those achieving favorable outcomes, CVT can remain a disabling condition leading to at least neuropsychiatric complaints.
Conclusion: CVT is a rare and important cause of stroke accounting for less than 1% of all strokes mainly affecting young females. With regard to the rarity of the disease and novelty of the therapeutic approaches, there is a long way to fully identify the best diagnostic and therapeutic approaches to the disease
... Cerebral venous sinus thrombosis (CVST) has received an increasing attention due to its poor clinical consequences [1]. Visual damage is one of the most common and severe complication of CVST-associated intracranial hypertension (IH), and treatment delay may result in irreversible visual impairment and even blindness, which can seriously affect the quality of life in patients with CVST [2]. ...
Background:
Visual damage is one of the most common complications of cerebral venous sinus thrombosis (CVST)-associated intracranial hypertension (IH). This study is aimed at stratifying the risk of IH-induced visual damage in an attempt to predict its deterioration and prevent high-risk patients from irreversible eyesight impairment promptly.
Methods:
A total of 94 patients with confirmed diagnosis of CVST were eligible for enrollment in this study. According to cerebrospinal fluid pressure at admission, the involved patients were classified into mild IH (< 250 mmH2O), moderate IH (250-330 mmH2O), and severe IH (≥330 mmH2O) groups.
Results:
The ratio of visual deterioration in the severe IH group was 75%, which was significantly higher than in either the moderate (44.4%) or the mild groups (14.3%). As regards subjects without visual symptoms at admission, visual deterioration occurred in 9.4 ± 4.5 days after admission in the severe group while it occurred in 30.5 ± 16.8 days in the moderate group (p = 0.024). The conditional inference tree and random forest revealed that severe IH might be considered as an index of visual deterioration. Visual field defect, fading eyesight, and papilledema were significantly worse in patients with severe IH as compared to patients with mild or moderate IH, all p < 0.01.
Conclusions:
IH ≥330 mmH2O may be a cut-off value to predict the deterioration of visual damage in CVST, revealing that ophthalmologic interventions should be considered in a timely manner in this condition, particularly when recanalization of cerebral venous sinus cannot be achieved within a short time.
Cerebral venous sinus thrombosis is a rare disorder characterized by thrombosis in the venous channels of the brain. Nonspecific symptoms such as headache, focal neurologic deficits and seizures make this condition difficult to diagnose. We describe a case of cerebral venous thrombosis in the sigmoid and transverse sinuses which presented as reversible, rapid-onset proptosis and loss of vision in a patient of subclinical hypothyroidism. Such an occurrence of proptosis due to thrombosis of the sigmoid and transverse sinuses has never been reported in literature. The diagnosis of cerebral venous thrombosis in this patient was arrived at in a stepwise fashion. Thus, this case in addition to reporting a novel finding, stresses by example, the importance of acute suspicion on part of the physician for timely diagnosis and treatment of this eluding yet reversible disease.
Background:
Recent studies have noted concern for increased thromboembolic events in the setting of Coronavirus Disease 2019 (COVID-19). Cerebral venous sinus thrombosis (CVST) is a form of thromboembolism that has been observed as a neuro-ophthalmologic complication of COVID-19.
Methods:
Review of the scientific literature.
Results:
In this article, we report an overview of CVST epidemiology, clinical presentation, diagnostics, disease pathophysiology, and management in the setting of COVID-19.
Conclusion:
CVST is an uncommon thromboembolic event with variable phenotypes and multiple etiologies. Neurologic complications can be severe, including significant visual deficits and death. Current observations suggest that the risk of CVST may be profoundly impacted by this novel COVID-19 pandemic, thus prompting increased attention to disease presentation, pathogenesis, and management.
