Risk factors for the development of secondary intracranial hypertension in acute cerebral venous thrombosis.

PURPOSE: Intracranial hypertension (IH) can complicate cerebral venous thrombosis (CVT), potentially causing permanent visual loss. Current knowledge on risk factors for the development of IH following CVT is scarce. We applied a compound classifier (CSF opening pressure > 25 cmH2O, papilledema, or optic disc protrusion on MRI) as a surrogate for IH and studied the predictive value of thrombus location, the number of thrombosed segments, and thrombus volume. METHODS: We prospectively included 26 patients with acute CVT and complete MRI data. IH was defined by CSF opening pressure > 25 cmH2O, papilledema, or optic disc protrusion on MRI. Using high-resolution contrast-enhanced venography, we determined the thrombus location, number of thrombosed segments, and thrombus volume. We analyzed their association with IH by logistic regression, their predictive power by the area under the receiver operating characteristic curve, and their association with CSF opening pressure by linear regression. RESULTS: IH occurred in 46% of CVT patients and was associated with higher thrombus volume (AUC 0.759, p = 0.025) and superior sagittal sinus thrombosis both alone (OR 2.086, p = 0.049) and combined with transverse sinus thrombosis (OR 2.014, p = 0.028). Effects in patients presenting CSF opening pressure > 25 cm H2O and the compound classifier were consistent. Thrombus volume > 4 ml was the single most important predictor of higher CSF opening pressure (ß = 0.566, p = 0.035), increasing IH risk. CONCLUSION: Larger thrombus volume, dominant transverse sinus occlusion, and extensive superior sagittal combined with transverse sinus thrombosis were associated with IH. Thrombus volumetry might identify patients at risk for IH and direct further clinical evaluation.

Pediatric otitic hydrocephalus: Report of two unusual cases and literature review.

Otitic hydrocephalus (OH) comprises a form of benign intracranial hypertension, which is secondary to otogenic lateral sinus thrombosis (LST). Only a small percentage of the patients with otogenic LST go into developing OH, and this may be associated with the multiplicity of anatomic variations of the cerebral venous drainage pathways. We present two pediatric cases of OH, along with a comprehensive review of the relevant literature. Both cases discussed in this article had concomitantly a rather rare anatomical variation; a high-riding, dehiscent jugular bulb, which might have played a role in the development of their clinical syndrome. The pediatric population with this particular imaging finding should receive special attention. Clinical implications of this concurrence are fitly discussed.

Headache attributed to cranial venous sinus stenting: A case series and literature review.

BACKGROUND: The diagnostic criteria for headache attributable to cranial venous sinus stenting were first formalized in the recently published third edition of the International Classification of Headache Disorders (ICHD-3). However, the diagnostic criteria for headache caused by cranial venous sinus stenting are based on very few data and the condition is poorly characterized. OBJECTIVES: To validate the diagnostic criteria for cranial venous sinus stenting headache by retrospectively studying the characteristics of headache in patients with isolated pulsatile tinnitus who underwent curative cranial venous sinus stenting and who had not previously complained of headache. PATIENTS AND METHODS: We retrospectively studied clinical, radiological, and manometric data from patients with isolated venous pulsatile tinnitus who had not previously reported headache. All patients underwent lateral sinus stenting in our institution between October 2010 and February 2018. RESULTS: Forty eight patients, 47 females and one male, were enrolled. The mean age at symptom onset was 36.2 ± 8.7 years and the mean body mass index was 24.0 ± 3.2 kg/m2. Lateral sinus stenosis was evident in 47 patients and a sigmoid diverticulum in one. Fourteen patients experienced headaches after recovering from general anesthesia. All were female, with a mean age of 35.5 ± 9.6 years. Headache persisted for less than 3 days in six patients (42.8%); for 3 days to 3 months in four (28.6%); and for longer than 3 months in four (28.6%). The headaches were located on the same sides as the cranial venous sinus stents in 13 patients (92.9%) and were principally occipital, being oppressive in nine patients (64.3%) and of moderate intensity in seven (50%). Age at onset of pulsatile tinnitus and body mass index were significantly associated with headache (p < 0.05; t-test). CONCLUSION: To the best of our knowledge, this is the first study to describe cranial venous sinus stenting headache in detail. We found that de novo headache developed after cranial venous sinus stenting, and was usually mild to moderate, unilateral, but oppressive; almost one-third of such headaches persisted for more than 3 months. Researchers and clinicians must become familiar with this headache spectrum; further prospective studies are required.

Multimodal Regional Brain Monitoring of Tissue Ischemia in Severe Cerebral Venous Sinus Thrombosis.

BACKGROUND: Comatose critically ill patients with severe diffuse cerebral venous thrombosis (CVT) are at high risk of secondary hypoxic/ischemic insults, which may considerably worsen neurological recovery. Multimodal brain monitoring (MBM) may therefore improve patient care in this setting, yet no data are available in the literature. METHODS: We report two patients with coma following severe diffuse CVT who underwent emergent invasive MBM with intracranial pressure (ICP), brain tissue oximetry (PbtO2), and cerebral microdialysis (CMD). Therapy of CVT consisted of intravenous unfractionated heparin (UFH), followed by endovascular mechanical thrombectomy (EMT). EMT efficacy was assessed continuously at the bedside using MBM. RESULTS: Despite effective therapeutic UFH (aPTT two times baseline levels in the two subjects), average CMD levels of lactate and glucose in the 6 h prior to EMT displayed evidence of regional brain ischemia. The EMT procedure was associated with a rapid (within 6 h) improvement in both CMD lactate (6.42 ± 0.61 4.89 ± 0.55 mmol/L, p = 0.02) and glucose (0.49 ± 0.17 vs. 0.96 ± 0.32 mmol/L, p = 0.0005). EMT was also associated with a significant increase in PbtO2 (22.9 ± 7.5 vs. 30.1 ± 3.6 mmHg, p = 0.0003) and a decrease in CMD glutamate (12.69 ± 1.06 vs. 5.73 ± 1.76 µmol/L, p = 0.017) and intracranial pressure (ICP) (13 ± 4 vs. 11 ± 4 mmHg (p = 004). Patients did not require surgical decompression, regained consciousness, and were discharged from the hospital with a good neurological outcome (modified Rankin score 3 and 4). CONCLUSIONS: This study illustrates the potential utility of continuous bedside MBM in patients with coma after severe brain injury, irrespective of the primary acute cerebral condition. Despite adequate ICP and PbtO2 control, the presence of CMD signs of regional brain cell ischemia triggered emergent EMT to treat CVT, which was associated with a significant and clinically relevant improvement of intracerebral physiology.

Acute otitis media associated with Gradenigo syndrome and transverse sinus thrombosis: a case report.

Gradenigo syndrome is associated with middle ear infection that extends to the petrous apex, leading to pain at the innervation site of the ophthalmic and maxillary branches of the trigeminal nerve and the development of abducens nerve palsy. Cerebral venous sinus thrombosis is a serious neurological complication of otitis media and occurs secondary to spread of the infection to the underlying bone. We herein report a pediatric case of otitis media associated with Gradenigo syndrome and ipsilateral sigmoid-transverse sinus thrombosis with magnetic resonance imaging findings.

Surgical Thrombectomy Combined with Bilateral Decompressive Craniectomy in a Life-Threatening Case of Coma from Cerebral Venous Sinus Thrombosis: Case Report and Literature Review.

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a rare cause of stroke. Medical and neurointerventional strategies, such as in situ thrombolysis, are standard therapies. These techniques are insufficient when clinical deterioration arises from mass-associated effects and transtentorial herniation; in such cases, decompressive hemicraniectomy may be indicated. To the best of our knowledge, the association of open surgical thrombectomy with bilateral decompressive craniectomy has not been reported to date. CASE DESCRIPTION: A 45-year-old woman presented with extensive cerebral venous sinus thrombosis that was resistant to anticoagulation and endovascular therapies. Her clinical condition deteriorated until she became comatose, and bilateral hemicraniectomy combined with open surgical thrombectomy through the superior sagittal sinus was indicated. Computed tomography angiography confirmed postoperative maintenance of sinus permeability. The patient's clinical status improved dramatically, and she had a favorable outcome, including recovery of her functional independence to perform all activities of daily living (modified Rankin Scale score = 0). The follow-up period was 5 years. CONCLUSIONS: Open surgical thrombectomy combined with decompressive craniectomy is a lifesaving procedure that can lead to favorable outcome and should be considered for treatment of refractory malignant cerebral venous sinus thrombosis.

Perimesencephalic nonaneurysmal subarachnoid hemorrhage caused by transverse sinus thrombosis: A case report and review of literature.

RATIONALE: Perimesencephalic nonaneurysmal subarachnoid hemorrhage (PNSAH) is characterized by a pattern of extravasated blood restricted to the perimesencephalic cisterns, normal angiographic findings, and an excellent prognosis with an uneventful course and low risks of complication. The precise etiology of bleeding in patients with PNSAH has not yet been established. The most common hypothesis is that PNSAH is venous in origin. Intracranial venous hypertension has been considered as the pivotal factor in the pathogenesis of PNSAH. The underlying venous pathology such as straight sinus stenosis, jugular vein occlusion may contribute to PNSAH. We describe a patient in whom transverse sinus thrombosis preceded intracranial venous hypertension and PNSAH. These findings supported that the source of the subarachnoid hemorrhage is venous in origin. PATIENT CONCERNS AND DIAGNOSES: A 45-year-old right-handed man was admitted to the hospital with a sudden onset of severe headache associated with nausea, vomiting, and mild photophobia for 6 hours. The patient was fully conscious and totally alert. An emergency brain computed tomography (CT) revealed an acute subarachnoid hemorrhage restricted to the perimesencephalic cisterns. CT angiography revealed no evidence of an intracranial aneurysm or underlying vascular malformation. Digital subtraction angiography of arterial and capillary phases confirmed the CT angiographic findings. Assessment of the venous phase demonstrated right transverse sinus thrombosis. Magnetic resonance imaging confirmed the diagnosis of cerebral venous sinus thrombosis (CVST). Lumbar puncture revealed an opening pressure of 360 mmH2O, suggestive of intracranial venous hypertension. Grave disease was diagnosed by endocrinological investigation. INTERVENTIONS: Low-molecular-weight heparin, followed by oral warfarin, was initiated immediately as the treatment for cerebral venous sinus thrombosis and PNSAH. OUTCOMES: The patient discharged without any neurologic defect after 3 weeks of hospital stay. MR venography revealed recanalization of right transverse sinus at the 6-month follow-up. No clinical or neuroimaging evidence of relapse was detected at 12 months follow-up. LESSONS: Hyperthyroidism may contribute to the development of CVST. The presence of acute transverse sinus thrombosis, as a cause of PNSAH, provides further support for the hypothesis that the source of PNSAH is venous in origin and intracranial venous hypertension plays a critical role in the pathogenesis of PNSAH.

Venous sinus stenting is a valuable treatment for fulminant idiopathic intracranial hypertension.

Over the past 10 years, transverse sinus stenting has grown in popularity as a treatment for idiopathic intracranial hypertension. Although promising results have been demonstrated in several reported series, the vast majority of patients in these series have been treated on an elective basis rather than in the setting of fulminant disease with acute visual deterioration. We identified four patients who presented with severe acute vision loss between 2008 and 2012 who were treated with urgent transverse sinus stenting with temporary cerebrospinal fluid (CSF) diversion with lumbar puncture or lumbar drain as a bridge to therapy. All patients presented with headache, and this was stable or had improved at last follow-up. Three patients had improvement in some or all visual parameters following stenting, whereas one patient who presented with severe acute vision loss and optic disc pallor progressed to blindness despite successful stenting. We hypothesize that she presented too late in the course of the disease for improvement to occur. Although the management of fulminant idiopathic intracranial hypertension remains challenging, we believe that transverse sinus stenting, in conjunction with temporary CSF diversion, represents a viable treatment option in the acute and appropriate setting.

Simultaneous appearance of cerebral venous thrombosis and subdural hematomas as rare cause of headache in puerperium following epidural analgesia: a case report.

The aim of this study is to report the first case of simultaneous appearance of cerebral venous thrombosis (CVT) and bilateral subdural hematomas (SDHs) following epidural analgesia for labor and delivery and to point out the difficulty of establishing such a diagnosis in the presence of postpartum headache. A 26-year old primigravida with a history of epilepsy received epidural analgesia for delivery. Three days after the uneventful spontaneous vaginal delivery she complained about the headache. Patient responded very well to the pain medication and oral hydration, and the headache was relieved. Ten days after the delivery, the headache reoccurred, and an epidural blood patch was performed that successfully relieved her symptom. Stronger progressive headache with nausea reappeared two days later and the parturient was readmitted to hospital. Urgent neuroimaging examinations detected CVT of right the transverse sinus, ipsilateral cortical veins, and partially occluded superior sagittal sinus, as well as bilateral subacute/chronic SDHs. The treatment of the patient with low molecular weight heparin and antiaggregation therapy was effective. In this case, the diagnosis was delayed because of atypical clinical presentation and potentially confounding events (epidural analgesia and assumption that it was a case of PDPH). It is important to carefully observe patients in such conditions and promptly conduct suitable diagnostic tests. Otherwise, unrecognized intracranial complications and delay of appropriate therapy could be life-threatening.

Pediatric otogenic lateral sinus thrombosis: role of anticoagulation and surgery.

OBJECTIVES: 1. To discuss the presentation, work up, and treatment of a series pediatric patients presenting with acute otogenic lateral sinus thrombosis (OLST). 2. To review the literature in discussing surgical treatment and the role of anticoagulation for sinus thrombosis from complicated otomastoiditis. STUDY DESIGN: Retrospective case series. METHODS: Retrospective case series of seven patients with otomastoidits and lateral sinus thrombosis were included in the study. Type of anticoagulation used and both clinical and radiographic outcomes were compared. Pediatric literature review was conducted using PubMed search terms "thrombosis and otitis media and anticoagulation" limited to English. RESULTS: Seven patients presented with acute otomastoiditis with sigmoid sinus thrombosis. Six patients were treated with anticoagulation for 1.5-12 months. Six patients underwent myringotomy with tube and 4 patients underwent cortical mastoidectomy without thrombectomy. Six patients had resolution of thrombosis by imaging in less than 6 months. Literature review of 19 pts with OLST showed that 95% had mastoidectomy and 84% had myrigotomy with tube. All 19 patients received anticoagulation. Sixteen patients had complete clinical recovery with recanalization or resolution of clot in 3 patients. CONCLUSIONS: The treatment of OLST is controversial. Most (23/26) patients had complete clinical recovery despite clot resolution in 9 of the 26 patients. Four patients had bleeding complication with anticoagulation. This series and literature review highlights the controversy of surgery and use of anticoagulation in the treatment of OLST and the need for further investigation.

Pediatric otogenic lateral sinus thrombosis recanalization.

OBJECTIVE: To describe the recovery outcomes in pediatric patients with otogenic lateral sinus and internal jugular vein thrombosis. METHODS: An inpatient database from a tertiary care pediatric hospital was queried from 1999 to 2010 for the diagnosis code [325] thrombosis of intracranial venous sinus. Demographics, extent of thrombosis, surgical intervention, use of anticoagulation, and the presence of recanalization on follow-up imaging was collected. RESULTS: Fifteen patients (10 male, 5 female) were identified with otogenic lateral sinus thrombosis. Eleven patients (73.3%) had evidence of thrombus in the transverse sinus, while 10 patients (66.7%) had thrombus in the internal jugular vein, and one patient (6.7%) had thrombus in the cavernous sinus. Five patients (33%) had otitic hydrocephalus. Twelve patients (80%) underwent operative manipulation of the lateral sinus including: three decompressions 20%, three needle aspirations 20%, and six venotomies with evacuation of clot or pus 40%. Twelve patients were anticoagulated with low molecular weight heparin, and three patients were not anticoagulated. Ten of fourteen patients (71.4%) who underwent follow-up magnetic resonance venography had evidence of partial (57.1%) or complete (14.3%) recanalization of the lateral sinus. All five patients with otitic hydrocephalus recovered as determined by a normal fundoscopic exam and recovery of abducens paresis. CONCLUSION: Recanalization of the lateral intracranial venous sinus occurred in the majority of the patients in this series. The role of operative intervention and/or anticoagulation remains unclear.

Cortical subarachnoid hemorrhage caused by cerebral venous thrombosis.

Patients with non-traumatic, non-aneurysmal, and non-perimesencephalic subarachnoid hemorrhage (SAH) tend to have clots circumscribed along the cortical convexity, a condition referred to as acute cortical SAH. Cerebral venous thrombosis (CVT) is a potential cause of cortical SAH. The study tried to establish the diagnosis and management of cortical SAH caused by CVT. Retrospective review of 145 patients with non-traumatic SAH identified 15 patients with no ruptured aneurysm. Clinical features were investigated with a specific focus on patients with SAH caused by CVT. Eight of the 15 patients had perimesencephalic SAH, and 7 had cortical SAH. SAH caused by CVT was diagnosed in 4 of the 7 patients with cortical SAH. The cortical SAH involved the unilateral convexity or sylvian cistern and spared the basal cistern on computed tomography in all 4 patients. CVT occurred in the transverse sinus and cortical vein (1 patient), insular vein (1 patient), and cortical vein (2 patients). Identification of thrombosed veins or sinuses was established directly by T(2)*-weighted and diffusion-weighted magnetic resonance (MR) imaging in the acute stage and diffusion-weighted and T(1)-weighted MR imaging in the subacute stage. All patients had cortical swelling without findings of venous hemorrhagic infarction on T(2)*-weighted MR imaging. None of the 4 patients received active treatment, and all had favorable outcomes. CVT in patients with non-traumatic cortical SAH should be first excluded as a potential hemorrhagic cause by MR imaging for thrombosed veins or sinuses before initiating antifibrinolytic therapy.

Otitic hydrocephalus: a report of 2 cases.

Otitic hydrocephalus is a rare intracranial complication of otitis media. It is characterized by elevated cerebrospinal fluid (CSF) pressure with normal CSF biochemistry and without any focal neurologic abnormality other than those related to the increased intracranial pressure. The pathophysiology of this rare condition is unknown. The incidence of intracranial complications was reduced dramatically with the introduction of newer antibiotics, but complicated cases still occur. Serious consequences can ensue if complications are not identified and treated properly. The author reviews the literature on otitic hydrocephalus and describes 2 new cases.

Post-partum transverse sinus thrombosis presenting as acute psychosis.

Whilst cerebral vascular disease and mental illness in the post-partum period are well recognised, their co-existence and the concept of organic psychoses in pregnancy, parturition and the puerperium remains poorly appreciated (Brockington 2006; Brockington Arch Women's Ment Health 10: 177-178, 2007a; Brockington Arch Women's Ment Health 10: 305-306, b). We report a woman who was referred to the Medical team on-call with a mixed presentation of euphoria, mutism and aggressive behaviour but ultimately demonstrated to have a transverse sinus thrombosis and recovered well with anti-coagulation. This serves an important reminder of the implications of a missed medical diagnosis in this high-risk and vulnerable group of patients.

Anticoagulation for cerebral venous thrombosis with subarachnoid hemorrhage: a case report.

OBJECTIVES: To report the success of anticoagulation (AC) treatment in a case of cerebral venous thrombosis (CVT) with subarachnoid hemorrhage (SAH) in view of the limited evidence seen in the literature supporting such a treatment option. CLINICAL PRESENTATION AND INTERVENTION: A 38-year-old lady with CVT and SAH presented 12 h after the onset of symptoms. AC with low-molecular-weight heparin was started 4 days later, when the repeated brain CT showed regression of the SAH. Heparin was changed to warfarin, and she was asymptomatic over a 12-month follow-up period. DISCUSSION: In a limited number of small studies, AC has been found to be beneficial for cases of CVT with hemorrhagic complications. The proper time to start AC in such cases was not clearly defined, and a delay of 4-33 days was observed after the onset of symptoms. In cases of spontaneous intracranial hemorrhage (ICH) in general, active bleeding is usually confined to the first 6 h, and chances of hematoma enlargement are higher in the first 24 h. On the other hand, it has been advised to rule out a coincidental vascular malformation and to radiologically confirm regression (or at least non-progression) of the ICH before starting AC. CONCLUSION: AC for cases of CVT may remain beneficial in the presence of SAH. The time to start AC for CVT with hemorrhagic complications is unclear; however, AC was successful when given 4 days after the onset of symptoms in our case. It may be wise to repeat CT after at least 24 h from the onset of symptoms (to confirm regression or at least non-progression of the ICH) before starting AC. It may also be prudent to perform magnetic resonance angiography, or digital subtraction angiography to rule out a coincidental intracranial aneurysm before AC.

Posterior vitreous contraction after cerebral sinus thrombosis.

The authors describe a 34-year-old woman who presented with headaches and marked papilledema after she had been taking oral hormonal therapy for the treatment of dysfunctional uterine bleeding. Neurological examination led to the diagnosis of a cerebral venous sinus thrombosis. After thrombolytic treatment, the headaches vanished and the papilledema in the right eye disappeared. Visual acuity increased to 20/25 in the right eye and 20/32 in the left eye. However, the marked papilledema in the left eye had resulted in a pronounced contraction of the posterior vitreous at the optic nerve head associated with a secondary folding of the macula and subretinal pigmentary irregularities in the foveal region.

Nonseptic lateral sinus thrombosis: the role of the otolaryngologist.

Nonseptic lateral sinus thrombosis differs from septic lateral sinus thrombosis in that it is not associated with ear or sinus infection. Mastoid changes are frequently detected on computed tomography or magnetic resonance imaging in cases of nonseptic lateral sinus thrombosis. An otolaryngologic evaluation is usually required to exclude coexisting mastoiditis.