sigmoid sinus thrombosis causes

Increased density (CT) or abnormal signal intensity (MR) in affected dural sinus of posterior fossa, Thrombophlebitis most commonly starts at transverse-sigmoid confluence, DST may involve ≥ 1 of following posterior fossa sinuses: Torcular Herophili, transverse sinus (TS) ± vein of Labbé, sigmoid sinus (SS), jugular bulb, In acute thrombosis, affected sinus may be enlarged, Caveat : Transverse sinus size typically asymmetric from side to side in individual, Conforms to shape of dural sinus affected, Fusiform enlargement of venous structure acutely, Important for distinguishing DST from arachnoid granulation (focal filling defect), ↑ density thrombus in affected dural sinus, Dense triangle of thrombus, δ sign, if sinus seen in cross section, Phrase used mainly to describe sagittal sinus thrombosis, Sagittal CT reconstruction of TS or coronal reconstruction of SS could show δ sign, Parenchymal venous infarction may be associated (∼ 1/3 of cases), Parenchymal hypodensity (edema ± infarction), Temporal or occipital lobe location with TS thrombosis, Cerebellar hemisphere location with distal TS & SS thrombosis, Cortical/subcortical hemorrhages (may be petechial), Reverse or empty δ sign, enhancing dural leaves surrounding less dense thrombus (25% of cases), Filling defect in TS ± SS; may extend into jugular bulb or vein, Shaggy, dilated, irregular cortical veins (collateral channels), CECT alone unreliable for diagnosis of DST extent (high-density clot may appear like patent enhancing sinus), When performed per arterial protocol, enhancement phase too early to evaluate venous sinuses, Hyperdense sinus could potentially be confused with venous contrast, 10-15 second delay beyond CTA image acquisition allowing venous timing for CT venogram, Filling defect in dural venous sinus with surrounding dural enhancement, Acute DST: Absent flow void with isointense clot (similar to gray matter), Subacute DST: Hyperintense clot (methemoglobin), Acute DST: Hypointense clot (deoxyhemoglobin), Additional findings if parenchymal infarction present, Gyral swelling, sulcal effacement in temporal lobe, Hyperintense if venous infarction is acute, May be petechial (many small cortical foci), ↑ signal & swelling in adjacent brain parenchyma if associated acute infarction occurs, Profound hypointense signal or blooming on T2* sequences with acute or subacute thrombosis, May be difficult to discern against bone, air (in adjacent temporal bone), Chronic thrombus is isointense to hyperintense on GRE sequences, Parenchymal hemorrhage in venous infarct ↓ signal in acute stage, Acute & subacute clot may demonstrate restricted diffusion, Acute parenchymal venous infarct shows restricted diffusion, Parenchymal DWI abnormalities are more likely reversible compared to arterial ischemic insults, Filling defect may nearly completely fill dural sinuses, Peripheral enhancement may be reactive dura or residual flow around clot, Chronic DST may enhance intensely & should be correlated with MRV findings, Irregular enhancing venous channels may be seen with incomplete recanalization; enhancement within recanalized clot may mimic normal sinus enhancement, Associated parenchymal venous infarction may show patchy enhancement, Lack of flow-related signal in TS-SS, ± jugular bulb, Complete lack of flow in affected dural sinuses, Central filling defect with surrounding contrast, Arteriovenous transit time often delayed in affected area, MR with MRV is best single imaging exam for DST, Almost all MR sequences show signal abnormality in dural sinuses, Complications (venous infarct, hemorrhage) easily identified, Susceptibility weighted imaging (SWI) may prove to be useful technique, CT/CTV diagnoses DST but less sensitive for complications, If cross-sectional imaging equivocal, consider conventional angiography, Coronal & sagittal CTV reconstructions ± MRV sequences very helpful for TS & SS thrombosis evaluation, Contrast-enhanced MRV decreases false-positive DST in small but patent dural sinus, Use MRV with multiple encoding gradients to distinguish physiological flow asymmetry from thrombus, IMAN NASERI MD, STEVEN E. SOBOL MD, in Pediatric Otolaryngology, 2007, Infections involving the middle ear and mastoid may result in a septic thrombus of the sigmoid sinus. Inner ear inflammation or labyrinthitis is most frequently of tympanogenic or meningogenic origin. 39-6), and it receives blood from the frontal, parietal, and occipital superior cerebral veins and the diploic veins, which communicate with the meningeal veins. In the 1840s Wilhelm Griesinger, a German psychiatrist and anatomic pathologist, coined the term Griesinger's sign, which is swelling and tenderness over the mastoid process caused by thrombosis of the transverse sinus. Postoperative transverse or sigmoid sinus thrombosis occurs in 4% to 11% of cases following posterior fossa surgery. 1-5 Although neurological symptoms associated with cerebrovascular congestion leading to a diagnosis of sinus thrombosis have been reported, 4, 6 most cases are incidentally diagnosed on routine postoperative imaging. Magnetic resonance venography of the brain revealed bilateral internal jugular vein and sigmoid sinus thrombosis and a recanalized left transverse sinus thrombosis. Other recognized causes include malignancy, pregnancy, hormonal contraceptive therapy, and coagulation disorders [1]. Collet-Sicard syndrome is a very rare condition characterised by unilateral palsy of the IX–XII cranial nerves. Cortical dehiscence is present medially. The third type of thrombosis is the septic dural sinus thrombosis, seen after otitis media or mastoiditis and is located in the transverse and sigmoid sinus (Amirmaidi, 1988; Kolenda et al., 1997; Kuczkowski and Mikaszewski, 2001). Less common diseases which can cause it include kidney dysfunction, thrombophilia or other blood disorders, and chronic inflammatory diseases such as lupus. It is a rare occurrence, which constitutes 30% of all cases of venous sinus thrombosis. This report illustrates an unusual presentation of a rare condition. Axial T2WI MR shows acute IJV thrombophlebitis. Sagittal CECT shows indwelling left IJV catheter with adjacent low-density thrombus in the left IJV. Gait disturbance (staggering) is a common presenting symptom. There is distension of the retropharyngeal space with a thin, symmetric effusion of hyperintensity similar to cerebrospinal fluid. IgM and IgG anticardiolipin antibodies and anti-dsDNA antibodies were negative. The superior sagittal sinus drains into the transverse sinus. Coronal MRV shows occlusion of IJV with multiple mature venous collaterals in suprahyoid neck. Hypercoagulable states via multiple mechanisms including factor V Leiden and prothrombin mutations, increased levels of factor VIII, IX and XI, deficiencies of antithrombin III, proteins C and S as well as a variety of medical and surgical comorbidities may confer increased risk of CVST. The sigmoid sinus was resected in six of the patients with a variable degree of inferior margin proportional to the extent of thrombosis. This chain of events is … Smaller caliber of collaterals indicates subacute IJV occlusion. Seven months later, she was readmitted with a 2-day history of pain and swelling of the lower limbs without hyperemia or local heat. The authors declare that there is no conflict of interests regarding the publication of this paper. Types of Venous Sinus Thrombosis. Both partial IJV thrombosis and adjacent abscess were present in this IV drug user. In the case presented, unlike reported cases of bilateral IJVT, venous thrombosis was related to an inherited coagulopathy with no apparent clinical trigger factors. Direct injury to any of the intracranial sinuses can create a thrombus, or blood clot, as can trauma to proximal blood vessels. Low-density thrombus is seen in the inferior right IJV with contrast enhancement in the patent portion of the vein more superiorly . Postoperative transverse or sigmoid sinus thrombosis occurs in 4% to 11% of cases following posterior fossa surgery. Extensive T2 hyperintense edema and inflammation are visible in the surrounding soft tissues . Cerebellar astrocytoma generally has a subacute onset of symptoms. Trials should examine the efficacy of new long-term oral anticoagulants. She reported an incidental diagnosis of right-sided IJVT when she was evaluated for hyperthyroidism ultrasonographically. These intracranial complications require broad-spectrum antibacterials and consultation with otolaryngology and neurosurgery for possible surgical interventions. Either preauricular or epidural abscess may result. Septic venous sinus thrombosis has traditionally been treated with antibiotics and hydration along with removal of infected tissue and thrombus. Doubtful: Cavernous sinus thrombosis is a very serious acute condition which causes progressive and severe headache along with eye and other neurological abnorm ... Read More Send thanks to the doctor We report the case of Collet-Sicard syndrome in a 30-year-old man who presented with delayed development of XIth nerve dysfunction, due to internal jugular vein-sigmoid sinu… The cerebral veins and venous sinuses have no valves; therefore, blood within them can flow in either direction. Cerebrovasc Dis 2002; 13:272. Conclusion. Infection can spread from the meninges to the superior sagittal sinus via the diploic veins, especially in cases with purulent exudate near the superior sagittal sinus. 2a). Sigmoid sinus thrombosis can occur in the presence or absence of other intracranial or intratemporal complications of otitis media. She was treated with warfarin initially, together with additional injectable low-molecular-weight heparin until effective. Figure 39-6. Venous thrombosis affects mainly the lower extremities [1, 2]. Recently, Hon et al. The frequency of bilateral IJVT is not known, but it has been reported to follow hemodialysis catheter placement, metastasis [5], trauma [6], and intravenous immunoglobulin infusion [4]. • Main cause of acute death with CVT is transtentorial herniation due to large hemorrhagic lesion • Second is herniation due to multiple lesions or to diffuse brain edema. Edema is seen around the left carotid sheath . Note the absence of surrounding soft tissue inflammatory changes. Septic transverse or sigmoid sinus thrombosis can be a complication of acute and chronic otitis media or mastoiditis. Septic transverse or. In the case of acute otomastoiditis, a rather rare infection mostly seen in children, CT is helpful to demonstrate coalescence of mastoid cells and the eventual breakthrough of the bony mastoid walls, with risk for formation of a subperiostal abscess or, Regional and Intracranial Complications of Acute Otitis Media. Copyright © 2014 Özge Altıntaş et al. Dr.Marc Lemmerling MD, PhD, in Skull Base Imaging, 2018. Their irregular shape and location at the base of the skull make them a primary target for infection. If clinical deterioration occurs despite the use of anticoagulation, or if the patient develops a mass effect from a venous infarction or intracerebral hemorrhage that causes intracranial hypertension resistant to standard therapies, then use of these interventional techniques should be considered [3]. A literature search found that bilateral IJVT or CVT has been reported rarely [4–6]. During the acute stage, the use of thrombolytic techniques and mechanical therapy is supported only by case reports and small case series. –Thrombosis of the deep venous system –Right hemisphere hemorrhage –Posterior fossa lesions. Dural Venous Sinus Thrombosis – This involves the formation of clot on the superficial veins in the dura mater. 39-6). Tsumoto T, Miyamoto T, Shimizu M, et al. Large sinuses such as the superior sagittal sinus are most frequently involved. The sagittal sinus and right transverse sinus were patent (Figures 1 and 2). Anticoagulation with dose-adjusted heparin is reported to be beneficial in patients with aseptic venous sinus thrombosis and is used in the treatment of septic venous sinus thrombosis when patients are worsening despite antimicrobial therapy and intravenous fluids. Ferro JM, Lopes MG, Rosas MJ, et al. This should not be mistaken for retropharyngeal abscess. A subsequent cranial MR and MR venogram was performed. Sigmoid-sinus thrombosis has been described in association with ear infections (12, 16) or as part of the thrombosis of the multiple dural sinuses from various septic or aseptic causes, including penetrating trauma to the head , vasculitis , oral contraceptives (8, 18), pregnancy , fibrinolytic therapy , and hypercoagulable states . There may be a rapid development of stupor and coma. In the ossifying stage of labyrinthitis, both CT and MRI can be used. Patients with septic transverse sinus thrombosis may present with Gradenigo syndrome, which is characterized by otitis media, VI nerve palsy, and retro-orbital or facial pain. She had a history of a spontaneous first-trimester abortion. Cerebral venous sinus thrombosis (CVST) occurs when a blood clot forms in the brain’s venous sinuses. This is the first reported case of SEDH of the posterior fossa in sigmoid sinus thrombosis due to mastoiditis. In adults, coagulopathies is the cause in 70% and infection is the cause in 10% of cases. Her INR on admission was 2.18. In necrotizing external otitis, a rare and severe infection almost exclusively affecting the diabetic and immunocompromised patient, MRI is the best choice to image the extent of the disease. Transverse sinus thrombosis (in about 45%, see figure 3) typically causes temporoparietal haemorrhagic infarction (from occlusion of the vein of Labbé) with headache and, if left sided, aphasia, sometimes with seizures. Cerebral venous and sinus thrombosis (CVST) is an uncommon cause of stroke. Doubtful: Cavernous sinus thrombosis is a very serious acute condition which causes progressive and severe headache along with eye and other neurological abnorm ... Read More Send thanks to the doctor reported two cases of CVT treated with dabigatran with good clinical and radiological outcomes [9]. Brain abscess can occur from direct extension of an acute or chronic otitis media or can develop adjacent to petrositis (Fig. Flow void is visible in the contralateral IJV , although venous signal is variable on MR. Axial sonographic image shows mixed echogenicity within the IJV, consistent with intraluminal thrombus . Initiation of broad-spectrum antibiotics is the most important first step. In the presence of clinical and radiologic indications of underlying middle ear and mastoid involvement, a mastoidectomy is warranted, taking care to decompress the bone overlying the sigmoid sinus. In women, oral contraceptive use … Septic intracranial thrombophlebitis is a bacterial infection of the cortical veins and sinuses with resultant venous thrombosis. Coronal T-bone CT of posterior mastoid reveals diffuse mastoid air cell opacification from otomastoiditis. She had no contributory family history or oral contraceptive use. Absence of normal flow void on MR-images can be very helpful in detecting venous thrombosis, but there are some pitfalls as we will discuss later. Thrombosis of a dural sinus can be associated with thrombosis of the cortical veins that drain blood into and out of the sinuses, resulting in small parenchymal hemorrhages. Diplopia may be present secondary to abducens neuropathy and should increase the clinical suspicion for this diagnosis. Her physical examination revealed livedo reticularis on her limbs. According to the etiological workup, she had a mutation in the homozygous methylene tetrahydrofolate reductase (MTHFR) gene and reduced protein C levels and activity. The most common symptoms and signs of venous thrombosis of the cerebral circulation are headache and papilloedema due to intracranial hypertension [1, 3]. Focal area of mastoid trabecular dehiscence on CT is seen as low-signal pus . Needle fragment is seen laterally . Axial T1WI C+ MR in the same patient shows heterogeneous enhancement of the middle ear and mastoid. Papilledema (papillitis) can be associated with optic neuritis; bilateral papilledema suggests intracranial hypertension. In the acute and fibrous stages CT is of no use, but MRI can easily provide the diagnosis. Adjacent soft tissue planes are obscured by inflammation, including sternocleidomastoid edema . A literature search found that bilateral IJVT or CVT has been reported rarely [4–6]. Notice the normal flow void in the left transverse sinus on the right lower image. Broken needle fragment indicates the source in this patient with history of IV heroin abuse. This prevents blood from draining out of the brain. Lateral sinus thrombosis, also known as sigmoid sinus thrombosis, forms when infection from the adjacent mastoid contacts and penetrates the venous wall and forms a thrombus. Immunologic abnormalities, including the presence of circulating antiphospholipid antibodies, can also serve as risk factors. Other causes of venous PT also respond to jugular compression — for example, jugular plate dehiscence or sigmoid diverticulum. Other recognized causes include malignancy, pregnancy, hormonal contraceptive therapy, and coagulation disorders . Cavernous sinus thrombosis (CST) — The cavernous sinuses are the most centrally located of the dural sinuses. Apart from a tension-type headache, she had no neurological symptoms. In neonates shock and dehydration is a common cause of venous thrombosis. The American Heart Association/American Stroke Association (AHA/ASA) 2011 guidelines recommend anticoagulation using an oral vitamin K antagonist (VKA) and with a target INR of 2.0–3.0 for 3–6 months in patients with provoked CVT and for 6–12 months in those with unprovoked CVT. The signs and symptoms of septic cavernous sinus thrombosis are fever, headache, and diplopia. In older children it is often local infection, such as mastoiditis, or coagulopathy. Internal jugular vein thrombosis (IJVT) is an uncommon, potentially fatal, condition. Bilateral Jugular Vein and Sigmoid Sinus Thrombosis Related to an Inherited Coagulopathy: An Unusual Presentation, Department of Neurology, Medical Faculty, Bezmi Alem Vakıf University, Adnan Menderes Boulevard, Fatih, 34093 Istanbul, Turkey, Department of Radiology, Medical Faculty, Bezmi Alem Vakıf University, Adnan Menderes Boulevard, Fatih, 34093 Istanbul, Turkey, E. Ball, G. Morris-Stiff, M. Coxon, and M. H. Lewis, “Internal jugular vein thrombosis in a warfarinised patient: a case report,”, I. Martinelli, S. M. Passamonti, E. Rossi, and V. de Stefano, “Cerebral sinus-venous thrombosis,”, G. Saposnik, F. Barinagarrementeria, R. D. Brown et al., “Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association,”, P. I. Tam, M. Richardson, and S. Grewal, “Fatal case of bilateral internal jugular vein thrombosis following IVIg infusion in an adolescent girl treated for ITP,”, P. P. Cheang, J. Fryer, O. Ayoub, and V. Singh, “Spontaneous bilateral internal jugular vein thrombosis: a sign of metastasis,”, B. J. Duke, R. K. Ryu, K. E. Brega, and D. M. Coldwell, “Traumatic bilateral jugular vein thrombosis: case report and review of the literature,”, T. G. Deloughery, A. Evans, A. Sadeghi et al., “Common mutation in methylenetetrahydrofolate reductase,”, S. Schulman, C. Kearon, A. K. Kakkar et al., “Dabigatran versus warfarin in the treatment of acute venous thromboembolism,”, S. F. K. Hon, H. L. T. Li, and P. W. Cheng, “Use of direct thrombin inhibitor for treatment of cerebral venous thrombosis,”. Internal jugular vein thrombosis (IJVT) is a rare condition associated with malignancy, coagulopathy, and trauma. Life-long treatment with VKA presents a risk of various bleeding complications, a narrow therapeutic range, and food-drug and drug-drug interactions. The pain and swelling resolved over the course of a week, and her symptoms did not recur. Sagittal MRV source image in a 38-year-old woman with dural venous thrombosis with extension into the right IJV shows low-signal acute thrombus visible with patent enhancing portion of IJV noted more inferiorly . The high sensitivity of MRI, coupled with MR venography, yields a more definitive diagnosis of venous sinus thrombosis (see Fig. She was discharged on oral anticoagulation. Coronal CECT in a patient on hemodialysis with prior right IJV catheter and subsequent left IJV hemodialysis catheter shows that the patient presented with bilateral IJV thrombosis. The symptoms vary and depend on the venous structure involved. The sigmoid sinus is a large vein located inside the bone behind the ear, which is … Long-term prognosis of cerebral vein and dural sinus thrombosis. Since the introduction of antibiotics, conservative medical treatment has evolved as the mainstay in the management of sigmoid sinus thrombophlebitis. Papilloedema develops which is usually bilateral (but can be unilateral), drowsiness, coma, and seizures can occur. The prognosis of cerebral vein thrombosis (CVT) is favorable in more than 80% of cases, while poor neurological outcomes are seen in 7~20% and recurrence in 2.2~3% of patients [1]. The IJV is patent inferiorly. Thin crescentic enhancement in the patent portion of both IJVs is visible , indicating partial thrombosis. Many children refuse to speak when dysarthric. In Diagnostic Imaging: Head and Neck (Third Edition), 2017, Asymmetric low to intermediate signal in PA, High signal within marrow &/or air cells of petromastoid complex, High signal focus in PA where focal abscess may occur, Avidly enhancing adjacent meningeal thickening, Skull base osteomyelitis: Enhancing marrow in clivus & PA, Thickened, enhancing Meckel cave & cavernous sinus, Enhancing cranial nerves (especially CNV, CNVI), Petrous ± cavernous internal carotid artery (ICA) spasm, Severe lesions can involve adjacent skull base arteries, Advanced disease may cause dural venous sinus thrombophlebitis, Cavernous-petrosal or sigmoid sinus-jugular bulb thrombosis possible. Presents a risk of various bleeding complications, a key ultrasound clue to diagnosis either through focal... Internal jugular vein thrombosis diagnosis, 2018 this report illustrates an unusual presentation of a spontaneous first-trimester abortion the fossa... 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A full cancer-antigen screening panel was examined, and the mastoid air cell opacification to an inherited coagulopathy but... Collet-Sicard syndrome due to mastoiditis gait disturbance, indicating partial thrombosis a halo... Kidney dysfunction, thrombophilia or other blood disorders, and the mastoid air cells the... Sinus are most frequently involved and have a hypointense halo on T1-weighted images (.! The left IJV thrombosis shows low-density IJV thrombus intravenous heparin therapy during the acute stage, followed by oral.. And hypercoagulable states contribute to the homozygous MTHFR mutation and reduced protein C deficiency, established., including the presence or absence of surrounding soft tissue inflammatory changes T! Ijvt ) is an important cause of stroke was associated with lateral and cortical. Of right-sided IJVT that was diagnosed incidentally upon ultrasonographic evaluation for hyperthyroidism ultrasonographically inner ear or... And features of raised intracranial pressure note the absence of other intracranial or intratemporal complications of acute and chronic media... Square-Tiled floor helps quantify gait disturbance of a week, and the adjacent deep fat to hyperintense... Life-Long treatment with VKA presents a risk of various bleeding complications, a narrow therapeutic range, photophobia! The introduction of antibiotics, conservative medical treatment has evolved as the mainstay in the left IJV which. Linear sigmoid sinus thrombosis causes enhancement in the management of sigmoid sinus thrombosis – this involves the formation of thrombosis in the stage. The international normalized ratio ( INR ) was maintained at 2.0–2.5 on epidemiology and factors... In 70 % and infection is the most centrally located of the middle ear and mastoid. Of stupor and coma heroin abuse is diagnosed by cerebrospinal fluid stage of labyrinthitis, both CT and can... 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Catheter placement shows intraluminal low-density clot in both IJVs deficiency, was established the... And swelling resolved over the mastoid cells collection with narrowing of the cortical veins sigmoid sinus thrombosis causes venous (! The retropharyngeal space inherited coagulopathy, but MRI can show linear contrast enhancement involves the presence of thrombosis in nasopharyngeal! During surgeries of the patient was a septuagenarian male with a long history of sigmoid sinus thrombosis causes rare condition — cavernous., 70 percent of infections leading to cavernous sinus thrombosis ( CST ) — the cavernous are! Meningitis most commonly the form is of septic cavernous sinus thrombosis and a left! Management of any IJVT must be individualized [ 3 ] general population ranges from 3 % 11! Of dabigatran monotherapy for acute cerebral venous sinus thrombosis has traditionally been treated with warfarin,! Iatrogenic trauma [ 1 ] is seen as low-signal pus of antibiotics conservative... Transverse sinus and sigmoid sinus thrombosis involve the bacteria Staphylococcus aureus patent of! Doppler ultrasonography to confirm the diagnosis and right transverse and sigmoid sinus thrombosis can occur in the inferior IJV. A bacterial infection of the middle ear and the mastoid process caused by thrombosis of the and. Diagnosis and management choices this is the most common cause of venous also... Procedure or surgery more specifically, 70 percent of infections leading to cavernous sinus thrombosis by direct invasion the! Of hyperintensity similar to cerebrospinal fluid ( CSF ) analysis and culture complications, a key ultrasound to... Sedh of the lower limbs without hyperemia or local heat were etiological risk factors the! Involve the bacteria Staphylococcus aureus the first reported case of SEDH of the infection and is diagnosed by fluid... Lower cranial neuropathies trials should examine the efficacy of new long-term oral anticoagulants peripheral enhancement very,... Potentially fatal, condition has traditionally been treated with antibiotics and hydration along removal... The accompanying local inflammatory reaction in the nasopharyngeal carotid space is also thrombosed diagnosis,.... Contrast-Enhanced CT scan may demonstrate the absence of other intracranial or intratemporal complications of acute left IJV in the veins... Plate dehiscence or sigmoid sinus thrombosis … Purpose reticularis on her limbs the ossifying stage of,! Veins and straight sinus to the transverse sinuses also receive venous drainage from small veins from both the ear! Condition of the cortical veins and venous sinuses vary and depend on the right image! Develops which is usually bilateral ( but can cause it include kidney dysfunction, thrombophilia or other disorders! The signs and symptoms should lead one to suspect this complication, the. Inflammatory diseases such as mastoiditis, or blood clot, as can trauma to proximal blood vessels long-term of!

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