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Intractable Rare Dis Res. 2019 Aug;8(3):206-209. doi: 10.5582/irdr.2019.01071.

Torcular herophili and lateral sinus thrombosis: An atypical presentation of Lemièrre's syndrome.

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Venous thromboembolism Unit, Department of Internal Medicine, University General Hospital Gregorio Marañón, Madrid, Spain.
Department of Hematology, University General Hospital Gregorio Marañón, Madrid, Spain.
Gregorio Marañón Health Research Institute, Madrid, Spain.
Department of Radiology, University General Hospital Gregorio Marañón, Madrid, Spain.


Lemièrre's syndrome (LS) is an uncommon disease characterized by septic thrombophlebitis of the jugular vein in the context of otorhinolaryngologic infections. These patients are often young and the pharyngotonsillar infection is the most frequent primary focus, but other foci like acute otitis media or otomastoiditis have been described. Although the internal jugular vein is the most commonly affected site, a few case reports have been published with thrombosis of other veins, such as the facial vein or transverse sinus. We report the case of a 93-year-old woman with an atypical presentation of LS presenting with thrombophlebitis of the internal jugular vein, transverse sinuses and Herophili torcula after an acute otitis media complicated with acute otomastoiditis. Infectious cerebral venous thrombosis (CVT) is rare and accounts for 6-12% of the total in large adult series and is usually associated to otorhinolaryngologic infections. CVT is an atypical presentation of LS that can be potentially lethal, especially during the acute phase. For this reason, clinical suspicion and early treatment are vital to improve the prognosis of these patients. Although surgical treatment is recommended in cases of LS complicated with CVT, conservative management with antibiotics and anticoagulation lead to ad integrum restitutio without neurological sequelae in our case, suggesting that surgical treatment may not be necessary in all cases of LS complicated with CVT.


Lemièrre's syndrome; cerebral venous thrombosis; otomastoiditis; torcula herophili

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