Version October 2024
INTRODUCTION — Septic lateral sinus thrombosis most commonly occurs as a complication of acute or chronic otitis media. It is now uncommon, except in resource-limited countries [1].
The lateral sinuses, also called transverse sinuses, are a pair of venous sinuses within the dura of the brain that course laterally along the interior surface of the occipital bone. They drain into the sigmoid sinuses which ultimately drain into the internal jugular vein. When thrombosis occurs, headache, increased intracranial pressure, and neurologic symptoms may ensue [2-4].
This topic discusses septic lateral sinus thrombosis. Septic dural venous sinus thrombosis affecting other sites and aseptic dural venous sinus thrombosis are discussed separately:
●(See "Septic cavernous sinus thrombosis".)
●(See "Cerebral venous thrombosis: Etiology, clinical features, and diagnosis".)
●(See "Cerebral venous thrombosis: Treatment and prognosis".)
PATHOGENESIS — Lateral sinus thrombosis most often occurs as a complication of acute or chronic otitis media. If otitis media is not treated appropriately in a timely manner, the infection can spread to the mastoid and ultimately to the epidural space. Mastoid air cell infection can lead to thrombosis of the lateral sinus, which may then propagate to the internal jugular vein and other dural venous sinuses [3,5,6]. (See "Acute otitis media in children: Epidemiology, microbiology, and complications", section on 'Complications and sequelae'.)
In rare cases, lateral sinus thrombosis may occur in the setting of septic thrombosis of the internal jugular vein (Lemièrre's syndrome), presumably as a result of retrograde extension [7]. (See "Lemierre syndrome: Septic thrombophlebitis of the internal jugular vein".)
Interruption of venous circulation may lead to increased intracranial pressure [6].
Septic pulmonary embolism may also occur as a complication of lateral sinus thrombosis [8-10].
In children, hypercoagulable conditions appear to be a risk factor. Although the magnitude of this risk has not been quantified systematically, in one series of septic dural venous sinus thrombosis in the setting of otitis media, more than 70 percent had a prothrombotic condition [11].
MICROBIOLOGY — Infection is often polymicrobial, containing both aerobes and anaerobes. The principal pathogens associated with this complication reflect the bacteriology of chronic otitis media.
Traditionally, Proteus spp have been most common, followed by S. aureus, Escherichia coli, and anaerobes (Bacteroides fragilis and anaerobic streptococci) [3]; however, more recent case series suggest that streptococcal infections are increasing in incidence [12]. The anaerobic pathogen Fusobacteriaum necrophorum is associated with a higher incidence of lateral sinus thrombosis that other pathogens associated with masotiditis [13]. Pseudomonas has been identified as a pathogen in intraoperative cultures in a few cases; however, when Pseudomonas is cultured from the external ear canal, it cannot be assumed that this is the infecting organism [5,14].
CLINICAL MANIFESTATIONS — Septic lateral sinus thrombosis has a subacute onset. Symptoms generally begin several weeks before presentation and reflect the underlying infection as well as the development of septic lateral sinus thrombosis.
Symptoms — Symptoms include [3]:
●Earache is generally the first symptom, persisting for several weeks prior to the onset of headache.
●Headache is generally severe, persistent, and localized to the side of the ear infection. Pain is thought to be a manifestation of a developing epidural abscess, irritation of the fifth cranial nerve, or thrombosis of the lateral sinus. (See "Intracranial epidural abscess".)
●Nausea and vomiting develop in nearly half of cases and can be mistaken for severe gastroenteritis. This manifestation may reflect irritation of brainstem nuclei or cranial nerves, or elevated cerebrospinal fluid (CSF) pressure.
●Other symptoms include vertigo, diplopia, photophobia, and neck pain and stiffness.
●Less commonly, patients can present with headache and neurologic manifestations in the absence of ear pain and have radiologic findings consistent with chronic otitis media that may be accompanied by a cholesteatoma [15].
●Some patients develop cough productive of bloody sputum that results from septic pulmonary emboli, an uncommon complication of lateral sinus thrombosis [10].
Physical findings
●Fever is present in 80 percent of cases, and patients often appear ill [3]. High-spiking "picket" fence fevers are considered characteristic [6]; however, fever may be less prominent in those associated with chronic otitis media [12].
●An abnormal ear examination is found in most patients; 40 percent have a ruptured tympanic membrane and 20 percent have a dull, erythematous tympanic membrane [3].
Posterior auricular swelling is found in over half of the cases and is caused by occlusion of the mastoid emissary veins. This finding is called the Griesinger sign and is considered pathognomonic for septic lateral sinus thrombosis [6].
●Bilateral papilledema (the result of elevated CSF pressure) is also present in half of the cases and may be associated with retinal hemorrhage [3]. Accompanying loss of visual acuity develops in 15 percent.
●Unilateral sixth nerve dysfunction is reported in over a third of cases and is thought to be caused by compression of the nerve by swelling of the inferior petrosal sinus within the closed space of Dorello canal [3,12].
●Otitis media, sixth nerve paralysis, and fifth cranial nerve irritation (temporoparietal and retroorbital pain) are known as Gradenigo syndrome. This is rare, but when present, this symptom complex provides strong evidence for lateral sinus thrombosis or inflammation of the petrous ridge of the temporal bone [16-18].
●Nuchal rigidity is present in one-third to one-half of patients and is probably the result of meningeal inflammation [3,12].
●Depression in mental status is noted in 14 percent of cases and may be more common in older adults.
Imaging findings — Mastoid radiographs are abnormal in all patients with septic lateral sinus thrombosis, showing increased density with loss of mastoid trabeculae, bony sclerosis, and lytic lesions of the temporal and parietal bones.
Otomastoid infection can be confirmed on either computed tomography (CT) or enhanced magnetic resonance imaging (MRI). Contrast-enhanced CT or MRI with venography (CTV or MRV) detect filling defects from thrombi and absent flow in the dural venous sinus, respectively, in patients with septic lateral sinus thrombosis (image 1). Three-dimensional magnetic resonance gradient-echo cerebral venography has been shown to be superior to two-dimensional magnetic resonance [19].
Chest radiography rarely reveals rounded densities. These lesions are caused by septic emboli passing from the lateral sinus into the internal jugular vein and then passing into the pulmonary venous circulation.
EVALUATION AND DIAGNOSIS — The diagnosis of septic lateral sinus thrombosis should be suspected in a patient with otitis media who develops headache and neurologic signs such as vertigo and diplopia. Either contrast-enhanced MR with MRV or contrast-enhanced CT with CTV should be performed and will confirm the diagnosis, differentiating this from other complications including mastoiditis and abscess formation. (See "Chronic otitis media and cholesteatoma in adults", section on 'Mastoiditis'.)
Lumbar puncture and opening pressure measurement should be performed in all cases after MRI or CT has excluded the possibility of brain abscess. CSF pressures are commonly elevated to the range of 450 to 500 mm H2O in 75 percent of cases. Increased CSF pressure results from interference with CSF resorption by arachnoid villi draining into the superior sagittal sinus. The CSF analysis is normal in two-thirds of cases and reflects parameningeal inflammation in the other third. CSF should be sent for Gram stain and culture.
Two sets of blood cultures should be sent prior to initiation of antibiotics.
We do not routinely evaluate adult patients for hypercoagulable conditions, particularly in the typical setting in which there is a documented otitis which explains the occurrence of thrombosis. However, case series in pediatric patients suggest that a high percentage (33 to 96 percent) have a hypercoagulable condition when tested [15,20,21].
TREATMENT — The mainstays of treatment of septic lateral sinus thrombosis are antibiotics and, in the absence of rapid defervescence, surgery.
Antibiotics — Intravenous (IV) antibiotics need to be administered immediately [3,22,23]. The regimens listed below are for patients with normal renal function. Dose adjustments for patients with reduced kidney function can be found in the Lexicomp drug information topics within UpToDate.
An appropriate empiric parenteral regimen includes:
●Ceftriaxone 2 g IV every 12 hours OR Cefepime 2 g IV every 8 to 12 hours
AND
●Metronidazole 500 mg IV every six to eight hours
Cefepime (2 g IV every eight hours) plus IV metronidazole should be used if Pseudomonas is suspected (eg, patients with chronic otitis media and known colonization with Pseudomonas).
Meropenem (2 g IV every eight hours) is an acceptable alternative to one of the above regimens for most patients and is also active against Pseudomonas. If meropenem is not available, imipenem (1 g IV every six hours) can be used, but if both agents are available, we prefer meropenem since imipenem may increase the risk of seizures. Patients with severe beta-lactam allergies (eg, Stevens Johnson syndrome) may require a different regimen. (See "Penicillin allergy: Delayed hypersensitivity reactions" and "Allergy evaluation for immediate penicillin allergy: Skin test-based diagnostic strategies and cross-reactivity with other beta-lactam antibiotics" and "Immediate cephalosporin hypersensitivity: Allergy evaluation, skin testing, and cross-reactivity with other beta-lactam antibiotics" and "Cephalosporin hypersensitivity: Clinical manifestations and diagnosis".)
Methicillin-resistant S. aureus (MRSA) is rare in patients with septic lateral sinus thrombosis; however, the empiric addition of vancomycin (table 1) is typically warranted in those who have had MRSA detected in prior cultures of the mastoid and in those who are acutely ill. (See "Vancomycin: Parenteral dosing, monitoring, and adverse effects in adults".)
If an organism is identified based upon cultures from myringotomy or intraoperative specimens, therapy should be changed to target the identified organisms based upon their antibiotic sensitivities.
Three to four weeks of antibiotics are generally recommended, as mastoiditis is the most common primary infection; if the infection has been resected, a shorter duration of 10 to 14 days may be sufficient. We typically document resolution of the thrombosis with imaging.
Surgery — Patients diagnosed with lateral sinus thrombosis should have an urgent surgical consult [12,24,25]. Radical mastoidectomy should be strongly considered in all patients who fail to defervesce within 12 to 24 hours of the initiation of antibiotic treatment. The basis for this recommendation is the understanding that mortality rates were substantial prior to the institution of this practice. In a meta-analysis of observational studies including children with complicated mastoiditis, overall complication rates were improved in surgically treated compared with medically managed patients [26].
During surgery, the area overlying the lateral sinus should be explored so that infected granulation tissue or purulent collections can be drained and sent for culture. Removal of thrombus from the lateral sinus is considered in some cases; however, recanalization or the development of collateral venous drainage can occur without this intervention once the infection has been controlled [12,27,28]. Ligation of the jugular vein, a common procedure in the preantibiotic era, is not recommended in most cases [12].
Not all cases of lateral sinus thrombosis require mastoidectomy with drainage; in some cases, expeditious myringotomy may be appropriate and sufficient. In one pediatric series, three of five patients responded quickly to antibiotic therapy and myringotomy for the underlying otitis, fully recovering without more aggressive surgical intervention [29]. Other patients may recover without surgical intervention; however, in the absence of prompt defervescence, surgical intervention is recommended.
Anticoagulation — Anticoagulation is NOT part of the routine management of septic lateral sinus thrombosis.
Potential indications for anticoagulation include:
●Failure to improve clinically with extension of thrombosis despite antibiotics and surgical drainage
●Patients with a documented hypercoagulable state
The role of anticoagulation in septic lateral sinus thrombosis remains controversial. Although there may be a theoretic benefit, the stimulus precipitating thrombosis in septic cases is self-limited, resolving with antibiotic treatment, and thrombotic vessels often undergo recanalization without anticoagulation [20].
Also, anticoagulation carries a potential risk of intracerebral hemorrhage. One case series describes a patient with septic lateral sinus thrombosis and cortical vein thrombosis who suffered a fatal hemorrhage while receiving warfarin [3]. Hemorrhagic complications have been reported in at least three patients who were receiving low-molecular-weight heparin for septic lateral sinus thrombosis [10,30]. In contrast, in an another case series, no hemorrhagic complications were observed in 11 pediatric patients with sinus- or otogenic-associated septic cerebral venous sinus thrombosis who received anticoagulation [31].
If anticoagulation is started, the duration is unclear. Neuroimaging can be used to document persistence or resolution of the thrombosis to guide treatment decisions. If a hypercoagulable state is documented, anticoagulation should be continued for at least six months [15]. Prolonged anticoagulation should also be maintained for pediatric patients whose lateral sinus fails to recanalize [15]. For others, anticoagulation can be continued until the infection as well as the symptoms and signs of lateral sinus thrombosis have resolved or significantly improved. (See "Cerebral venous thrombosis: Treatment and prognosis", section on 'Long-term anticoagulation'.)
Treatment of elevated intracranial pressure — For patients with elevated intracranial pressure, serial lumbar punctures every 48 hours and/or ventricular drain may be required to lower the CSF pressure and relieve papilledema. Persistent communicating hydrocephalus may require placement of a ventricular shunt. In one series of 11 children, 45 percent required shunt placement [32]. Follow-up imaging and intervention should be guided by the patient's clinical examination, especially their level of consciousness. (See "Evaluation and management of elevated intracranial pressure in adults".)
OUTCOME — The outcome is usually favorable in this disease, with a reported mortality of less than 1 percent in the past decade [3,33]. The majority of patients fully recover, although 10 to 15 percent suffer chronic sequelae including hydrocephalus, decreased visual acuity, impaired hearing, and, rarely, residual hemiparesis [25].
SUMMARY AND RECOMMENDATIONS
●Pathogenesis - Septic lateral sinus thrombosis most often occurs as a complication of acute or chronic otitis media. Infection is often polymicrobial, containing both aerobes and anaerobes, reflecting the bacteriology of chronic otitis media. (See 'Pathogenesis' above and 'Microbiology' above.)
●Clinical features - Along with symptoms of ear infection and mastoiditis, patients develop headache along with neurologic signs such as vertigo and fifth and sixth nerve impairment. Some patients develop increased intracranial pressure. (See 'Clinical manifestations' above.)
●Diagnosis - The diagnosis of septic lateral sinus thrombosis is made by contrast-enhanced MRI. Other essential tests include lumbar puncture and blood cultures. (See 'Evaluation and diagnosis' above.)
●Treatment - Treatment consists of antibiotic therapy and surgery in most cases; some patients require management of elevated intracranial pressure:
•Urgent empiric antibiotic therapy is required. For most patients, we suggest a regimen that includes metronidazole plus either ceftriaxone or cefepime (Grade 2C). Meropenem is an acceptable alternative. (See 'Antibiotics' above.)
•In addition, we recommend surgical treatment unless the patient is responding rapidly to antibiotic treatment within 12 to 24 hours (Grade 1C). (See 'Surgery' above.)
•We suggest against anticoagulation in most patients with lateral sinus thrombosis, unless a hypercoagulable state is documented (Grade 2C). However, in patients not responding to antibiotic and surgical treatment, it is reasonable to provide anticoagulation treatment along with monitoring for hemorrhagic complications of anticoagulation. (See 'Anticoagulation' above.)
•For patients with increased intracranial pressure, monitoring and interventions are guided by the clinical examination. (See "Evaluation and management of elevated intracranial pressure in adults".)
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