Danny R. Rose, Jr., MD

Press CA, Lindsay A, Stence NV, Fenton LZ, Bernard TJ, and Mirsky DM. Cavernous Sinus Thrombosis in Children: Imaging Characteristics and Clinical Outcomes. Stroke. 2015
Cavernous sinus thrombosis (CST) is a rare, but potentially serious complication of a variety of conditions including meningitis, sinusitis, and acute otitis media. Given its proximity to the internal carotid artery (ICA), cerebrovascular sequelae are common in this setting and include carotid artery narrowing, vasospasm, embolic and hypoperfusion-related infarcts. The widespread use of antibiotics has likely reduced the incidence of CST, and because of its rarity, determining both the optimal treatment regimen and current morbidity and mortality is challenging. To shed some light on the natural history of CST in children, Press et al. conducted an analysis of a retrospective cohort with CST cared for at a single pediatric tertiary care center focusing on clinical presentation, risk factors, imaging, treatment, incidence of infarct, complications of treatment and outcome.

The study included ten patients identified between 2003-2014, ranging in age from 3-17 years. The most common presenting symptom was headache, which was found in seven of the patients. Fever and vomiting were the second most common presenting symptoms occurring in six. Streptococcus/staphylococcus species were found in the vast majority of cases, with S. anginosus being the most common pathogen. Despite a prothrombotic evaluation conducted for all of the patients, only one patient had a coagulation abnormality (heterozygous for a Factor V Leiden mutation).

Radiographically, expansion of the cavernous sinus and filling defects on contrasted studies were the most common findings. Additionally, subdural empyema and narrowing of the ICA with arterial wall enhancement was present in every case, the majority of which had narrowing of the ICA by greater than 50% by WASID criteria. This narrowing completely resolved in six of the patients, and only two had a residual stenosis greater than 50% on their last available imaging study. Thromboses of additional veins/venous sinuses was frequent, with thromboses of the superior ophthalmic vein, draining and tributary veins of the CS, internal jugular vein and sigmoid sinus also reported. These thromboses were usually associated with restricted diffusion. Multiple areas of parenchymal restricted diffusion suggesting embolic and/or hypoperfusive infarction was found in six of the patients, and one had a complete middle cerebral artery territory infarction. 

Medical management involved a combination of antibiotics, anticoagulation, anti-platelets, nimodipine and/or steroids. Antibiotics were used on all of the children, with appropriate broad-spectrum antibiotics and narrowing based on microbial sensitivities. Eight patients were treated with anticoagulation. Three of the patients developed new infarcts while anticoagulated and half of the anticoagulated patients developed bleeding complications. Bleeding was most often post-procedural and was not catastrophic, with one extracranial bleed and three <20mL intracerebral hemorrhages with minimal intraventricular extension. Nimodipine was used to treat presumed vasospasm in two patients who had developed infarctions while on anticoagulation, with one of the two developing further infarcts while on nimodipine. The majority of the patients were managed operatively with endoscopic sinus surgery, with a total of six having more extensive procedures that included ventriculostomy, orbital decompression, mastoidectomy, orbital exenteration and cerebral debridement.

Four patients became blind in the affected eye (one bilateral) due to either injury to the optic nerve or orbital exenteration. Seven patients were discharged with a mRS of ≤ 1, with a median mRS of 1 at the time of last follow up (range 0-3). One patient had care withdrawn due to stroke-related neurologic injury.

This cohort provides valuable insights into the current state of childhood CST diagnosis and management. Despite a high incidence of infarction and significant ICA narrowing, the majority of patients had a favorable outcome. This is likely due in part to current aggressive, multimodal CST treatment. It is also worth noting that the only death in this study was a patient who had a large infarction that was identified before initiation of anticoagulation or antiplatelet therapy. Although bleeding complications were relatively common, these did not appear to have adversely affected the outcome of the affected patients.

Infarction was a common finding in this study, and may be more common than previously assumed. The precise mechanism of cerebrovascular sequelae in CST remains unclear, but likely involves a combination of thrombotic, hemodynamic and inflammatory arteritic causes. Given this uncertainty and the lack of sufficient patients for comparative study, the optimal management for prevention of cerebrovascular complications in CST has yet to be determined. Despite this limitation, in comparison to the historically poor aftermath of CST, this study provides additional evidence that outcomes can be good with aggressive treatment. Additional multicenter studies will likely be needed to provide more clarity on the issue.