Cerebral Venous Sinus Thrombosis in Adults with Prothrombotic Conditions: A Systematic Review and a Case from Our Institution

Abstract

Cerebral venous sinus thrombosis (CVST) is a rare condition characterized by elevated intracranial pressure due to impaired cerebral venous drainage, potentially leading to life-threatening consequences. We searched the PubMed electronic database for 'cerebral venous sinus thrombosis' and 'prothrombotic' cases reported in adults (19+ years) and conducted a systematic review for the published literature in the English language pooled with a case from our institution. Data were analyzed regarding patient demographics, risk factors, clinical features, treatment modalities, and outcomes when available.Thirty cases of CVST were identified (29 case reports, of whom two were described in a case series, and the one case from our institution). The patients' mean age was 39 years (range: 19 -65). The male: female ratio was 1.14:1. The majority (73.3%) had at least one preexisting risk factor, with prescription drug use being the most common risk factor (33.3%) shared among all patients. Most patients (83.3%) presented with at least two symptoms. The most common presenting symptoms were headache (70%), gastrointestinal disturbance (50%), and seizures (40%). Focal deficits (36.7%), vision disturbances (30%), and altered consciousness (20%) were the remaining presenting complaints. Twelve cases (40%) commented on papilledema, with 10 (83.3%) having papilledema present. Anticoagulation abnormalities were examined in 26 cases (86.7%), out of which four cases (15.4%) had isolated protein S (PS) deficiency, three cases (11.5%) had isolated antithrombin III (ATIII) deficiency, and one case (3.8%) had isolated protein C (PC) deficiency. The most common initial imaging modality (22 cases, 73.3%), and most commonly used overall (23 cases, 76.7%), was computed tomography (CT). Magnetic resonance imaging (MRI) was the second most common imaging modality for initial use (five cases, 16.7%), diagnosis or confirmation of CVST (eight cases, 26.7%), and overall (21 cases, 70%). Heparin treatment was involved in the treatment of 18 cases (60%), and warfarin treatment was used in 10 cases (33.3%). Heparin-warfarin combination treatment was utilized in eight cases (26.7%). Most patients survived (28 cases, 93.3%), while the two remaining patients died secondary to brain death from the CVST (6.7%).The findings from this study highlight the clinical characteristics of CVST. Therefore, this study aims to increase awareness of this rare entity. Physicians should maintain a high index of suspicion in order to diagnose patients presenting in the proper clinical context, given this case shares various forms of presentations with other common clinical conditions but requires long-term anticoagulation.

Clinical presentations, radiological characteristics, and biological risk factors of cerebral venous thrombosis at a University Hospital in Saudi Arabia

“…13 , 18 Underlying prothrombotic disorder was found in 33.3% of our patients, which is higher than mentioned in some studies (19.8%, 13 26.4%, 16 6.7% 26 ) but in agreement with a study by Ferro et al 14 (34%). 27 We did not find protein C deficiency in any of our patients. Bechet’s disease was found in 25% of cases by Daif et al, 4 9.4% by Duman et al 24 and in none by Kajtazi et al 19 It was found in 2 of our patients, which is similar to a finding by Algahtani et al 13 Infection as the etiology was less frequent than reported in another study from KSA.…”

Not Just Another Headache: Cerebral Venous Sinus Thrombosis in a Patient With Isolated Antithrombin III Deficiency

“…Therefore, the clinician must have a high suspicion for diagnosis if a patient presents with a headache and has one or more known risk factors for CVST [4,10]. This case illustrates how in those without typical or previously identified risk factors, a comprehensive investigation through a detailed history, physical examination including ophthalmoscopic examination, and pertinent laboratory studies under most recent guidelines are warranted [4,9,10].…”

“…The presentation of CVST is highly variable; however, it commonly presents with at least one of a few symptoms. The most common presenting symptoms include headache, focal neurological deficits, and seizures [4,5,9]. Headache is by far the most common, with 70% to 90% of patients presenting with this symptom [4,9,11].…”

“…Despite this, it has been inconsistently reported in patients diagnosed with CVST. One systematic review found that only 12 out of 30 case reports commented on papilledema and that papilledema was present in 10 out of those 12 cases [9]. Risk factors for CVST have also been described extensively, but may not be present in up to 15% of cases [4].…”

“…The most common presenting symptom, however, is an uncharacteristic headache which may mimic many other conditions [5,7,8]. In a systematic review, papilledema was found in more than 80% of patients in which direct ophthalmoscopic examination was performed [9]. Basic laboratory studies, including coagulation studies and screening for prothrombotic conditions, are recommended in the initial workup [7].…”