What are the risk factors for Cerebral Sinus Venous Thrombosis (CSVT)?

Risk Factors for Cerebral Sinus Venous Thrombosis (CSVT)

CSVT risk factors are predominantly prothrombotic conditions (both inherited and acquired), female-specific hormonal factors, and local precipitants, with the majority of patients having identifiable risk factors that are often multifactorial. 1, 2

Prothrombotic Conditions

Inherited Thrombophilias

• Protein C deficiency carries a combined odds ratio of 11.1 for CSVT and represents one of the most significant inherited risk factors. 1
• Protein S deficiency and antithrombin III deficiency are additional significant inherited thrombophilias associated with CSVT. 1, 2
• Factor V Leiden mutation and prothrombin G20210A mutation increase CSVT risk, particularly in children and young adults. 1, 3
• Hyperhomocysteinemia is another important prothrombotic risk factor for CSVT. 1, 3

Acquired Prothrombotic States

• Antiphospholipid antibodies represent an acquired thrombophilic condition associated with CSVT. 3
• Nephrotic syndrome can create an acquired prothrombotic state predisposing to CSVT. 4
• Myeloproliferative neoplasms may present initially as CSVT in a small proportion of patients. 3

Female-Specific Hormonal Risk Factors

• Oral contraceptive use is one of the most common risk factors for CSVT, contributing to the female predominance of this condition. 2, 4, 3
• Pregnancy and the postpartum period (puerperium) are major risk factors, making CSVT particularly relevant in reproductive-age women. 2, 4, 3
• Hormone replacement therapy increases CSVT risk. 4

Local and Mechanical Precipitants

• Head trauma can precipitate CSVT through local vessel wall injury. 4, 5
• Jugular catheterization and central venous catheters represent iatrogenic mechanical risk factors. 4, 6
• Neurosurgical procedures and lumbar puncture can serve as mechanical precipitants. 4
• Brain tumors and cerebral infections are local risk factors that directly affect venous structures. 3

Infectious and Inflammatory Conditions

• Local and generalized infections are common acquired risk factors, particularly in pediatric populations where infections represent one of the most frequent precipitants. 4, 6, 5
• Vasculitis and inflammatory bowel disease can predispose to CSVT. 4
• In neonates specifically, sepsis is an age-specific risk factor. 6

Hematological and Systemic Disorders

• Malignancy is associated with CSVT through multiple mechanisms including hypercoagulability. 2, 4, 5
• Dehydration contributes to blood stasis and increased thrombotic risk, particularly in children. 1, 6
• Autoimmune hemolytic anemia has been identified as a risk factor in pediatric cases. 5

Age-Specific Considerations

• CSVT shows a bimodal age distribution with peaks in infancy (approximately 20% of pediatric cases) and adolescence (around 50% occurring in ages 11-18 years). 1
• Overall, 78% of CSVT cases occur in patients under 50 years of age, making it predominantly a disease of younger individuals. 1, 7
• In neonates, umbilical catheterization and birth asphyxia represent age-specific risk factors. 6, 5

Multifactorial Nature

• The etiology of CSVT is frequently multifactorial, with over 60% of patients with inherited thrombophilia having at least one additional risk factor. 4
• Multiple prothrombotic factors occur together in a substantial proportion of patients, emphasizing the need for comprehensive evaluation. 4
• In 15-20% of patients, CSVT remains unprovoked without identifiable predisposing factors. 3

Clinical Pitfall

• Clinicians must maintain high suspicion for CSVT in at-risk patients, as presentation may be nonspecific with no apparent neurological manifestations, and delayed diagnosis is common with a median 7 days from symptom onset to diagnosis. 2, 6