Central Venous Sinus Thrombosis

Published Date: 19th July 2016

Publication Authors: Clarke R, Ismail N

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Objectives: 

To describe the predisposing factors clinical presentation, neuroimaging, management and outcome in a cohort of children with cerebral sinus venous thrombosis (CVST). 

Methods: 

Retrospective analysis of case notes of patients up to 17 years of age with a diagnosis of CVST, between January 1997 to December 2009 in a tertiary hospital.

Results: 

Seventeen patients were identified (10 male, 7 female). The median age at presentation was 5.5 years (range 2d-17yrs). Headache (9/17) with vomiting (6/17) was the dominant presenting symptom often associated with papilloedema (5/17). Neurological signs (11/17). Six patients had antecedent infection of the ear or mastoid, three children had leukaemia and two had central lines. One 14-year-old girl was on the contraceptive pill. One patient had transposition of the great vessels. Two patients had pneumonia, one was dehydrated, and the other had associated sphenoethmoiditis. Two children out of 7 showed heterozygosity for factor V mutation. All children had MRI and MRV. The most common sinuses involved were superficial (transverse and superior sagittal) followed by the deep sinuses (sigmoid and straight sinuses). Fourteen children had sinus thrombosis, there were cerebral infarcts in four, while two children had cerebral haemorrhages. Six patients received anticoagulation along with other supportive measures. Follow up data were available for 15 patients. Two died first was due to marked deterioration of brain and brainstem function, and the other had CHARGE association and perinatal hypoxic insult. Seven recovered completely, while three children had residual permanent neurological deficit. Two suffered persistent headache one of those was associated with raised intracranial pressure and required intervention. One patient had persistent middle ear effusion.  

Conclusion: 

It is important to maintain a high index of suspicion to diagnose this relatively rare condition, especially where the risk factors for thrombosis do not exist. The evidence for optimal treatment of CVST remains to be elucidated.

Ismail, N; Clarke, R; John, C. (2013).  Central venous sinus thrombosis: A case series from a regional paediatric UK hospital . Developmental Medicine and Child Neurology. 55 (Supplement S1)

 

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