Cerebral sinovenous thrombosis in children: Thrombophilia and clinical outcome

Eleven cases of cerebral sinovenous thrombosis (CSVT) were identified (9 males, 2 females). Clinical onset was in the neonatal age (mean 3.2 days; median 4.0 days; range 1–6 days) in 5 patients and between 2 and 6.3 years in the six non-neonates (mean 4.5 years; median 4.6 years). Among neonates CSVT presented with seizures in 4/5; aspecific symptoms such as lethargy and respiratory distress were present in one patient. Five out of 6 nonneonates developed signs of intracranial hypertension (vomiting, headache and abducens palsy variably associated; papilledema in all cases), one patient presented with epileptic status. In the group of neonates, risk factors were congenital heart diseases (2/5), perinatal asphyxia (2/5), sinus compression by subdural haematoma (in a patient affected by hemophilia A) or vacuum extractor (2/5). Among non-neonates, 3 out of 6 patients presented CSVT after an episode of properly treated mastoiditis, one developed CSVT during an episode of gastroenteritis, one during a relapse of nephrotic syndrome. As for the presence of thrombophilia, 1 out of 5 investigated nonneonates (20%) and 4 out of 5 neonates (80%) presented one or more prothrombotic abnormalities.In conclusion, CSVT is a potentially serious disorder in pediatrics, particularly in the neonatal age. We observed neurological sequelae in neonates and in all patients with transient or inherited prothrombotic abnormalities at the baseline. Further studies are needed to understand if the early insult to immature brain or the thrombophilic state plays the major role as the negative prognostic factor. An Italian Childhood Thrombosis Registry is ongoing to improve our understanding of epidemiology, clinical features and outcome of childhood CSVT in Italy.