Long-term antikoagulation in a neonate with sinovenous thrombosis and stroke. Case presentation and review of the literature

Neonatal stroke from cerebral sinovenous thrombosis (CSVT) is a common cause of acute neonatal encephalopathy. Altered mental status, seizures and sensorimotor deficits are typical at presentation, chronic neurologic disability are a frequent feature. Neonatal risk for CSVT begins prenataly and extends into postnatal life, influenced by a developing haemostatic system, systemic derangements associated with neonatal complications, and acquired risk factors from medical interventions besides congenital prothrombotic disorders. Modern diagnostic possibilities make diagnosis of CSVT in newborns easy, but the discussion on therapeutic approaches is still controversial. Despite advances in our understanding of the pathophysiology of CSVT, significant deficiencies in our knowledge remain related to optimal therapy in this group.

We report on a neonate with CSVT (sinus rectus and partially internal cerebral veins, superior sagittal sinus) with secondary bilateral haemorrhage of thalami and basal ganglia on the basis of LP (a)-hyperlipoproteinemia, presenting with acute loss of consciousness, seizures, and generalized increase of muscle-tone at day 10 of life. After initial stabilization we started anticoagulation with LMWH, which is still ongoing. During the clinical course the child developed an accentuated spastic tetraparesis, West-Syndrom and a combined developmental delay. Nevertheless, actually she is predominantly seizure-free, ambulatory and mentally moderately retarded.

Discussion: Treatment options for symptomatic children with infarctation and hemorrhage after CSVT are still under discussion. In many children, like in the one presented, a clear individual estimation of an expected recurrance-risk can not be obtained despite considerable efforts. The poor database of pediatric standard values for many specific coagulation tests is an additional alienating factor, that complicates the decision when to stop anticoagulation. Recommendations on therapeutic regimens in children with prothombotic risk factors and history of CSVT are sparse.