Cerebral venous thrombosis in infants and children

Mahendranath D Moharir MD MSc (Dr. Moharir of the University of Toronto has no relevant financial relationships to disclose.)
Gabrielle deVeber MD MHSc (Dr. deVeber of the University of Toronto has no relevant financial relationships to disclose.)
Bernard Maria MD, editor. (Dr. Maria of Icahn School of Medicine at Mount Sinai and Director of Pediatric Neurology and Developmental Medicine at Goryeb Children)
Originally released February 3, 1994; last updated May 16, 2016; expires May 16, 2019

This article includes discussion of cerebral venous thrombosis in infants and children, cerebral sinovenous thrombosis, dural sinus thrombosis, sagittal sinus thrombosis, and sinus thrombosis. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Cerebral sinovenous thrombosis is often unrecognized in neonates and children due to nonspecific clinical features and challenges in radiographic diagnosis. Risk factors for cerebral sinovenous thrombosis in neonates are different from those in older children. Treatment with anticoagulants, a well-established practice in adults, is increasingly offered in children and, to a lesser extent, in neonates. The safety of anticoagulants and recanalization outcomes in cerebral sinovenous thrombosis has been established in children as well as in neonates based on consecutive cohort studies. The clinical outcome from cerebral sinovenous thrombosis is worse in neonates than in children.

Key points

 

• The clinical and radiographic diagnosis of cerebral sinovenous thrombosis is challenging in neonates and children.

 

• Cerebral sinovenous thrombosis should be considered in any child with unexplained seizures and/or encephalopathy.

 

• Anticoagulant therapy is safe in the absence of significant intracranial hemorrhage in neonates and children. It is probably safe in the presence of intracranial hemorrhage as well, at least in older children.

 

• Anticoagulant therapy prevents worsening of thrombosis in both neonates and children.

 

• Clinical outcomes are poor in the majority of neonates with cerebral sinovenous thrombosis.

Historical note and terminology

Early literature on cerebral sinovenous thrombosis is mainly based on autopsy studies by French physicians. In 1873, Parrot recognized sagittal sinus thrombosis in neonates. In 1884, Bouchut observed cerebral sinovenous thrombosis in infants who had seizures during febrile or debilitating illnesses. The same year, Rilliet and Barthez reported infection as a predisposing factor in young children. Lhermitte described the association of cerebral sinovenous thrombosis with congenital heart disease. Several years later, Symonds described “otitic hydrocephalus” referring to hydrocephalus and raised intracranial pressure in children with chronic otitis media and mastoiditis (Symonds 1931). This probably represents the first description of childhood cerebral sinovenous thrombosis in modern English literature. Because the condition was considered infrequent and difficult to diagnose, systematic literature was rare before the advent of modern neuroimaging techniques.

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