Cеrebral venous thrombosis in children – case presentation

Abstract

Cеrebral venous thrombosis (CVT) in children is rare with a multifactorial etiology, a variable clinical presentation, but with serious neurological consequences and significant mortality. The clinical presentation depends on the location and the degree of thrombosis, the age of the patients and the presence of underlying diseases. The diagnosis of CVT is confirmed by neuroimaging: CT venography and MRI venography.
We present three cases of children with CVT, diagnosed in MHATNP „St. Naum”. The first case is of a 11 year old girl with childhood absence epilepsy and thrombophilia: mutation in the plasminogen activator inhibitor (PAI-1) – homozygous 4G/4G and a mutation in methylene tetrahydrofolate reductase (MTHFRC677T) – homozygous T/T. The girl was admitted in MHATNP with symptoms of increase intracranial pressure – headache, vomiting, diplopia and evidence from fundoscopy for congestive papillae with a prominence of about 1.5 D. MRI of the brain showed subacute thrombosis of the upper sagittal sinus and cortical veins along the cerebral convexity bilaterally. Antiedema and anticoagulant therapy was initiated that led to complete normalization of the neurological status and disappearance of the papilledema. In the MRI of the brain performed after 1.5 months, a restored blood flow in the dural sinuses and cerebral veins was visualized.
The second case is of a 6 year old boy, who, on the background of purulent otitis, presented with symptoms of an increase intracranial pressure (headache, single vomiting and bilateral abducens nerve palsy). The CT scan showed a normal image of the brain and mastoiditis. Initially, the case was discussed as idiopathic intracranial hypertension (IIH) until MRI was performed that showed thrombosis of the right transversal and sigmoid sinus and the right jugular bulb. Antiedema and anticoagulant therapy was initiated with gradual improvement and after 3 month there was a complete recovery of the volume of eye movements and a partial recanalization of the thrombotic sinuses.
The third case is of a 14 year old girl with thrombosis of the left transverse and sigmoid sinus, and internal jugular vein during treatment with progesterone and estrogen therapy due to severe menorrhagia and iron deficiency anemia. Due to the syndrome of increasing intracranial pressure with severe headache, quantitative disturbances of consciousness, fluctuating sensory aphasia, CT and MRI of the brain with venography were performed that showed thrombosis of the left sinus transversus, sinus sigmoideus and vena jugularis interna, as well as hemorrhage stroke in the left temporal lobe with edema and compression of the lateral ventricle. Leiden factor V and prothrombin were with normal genotype, protein C and S were normal too. Anticoagulant, antiedema and anticonvulsant therapy were performed, with disappearance of the aphasia and intracranial hypertension. MRI performed 3 months later demonstrated partial recanalization of thrombotic sinuses and vessels, as well as postischemic gliosis and cysts of the left temporal lobe.
The differential diagnosis of CVT from other neurological diseases and the timely initiation of adequate therapy is crucial for the prognosis.