Constrictions of cerebral venous sinus secondary to idiopathic intracranial hypertension

Pseudotumor cerebri (PTC) is a rare disease of heterogenous etiology. As strictures of the cerebral venous sinus are found repeatedly, impaired venous drainage with secondary dysfunction of CSF drainage has been hypothesized. However, the strictures might also be a consequence of the intracranial hypertension. Distinct patients with strictures have already been treated by stent implantation.

A ten-year-old girl was diagnosed to suffer from PTC in 2/2006 because of headaches and elevated intracerebral pressure (30cm H₂O). The MRI showed normal parenchymatous findings with bilateral strictures at the transverse-sigmoid junction. After a lumbar puncture she was free of symptoms for eight months. Then the headaches reappeared, now combined with attention problems. Again the intracerebral pressure was elevated (30cm H₂O). A treatment trial with acetacolamide failed so that repeated and frequent lumbar punctures became necessary. Thereupon, a ventriculoperitoneal shunt was inserted resulting in complete clinical recovery. A subsequent MRI control revealed disappearance of the former strictures.

The presented case demonstrates that strictures of the cerebral venous sinus can be a consequence of elevated intracerebral pressure. Invasive intervention such as dilation or venous stenting should be done restrainedly.