Blue rubber-bleb nevus syndrome: report of a familial case with a dural arteriovenous fistula
Sílvia Carvalho, Vieira Barbosa, Nuno Santos, Egidio Machado, Sílvia Carvalho, Vieira Barbosa, Nuno Santos, Egidio Machado
Abstract
We report a case of a 45-year-old woman known to have a familial form of blue rubber-bleb nevus syndrome (BRBNS). The patient developed severe occipital headaches and bilateral retroauricular bruits. Cerebral angiography showed a large dural arteriovenous fistula in the torcular region. Central nervous system involvement in BRBNS is not often reported, and most cases of BRBNS are sporadic.
Figures
![F ig 1.](https://www.ncbi.nlm.nih.gov/pmc/articles/instance/7976306/bin/j40830733001.jpg)
MR images obtained on admission. A, Axialproton density-weighted image (2530/25 [TR/TE]) shows enlarged veins in the posterior fossa. B, Sagittal T1-weighted image (450/15) shows an enlarged straight sinus and the presence of a falcine sinus. Note the tonsillar prolapse.
![F ig 2.](https://www.ncbi.nlm.nih.gov/pmc/articles/instance/7976306/bin/j40830733002.jpg)
Carotid and vertebral angiograms. A, Left common carotid artery angiogram demonstrates a DAVF in the torcular region. It is supplied by both the occipital and middle meningeal arteries and drains in a retrograde fashion in the superior sagittal and straight sinuses. Note the presence of a falcine sinus. B, Venous phase of a left vertebral angiogram shows substantial venous stagnation in the posterior fossa. Note the dilated premedullary and retromedullary veins.
Source: PubMed