Abstract
Objective: We report a case of giant aneurysm of the petrous internal carotid artery in a young adult man with cutaneous findings of phakomatosis pigmentovascularis (PPV), treated with endovascular trapping of the internal carotid artery.
Case presentation: A 24-year-old male with PPV presented with headache and left abducens nerve palsy. The skin anomaly was on the left side of his body since birth. A brain magnetic resonance imaging (MRI) revealed a giant thrombosed aneurysm located at the petrous and extending to the cavernous portion of the left internal carotid artery (ICA). After thorough evaluation of hemodynamic tolerance, endovascular left ICA trapping was performed with detachable coils. The postoperative course was uneventful and the symptoms were resolved in 3 months from embolization. At one year after the treatment, MRI showed an obvious decrease in the size of the aneurysm.
Conclusions: PPV is a rare syndrome characterized by the association of a widespread vascular nevus with an extensive pigmentary nevus. Though the correlation of PPV with cerebral aneurysm is still unclear, PPV can accompany abnormalities in cerebral vessels similar to those seen in Klippel-Trenaunay syndrome and Sturge-Weber syndrome. Furthermore, the abrupt ICA occlusion may change the hemodynamics in the circle of Willis and these abnormal conditions may predispose patients to the formation of de novo aneurysms at these sites. Close and continuous follow up is particularly important.
