2018 Volume 40 Issue 1 Pages 29-34
We here report a very rare case of a 54-year-old man presenting with pseudoxanthoma elasticum (PXE), a primary metabolic disorder characterized by the progressive accumulation of calcium phosphate and proteoglycan deposits in the connective tissues and fragmentation of the elastic fibers, along with concomitant carotid rete mirabile (CRM), an abnormal collateral anastomosis between the internal and external carotid arteries, generally located around the cavernous sinus. At the 19-month follow-up, angiography revealed that the flow of the CRM in the left internal carotid artery was decreased; accordingly, cerebral revascularization surgery with superficial temporal artery and middle cerebral artery anastomosis was successfully performed. There is currently no report on superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis for PXE with CRM, and this is the first report of the midterm outcomes of revascularization surgery for such a patient. The STA may be used as the donor artery for revascularization surgery; however, the effects of harvest and anastomosis on the disease process of PXE in the STA and long-term patency of the STA graft remain unknown. The histological findings in the STA of the present case revealed no apparent impairment and accumulation of calcium phosphate, indicating that the STA is a suitable donor artery.
