Microvascular decompression (MVD) is an effective and safe treatment option that offers the prospect of definitive cure for hemifacial spasm (HFS). However, there are potential risks of complications for MVD associated with retromastoid suboccipital craniectomy (RmSOC) and cranial nerves in particular. The purpose of this study was to identify clinical characteristics of possible complications after MVD for HFS and to establish appropriate management concept of these complications. We retrospectively reviewed medical records of 2040 patients who underwent RmSOC with MVD for HFS at Samsung medical center between January 1998 and March 2013. Of 2040 patients, 2027 were followed (99.4%). Of the 2027 patients, 1841 (90.8%) exhibited complete relief or minimal symptoms, and 113 (5.6%) reported improved spasm but had mild remnant symptoms. After operation, the most frequently developed complications were facial nerve palsy (8.19%), followed by middle ear effusion (4.90%) and hearing loss (3.63%). There were two cases of supratentorial subdural hemorrhage, three cases of infarction. MVD was found to be safe and effective treatment for HFS, in consistent with previous reports. Some of the complications such as facial nerve palsy, middle ear effusion, and hearing loss are relatively common. However, they have mild clinical courses that are usually transient.

No abstract

Background RNF213 is a major susceptibility gene for moyamoya disease (MMD), characterized by chronic progressive steno‐occlusion of the intracranial arteries. However, coincidental extracranial arteriopathy is sporadically described in a few cases and in children with MMD. Methods and Results This study prospectively enrolled 63 young adults (aged 20–49 years) without a known history of systemic vascular diseases who were confirmed to have definite (bilateral, n=54) or probable (unilateral, n=9) MMD, as per typical angiographic findings. Coronary and aorta computed tomography angiography was performed to characterize extracranial arteriopathy and investigate its correlation with clinical characteristics and MMD status, including the RNF213 p.Arg4810Lys variation (c.14429G>A, rs112735431). Altogether, 11 of 63 patients (17%) had significant (>50%) stenosis in the coronary (n=6), superior mesenteric (n=2), celiac (n=2), renal (n=1), and/or internal iliac artery (n=1). One patient showed both mesenteric and iliac artery stenosis. Patients with extracranial arteriopathy were more likely to have diabetes mellitus and posterior cerebral artery involvement. Moreover, a higher prevalence of extracranial arteriopathy was observed in the presence of the RNF213 p.Arg4810Lys variant (67% in homozygotes). After controlling for diabetes mellitus and posterior cerebral artery involvement, the p.Arg4810Lys variant was independently associated with extracranial arteriopathy (additive model; P =0.035; adjusted odds ratio, 4.57; 95% CI, 1.11–27.20). Conclusions Young adults with MMD may have concomitant extracranial arteriopathy in various locations. Patients with RNF213 variants, especially the p.Arg4810Lys homozygous variant, should be screened for systemic arteriopathy.