Moyamoya disease is a unique cerebrovascular disease that is characterized by progressive bilateral stenotic alteration at the terminal portion of the internal carotid arteries. These changes induce the formation of an abnormal vascular network composed of collateral pathways known as moyamoya vessels. In quasi-moyamoya disease, a similar stenotic vascular abnormality is associated with an underlying disease, which is sometimes an inflammatory disease. Recent advances in moyamoya disease research implicate genetic background and immunological mediators, and postulate an association with inflammatory disease as a cause of, or progressive factor in, quasi-moyamoya disease. Although this disease has well-defined clinical and radiological characteristics, the role of inflammation has not been rigorously explored. Herein, we focused on reviewing two main themes: (1) molecular biology of inflammation in moyamoya disease, and (2) clinical significance of inflammation in quasi-moyamoya disease. We have summarized the findings of the former theme according to the following topics: (1) inflammatory biomarkers, (2) genetic background of inflammatory response, (3) endothelial progenitor cells, and (4) noncoding ribonucleic acids. Under the latter theme, we summarized the findings according to the following topics: (1) influence of inflammatory disease, (2) vascular remodeling, and (3) mechanisms gleaned from clinical cases. This review includes articles published up to February 2019 and provides novel insights for the treatment of the moyamoya disease and quasi-moyamoya disease.

Acute coagulopathy is common after traumatic brain injury (TBI), particularly in severe cases of acute subdural hemorrhage (ASDH). Although acute coagulopathy is associated with poor outcomes, the optimal treatment strategy remains unknown. Here, we report the initial results of an empirical cryoprecipitate transfusion strategy that we developed as an early intervention for acute coagulopathy after TBI. We performed chart reviews of adult patients (aged ≥18 years) who received early cryoprecipitate transfusion after admission to our institution with a diagnosis of severe TBI (Glasgow Coma Scale ≤8) and ASDH from March 2013 to December 2016. We compared the outcomes of these patients with those who were treated before the implementation of the cryoprecipitate transfusion strategy (January 2011–February 2013). During the study period, 33 patients received early cryoprecipitate transfusion and no acute transfusion-related adverse event was reported. The rate of coagulopathy development within 24 h after admission was lower in these patients (23%) than in the controls (49%), but the difference was not significant (P = 0.062). The in-hospital mortality rate was 36% in patients receiving early cryoprecipitate transfusion and 52% in controls. After adjusting for confounding factors, the in-hospital mortality rate was significantly lower in the intervention period [adjusted odds ratio: 0.25, 95% confidence interval (CI): 0.08–0.78, P = 0.017]. In summary, we analyzed initial results of a cryoprecipitate transfusion strategy in patients with severe isolated TBI and ASDH. No acute transfusion-related adverse event was observed, and early transfusion of the in-house-produced cryoprecipitate may have reduced rates of coagulopathy development and in-hospital mortality.

Formalin or formaldehyde is commonly used for cadaver fixation, which is, however, not suitable for endoscopic transnasal skull base approach because of consequent hardening of the soft tissue. Several alternatives have been reported, but each of them also has some limitations. We applied a novel fixation method using N-vinyl-2-pyrrolidone (NVP), a precursor of the water-soluble macromolecular polymer, for endoscopic transnasal skull base approach in six donated cadaver specimens. In four cadavers, elasticity of the soft tissue in the nasal cavity was almost similar to that of living tissue, and a surgical approach similar to the real surgical field was possible. However, the soft tissue was moderately stiffer than living tissue in two specimens so that surgical manipulation was hindered to some extent while NVP concentration was 10% in all the cadavers. Since the brain tissue was too soft and pliable for surgical manipulation in NVP, more careful surgical manipulation than real surgical field was mandatory in order to prevent damage in the brain tissue. Therefore, this concentration of NVP was considered to be appropriate. In conclusion, NVP embalming was effective for endoscopic transnasal skull base approach in cadaver dissection, providing environment similar to the real surgical field.

Precautious balloon test occlusion (BTO) is sometimes performed in cases of high-risk intraoperative internal carotid artery injury. We investigated whether magnetic resonance angiography (MRA) findings could predict BTO results to thus avoid the use of precautious BTO. This retrospective study, included 96 patients who underwent BTO, eight of whom underwent bilateral BTO. The relationship between the BTO results for 104 internal carotid arteries and the MRA findings obtained in 96 patients were retrospectively evaluated. On MRA, anterior cerebral artery (A1)–anterior communicating artery–A1 was defined as anterior collateral circulation (ACC), and posterior cerebral artery–posterior communicating artery was defined as posterior collateral circulation (PCC). BTO was tolerated in all 27 sides with thick ACC regardless of PCC thickness. In 31 of 44 cases with a thin ACC, the tested sides were BTO-tolerant (70.5%). Of these 44 tested sides, all five with a thick PCC were BTO-tolerant, but eight with a thin PCC and 31 with an invisible PCC showed results other than tolerance. Among cases with an invisible ACC, 10 of 33 tested sides were BTO-tolerant (30.3%). Among these 33 tested sides, outcomes other than tolerance were observed regardless of PCC thickness. Thick, thin, and invisible ACCs were assigned 3, 1, and 0 points, respectively; and thick, thin, and invisible PCCs were assigned 2, 1, and 0 points, respectively. A sum of 3 points in the ACC and PCC indicated that all sides were BTO-tolerant. In conclusion, a thick ACC or a thin ACC with a thick PCC indicates BTO-tolerance. The BTO prediction score is useful for predicting results of BTO.

Surgery for neoplastic or vascular lesions at the craniovertebral junction remains one of the major challenges for neurosurgeons, because of issues such as the complex functional anatomy and vascular structures. We present three cases in which the high cervical lateral approach was used to safely remove the cystic retro-odontoid pseudotumor, not associated with rheumatoid arthritis, severely compressing the spinal cord. The mean age of patients was 74.7 years (range, 73–77 years). Neurological condition was assessed based on the neurosurgical cervical spine scale. A high cervical lateral approach was applied to remove the pseudotumor safely. Mean duration of follow-up after surgery was 21.3 months (range, 18–24 months). Mean recovery rate was 77.8%. All patients showed acceptable or satisfactory functional recovery, although one patient (Case 2) developed mild paralysis of the facial and spinal accessory nerve on the surgical approach side, but that completely recovered within about 1 month after surgery. Postoperative assessment at the recent follow-up suggested no significant aggravation of neck movement. This technical note suggests that the high cervical lateral approach can be considered as a surgical option for cystic retro-odontoid pseudotumor, not associated with rheumatoid arthritis, severely compressing the spinal cord. Safe management of the vertebral artery is one of the key considerations.