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Measuring regional cerebral blood flow (rCBF) after revascularization for moyamoya disease, as a type of ischemic cerebrovascular disease, is crucial. This study aims to validate our novel technology that combines near-infrared spectroscopy (NIRS) with a frequency filter to extract the arterial component. We measured rCBF before and after revascularization for moyamoya disease and at the end of the surgery using NIRO-200NX (Hamamatsu Photonics, Japan) and indocyanine green (ICG). rCBF was calculated using Fick's principle, change in arterial ICG concentrations, and maximum arterial ICG concentration. rCBF measured with NIRS (rCBF_N) was compared with pre- and postoperative rCBF measured with SPECT (rCBF_S). Thirty-four procedures were analyzed. rCBF_N increased from baseline to end of the surgery (mean difference (MD), 2.99 ml/min/100 g; 95% confidence interval (CI), 0.40-5.57 ml/min/100 g on the diseased side; MD, 4.94 ml/min/100 g; 95% CI, 2.35-7.52 ml/min/100 g on the non-diseased side). Similar trends were observed for rCBF_S (MD, 3.98 ml/min/100 g; 95% CI, 2.30-5.67 ml/min/100 g on the diseased side; MD, 2.77 ml/min/100 g; 95% CI, 1.09-4.45 ml/min/100 g on the non-diseased side). Intraclass correlations 3 (ICC3s) between rCBF_N and rCBF_S were weak on the diseased side (ICC3, 0.25; 95% CI, -0.03-0.5; p = 0.07) and the non-diseased side (ICC3, 0.24; 95% CI, -0.05-0.5; p = 0.08). rCBF measurements based on this novel method were weakly correlated with rCBF measurements with SPECT., (© 2022. The Author(s), under exclusive licence to Springer Nature B.V.)

Background: Moyamoya disease is a cerebrovascular disease characterized by bilateral stenosis of the intracranial internal carotid arteries and an abnormal collateral vascular network at the base of the brain. Transient neurological events (TNEs), which are episodes of neurological dysfunction lasting <24 hours, are associated with stroke in pediatric patients with Moyamoya disease. Perioperative agitation often occurs in pediatric patients. We hypothesized that anesthetic technique and postoperative sedation would modify the association between TNE and superficial temporal artery-middle cerebral artery (STA-MCA) bypass in pediatric patients with Moyamoya disease., Methods: We retrospectively reviewed the medical records of patients with Moyamoya disease aged 15 years and below who underwent STA-MCA bypass under general anesthesia at a single cerebrovascular center in Japan between January 1999 and March 2016. The primary outcome was TNE. Mixed-effects logistic regression was used to evaluate whether postoperative sedation and anesthetic agents were associated with TNE., Results: Among 277 hemispheres in 154 pediatric patients who underwent STA-MCA bypass, 107 patients (39%) experienced TNE within 1 week after surgery. Crying (adjusted odds ratio, 3.11; 95% confidence interval, 1.01-9.59; P=0.048) was an independent risk factor for TNE. Postoperative sedation was associated with a lower incidence of TNE (adjusted odds ratio, 0.514; 95% confidence interval, 0.264-0.997; P=0.049), but premedication and anesthetic agents were not associated with TNE., Conclusion: In pediatric patients with Moyamoya disease, crying was associated with increased TNE and postoperative sedation is associated with decreased TNE.

Moyamoya disease is a chronic cerebrovascular occlusive disease, occurring predominantly in young populations, that causes cerebral ischemia and hemorrhage. Patients with moyamoya disease are at high risk of neurological complications during cardiac surgery because of perioperative hemodynamic changes. However, there is no established evidence on temperature management during cardiopulmonary bypass. Previous reports described normothermia or mild to moderate hypothermia during cardiopulmonary bypass in patients with moyamoya disease; however, surgical conditions, such as not having enough space to clamp the aorta or a clean surgical field, sometimes force us to use deep hypothermic circuratory arrest. We report a successful case of a pediatric patient with moyamoya disease who underwent deep hypothermic circulatory arrest (18 °C) for hemiarch replacement without neurological complications. Deep hypothermia may be an alternative technique for achieving cerebral protection in the context of moyamoya disease.

We report on a child with moyamoya disease combined with von Gierke's disease. A 7-year-old girl with von Gierke's disease had a stroke associated with moyamoya disease. She had had many episodes of hypoglycemia and severe metabolic acidosis before surgery. General anesthesia was induced with midazolam 3 mg and fentanyl 100 microg followed by rocuronium 12 mg. After tracheal intubation, anesthesia was maintained with sevoflurane 2.5% in 33% oxygen and 66% nitrous oxide. We used mainly mixture of saline and glucose as intraoperative fluid instead of acetated Ringer solution, and controlled administration of glucose according to blood glucose levels. The patient's plasma lactate levels and base excess during operation showed changes compared with those before operation, because sodium bicarbonate was used during the surgery. The duration of anesthesia was 374 minutes. The patient woke up and spontaneous respiration returned, and the trachea was extubated in the operating room. We were able to manage this case safely without any complications.