The perioperative transition of serum biomarkers of a 1.5-year-old boy with very-long-chain acyl-CoA dehydrogenase deficiency.

Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCADD, OMIM 201475) is a congenital fatty acid oxidation disorder. Individuals with VLCADD should avoid catabolic states, including strenuous exercise and long-term fasting; however, such conditions are required when undergoing surgery. The perioperative management of VLCADD in infants has rarely been reported and details regarding the transition of serum biomarkers reflecting catabolic status have not been disclosed. Herein, we present the perioperative clinical and biological data of cryptorchidism in a 1.5-year-old boy with VLCADD. The patient was diagnosed through newborn screening and his clinical course was very stable. Genetic testing of ACADVL revealed compound heterozygous variants c.506 T > C (p.Met169Thr) and c.606-609delC (p.L216*). The enzyme activity of the patient with VLCAD was only 20% compared to that of healthy control. Left orchiopexy for the pediatric cryptorchidism was planned and performed at 1 and a half year of age. Induction anesthesia involved thiopental, fentanyl and rocuronium. The glucose infusion rate was maintained above 6.6 mg/kg/min starting the day before surgery until the operation was completed. Anesthesia was maintained with sevoflurane at approximately 2%. The serum concentration of tetradecenoylcarnitine were stable during the operation, ranging between 0.08 and 0.19 µM (cutoff <0.2 µM), and never deviated from the reference range. Concentration of other serum biomarkers including free fatty acid, 3-OH-butyrate, and creatine kinase, remained similarly unchanged. In this report, we describe the uneventful perioperative management of unilateral orchiopexy for left cryptorchidism in a 1.5-year-old boy with VLCADD using sufficient glucose infusion and volatile anesthesia.

Anesthetic management and associated complications of peroral endoscopic myotomy: A case series.

AIM: To investigate the anesthetic management of peroral endoscopic myotomy (POEM) and its associated complications. METHODS: This study was a single-center, retrospective, observational study comprising a case series of all patients who underwent POEM in our hospital from April 2015 to November 2016. We collected data regarding patient characteristics, anesthetic methods, surgical factors, and complications using an electronic chart. RESULTS: There were 86 patients who underwent POEM in our hospital during the study period. Preoperatively, patients were maintained on a low residue diet for 48 h prior to the procedure. They were fasted of solids for 24 h before surgery. There was one case of aspiration (1.2%). During POEM, patients were positioned supine with the upper abdomen covered by a clear drape so that pneumoperitoneum could be timeously identified. In three cases, the peak airway pressure exceeded 35 cmH2O during volume controlled ventilation with tidal volumes of 6-8 mL/kg and subsequent impairment of ventilation. These cases had been diagnosed with spastic esophageal disorders (SEDs) and the length of the muscular incision on the esophageal side was longer than normal. CONCLUSION: In the anesthetic management of POEM, it is important to prevent aspiration during induction of anesthesia and to identify and treat complications associated with CO2 insufflation.

Constant Current vs. Constant Voltage Systems for Temporal Spinal Cord Stimulation for Intractable Pain.

Although spinal cord stimulation (SCS) is a useful treatment for chronic intractable pain, the optimal method of stimulation has not yet been established. In this prospective, crossover study, we compared the efficacy of using a constant current (CC) system with that of a constant voltage (CV) system for temporal SCS. Twenty patients were enrolled and divided into two groups. For 10 patients, a CV system was applied on Days 1-5, followed by the use of a CC system on Days 6-10. For the other 10 patients, a CC system was applied for the first five days, followed by a CV system for the subsequent five days. We evaluated the alteration of pain intensity using a visual analogue scale (VAS), the area of stimulation, the stability of effect, and patient satisfaction regarding treatment. The pain scores decreased significantly after the start of the SCS. There was no significant difference in the change in VAS between the two systems. The stimulation method used for temporal SCS did not affect the reduction of pain intensity. Patients felt a wider stimulation area by the CC system compared to the CV system.

[Controversy in the treatment of acid-base abnormalities].

Sodium bicarbonate has been standard therapy for the treatment of acidosis. In lactic acidosis and hypercapnic acidosis, however, there is no clinical data supporting its effectiveness. We reviewed the literature of the efficacy of sodium bicarbonate on lactic acidosis and hypercapnic acidosis. On both conditions, we have no solid evidence supporting its beneficial effect. Conversely, acidosis or hypercapnia might be protective in acute lung and systemic organ injury. Therefore, the unprepared use of bicarbonate might be harmful in terms of fluid and sodium overload and excess lactate concentrations. According to current literature, we cannot recommend sodium bicarbonate administration for patients with lactic acidosis and hypercapnic acidosis.