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Background: Anesthetic management of pheochromocytoma and paraganglioma with Fontan circulation is challenging for physicians, with attention to cardiovascular physiology., Case Presentation: We performed anesthetic management for pheochromocytoma and paraganglioma in three patients with Fontan circulation. We maintained intraoperative central venous pressure at preoperative level under fluid infusion and administrating nitric oxide to decrease pulmonary arterial resistance. We administered noradrenaline or vasopressin if low blood pressure was present despite adequate central venous pressure. Although noradrenaline is prevalent for the case of noradrenaline-secreting tumor especially after resection, we could maintain blood pressure to administrate vasopressin without increasing central venous pressure. Retroperitoneal laparoscopic approach which could avoid intra-abdominal adhesions might be selectable as case 3., Conclusions: Sophisticated management is required for pheochromocytoma and paraganglioma with Fontan circulation., (© 2023. The Author(s).)

Background: Hepatectomy for patients with Fontan circulation consists of high central venous pressure and low pulmonary vascular resistance, and is challenging for physicians., Case Presentation: We performed anesthetic management for hepatectomy in three patients with Fontan circulation. Massive bleeding and transfusion as well as careful management were needed. Open abdominal surgery had to be conducted instead of laparoscopic surgery for controlling bleeding in one case. We successfully performed general anesthesia using nitric oxide and inotropes while monitoring arterial pressure and central venous pressure in all the cases., Conclusions: To maintain Fontan circulation during hepatectomy, it is important to manage central venous pressure and ensure appropriate circulatory volume., (© 2022. The Author(s).)

Background: Carbamoyl phosphate synthetase deficiency (CPS1D) is a urea-cycle disorder (UCD). We report successful perioperative management of pediatric living donor liver transplantation (LDLT) in a CPS1D patient., Case Presentation: A 10-year-old female patient with CPS1D underwent LDLT. Proper administration of dextrose 50% and 60 kcal/kg/day with L-arginine and L-carnitine resulted in the avoidance of intraoperative hyperammonemia induced by hypercatabolism. Serum ammonia level transiently increased to 61 mmol/L in the anhepatic phase and decreased to 44 mmol/L after reperfusion., Conclusions: We suggest anesthesia management with administration of dextrose to avoid hyperammonemia during LDLT in patients with CPS1D., (© 2022. The Author(s).)

Background: Cardiac sarcoidosis (CS) causes severe conduction abnormalities and arrhythmias. CS patients are increasingly being treated with cardiac resynchronization therapy-defibrillators (CRT-Ds). For the first time, we report the anesthetic management of a CS patient with a CRT-D., Case Presentation: A 65-year-old male with an implanted CRT-D due to CS was scheduled for a laparoscopy-assisted total proctocolectomy for his transverse colon cancer. His left ventricular ejection fraction was 32.0%, and his physical status was a New York Heart Association class III. General and epidural anesthesia were performed while using standard monitors and a FloTrac TM system. The dual-chamber pacing (DDD) modality of the CRT-D was unchanged, and its defibrillation function was deactivated before surgery. The surgery was successfully performed, and the patient was discharged without worsening of his cardiac condition., Conclusions: A detailed understanding of this patient's condition, as well as sarcoidosis, helped to facilitate successful anesthetic management of this patient.

Background: Bleeding and carbon dioxide (CO 2 ) gas embolism have been reported as serious complications associated with laparoscopic surgery. We present a case of cerebral infarction presumably caused by CO 2 gas embolism during laparoscopic hepatectomy., Case Presentation: During liver resection, the end-tidal CO 2 suddenly dropped from 40 to 21 mmHg. Simultaneously, ST elevation in lead II and ST depression in lead V5 of the electrocardiogram were observed. After improvement of these electrocardiographic changes, surgery was continued. Postoperatively, incomplete paralysis was present in the right arm and leg. Magnetic resonance imaging revealed cerebral infarction in the broad area of the left cerebral cortex. These complications might have been caused by paradoxical embolism., Conclusion: We should always keep in mind the risk of cerebral infarction with neurological deficits in the case of laparoscopic surgery. Careful monitoring and appropriate treatment for gas embolism are necessary during laparoscopic surgery.

Background: Intestinal pseudo-obstruction, which is a rare complication of pheochromocytoma, can be caused by hypersecreted catecholamines., Case Presentation: A 45-year-old woman was admitted for local recurrence of pheochromocytoma complicated by intestinal pseudo-obstruction. The intestinal pseudo-obstruction showed poor response to α-adrenergic receptor blocker and she was scheduled for surgical resection of pheochromocytoma. The surgery was uneventfully accomplished with general anesthesia combined with epidural anesthesia. The latter was performed with the aim of not only perioperative pain management but also of promoting intestinal peristalsis. The anticipated effect for intestinal peristalsis was not apparent in the early postoperative phase. The abdominal symptoms were gradually relieved over the course of about 1 month., Conclusions: For intestinal pseudo-obstruction induced by pheochromocytoma, although inhibition of the sympathetic system by epidural infusion of local anesthetics may be promising, short-term usage of epidural local anesthetics infusion did not provide a quick recovery after pheochromocytoma removal surgery.