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Key Clinical Message: The perioperative control of ammonia, reduction of stress, and administration of drugs tolerated in type 1 citrullinemia and Brugada pattern allowed the successful and uneventful management of general anesthesia in the study patient. The aim of this study was to report the targeted perioperative management of general anesthesia (GA) adopted for dental extractions in a rare patient with type 1 citrullinemia and Brugada pattern. A male, Caucasian, adult type 1 citrullinemia patient needed dental extractions under GA. The medical history showed neurodevelopmental impairment, growth retardation, epilepsy, and a Type 2 Brugada electrocardiographic pattern in the second precordial lead. The authors focused the anesthesiologic protocol on the prevention of hyperammonemia and fatal arrhythmias. Changes in diet and 10% glucose solution administration prevented protein catabolism due to the fasting period (ammonia was 44 μmol/L preoperatively and 46 μmol/L postoperatively; glycemia was 120 g/dL preoperatively and 153 g/dL postoperatively). The patient received a continuous electrocardiogram, noninvasive blood pressure, pulse oximeter, entropy monitoring, train‐of‐four monitoring, and external biphasic defibrillator pads. Midazolam, remifentanil, and dexamethasone were administered for pre‐anesthesia; thiopental and rocuronium for induction; remifentanil and desflurane for maintenance; sugammadex for decurarization. After the intraligamentary injection of lidocaine 2% with epinephrine 1:100,000 for local anesthesia, the patient developed a transient Type 1 Brugada pattern that lasted a few minutes. The whole procedure lasted 30 min. The patient's discharge to ward occurred 3 h after the end of GA. The perioperative management of ammonia, reduction of stress, and administration of drugs tolerated in Type 1 citrullinemia and Brugada pattern allowed the successful and uneventful administration of GA in the study patient. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Oral maxillary exostoses are proliferating bone lesions with an unknown etiology occurring on the cortical plates both in the maxilla and in the mandible of young individuals, showing a typical slow but continuous enlargement. No treatment is usually required unless they create esthetic or functional limitations during follow‐up; the biopsy is needed only for doubtful lesions. Furthermore, it is mandatory to collect an accurate familiar history of patients affected by exostosis, especially when occurring with atypical clinical presentation, in order to exclude or prevent potentially associated systemic diseases. Oral maxillary exostoses are proliferating bone lesions with an unknown etiology occurring on the cortical plates both in the maxilla and in the mandible of young individuals, showing a typical slow but continuous enlargement. No treatment is usually required unless they create esthetic or functional limitations during follow‐up; the biopsy is needed only for doubtful lesions. Furthermore, it is mandatory to collect an accurate familiar history of patients affected by exostosis, especially when occurring with atypical clinical presentation, in order to exclude or prevent potentially associated systemic diseases. [ABSTRACT FROM AUTHOR]