Anesthetic Management of a Patient With Catecholaminergic Polymorphic Ventricular Tachycardia.

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited arrhythmogenic disorder induced by adrenergic stress. Electrophysiologically, it is characterized by emotional stress- or exercise-induced bidirectional ventricular tachycardia that may result in cardiac arrest. Minimizing perioperative stress is critical as it can reduce fatal arrhythmias in patients with CPVT. Dexmedetomidine (DEX), a centrally acting sympatholytic anesthetic agent, was used in the successful intravenous (IV) moderate sedation of a 27-year-old female patient with CPVT, a history of cardiac events, and significant dental fear and anxiety scheduled to undergo mandibular left third molar extraction. Oral surgery was successfully performed under DEX-based IV sedation to reduce stress, and no arrhythmias were observed. IV sedation with DEX provided a sympatholytic effect with respiratory and cardiovascular stability in this patient with CPVT who underwent oral surgery.

Hemodynamic Impact of Drug Interactions With Epinephrine and Antipsychotics Under General Anesthesia With Propofol.

OBJECTIVE: Antipsychotic drugs exhibit α-1 adrenergic receptor-blocking activity. When epinephrine and antipsychotic drugs are administered in combination, ß-2 adrenergic effects are thought to predominate and induce hypotension. This study aimed to assess hemodynamic parameters in patients regularly taking antipsychotics who were administered epinephrine-containing lidocaine under general anesthesia in a dental setting. METHODS: Thirty patients taking typical and/or atypical antipsychotics and scheduled for dental procedures under general anesthesia were enrolled. Five minutes after tracheal intubation, baseline systolic blood pressure (SBP), diastolic blood pressure (DBP), heart rate (HR), and percutaneous oxygen saturation (SpO2) measurements were taken. The SBP, DBP, HR, and SpO2 measurements were repeated 2, 4, 6, 8, and 10 minutes after the injection of 1.8 mL of 2% lidocaine (36 mg) with 1:80,000 epinephrine (22.5 mcg) via buccal infiltration. RESULTS: Differences between the baseline measurements and those of each time point were analyzed using Dunnett test, and no statistically significant changes were observed. CONCLUSIONS: Our findings demonstrate that the use of epinephrine at a clinically relevant dose of 22.5 mcg for dental treatment under general anesthesia is unlikely to affect the hemodynamic parameters of patients taking antipsychotic medications.

Anesthetic Management of a Juvenile Hyaline Fibromatosis Patient With Trismus and Cervical Movement Limitation.

Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease characterized by the presence of tissue nodules, joint contractures, and gingival hyperplasia. With a 1-year-9-month-old female patient scheduled for a gingivectomy and excision of a lower lip mass under general anesthesia, it was anticipated that airway management would be difficult because of trismus and limited cervical movement. Intubation with video-laryngoscopic assistance could not be achieved because gingival hyperplasia and trismus prevented blade insertion and manipulation. Therefore, 2 endotracheal tubes were used: 1 used as a nasopharyngeal airway for assisted ventilation, and 1 used for intubation along with a flexible fiberoptic scope. This case demonstrated a useful method for managing ventilation and intubation in patients with JHF, particularly when the use of oral airway devices is difficult.

Anesthetic Management of a Pediatric Patient With Cardiofaciocutaneous Syndrome.

Cardiofaciocutaneous (CFC) syndrome is a rare condition characterized by congenital heart disease, craniofacial dysmorphology, and dermatological abnormalities. CFC syndrome is one of the RASopathies, a family of syndromes that also includes Noonan and Costello syndromes, all with underlying gene mutations involving the Ras/mitogen-activated protein kinase pathways. Important considerations for anesthesiologists caring for these patients include the need to evaluate for possible cardiac defects, anticipating and planning for potentially difficult airway management, and the consideration of potential weakness of the respiratory muscles. Musculoskeletal abnormalities, such as muscle weakness and decreased muscle mass, are observed in all RASopathies, but are particularly prominent in CFC syndrome. In patients with CFC syndrome who experience respiratory muscle weakness, the use of desflurane and remifentanil may aid in a faster recovery and effectively help reduce the risk of respiratory complications, such as respiratory depression, following general anesthesia because of their rapid metabolism or elimination.

Anesthetic Management of a Patient With Systemic Sclerosis and Microstomia.

Systemic sclerosis (SSc) is an autoimmune disease that can cause fibrosis in vital organs, often resulting in damage to the skin, blood vessels, gastrointestinal system, lungs, heart, and/or kidneys. Patients with SSc are also likely to develop microstomia, which can render dental treatment difficult and painful, thereby necessitating advanced anesthetic management. This is a case report of a 61-year-old woman with a history of SSc with microstomia, interstitial pneumonia, and gastroesophageal reflux disease in whom intravenous moderate sedation was performed using a combination of dexmedetomidine and ketamine for dental extractions. Both anesthetic agents are known to have analgesic effects while minimizing respiratory depression. Consequently, the increased discomfort caused by opening the patient's mouth and stretching the buccal mucosa was sufficiently managed, permitting an increase in maximum interincisal opening and completion of treatment without complications. Patients with SSc present with serious comorbidities that can negatively impact anesthetic management, so the implementation of an anesthetic plan that takes such risks into account is required. Furthermore, emergency airway management is likely to be difficult in patients with microstomia. For intravenous moderate sedation, combined use of dexmedetomidine and ketamine, which have analgesic effects while minimizing respiratory depression, may be particularly effective in patients with SSc and microstomia.

Anesthetic management in corticobasal degeneration with central sleep apnea: A case report.

Corticobasal degeneration (CBD) is a rare neurodegenerative disease characterized by dystonia, cognitive deficits, and an asymmetric akinetic-rigid syndrome. Little information is available regarding anesthetic management for CBD patients. Our patient was a 55-year-old man with CBD complicated by central sleep apnea (CSA). Due to the risk of perioperative breathing instability associated with anesthetic use, a laryngeal mask airway was used during anesthesia with propofol. Spontaneous respiration was stable under general anesthesia. However, respiratory depression occurred following surgery, necessitating insertion of a nasopharyngeal airway. Since no respiratory depression had occurred during maintenance of the airway using the laryngeal mask, we suspected an upper airway obstruction caused by displacement of the tongue due to residual propofol. Residual anesthetics may cause postoperative respiratory depression in patients with CBD. Therefore, continuous postoperative monitoring of SpO2 and preparations to support postoperative ventilation are necessary.

General Anesthesia in a Glucose-6-Phosphate Dehydrogenase Deficiency Child: A Case Report.

We performed general anesthesia on a 3-year-old boy with glucose-6-phosphate dehydrogenase (G6PD) deficiency. Patients with G6PD deficiency exhibit jaundice and anemia due to hemolysis caused by a lack of the G6PD enzyme. To maintain anesthesia, we used propofol and remifentanil, which may prevent hemolytic attacks by exerting an antioxidant effect. In addition, because the patient was in a high-risk group for the development of methemoglobinemia, we used mepivacaine as a local anesthetic. We liaised with the patient's attending physician to make sufficient arrangements, such as securing an emergency transfer on the day of anesthesia. The patient did not develop hemolytic attacks during or after the procedure, and he progressed well without problems.

Intravenous Sedation in Arnold-Chiari Malformation With Respiratory Failure.

Type II Arnold-Chiari malformation (ACM) is an abnormality in which the cerebellum, pons, and medulla oblongata are displaced downward into the spinal cord. Type II ACM is often complicated by respiratory depression, sleep-disordered breathing, and deglutition disorder as a result of medullary dysfunction and impairment of the lower cranial nerves. Bending and stretching of the neck is restricted, and anesthetic management is problematic in patients with the disorder. We performed dental treatment twice under intravenous sedation in a patient with intellectual disability with type II ACM complicated by hypercapnic respiratory failure. Propofol was used for the first sedation procedure. Repeated bouts of respiratory depression occurred on that occasion, so the airway was managed manually by lifting the jaw. However, aspiration pneumonitis occurred postoperatively. A combination of dexmedetomidine and midazolam was used for sedation on the second occasion, and the intervention was completed uneventfully without any respiratory depression. Our experience with this patient highlights the need for selection of an agent for intravenous sedation that does not require neck extension and has minimal effect on respiration in patients with type II ACM, who are at high risk of respiratory depression and pulmonary aspiration.