Anesthesia for Kearns Sayre syndrome

case report

Abstract

Introduction: Introduction: Kearns-Sayre syndrome (KSS) is a very rare multisystem mitochondrial disease that occurs before the age of 20 and is characterized by a typical clinical triad: progressive external ophthalmoplegia with ptosis, pigmentary retinopathy and cardiac conduction abnormalities, including heart block. Other clinical manifestations may also include muscle weakness, symptoms of neurological dysfunction such as cerebellar ataxia, impaired intellectual and cognitive function, sensorineural hearing loss and neuropathy, various endocrine abnormalities, nephropathy, and dental anomalies. Case Report: Female patient, 62 years old, with heart disease using a bicameral pacemaker, type II diabetic, sedentary, with KSS, was admitted in Goiânia, Goiás on 05/06/2024 after desaturation and decreased level of consciousness, being diagnosed with pneumonia in the emergency room. She was intubated and maintained on mechanical ventilation until May 16th and then a tracheostomy (TQT) was performed. After the TQT, the patient was already in the infirmary bed (with TQT in a tent) where the patient’s pulmonary investigation was continued with bronchoscopy under light sedation (midazolam, propofol and fentanyl). After the procedure, the patient was kept in the post-anesthesia recovery room for approximately sixty minutes and taken to the infirmary bed. Approximately six hours after bronchoscopy, the patient began to experience desaturation, cyanosis of the extremities and central region, and a lower level of consciousness. She was taken to the ICU and maintained on BIPAP support. Bronchoscopy result was negative. During her stay in the ICU, she was diagnosed with two bacterial pneumonias and one viral pneumonia due to Influenza A. The TQT was changed on 06/04/24 and 06/17/24. On 06/19/24, the patient underwent gastrostomy under light sedation using fentanyl 15 mcg, midazolam 3 mg, ketamine 5 mcg without complications and was subsequently sent to the ICU, where she remained without adverse events. Discussion: KSS is a rare mitochondrial disease that is difficult to diagnose early. Little is known about the behavior of the disease in the face of surgical procedures, which is why it is a challenge. In patients diagnosed with KSS, toxic mitochondrial medications should be avoided, such as propofol, aminoglycosides, linezolin, metformin and nucleoside analogues.