Research Information System
MEDICINE, vol.99, no.38, 2020 (SCI-Expanded)
Introduction: Bardet-Biedl syndrome, which compromises airway management and the cardiovascular and renal systems, is a rare ciliopathic syndrome characterized by multisystem involvement and varying genetic etiologies and clinical manifestations. Patient concerns: A 13-year-old female patient had a history of chronic renal failure, hypothyroidism, mental retardation, hypogonadotropic hypogonadism, obesity, and retinitis pigmentosa and was undergoing 4-hour hemodialysis 3 days a week. Diagnosis: We diagnosed Bardet-Biedl syndrome based on the results of genetic tests. Interventions: We performed renal transplantation under general anesthesia while considering the perioperative risks of airway obstruction and hypothermia. Outcomes: Multidisciplinary preoperative evaluation is crucial to avoid perioperative complications. The risk of an obstructed airway should be considered. Hypothyroidism is a rare consequence of Bardet-Biedl syndrome. Rocuronium and sugammadex are safe for anesthetic management during renal transplantation to address Bardet-Biedl syndrome. Conclusion: Safe anesthetic management can be achieved with the rigorous preoperative assessment of perioperative complications.