
Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation
Author(s) -
Ferda Yaman,
Nedim Çekmen
Publication year - 2020
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000022300
Subject(s) - medicine , transplantation , kidney transplantation , intensive care medicine , surgery
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.