Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report.

Medicine (Baltimore)

Department of Anesthesiology and Reanimation, Istanbul Nisantasi University, İstanbul, Turkey.

Published: September 2020

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.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7505336PMC
http://dx.doi.org/10.1097/MD.0000000000022300DOI Listing

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