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

Abstract:

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.

journal_name

Medicine (Baltimore)

journal_title

Medicine

authors

Yaman F,Çekmen N

doi

10.1097/MD.0000000000022300

subject

Has Abstract

pub_date

2020-09-18 00:00:00

pages

e22300

issue

38

eissn

0025-7974

issn

1536-5964

pii

00005792-202009180-00087

journal_volume

99

pub_type

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