Cargando…
Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report
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...
Autores principales: | , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Lippincott Williams & Wilkins
2020
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7505336/ https://www.ncbi.nlm.nih.gov/pubmed/32957390 http://dx.doi.org/10.1097/MD.0000000000022300 |
_version_ | 1783584789039677440 |
---|---|
author | Yaman, Ferda Çekmen, Nedim |
author_facet | Yaman, Ferda Çekmen, Nedim |
author_sort | Yaman, Ferda |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-7505336 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Lippincott Williams & Wilkins |
record_format | MEDLINE/PubMed |
spelling | pubmed-75053362020-09-24 Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report Yaman, Ferda Çekmen, Nedim Medicine (Baltimore) 3300 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. Lippincott Williams & Wilkins 2020-09-18 /pmc/articles/PMC7505336/ /pubmed/32957390 http://dx.doi.org/10.1097/MD.0000000000022300 Text en Copyright © 2020 the Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0 (https://creativecommons.org/licenses/by/4.0/) |
spellingShingle | 3300 Yaman, Ferda Çekmen, Nedim Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report |
title | Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report |
title_full | Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report |
title_fullStr | Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report |
title_full_unstemmed | Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report |
title_short | Anesthetic management of a patient with Bardet-Biedl syndrome undergoing renal transplantation: A case report |
title_sort | anesthetic management of a patient with bardet-biedl syndrome undergoing renal transplantation: a case report |
topic | 3300 |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7505336/ https://www.ncbi.nlm.nih.gov/pubmed/32957390 http://dx.doi.org/10.1097/MD.0000000000022300 |
work_keys_str_mv | AT yamanferda anestheticmanagementofapatientwithbardetbiedlsyndromeundergoingrenaltransplantationacasereport AT cekmennedim anestheticmanagementofapatientwithbardetbiedlsyndromeundergoingrenaltransplantationacasereport |