Perioperative Care of a Patient With Waardenburg Syndrome.
Airway
Anesthesia
Sensorineural hearing loss
Waardenburg syndrome
Journal
Journal of medical cases
ISSN: 1923-4163
Titre abrégé: J Med Cases
Pays: Canada
ID NLM: 101551824
Informations de publication
Date de publication:
Oct 2021
Oct 2021
Historique:
received:
15
07
2021
accepted:
29
07
2021
entrez:
25
10
2021
pubmed:
26
10
2021
medline:
26
10
2021
Statut:
ppublish
Résumé
Waardenburg syndrome is a genetic disorder, resulting in defective control of the division and migration of neural crest cells including the melanocyte lineage during embryonic development. Primary involvement of melanocytes results in the characteristic phenotypic involvement including a white forelock, vitiligo, and heterochromia. Involvement of the organ of Corti leads to sensorineural hearing loss. Involvement of the craniofacial mesenchyme results in abnormal facial features, airway abnormality, and upper limb involvement malformations. Given the potential for end-organ involvement, surgical intervention may be required. Specific concerns during anesthetic care include the potential for difficulties with endotracheal intubation, tracheal involvement, impairment of communication related to deafness, and associated congenital heart disease.
Identifiants
pubmed: 34691332
doi: 10.14740/jmc3751
pmc: PMC8510664
doi:
Types de publication
Case Reports
Langues
eng
Pagination
381-385Informations de copyright
Copyright 2021, Holbrook et al.
Déclaration de conflit d'intérêts
None to declare.
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