Case report: Single-stage facial reanimation with bilateral lengthening temporalis myoplasties for immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome related developmental facial palsy.
Facial animation
Facial palsy
IPEX syndrome
Myopathic facies
Temporalis myoplasty
Journal
JPRAS open
ISSN: 2352-5878
Titre abrégé: JPRAS Open
Pays: Netherlands
ID NLM: 101680420
Informations de publication
Date de publication:
Jun 2023
Jun 2023
Historique:
received:
12
01
2023
accepted:
26
02
2023
medline:
4
4
2023
entrez:
3
4
2023
pubmed:
4
4
2023
Statut:
epublish
Résumé
Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare primary immunodeficiency, typically associated with clinical features of intractable diarrhoea, type 1 diabetes mellitus and eczema. We present a case of IPEX syndrome referred to our regional facial palsy service for smile restoration surgery. The patient presented with dissatisfaction of facial appearance, including mask-like facies and no functional smile. Pre-operative electromyography confirmed normal temporalis muscle activation. Consequently, the patient was offered single-stage bilateral lengthening temporalis myoplasties. The patient reported improved satisfaction with facial appearance. Surgery resulted in good early resting and voluntary symmetry. Oral commissures were elevated at rest improving oral incompetence. This is the first description of facial animation surgery in the context of IPEX syndrome. With careful consideration and patient selection, successful surgical restoration of resting symmetry and dynamic commissural smile can be achieved in this complex cohort of patients.
Identifiants
pubmed: 37009631
doi: 10.1016/j.jpra.2023.02.003
pii: S2352-5878(23)00006-2
pmc: PMC10060169
doi:
Types de publication
Case Reports
Langues
eng
Pagination
19-23Informations de copyright
© 2023 The Authors. Published by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive and .
Déclaration de conflit d'intérêts
None declared.
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