Anesthesia in Mowat-Wilson syndrome: Information on 11 Italian patients

Marianna Spunton, Livia Garavelli, Paola Cerutti Mainardi, Uta Emmig, Enrico Finale, Andrea Guala

Research output: Contribution to journalArticle

Abstract

Mowat-Wilson syndrome is a genetic disease caused by heterozygous mutations or deletions of the ZEB2 gene and characterized by typical clinical features. The congenital malformations typical of this syndrome call for early diagnostic and surgical procedures requiring general anesthesia, but few information about the anesthesiology management of such patients is available. We enrolled 11 families of patients with Mowat-Wilson syndrome who had undergone surgical or diagnostic procedures requiring general anesthesia, and sent them a retrospective questionnaire including 16 open questions about the procedures. They were further contacted by phone for a semistructured interview. A total of 37 procedures requiring general anesthesia was reported in 11 patients. Only two patients reported anesthesia-related complications during the procedure. No true additional anesthesiarelated risk was present for the patients with MW syndrome, besides difficult intubation, weaning and lower respiratory tract infection. Perception of risk, however, is derived by non-medical observation on the part of the parents.

Original languageEnglish
Pages (from-to)14-16
Number of pages3
JournalPediatric Reports
Volume10
Issue number1
DOIs
Publication statusPublished - Jan 1 2018

Fingerprint

Anesthesia
General Anesthesia
Inborn Genetic Diseases
Anesthesiology
Sequence Deletion
Gene Deletion
Weaning
Intubation
Respiratory Tract Infections
Parents
Mowat-Wilson syndrome
Interviews

Keywords

  • General anesthesia, anesthesia-related risk
  • Mowat Wilson syndrome

ASJC Scopus subject areas

  • Pediatrics

Cite this

Spunton, M., Garavelli, L., Mainardi, P. C., Emmig, U., Finale, E., & Guala, A. (2018). Anesthesia in Mowat-Wilson syndrome: Information on 11 Italian patients. Pediatric Reports, 10(1), 14-16. https://doi.org/10.4081/pr.2018.7514

Anesthesia in Mowat-Wilson syndrome : Information on 11 Italian patients. / Spunton, Marianna; Garavelli, Livia; Mainardi, Paola Cerutti; Emmig, Uta; Finale, Enrico; Guala, Andrea.

In: Pediatric Reports, Vol. 10, No. 1, 01.01.2018, p. 14-16.

Research output: Contribution to journalArticle

Spunton, M, Garavelli, L, Mainardi, PC, Emmig, U, Finale, E & Guala, A 2018, 'Anesthesia in Mowat-Wilson syndrome: Information on 11 Italian patients', Pediatric Reports, vol. 10, no. 1, pp. 14-16. https://doi.org/10.4081/pr.2018.7514
Spunton M, Garavelli L, Mainardi PC, Emmig U, Finale E, Guala A. Anesthesia in Mowat-Wilson syndrome: Information on 11 Italian patients. Pediatric Reports. 2018 Jan 1;10(1):14-16. https://doi.org/10.4081/pr.2018.7514
Spunton, Marianna ; Garavelli, Livia ; Mainardi, Paola Cerutti ; Emmig, Uta ; Finale, Enrico ; Guala, Andrea. / Anesthesia in Mowat-Wilson syndrome : Information on 11 Italian patients. In: Pediatric Reports. 2018 ; Vol. 10, No. 1. pp. 14-16.
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