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

Marianna Spunton, Livia Garavelli, Paola Cerutti Mainardi, Uta Emmig, Enrico Finale, Andrea Guala
  • Marianna Spunton
    Department of Pediatrics, Castelli Hospital, Verbania, Italy
  • Livia Garavelli
    Clinical Genetics Unit, Obstetric and Pediatric Department, S. Maria Nuova Hospital, Reggio Emilia, Italy
  • Paola Cerutti Mainardi
    Department of Pediatrics, S. Andrea Hospital, Vercelli, Italy
  • Uta Emmig
    Department of Anesthesia, S. Biagio Hospital, Domodossola, Italy
  • Andrea Guala
    Department of Pediatrics, Castelli Hospital, Verbania, Italy

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 semi-structured 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 anesthesia-related 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.

Keywords

Mowat Wilson syndrome; general anesthesia, anesthesia-related risk

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Submitted: 2017-11-30 16:34:00
Published: 2018-03-29 15:52:54
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