Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
, 10 (1), 7514
eCollection

Anesthesia in Mowat-Wilson Syndrome: Information on 11 Italian Patients

Affiliations

Anesthesia in Mowat-Wilson Syndrome: Information on 11 Italian Patients

Marianna Spunton et al. Pediatr Rep.

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.

Keywords: Mowat Wilson syndrome; anesthesia-related risk; general anesthesia.

Conflict of interest statement

Conflict of interest: the authors declare no potential conflict of interests.

Figures

Figure 1.
Figure 1.
The age of patients as a percentage. At the time of general anesthesia, the age of patients ranged from month to 21 years.
Figure 2.
Figure 2.
Type of surgery and diagnostic procedures in 11 Italian patients with MWs.

Similar articles

See all similar articles

References

    1. Adam MP, Bean LJH, Ranger Miller V. Mowat-Wilson sindrome. Genereviews www.genetests.org
    1. Bissonnette B, Luginbruehl I, Marciniak B, et al. Syndromes: rapid recognition and perioperative implications. McGraw-Hill, 2006.
    1. Cacheux V, Dastot-Le Moal F, Kääriäinen H, et al. Loss-of-function mutations in SIP1 Smad interacting protein 1 results in a syndromic Hirschsprung disease. Hum Mol Genet 2001;10:1503-10. - PubMed
    1. Cerutti Mainardi P, Pastore G, Zweier C, et al. Mowat-Wilson sindrome and mutation in the zinc finger homeo box 1B gene: a well defined clinical entity J Med Genet 2004;41:e16. - PMC - PubMed
    1. Dastot-Le Moal F, Wilson M, Mowat D, et al. ZFHX1B mutations in patients with Mowat-Wilson syndrome. Hum Mutat 2007:1-9. - PubMed

Grant support

Funding: none.
Feedback