Anesthesia in Cri du Chat syndrome: Information on 51 Italian patients

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.

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“…However, a similar investigation made for the MWS was done in the same manner for the Cri du Chat syndrome. 20 …”

Section: Discussionmentioning

confidence: 99%

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

Spunton¹,

Garavelli

Mainardi

et al. 2018

Pediatric Reports

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“…[ 1 ] The phenotypic manifestations are variable and often affect multiple organ systems with the most common features being the characteristic high-pitched cat-like cry (hence the name), dysmorphic features, and mental retardation. [ 2 3 ] The reported incidence of CdCs is between 1 in 15,000 and 1 in 50,000 live births,[ 2 3 ] and the children often require surgery for correctable anomalies. [ 3 ] Difficult airway management is a recognized challenge because of dysmorphic changes such as microcephaly, micrognathia, short neck, and the anatomical anomalies of the larynx.…”

mentioning

confidence: 99%

“…[ 2 3 ] The reported incidence of CdCs is between 1 in 15,000 and 1 in 50,000 live births,[ 2 3 ] and the children often require surgery for correctable anomalies. [ 3 ] Difficult airway management is a recognized challenge because of dysmorphic changes such as microcephaly, micrognathia, short neck, and the anatomical anomalies of the larynx. [ 2 3 ] However, there have not been any reports of increased risk or susceptibility to malignant hyperthermia (MH) with this condition.…”

mentioning

confidence: 99%

“…[ 3 ] Difficult airway management is a recognized challenge because of dysmorphic changes such as microcephaly, micrognathia, short neck, and the anatomical anomalies of the larynx. [ 2 3 ] However, there have not been any reports of increased risk or susceptibility to malignant hyperthermia (MH) with this condition. I present a child that developed airway management challenge and possible MH during anesthetic induction.…”

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confidence: 99%

“…Children with CdCs have variable multiple phenotypical manifestations that predispose them to a greater likelihood of surgical intervention and also that have significant anesthetic implications. [ 2 3 ] Diagnosis is clinical with confirmation by molecular cytogenetic tests such as fluorescent in situ hybridization. With increasing and widespread availability of cytogenetic studies, the anesthesia care provider is likely to see increasing numbers of patients with this relatively common chromosomal condition.…”

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confidence: 99%

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