Anesthesia in Mowat-Wilson syndrome: information on 11 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 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

ECMO for intractable status asthmaticus following atracurium

Intraoperative allergic reactions are rare but serious events associated with increased morbidity and mortality. We report the salvage of intraoperative anaphylaxis leading to extreme hypercapnic respiratory failure by veno-venous extracorporeal membrane oxygenation (ECMO). A 38-year-old woman undergoing thyroidectomy developed intractable bronchospasm after administration of atracurium, leading to extreme hypercapnic respiratory failure (PaCO2\uc2\ua0>\uc2\ua0250\uc2\ua0mmHg, pH 6.773). After the failure of conventional medical therapy and ventilatory optimization, the patient was connected to a veno-venous ECMO circuit. PaCO2of 45.6\uc2\ua0mmHg and pH of 7.25 were achieved in 1\uc2\ua0h, by slowly increasing sweep gas flows up to 3.5 L/min and using continuous end-tidal CO2monitoring to gauge the procedure. After extubation and disconnection from ECMO, the patient was discharged on the 6th day without sequelae. Rapid reversal of extreme hypercapnic acidosis by ECMO was feasible, without any neurologic sequelae. Veno-venous ECMO support may be a valuable option for the salvage of intraoperative anaphylaxis