Anesthesia for a 16-month-old patient with Prader–Willi syndrome

Prader–Willi syndrome (PWS) is a rare disorder of chromosome abnormalities in which the paternal genes in chromosome 15 are lacking. The clinical course is characterized by hypotonia, hyperphagia, and morbid obesity. Both general and regional anesthesia in these patients is difficult due to morbid o...

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Veröffentlicht in:Journal of anesthesia 2010-12, Vol.24 (6), p.949-950
Hauptverfasser: Meco, Basak C., Alanoglu, Zekeriyya, Cengiz, Ozgur Sinan, Alkis, Neslihan
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Sprache:eng
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Zusammenfassung:Prader–Willi syndrome (PWS) is a rare disorder of chromosome abnormalities in which the paternal genes in chromosome 15 are lacking. The clinical course is characterized by hypotonia, hyperphagia, and morbid obesity. Both general and regional anesthesia in these patients is difficult due to morbid obesity and hypotonia. We report our anesthetic management in a patient with PWS with a body mass index (BMI) of 29.43 kg/m 2 who underwent orchiopexy and hypospadias repair. The clinical course of the patient was uneventful during the procedure and postoperative period. However, arrangements with a pediatric intensive care setting for the postoperative period are recommended for patients with PWS undergoing surgery.
ISSN:0913-8668
1438-8359
DOI:10.1007/s00540-010-1005-3