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 obesity and hypotonia. We report our anesthetic management in a patient with PWS with a body mass index (BMI) of 29.43 kg/m² 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.
Meco
Basak C
BC
Department of Anesthesiology and ICM, Faculty of Medicine, Ankara University, Mesa Koza sitesi, 2.blok no:15, GOP, 06700 Ankara, Turkey. basakceyda@hotmail.com
Alanoglu
Zekeriyya
Z
Cengiz
Ozgur Sinan
OS
Alkis
Neslihan
N
D000768:Anesthesia, General; D015992:Body Mass Index; D006801:Humans; D006963:Hyperphagia / Q000209:etiology; D007021:Hypospadias / Q000601:surgery; D007223:Infant; D008297:Male; D016343:Monitoring, Intraoperative; D009123:Muscle Hypotonia / Q000209:etiology / Q000503:physiopathology; D009767:Obesity, Morbid / Q000209:etiology / Q000503:physiopathology; D056126:Orchiopexy; D010100:Oxygen / Q000097:blood; D011218:Prader-Willi Syndrome / Q000601:surgery*
D010100:Oxygen