title: Anesthesia for pediatric patients with Prader-Willi syndrome: report of two cases.
abstract: Prader-Willi syndrome (PWS) is a sporadic disorder of chromosome abnormalities with an estimated prevalence of 1 in 15,000. It mainly affects the central nervous system, and often involves the hypothalamus. Both general and regional anesthesia for these patients is difficult mainly due to morbid obesity. Other common problems include hypotonia, disturbance in thermoregulation, arrhythmia, cor pulmonale, diabetes mellitus, behavior problems, and convulsions. We report on 2 pediatric patients with PWS receiving general anesthesia. The first patient experienced life-threatening episodes of severe hypoxemia in the postanesthesia care unit (PACU) as well as in the pediatric intensive care unit (PICU). Nasal continuous positive airway pressure (CPAP) was suggested by the pediatric pulmonary medicine specialist, and thereafter the patient's condition improved. The clinical course of the second patient was uneventful except for transient intermittent episodes of bronchospasms during emergence. In addition, we discuss differences between these 2 cases and our strategy for the prevention of perioperative complications for PWS patients in the future.
authors.affiliation: Department of Anesthesiology, Chang Gung Children's Hospital, Taoyuan, Taiwan, R.O.C.
authors.forename: Chi-Hao
authors.initials: CH
authors.lastname: Tseng
authors.forename: Chit
authors.initials: C
authors.lastname: Chen
authors.forename: Chung-Hang
authors.initials: CH
authors.lastname: Wong
authors.forename: Shu-Yam
authors.initials: SY
authors.lastname: Wong
authors.forename: Kit-Man
authors.initials: KM
authors.lastname: Wong
mesh_terms: D000768:Anesthesia, General / Q000009:adverse effects*; D002675:Child, Preschool; D006801:Humans; D007384:Intermittent Positive-Pressure Breathing; D007431:Intraoperative Complications / Q000517:prevention & control; D008297:Male; D011218:Prader-Willi Syndrome / Q000150:complications*