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Anesthesia for Pediatric Patients with Prader-Willi Syndrome: Report of Two Cases

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Author(s): Chi-Hao Tseng | Chit Chen | Chung-Hang Wong | Shu-Yam Wong | Kit-Man Wong

Journal: Chang Gung Medical Journal
ISSN 2072-0939

Volume: 26;
Issue: 06;
Start page: 453;
Date: 2003;
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Keywords: Prader-Willi syndrome | obesity | obstructive sleep apnea syndrome | polysomnogram | continuous positive airway pressure

ABSTRACT
Prader-Willi syndrome (PWS) is a sporadic disorder of chromosome abnormalities withan estimated prevalence of 1 in 15,000. It mainly affects the central nervous system, andoften involves the hypothalamus. Both general and regional anesthesia for these patients isdifficult mainly due to morbid obesity. Other common problems include hypotonia, disturbancein 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 postanesthesiacare unit (PACU) as well as in the pediatric intensive care unit (PICU). Nasal continuouspositive airway pressure (CPAP) was suggested by the pediatric pulmonary medicinespecialist, and thereafter the patient's condition improved. The clinical course of the secondpatient was uneventful except for transient intermittent episodes of bronchospasms duringemergence. In addition, we discuss differences between these 2 cases and our strategy forthe prevention of perioperative complications for PWS patients in the future.

Tango Jona
Tangokurs Rapperswil-Jona

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