Sedation in a Patient with Prader-Willi Syndrome: A Case Report

Abstract

ÖzetPrader Willi sendromu (PWS), hipotoni, gelişme geriliği, karakteristik yüz görünümü, hipogonadizim, hiperfaji ve bunun sonucunda oluşan morbid obezite ile karekterize nadir görülen bir sendromdur. Bu hastalarda anestezi uygulaması, eşlik eden santral hipotoni, deformatif diş yapısı, mikrognati ve kısıtlı boyun hareketliliği gibi sebeplerden dolayı artmış peroperatif risk içermektedir. Bu yüzden PWS'li olgularda genel veya günübirlik anestezi uygulaması esnasında anestezistin en kötü senaryoya göre hazırlıklarını yapması ve hastayı yukarıda bahsettiğimiz nedenlerden dolayı peroperatif yakın takip altında tutması gerekmektedir. Bu olgu sunumunda; PWS'li hastaya elektromyelografi (EMG) Abs tractPrader-Willi syndrome (PWS) is a rare disorder characterized by hypotonia, growth retardation, characteristic face shape, hypogonadism, hyperphagia and related morbid obesity. Anesthesia application in these patients has increased risk of peroperative complication due the central hypotonia, abnormal teeth structure, and limited neck mobility. Therefore, clinicians should be prepared for the worst scenario before anesthesia application in patients with PWS during general or out-patient surgery. Here, in this case report, outpatient anesthesia performed in a patient with PWS for diagnostic electromyography is presented with the literature review. (The Me di cal Bul le tin of Ha se ki 2014; 52: 304-5)

Anesthesia Management in A Case with Prader-Willi Syndrome

“…In these patients, general and regional anesthesia may be more risky than normal due to the perioperative risks arising from hypotonia and morbid obesity, especially in general anesthesia. 4 If access to food and weight gain are not adequately controlled in PWS, diabetes mellitus, right heart failure, hypoventilation, obstructive sleep apnea (OSAS), and narrow airway, GI dysmotility, esophageal dysmotility, delayed gastric emptying may accompany the clinical picture associated with hyperphagia and obesity. The presence of conditions such as water intoxication may make anesthesia management more difficult.…”