Journals / Haseki Tıp Bülteni / 2014 / Cilt: 52 - Sayı: 4

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

Prader Willi Sendromlu Hastada Sedasyon: Olgu Sunumu

Pages
304–305
DOI
—
🏛️Bibliographic metadata and full text access for this article are provided via DergiPark.
DergiPark Record ↗
✨

AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Here, in this case report, outpatient anesthesia performed in a patient with PWS for diagnostic electromyography is presented with the literature review.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Prader-Willi syndrome (PWS) is a rare disorder characterized by hypotonia, growth retardation, characteristic face shape, hypogonadism, hyperphagia and related morbid obesity.
📊
Key Findings & Data:Anesthesia application in these patients has increased risk of peroperative complication due the central hypotonia, abnormal teeth structure, and limited neck mobility.
💡
Conclusion & Impact:Here, in this case report, outpatient anesthesia performed in a patient with PWS for diagnostic electromyography is presented with the literature review.
🔬Design:Review & Systematic Survey
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Prader-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.

Özet

Prader 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) çekimi için yaptığımız günübirlik anestezi uygulaması güncel literatür eşliğinde tartışılmıştır.

🔖

Cite This Article

DAŞKAYA, H., İNAL, F. Y., TOPTAŞ, M., YAZICI, Ü. (2014). Sedation in a Patient with Prader-Willi Syndrome: A Case Report. Haseki Tıp Bülteni, Cilt: 52 - Sayı: 4, 304–305. https://ajindex.com/articles/267892

📄 Full Text PDF Viewer

📄

PDF görüntüleyici hazırlanıyor...

References (7)

  1. Cassidy SB, McCandless SE. Prader-Willi syndrome. In: Cassidy SB, Allanson JE (eds). Management of Genetic Syndromes (2nd ed). New Jersey: Wiley-Liss 2005: 429-48
  2. Sıklar Z, Berberoğlu M. Syndromic disorders with short stature. J Clin Res Pediatr Endocrinol 2014;6:1-8
  3. Meco BC, Alanoglu Z, Cengiz OS, ve ark. Anesthesia for a 16-month-old patient with Prader-Willi syndrome. J Anesth 2010;24:949-50
  4. Legrand R, Tobias JD. Anesthesia and Prader-Willi syndrome: preliminary experience with regional anesthesia. Paediatr Anaesth 2006;7:712-22
  5. Mackenzie JW. Anaesthesia and the Prader-Willi syndrome. J R Soc Med 1991;84:239 6. Lirk P, Keller C, Rieder J et al. Anaesthetic management of the Prader-Willi syndrome. Eur J Anaesthesiol 2004;21:831-33
  6. Helfaer MA, Wilson MD. Obstructive sleep apnea, control of ventilation, and anesthesia in children. Pediatr Clin North Am 1994;41:131-51
  7. Mayhew JF, Taylor BT. Anaesthetic considerations in PraderWilli syndrome. Can Anaesth Soc J 1983;5:565-66.