Anesthesia Management in A Case with Prader-Willi Syndrome

Year 2023, Volume: 8 Issue: 3, 410 - 412, 06.09.2023

Yeliz Tunç , Erol Karaaslan , Ahmet Selim Özkan

https://doi.org/10.26453/otjhs.1159374

Abstract

Prader Willi (PWS) is the most common and rare genetic cause of obesity. Airway problems associated with obesity and hypotonia, increased risk of aspiration due to gastrointestinal (GI) motility and hyperphagia, obstructive sleep apnea syndrome (OSAS), difficult airway management, and postoperative respiratory failure risk due to narrow airway are the factors that complicate anesthesia management. It should be noted that there are many anatomical and physiological factors that may adversely affect perioperative anesthesia management in patients with PWS. For this reason, preoperative anesthesia evaluation and all preparations should be performed completely.

Keywords

Anesthesia management, difficult airway, prader-willi syndrome

Thanks

Doç. Dr. Sedat AkbaşSedat Akbaş

Prader Willi Sendromlu Bir Olguda Anestezi Yönetimi

Abstract

Prader Willi (PWS), obezitenin en yaygın ve nadir görülen genetik nedenidir. Obezite ve hipotoni ile ilişkili hava yolu sorunları, gastrointestinal (GI) motilite ve hiperfaji nedeniyle artan aspirasyon riski, obstrüktif uyku apne sendromu (OUAS), zor hava yolu yönetimi, dar hava yoluna bağlı postoperatif solunum yetmezliği riski anestezi yönetimini zorlaştıran faktörlerdir. PWS'li hastalarda perioperatif anestezi yönetimini olumsuz etkileyebilecek birçok anatomik ve fizyolojik faktör olduğu unutulmamalıdır. Bu nedenle ameliyat öncesi anestezi değerlendirmesi ve tüm hazırlıklar eksiksiz yapılmalıdır.

Keywords

Anestezi yönetimi, prader-willi sendromu, zor havayolu

References

• Butler MG, Miller JL, Forster JL. Prader willi syndrome-clinical genetics, diagnosis and treatment approaches: An Update. Curr Pediatr Rev. 2019;15(4):207-244. doi:10.2174/1573396315666190716120925
• Meco BC, Alanoglu Z, Cengiz OS, Alkis N. Anesthesia for a 16- month-old patient with prader-willi syndrome. J Anesth. 2010;24:949-950. doi:10.1007/s00540-010-1005-3
• Tuncalı B, Zeyneloğlu P, Yılmaz T. Anesthesia application in a morbid obese case with prader willi syndrome. Journal of anesthesia. 2015;23(4):261-266.
• Daşkaya H, Yılmaz İnal F, Toptaş M, Yazıcı Ü. Sedation in a patient with prader-willi syndrome: A Case Report. Med Bull Haseki. 2014;52:304-305.
• Tseng CH, Chen C, Wong CH, Wong SY, Wong KM. Anesthesia for pediatric patients with prader–willi syndrome: report of two cases. Chang Gung Med J. 2003;26:453–457.