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REFSUM HASTALIĞINDA ANESTEZİ UYGULAMASI

ANESTHETIC MANAGEMENT IN REFSUM DISEASE

Journal Name:

  • Selçuk Tıp Dergisi

Publication Year:

  • 2009

Keywords (Original Language):

Author NameUniversity of Author
Abstract (2. Language): 
Refsum disease (RD) is an autosomal recessive familial disorder that usually presents in childhood. In this case report, we examined anaesthetic approach for emergency surgery in a patient with Refsum Disease. A 24- yr- old man with RD presented with an acute abdomen. Past medical history included hypotonia, mental retardation and deafness. Anesthesia was induced intravenously with ketamin, propofol and rocuronium and maintained with sevoflurane in an oxygen / air mixture. The airway was secured after the modified rapid sequence induction (RSI) with Sellick maneuver with an 7 mm tracheal tube and the patent was ventilated with volume controlled mode. Tracheal intubation followed without complication. The anesthesia management was characterized by stabile hemodynamic conditions. The trachea was extubated with the patient awake and all reflexes were returned. The postoperative period was uneventful in the intensive care unit. Refsum Disease may present with different severity of hypotoni with respiratory insufficiency, gastroesophageal reflux, liver dysfunction and the anesthesia strategy should be focussed on the points.
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Abstract (Original Language): 
Genellikle çocukluk döneminde ortaya çıkan Refsum hastalığı (RH) otozomal resesif geçiş gösterir. Bu olguda, acil cerrahi gerektiren RH tanısı olan hastaya uyguladığımız anestezik yaklaşımı sunmayı amaçladık. 24 yaşında Refsum hastalığı tanısı olan, akut batın ön tanısı ile ameliyatı planlanan hastanın anamnezinde hipotoni, mental retardasyon, ve işitme kaybı mevcuttu. Anestezi indüksiyonu intravenöz ketamin, propofol ve roküronyum ile yapılıp Sellick manevrası ile hızlı ardışık indüksiyon sonrası 7 mm’lik trakeal tüple entübe edildi. Anestezi idamesinde sevofluran ve %50 O2- %50 Hava karışımı kullanılan hasta volüm kontrollü modda ventile edildi. Trakeal entübasyon komplikasyonsuz gerçekleştirildi. Anestezik yaklaşım sürecinde stabil hemodinamik durum korundu. Ekstübasyon hasta uyanıkken ve tüm refleksler geri döndükten sonra gerçekleştirildi. Postoperatif periyotda herhangi bir problemle karşılaşılmadı. Refsum hastalığı şiddetli hipotoni, respiratuar yetmezlik, gastroözefagial reflü ve karaciğer yetmezliği gibi farklı tablolar ile ortaya çıktığı için bu noktalar anestezi yönetiminde önemli olmalıdır.
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  • 2
94-97
  • Turkish

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