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Beckwith-Wiedemann Sendromlu Olguya Anestezik Yaklaşım: Olgu sunumu

Anesthetic Management of Beckwith-Wiedemann Syndrome Case: Report

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Abstract (2. Language): 
Beckwith-Wiedemann syndrome consists of various abnormalities, such as exomphalos, macroglossia, hypoglcemia, visceromegaly, omphalocele, gigantism. Patients with Beckwith-Wiedemann syndrome frequently require surgical intervention during infant period. Common problems associated with anesthetic management of Beckwith- Wiedemann syndrome are difficult airway and hypoglycemia. Macroglossia might cause difficult ventilation and intubation for that reason preparation for airway difficulty should be made before induction. Intraoperative plasma glucose monitoring is particularly important to prevent the neurologic sequelae. In this presentation, we addressed the use of laryngeal mask may be an alternation to the endotracheal tube patients with Beckwith-Wiedemann which consist potentially difficult airway anatomy.
Abstract (Original Language): 
Beckwith-Wiedemann sendromu; eksofalos, makroglossi, hipoglisemi, organomegali, omfalosel ve gigantizim gibi çeşitli anomalileri içerir. Beckwith-Wiedemann sendromlu olgular sıklıkla infant dönemde cerrahiye gereksinim duyarlar. Anestezi yönetimindeki temel problemler sıklıkla; zor hava yolu ve hipoglisemidir. Makroglossinin maske ventilasyonu ve entübasyonu zorlaştırabileceği akılda tutularak indüksiyondan önce zor hava yolu hazırlığı yapılmalıdır. Dikkatli intraoperatif glukoz takibi hipogliseminin neden olacağı nörolojik sekelleri önlemede oldukça önemlidir. Bu sunuda laringeal maskenin potansiyel zor hava yolu anatomisine sahip Beckwith-Wiedemann sendromlu olguda endotrakael tüpe alternatif olarak kullanılabileceğine değindik.
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