Aysun Ankay Yılbaş, Bengisu Ercan, Özgür Canbay, Ümitcan Ünver

Emanuel Sendromlu Çocuk Hastada Anestezik Yaklaşım

Emanuel sendromu; multipl konjenital anomaliler, kraniofasial dismorfizm, ciddi gelişme geriliği ve mental retardasyon ile karakterize oldukça ender, herediter resesif bir hastalıktır. Biz bu olgu sunumunda, izole yarık damak ameliyatı geçiren Emanuel sendromlu pediatrik hastamızdaki anestezi yaklaşımımızı tartışmayı amaçladık. Hastanın anestezi yönetiminde total intravenöz anestezi tercih edildi. Endotrakeal entübasyon videolaringoskopi ile gerçekleştirildi. Sorunsuz intraoperatif takibin ardından, ekstübasyon sonrası solunum sıkıntısı gelişmesi üzerinde hasta yine entübe edilerek yoğun bakım ünitesine devredildi. Hastamızda olduğu gibi, eşlik eden mikro- retrognati nedeniyle anesteziyologlar zor hava yolu olasılığına hazır olmalıdır. Ayrıntılı bir preope- ratif değerlendirme, zor hava yolu hazırlığı ve dikkatli hemodinamik monitorizasyon ile bu ender hastalığın anestezi yönetiminde sorunlar en aza indirgenebilir

Anesthetic Management of a Pediatric Patient with Emanuel Syndrome

Emanuel syndrome is a very rare recessive hereditary disorder characterized by multiple con- genital anomalies, craniofacial dysmorphism, significant developmental delay and mental retar- dation. In this case report, we discussed our anesthetic management of a pediatric patient with Emanuel Syndrome undergoing isolated cleft palate repair operation. General anesthesia was maintained with total intravenous anesthetic techniques. Videolaryngoscopy was used for endo- tracheal intubation. The patient was extubated at the operating room following an uneventful intraoperative period, however she developed respiratory distress and was reintubated and transferred to pediatric intensive care unit. Anesthesiologists should be prepared for difficult airway management because of concomitant micro-retrognathia, as in our patient. Most of the problems regarding anesthetic management of this extremely rare syndrome can be reduced by a detailed preoperative assesment, preparation for diffucult airway and a careful hemodynamic monitoring

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