Anesthetic Management of Syndactyly Operation for A Patient with Apert Syndrome
Begüm Nemika Gökdemir, Nedim Çekmen
Başkent Üniversitesi Ankara Hastanesi, Anesteziyoloji ve Reanimasyon Bilim Dalı
Giriş: Apert syndrome (AS) is autosomal dominant disease characterized by multiple craniofacial and limb deformities like syndactyly of feet and hands. We aim to highlight anesthetic considerations for AS and how to maintain anesthesia during the operation.
Olgu: A 10-year-old girl came into operation room for syndactyly repairment. She has no comorbidities other than AS. Her weight is 18 kg, ASA score was II. Her laboratory results, echocardiography and electrocardiogram were normal, but physical examination showed enlargement of the head and hypertelorism, flat and broad forehead, depressed nasal root, small mouth, micrognathia, and syndactyly of all extremities. Her mallampati grade was I, but because of her craniofacial anomalies, we predicted chance of difficult intubation and difficult airway cart which has different size of LMA, ETT, gum bougie, stile and videolaryngoscope was kept ready. Oral and written consent was obtained from the patient’s family. She was taken theOR where standard monitoring was instated. We inserted intravenous access line after multiple attempts. Anesthesia induction was maintained with lidocaine, propofol, fentanyl, rocuronium. After 3 min. preoxygenation, with cricoid pressure was applied and we intubated the patient with 5.5 endotracheal tube using direct laryngoscope at first attempt. Due to craniofacial anomalies, difficult intubation cart kept ready. Anesthesia maintenance was provided by infusion of remifentanil and inhaler sevoflurane and oxygen air mixture. Surgery was accomplished uneventful. Extubation was performed after she was able to breathe spontaneously. She was taken to the ward when she reached Aldrete score 8 from PACU.
Tartışma ve Sonuç: Anesthetic management of a patient with AS is challenging for anesthetist. So, comprehensive preoperative evaluation and careful anesthesia plan are imperative for these population. Because of the complexity of the syndrome a multidisciplinary approach and managing accordingly are essential in treating the psychological, aesthetic and functional problems.
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