A 7-month-old boy with Pena-Shokeir syndrome I underwent surgery under general anesthesia for the correction of inguinal hernia. Patients with this syndrome may present anesthetic problems involving difficulties in endotracheal intubation due to micrognathia, cleft palate and neck rigidity, as well as perioperative respiratory complications that relate to hypoplasia of the lung. General anesthesia was induced with sevoflurane (1-3%) and nitrous oxide in the oxygen, and we attempted to insert a size 1 laryngeal mask airway (LMA). The LMA was smoothly inserted and lung ventilation during the operation was assisted without any complications. After recovering from the anethetize, we removed the LMA and his postoperative recovery was uneventful. In this case, LMA is very useful for airway management.
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