| Summary: | Abstract Background Kagami-Ogata syndrome (KOS) is due to abnormal gene expression in the 14q32.2 imprinted region. Laryngomalacia and bell-shaped thorax of children with KOS can affect airway management of general anesthesia. Case presentation A 12-year-old girl with KOS had a mechanical ventilation history and underwent pectus excavatum repair for cosmetic reasons. Although she had undergone invasive thoracic surgery under general and epidural anesthesia, her respiratory rate and tidal volume were stable with adequate pain control mainly through epidural analgesia at the end of the surgery. We examined her larynx by a bronchoscope. Then, we successfully extubated her after confirming the normal movement of her larynx. Conclusions When patients with KOS undergo pectus excavatum repair, anesthesiologists should prevent postoperative respiratory failure by providing adequate postoperative analgesia. Evaluation of airway patency and respiratory pattern before extubation is critical.
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