Abstract
BackgroundKagami-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 presentationA 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.ConclusionsWhen 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.
Highlights
Kagami-Ogata syndrome (KOS) is due to abnormal gene expression in the 14q32.2 imprinted region
Yamagata K et al reported the successful anesthetic management of 2-year-old child with KOS complicated with marked tracheal deviation and small bell-shaped thorax who underwent orchiopexy for bilateral cryptorchidism [3]
The following report describes an older child with KOS who underwent general anesthesia for pectus excavatum repair
Summary
When patients with KOS undergo pectus excavatum repair, anesthesiologists should prevent postoperative respiratory failure by providing adequate postoperative analgesia.
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