Background: Kagami-Ogata syndrome (KOS) is a rare congenital imprinting disorder. The problems related to the anesthetic management of patients with KOS are respiratory distress and difficult endotracheal intubation.
Case presentation: A 2-year-old male was scheduled to undergo orchiopexy for bilateral cryptorchidism. Although he had a history of severe respiratory distress immediately after birth, his preoperative respiratory condition was stable. He also had marked tracheal deviation. General anesthesia was induced with nitrous oxide and sevoflurane in oxygen. A laryngeal mask airway (LMA) was inserted following rocuronium administration. Anesthesia was maintained with sevoflurane and simultaneous caudal anesthesia. His postoperative course was uneventful.
Conclusions: Patients with KOS should preferably undergo elective surgery only after infancy because their respiratory status is more stable as they grow older. Thorough preoperative evaluation of the respiratory tract is important even in KOS patients with a stable respiratory condition.
Keywords: Bell-shaped small thorax; Coat-hanger sign; Kagami-Ogata syndrome; Paternal uniparental disomy 14; Respiratory distress; Tracheal deviation.