Yamagata Kazuaki, Kawamura Atsushi, Kasai Satomi, Akazawa Mai, Takeda Michiru, Tachibana Kazuya
Department of Dental Anesthesiology, Field of Oral and Maxillofacial Rehabilitation, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima-shi, Kagoshima, 890-8544, Japan.
Department of Anesthesiology, Osaka Women's and Children's Hospital, 840 Murodo-cho, Izumi-shi, Osaka, 594-1101, Japan.
JA Clin Rep. 2018 Aug 31;4(1):62. doi: 10.1186/s40981-018-0199-5.
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.
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.
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.
加贺美 - 绪方综合征（KOS）是一种罕见的先天性印记障碍。与KOS患者麻醉管理相关的问题是呼吸窘迫和困难气管插管。
一名2岁男性计划因双侧隐睾症接受睾丸固定术。尽管他出生后立即有严重呼吸窘迫史，但术前呼吸状况稳定。他还存在明显的气管偏移。在氧气中用氧化亚氮和七氟醚诱导全身麻醉。给予罗库溴铵后插入喉罩气道（LMA）。用七氟醚和同时进行的骶管麻醉维持麻醉。他的术后病程顺利。
KOS患者最好仅在婴儿期后进行择期手术，因为随着年龄增长他们的呼吸状况更稳定。即使是呼吸状况稳定的KOS患者，术前对呼吸道进行全面评估也很重要。
