Anesthetic management of a child with Kagami-Ogata syndrome complicated with marked tracheal deviation: a case report
Anesthetic management of a child with Kagami-Ogata syndrome complicated with marked tracheal deviation: a case report
复制标题
DOI：
10.1186/s40981-018-0199-5
复制
发表时间：
2018-08-31
影响因子：
0.9
通讯作者：
Tachibana, Kazuya
中科院分区：
文献类型：
--
作者：
Yamagata, Kazuaki;Kawamura, Atsushi;Tachibana, Kazuya
背景：Kagami-Ogata 综合征（KOS）是一种罕见的先天性印记障碍。 KOS 患者的麻醉管理相关问题是呼吸窘迫和气管插管困难。病例介绍：一名 2 岁男性因双侧隐睾而计划接受睾丸固定术。虽然出生后即有严重呼吸窘迫病史，但术前呼吸状况稳定。他还存在明显的气管偏差。用一氧化二氮和七氟醚在氧气中诱导全身麻醉。给予罗库溴铵后插入喉罩气道（LMA）。用七氟烷维持麻醉并同时进行尾部麻醉。术后过程很顺利。结论：KOS 患者最好在婴儿期后才进行择期手术，因为随着年龄的增长，他们的呼吸状态会更加稳定。即使对于呼吸状况稳定的 KOS 患者，术前彻底评估呼吸道也很重要。
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.