Perioperative management of a patient with Coffin-Lowry syndrome complicated by severe obesity: A case report and literature review.
Perioperative management of a patient with Coffin-Lowry syndrome complicated by severe obesity: A case report and literature review.
复制标题
DOI：
10.1097/md.0000000000009026
复制
发表时间：
2017-12
期刊：
影响因子：
1.6
通讯作者：
Yorozuya T
中科院分区：
文献类型：
--
作者：
Hirakawa M;Nishihara T;Nakanishi K;Kitamura S;Fujii S;Ikemune K;Dote K;Takasaki Y;Yorozuya T
棺材-洛瑞综合征是一种罕见的遗传性疾病，具有特殊的临床特征，如智力低下、面部畸形和心脏异常。特别是，CLS特有的面部特征，包括下颌后倾和大舌头，与呼吸和/或插管困难有关，这是麻醉管理的一个严重问题。然而，有关CLS麻醉处理的病例报道非常有限，到目前为止只有两篇已发表的英文报道。在这个病例报告中，我们讨论了CLS患者的麻醉和术后注意事项，重点是困难的气道管理，并总结了过去的报告，包括一些日本文章。一名25岁的CLS患者由于黄色韧带钙化导致的进行性四肢瘫痪，正计划接受椎板切除术。我们怀疑他的呼吸道管理困难是因为他面部特征的几个因素，计算机断层扫描中甲状腺和胸骨距离短，严重肥胖和睡眠呼吸暂停综合征。怀疑有困难的呼吸道。然而，由于精神发育迟缓，清醒插管被认为是困难的。我们选择纤维支气管镜引导下经鼻气管插管，在适度镇静下使用异丙酚靶控输注维持自主呼吸。麻醉诱导期间进行安全的呼吸道管理。在许多CLS患者中，插管困难，麻醉诱导主要选择镇静或缓慢诱导维持自主呼吸。对于CLS患者，麻醉诱导期间应维持自主呼吸。
Coffin–Lowry syndrome (CLS) is a rare inherited disease with specific clinical features, such as mental retardation, facial dysmorphism, and cardiac abnormality. In particular, the characteristic facial features of CLS, including retrognathia and large tongue, are associated with difficult ventilation and/or intubation, which is a serious problem of anesthesia management. However, case reports on anesthesia management of CLS are very limited as there are only two published English reports till date. In this case report, we discuss anesthetic and postoperative considerations in patients with CLS, focusing on difficult airway management, and summarize past reports including some Japanese articles. A 25-year-old man with CLS was planning to undergo laminectomy because of progressive quadriplegia caused by calcification of the yellow ligament. We suspected difficulty in airway management because of several factors in his facial features, short thyromental and sternomental distances in computed tomography, severe obesity, and sleep apnea syndrome. Difficult airway was suspected. However, because of mental retardation, awake intubation was considered difficult. We selected bronchofiberscope-guided nasotracheal intubation, maintaining spontaneous breathing under moderate sedation with a propofol target-controlled infusion. Airway management was safely performed during anesthesia induction. In many patients with CLS, difficult intubation was reported, and sedation or slow induction maintaining spontaneous breathing was mainly selected for anesthesia induction. Spontaneous breathing should be maintained during anesthesia induction in case of CLS patients.