Department of Anaesthesia, Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust, Manchester, UK.
Paediatr Anaesth. 2021 Mar;31(3):260-267. doi: 10.1111/pan.14090. Epub 2020 Dec 12.
Mucolipidosis (ML) II and III are complex lysosomal storage disorders characterized by progressive multisystem pathology which can pose challenges to the anesthetist and increase the risks associated with general anesthesia. We sought to review the management of patients with ML II and III undergoing anesthesia in our institution in order to better define recommendations for the preoperative assessment and optimization of these children. We further elected to analyze the conduct of anesthesia, intraoperative management, and perioperative complications that our patients had experienced in order to allow improved informed consent and anesthetic planning. We performed a retrospective examination of the medical notes of those patients who had undergone anesthesia in our institution to identify their clinical features, anesthetic technique, airway management, and perioperative complications. Five children underwent 11 episodes of anesthesia. Fiber-optic or videolaryngoscopy was utilized in six out of seven intubations, with four out of seven requiring a change from the method initially chosen to enable intubation. Four of the five patients had an abnormal echocardiogram. Three patients had radiological evaluation of their cervical spine, with two demonstrating abnormalities. One patient had changes suggesting instability at the atlantoaxial junction. Children and babies with ML II and III present multisystem challenges to the anesthetist. Multidisciplinary planning and assessment, followed by a discussion of risk, should proceed any elective surgery. These complex children should undergo elective anesthesia delivered by an experienced (pediatric) anesthetist in an appropriate tertiary center with on-site pediatric ENT and critical care support.
黏脂贮积症（ML）II 型和 III 型是复杂的溶酶体贮积症，其特征为进行性多系统病变，这可能给麻醉师带来挑战，并增加全身麻醉相关的风险。我们旨在回顾在我院接受麻醉的 ML II 型和 III 型患者的管理情况，以便更好地确定这些儿童术前评估和优化的建议。我们还选择分析我们的患者在麻醉期间的麻醉管理、术中管理和围手术期并发症，以允许更好地知情同意和麻醉计划。我们对在我院接受麻醉的患者的病历进行了回顾性检查，以确定其临床特征、麻醉技术、气道管理和围手术期并发症。五名儿童接受了 11 次麻醉。七次插管中有六次使用了纤维光学或视频喉镜，七次中有四次需要从最初选择的方法改为能够插管。五名患者中有四名有异常的超声心动图。三名患者对其颈椎进行了影像学评估，其中两名有异常。一名患者的寰枢关节处有变化提示不稳定。患有 ML II 型和 III 型的儿童和婴儿对麻醉师提出了多系统挑战。任何择期手术前，都应进行多学科计划和评估，然后讨论风险。这些复杂的儿童应由有经验的（儿科）麻醉师在有现场儿科耳鼻喉科和重症监护支持的适当三级中心进行择期麻醉。
