Ammer Luise Sophie, Muschol Nicole Maria, Santer René, Lang Annika, Breyer Sandra Rafaela, Sasu Phillip Brenya, Petzoldt Martin, Dohrmann Thorsten
International Center for Lysosomal Disorders (ICLD), Department of Paediatrics, University Medical Centre Hamburg-Eppendorf, 20246 Hamburg, Germany.
Department of Anaesthesiology, University Medical Centre Hamburg-Eppendorf, 20246 Hamburg, Germany.
J Clin Med. 2022 Jun 24;11(13):3650. doi: 10.3390/jcm11133650.
Mucolipidosis (ML) type II, intermediate, and III are lysosomal storage disorders with progressive multiorgan manifestations predisposing patients to a high risk of perioperative morbidity. The aims of the study were to systematically assess disease manifestations relevant to anaesthesia as well as anaesthesia-related complications. This retrospective study includes ML patients who underwent anaesthesia in two centres between 2008 and 2022. We reviewed patients' demographics, medical history, disease manifestations, as well as procedure- and outcome-related data. A total of 12 patients (7 MLII, 2 ML intermediate, 3 MLIII) underwent 44 anaesthesia procedures (per patient: median 3, range 1-11). The median age was 3.3 years (range 0.1-19.1). At least one complication occurred in 27.3% of the anaesthesia procedures. The vast majority of complications (94%) occurred in children with MLII and ML intermediate. A predicted difficult airway was found in 100% and 80% of the MLII and ML intermediate patients, respectively. Accordingly, most complications (59%) occurred during the induction of anaesthesia. Altogether, respiratory complications were the most frequent (18%), followed by difficult airway management (14%). The risk for anaesthesia-related complications is alarmingly high in patients with ML, particularly in those with MLII and ML intermediate. Multidisciplinary risk-benefit analysis and thoughtful anaesthesia planning are crucial in these patients.
II型、中间型和III型粘脂贮积症（ML）是溶酶体贮积症，具有进行性多器官表现，使患者围手术期发病风险很高。本研究的目的是系统评估与麻醉相关的疾病表现以及麻醉相关并发症。这项回顾性研究纳入了2008年至2022年期间在两个中心接受麻醉的ML患者。我们回顾了患者的人口统计学资料、病史、疾病表现以及与手术和结局相关的数据。共有12例患者（7例MLII型、2例中间型ML、3例MLIII型）接受了44次麻醉手术（每位患者：中位数3次，范围1 - 11次）。中位年龄为3.3岁（范围0.1 - 19.1岁）。27.3%的麻醉手术发生了至少一种并发症。绝大多数并发症（94%）发生在MLII型和中间型ML的儿童患者中。分别在100%和80%的MLII型和中间型ML患者中发现预测的困难气道。因此，大多数并发症（59%）发生在麻醉诱导期间。总体而言，呼吸并发症最为常见（18%），其次是困难气道管理（14%）。ML患者麻醉相关并发症的风险高得惊人，尤其是MLII型和中间型ML患者。多学科风险效益分析和精心的麻醉计划对这些患者至关重要。
