From the *Department of Anesthesiology, †Mayo Clinic School of Medicine, and ‡Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota.
Anesth Analg. 2017 Mar;124(3):908-914. doi: 10.1213/ANE.0000000000001822.
Arthrogryposis syndromes are a heterogeneous group of disorders characterized by congenital joint contractures often requiring multiple surgeries during childhood to address skeletal and visceral abnormalities. Previous reports suggest that these children have increased perioperative risk, including hypermetabolic events discrete from malignant hyperthermia, difficult airway management, isolated hyperthermia, and difficult IV line placement. We sought to compare children with arthrogryposis multiplex congenita (AMC) versus the less severe, distal arthrogryposis syndromes (DAS) and to evaluate possible intraoperative hyperthermia of patients with AMC. We hypothesized that children with AMC had a greater incidence of intraoperative hyperthermia and more difficulty with airway management and IV access.
Children aged 0 to 25 years with arthrogryposis syndromes who underwent anesthesia from 1972 to 2013 were identified. The medical records were reviewed for demographics, arthrogryposis type, and anesthetic complications. AMC subjects were compared with DAS subjects. To evaluate the probability of hyperthermia and hypermetabolic responses of patients with AMC, we performed a post hoc case-control analysis. Patients with AMC were matched in a 1:2 ratio to patients without arthrogryposis to evaluate the primary outcome of maximum intraoperative temperature.
Forty-five patients with AMC and 16 patients with DAS underwent 264 and 105 unique anesthetics, respectively. There was no significant difference in intraoperative hyperthermia or hypermetabolic events (odds ratio [OR], 0.94; 95% confidence interval [CI], 0.36-2.47; P = .90). Children with AMC were more likely to have difficult IV access (OR, 7.1; 95% CI, 1.81-27.90; P = .005). Additional evidence suggested that difficult airway management (OR, 4.06; 95% CI, 1.01-16.39; P = .049) and hemodynamic instability (OR, 4.22; 95% CI, 1.03-17.26; P = .045) were more likely in children with AMC. From post hoc case-control analysis, there was no significant difference in the mean maximum intraoperative temperature (estimated difference +0.04°C; 95% CI, -0.14 to +0.22; P = .64) or odds of intraoperative hyperthermia (OR, 1.49; 95% CI, 0.78-2.82; P = .223) for patients with AMC compared with control subjects.
Children with arthrogryposis syndromes present challenges to the anesthesia and surgical teams, including greater neuromuscular disease burden and challenging peripheral IV placement, with additional evidence suggesting difficult airway management and intraoperative hemodynamic instability. Although more definitive studies are warranted, we did not find evidence of increased odds of intraoperative hyperthermia or hypermetabolic responses.
关节挛缩症是一组先天性关节挛缩的异质性疾病，常需要在儿童时期进行多次手术以解决骨骼和内脏异常。先前的报告表明，这些儿童的围手术期风险增加，包括与恶性高热不同的代谢亢进事件、气道管理困难、孤立性高热和静脉置管困难。我们旨在比较多发性关节挛缩症（AMC）患儿与病情较轻的远端关节挛缩症（DAS）患儿，并评估 AMC 患儿可能发生的术中高热。我们假设 AMC 患儿术中高热发生率更高，气道管理和静脉通路建立更困难。
我们回顾了 1972 年至 2013 年间接受麻醉的 0 至 25 岁关节挛缩症患儿的病历，以评估其人口统计学、关节挛缩症类型和麻醉并发症。将 AMC 患儿与 DAS 患儿进行比较。为了评估 AMC 患儿发生高热和代谢亢进反应的概率，我们进行了事后病例对照分析。将 AMC 患儿按照 1:2 的比例与无关节挛缩症患儿相匹配，以评估 AMC 患儿术中最高体温这一主要结局。
45 例 AMC 患儿和 16 例 DAS 患儿分别接受了 264 次和 105 次独特的麻醉。术中高热或代谢亢进事件无显著差异（比值比[OR]，0.94；95%置信区间[CI]，0.36-2.47；P =.90）。AMC 患儿更有可能出现静脉置管困难（OR，7.1；95%CI，1.81-27.90；P =.005）。进一步的证据表明，气道管理困难（OR，4.06；95%CI，1.01-16.39；P =.049）和血流动力学不稳定（OR，4.22；95%CI，1.03-17.26；P =.045）更可能发生在 AMC 患儿中。从事后病例对照分析来看，与对照组相比，AMC 患儿的术中最高体温平均值差异无统计学意义（估计差值+0.04°C；95%CI，-0.14 至 +0.22；P =.64）或术中高热的比值比（OR，1.49；95%CI，0.78-2.82；P =.223）。
关节挛缩症患儿给麻醉和外科团队带来挑战，包括更大的神经肌肉疾病负担和外周静脉置管困难，进一步的证据表明气道管理困难和术中血流动力学不稳定。尽管需要更明确的研究，但我们没有发现术中高热或代谢亢进反应的几率增加的证据。
