Isaacson Glenn, Drum Elizabeth T
Department of Otolaryngology - Head & Neck Surgery, Lewis Katz School of Medicine at Temple University, Philadelphia, PA, USA.
Department of Pediatrics, Lewis Katz School of Medicine at Temple University, Philadelphia, PA, USA.
World J Otorhinolaryngol Head Neck Surg. 2018 Jul 13;4(2):122-125. doi: 10.1016/j.wjorl.2018.04.003. eCollection 2018 Jun.
To review current evidence and experience with anesthesia and airway management issues in children and young adults with arthrogryposis.
Review of existing world literature and description of personal experience at a center for children's orthopedic surgery and rehabilitation over 2 decades.
Description of common problems and their solutions in this unusual and diverse group of patients.
Arthrogryposis multiplex congenital includes more than 400 conditions that lead to congenital joint contractures affecting more than one body area. Among the many causes of arthrogryposis, 50%-65% fall into two large categories - amyoplasia and distal arthrogryposis. There is general agreement that best function in children with arthrogryposis is achieved through early mobilization of joint contractures. Children with arthrogryposis average >5 operative procedures during childhood. Anesthesia for these procedures may be complicated by limited jaw mobility and mouth opening, restricted lung development, positioning difficulties, difficult venous access and concerns about increased risk for malignant hyperthermia. 75% of arthrogryposis patients do not have a difficult airway. For those with a history of airway problems or those meeting criteria for a difficult airway, careful advanced planning helps to assure safe and successful surgery. We describe several specialized techniques for endotracheal intubation of children with arthrogryposis.
Children and young adults with arthrogryposis are a diverse group. Many pose unique challenges for airway and surgical management. Review of individual anesthesia records and careful advanced planning by a coordinated, experienced airway team can lead to best outcomes from arthrogryposis surgery.
回顾有关先天性多发性关节挛缩症患儿及青年患者麻醉和气道管理问题的现有证据及经验。
检索世界范围内的现有文献，并描述一家儿童矫形外科及康复中心20多年来的个人经验。
描述这类特殊且多样的患者群体中的常见问题及其解决方法。
先天性多发性关节挛缩症包括400多种导致先天性关节挛缩、影响一个以上身体部位的病症。在关节挛缩症的诸多病因中，50%-65%可归为两大类——缺肢性发育不全和远端关节挛缩症。人们普遍认为，先天性多发性关节挛缩症患儿通过早期松解关节挛缩可实现最佳功能。先天性多发性关节挛缩症患儿在儿童期平均要接受超过5次手术。这些手术的麻醉可能会因下颌活动度和张口受限、肺发育受限、体位摆放困难、静脉穿刺困难以及恶性高热风险增加等问题而变得复杂。75%的先天性多发性关节挛缩症患者不存在气道困难。对于有气道问题病史或符合困难气道标准的患者，仔细的术前规划有助于确保手术安全、成功。我们描述了几种针对先天性多发性关节挛缩症患儿进行气管插管的特殊技术。
先天性多发性关节挛缩症患儿及青年患者群体多样。许多患者在气道和手术管理方面带来独特挑战。回顾个体麻醉记录并由经验丰富的气道团队进行仔细的术前规划，可使先天性多发性关节挛缩症手术取得最佳效果。
