Difficult airway management in children and young adults with arthrogryposis

Objective To review current evidence and experience with anesthesia and airway management issues in children and young adults with arthrogryposis. Data sources Review of existing world literature and description of personal experience at a center for children's orthopedic surgery and rehabilitation over 2 decades. Methods Description of common problems and their solutions in this unusual and diverse group of patients. Results 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. Conclusions 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.