Arthrogryposis multiplex congenita: classification, diagnosis, perioperative care, and anesthesia

Abstract

Arthrogryposis multiplex congenita (AMC) is a rare disorder characterized by non-progressive, multiple contractures. In addition to affected extremities, patients may also present microstomia, decreased temporomandibular joint mobility. Although the etiology of AMC is unclear, any factor that decreases fetal movement is responsible for AMC. Thus, accurate diagnosis and classification are crucial to the appropriate treatment of AMC. The development of ultrasound technology has enabled prenatal diagnosis. Very early treatment is favorable, and multidisciplinary treatment is necessary to improve the function of AMC patients. Most patients require surgery to release contracture and reconstruct joints. However, perioperative care is challenging, and difficult airway is the first concern of anesthesiologists. Postoperative pulmonary complications are common and regional anesthesia is recommended for postoperative analgesia. This review on AMC is intended for anesthesiologists. Thus, we discuss the treatment and perioperative management of patients undergoing surgery, as well as the diagnosis and classification of AMC.

Keywords

arthrogryposis / amyoplasia / distal arthrogryposis / anesthesia

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Compliance with ethics guidelines

Lulu Ma and Xuerong Yu declare that they have no conflict of interest. The manuscript is a review article and does not need ethical approval from the institutional review board. Images included in this article have been de-identified and the patients consented to the use of their medical records.

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