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

doi:10.1007/s11684-017-0500-4

Front. Med.

2017, Vol. 11

Issue (1) : 48-52 https://doi.org/10.1007/s11684-017-0500-4

REVIEW

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

Lulu Ma,Xuerong Yu(

)

Department of Anesthesiology, Peking Union Medical College Hospital, Beijing 100730, China

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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

Corresponding Author(s): Xuerong Yu

Just Accepted Date: 12 January 2017 Online First Date: 17 February 2017 Issue Date: 20 March 2017

Cite this article:

Lulu Ma,Xuerong Yu. Arthrogryposis multiplex congenita: classification, diagnosis, perioperative care, and anesthesia[J]. Front. Med., 2017, 11(1): 48-52.

URL:

https://academic.hep.com.cn/fmd/EN/10.1007/s11684-017-0500-4
https://academic.hep.com.cn/fmd/EN/Y2017/V11/I1/48

Fig.1 Clinical features of hand contractures in a 9-year-old female patient with AMC.

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