Background: Arthrogryposis Multiplex Congenita (AMC) is a rare condition that has not been extensively studied, and the need for further research has been strongly emphasized. Despite substantial functional impact resulting from AMC, minimal research has been completed regarding mid- to long-term outcomes in this population. The objective of this systematic review was to identify existing knowledge concerning outcomes of shoulder, elbow and wrist surgery with a minimum two year follow-up in individuals with AMC.
Methods and findings: A search was conducted in Medline, Embase and Web of Science databases and a hand search of the bibliographies of articles relevant to the research question was completed. Inclusion criteria were English articles with AMC participants only (excluding syndrome-related AMC) who underwent shoulder, elbow or wrist surgeries with a minimum follow-up of two years. 1289 articles were identified through electronic database and hand searches; after review, 12 articles met all inclusion criteria. No articles were identified that described shoulder surgery outcomes in those with AMC. Elbow surgeries discussed were posterior capsulotomy and triceps lengthening, pectoralis major muscle transfer, gracilis muscle transfer, Steindler flexorplasty, and brachial nerve exploration with or without nerve transfer. Wrist surgeries included proximal row carpectomy, osteotomy of radius and ulna, and dorsal carpal wedge osteotomy.
Conclusions: This systematic review identified those procedures with lasting benefit, those that resulted in additional complications or surgeries, and those which are currently recommended as treatment in patients with AMC. These conclusions were based on a small number of articles with low levels of evidence due to the obstacles of performing higher-level studies in this rare, heterogeneous population. To facilitate treatment and to prevent adverse effects on patient quality of life and strain on healthcare and social system resources, additional and continued research is necessary in this population. AMC is a rare congenital condition regarding which further research is required to ensure appropriate and successful treatment as well as expansion of the existing knowledge base.
Miller R and Sawatzky B
All Published work is licensed under a Creative Commons Attribution 4.0 International License
Copyright © 2018 All rights reserved. iMedPub LTD Last revised : May 23, 2018