S Asif1, M Umer, R Beg, M Umar. 1. Division of Orthopedics, Department of Surgery, The Aga Khan University Hospital, Karachi, Pakistan.
Abstract
PURPOSE: Arthrogryposis multiplex congenita (AMC) is a rare syndrome with multiple joint contractures. It is commonly believed that bilaterally dislocated hips associated with joint contractures should not be reduced, because movement is satisfactory, while open reduction leads to poor results. This report presents our experience with surgical management of bilateral dislocation of hips in children with AMC. METHODS: During the period 1990 to 2000, we performed open reduction on 8 hips of 4 children with AMC. The mean age at surgery was 23 months (range, 5-48 months). Open reduction and capsular plication without any bony procedure were performed in 4 hips (2 patients). De-rotation and varus osteotomy of the femur was performed in 4 hips, and Salter osteotomy of the innominate bone in 2 hips. The average acetabular index was 44 degrees, and the mean centreedge angle was -41 degrees preoperatively. RESULTS: The average follow-up period was 4 years (range, 2-9 years). The average acetabular index and centre-edge angle were 19 and 18 degrees, respectively at the time of last follow-up. All children could walk without support. One child required re-opening for redislocation of hip joint. The clinical results were good in 6 hips and fair in 2 hips, according to Severin's and McKay's classifications. CONCLUSION: Our experience shows that open reduction for bilateral dislocation of hips in children with AMC is a suitable option with generally good results. Surgery performed at earlier age gives the best functional outcome.
PURPOSE:Arthrogryposis multiplex congenita (AMC) is a rare syndrome with multiple joint contractures. It is commonly believed that bilaterally dislocated hips associated with joint contractures should not be reduced, because movement is satisfactory, while open reduction leads to poor results. This report presents our experience with surgical management of bilateral dislocation of hips in children with AMC. METHODS: During the period 1990 to 2000, we performed open reduction on 8 hips of 4 children with AMC. The mean age at surgery was 23 months (range, 5-48 months). Open reduction and capsular plication without any bony procedure were performed in 4 hips (2 patients). De-rotation and varus osteotomy of the femur was performed in 4 hips, and Salter osteotomy of the innominate bone in 2 hips. The average acetabular index was 44 degrees, and the mean centreedge angle was -41 degrees preoperatively. RESULTS: The average follow-up period was 4 years (range, 2-9 years). The average acetabular index and centre-edge angle were 19 and 18 degrees, respectively at the time of last follow-up. All children could walk without support. One child required re-opening for redislocation of hip joint. The clinical results were good in 6 hips and fair in 2 hips, according to Severin's and McKay's classifications. CONCLUSION: Our experience shows that open reduction for bilateral dislocation of hips in children with AMC is a suitable option with generally good results. Surgery performed at earlier age gives the best functional outcome.