Short-term functional outcome in children with arthrogryposis multiplex congenita after multiple surgeries at an early age

Abstract

Purpose: The purpose of this study is to report our short-term functional outcome for 14 children with arthrogryposis multiplex congenita (AMC) who underwent multiple surgical procedures at an early age.

Methods: During the period 2002–2010, 14 children (11 males and three females) with AMC underwent multiple surgical procedures to treat deformities of the lower and upper limbs. About 81 procedures were performed, at a rate of 5.9 procedures per child. The mean age at the last follow-up was 5.9 years. The average follow-up period was 3.6 years (range, 1.5–6 years). The functional outcome assessment included walking ability and the activities of daily living for the upper limb function.

Results: At the last follow-up visit, six (43%) children (four males, two females) with a mean age of 8.3 years (range, 4–15) were independent walkers. Three children (males) with a mean age of 3.5 years (range, 2.5–5) were able to walk, but with support. One child (male), 3 years old, was a household ambulator. Three children (two males, one female) with a mean age of 4.2 years (range, 2.5–6) were nonfunctional ambulators. The last child (male) was nonambulatory at the age of 5 years. Activities of daily living were severely affected in the nonambulatory child. One child in the nonfunctional ambulators group had limitations in the activities of daily living; however, upper limb function was not affected in the remaining 12 children.

Conclusion: We believe that aggressive surgical treatment using multiple operations at an early age can improve the short-term functional and clinical outcomes of children with AMC.