UPPER LIMB
Upper limbs are commonly involved in Arthrogyposis Multiplex Congenita. They may he involved in isolation or in combination with lower limbs. There are two patterns of involvement in upper limbs. The most common (type I) pattern presents with adduction and internal rotation at the shoulder, extension at the elbow, pronation of the forearm and flexion deformity of the wrist, indicating involvement of the C5 and C6 segments. These deformities can be quite disabling and may require surgery to help improve function. We present our long-term results with pectoralis major transfer procedure (as modified by senior author MJB) to restore elbow flexion in seven patients (ten procedures).
Results: Early results in all our patients were quite encouraging. Six patients retained useful power in transferred pectoralis major muscle and maintained the arc of flexion, which was attained following tricepsplasty. However, as children were followed up a gradually increasing flexion deformity and decreasing flexion arc were observed in eight elbows. The onset and progression of flexion deformity was gradual and progressive. The flexion deformity reached ninety degrees or more in all cases.
Conclusions: Results of pectoralis major transfer to treat extension contracture of the elbow in arthrogryposis deteriorate with time due to development of recalcitrant flexion deformity of the elbow. Presently we recommend this procedure on one side only in cases of bilateral involvement because if one procedure is carried out it would be possible for this hand to get to the mouth for feeding and the other unoperated side would be able to look after the perineal hygiene
Lahoti, O., Bell. M.J.
University Hospital Lewisham, London, U.K.
Copyright British Editorial Society of Bone & Joint Surgery 2003
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