Surgical treatment of upper extremities of cases arthrogryposis multiplex congenita]

Between 1990-1999 at the Polish-American Pediatric Institute 36 children with arthrogryposis were treated. In 10 cases 21 corrective surgical procedures of the upper extremities were performed. Corrective procedures of the upper extremities were performed. Corrective procedures aimed at contractures of the hand, wrist and forearm, consisting of capsulotomies, lengthening of the finger flexor tendons and transposition of wrist flexor on to the wrist extensors were performed 8 times. Correction of extension contracture of elbow joint, consisting of posterior capsulotomy and lengthening of the triceps tendon was performed 9 times. In 4 cases transposition of the pectoralis major onto the biceps tendon according to Clark or Zancolli in Carrol's modification was performed. The average age of the children was 4.7 years (age ranging from 1.5 to 9 years), the follow-up period was 4.25 years (ranging from 0.5 to 10 years). Palmer contracture of the wrist was an average 80 degrees (70-90 degrees) before surgery. Passive flexion in the elbow joint was on average 15 degrees (0-35 degrees). Active flexion of the elbow was absent in all cases. After corrective procedures in the wrist, in 5 cases physiological positioning of the was achieved. After lengthening of the triceps tendon passive flexion of elbow was 80-90 degrees, in one extremity 70 degrees. After the Clark or Zancolli procedures active flexion of the elbow was 80 degrees in one case, 70 degrees in another and 50 degrees six months post-op. The author's own experiences indicate that and multi-stage interdisciplinary approach in necessary from the beginning in these cases.