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[Surgical treatment of upper extremities of cases arthrogryposis multiplex congenita]
1Oddział Plastyczno-Rekonstrukcyjny i Oparzeń, Katedra Chirurgii Pediatrycznej Wydziału Lekarskiego Uniwersytetu Jagiellońskiego.
Insights
Surgical interventions for pediatric arthrogryposis can restore function in upper extremities. Procedures like tendon lengthening and capsulotomy significantly improved wrist and elbow joint mobility in children.
Area of Science:
- Orthopedic Surgery
- Pediatric Rehabilitation
- Congenital Musculoskeletal Disorders
Background:
- Arthrogryposis multiplex congenita is a rare condition characterized by multiple joint contractures.
- Upper extremity involvement is common, significantly impacting a child's functional independence.
- Early and effective surgical intervention is crucial for improving outcomes.
Observation:
- A study at the Polish-American Pediatric Institute treated 36 children with arthrogryposis between 1990-1999.
- 21 corrective surgical procedures were performed on the upper extremities of 10 children.
- Procedures included wrist/forearm contracture release, elbow extension contracture correction, and tendon transpositions.
Findings:
- Wrist contractures averaged 80 degrees, with 5 cases achieving physiological positioning post-surgery.
- Pre-operative passive elbow flexion was limited to 15 degrees, with no active flexion.
- Post-surgery, passive elbow flexion improved to 70-90 degrees, and active flexion ranged from 50-80 degrees in some cases.
Implications:
- Surgical correction of upper extremity contractures in arthrogryposis can yield significant functional gains.
- Multi-stage, interdisciplinary treatment approaches are essential for managing this complex condition.
- Restoring upper extremity function is vital for enhancing quality of life in affected children.
Abstract:
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.
