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Early Splinting Intervention and Active Motion Achievement In Child with Amyoplasia - A Case Report
Maria Candida M. Luzo, OT1; Rames Mattar Jr., MD, PhD2; Valeria Aquilino Reis, OTR3; Teng Hsiang Wei, MD, PhD4
1Occupational Therapy, Instituto de Ortopedia e Traumatologia do HCFMUSP, São Paulo, Brazil; 2Orthopedic Surgery, University of Sao Paulo, Sao Paulo, Brazil; 3Orthopaedic and Traumatology, University of Sao Paulo, sao Paulo, Brazil; 4Instituto de Ortopedia e Traumatologia/Hand Surgery and Microsurgery, Universidade de São Paulo, São Paulo, Brazil
Purpose: The purpose of this case report is to show the result of early intervention in a child diagnosed with Amyoplasia, treated with a splinting program that included day and night use, early developmental stimulation and family orientation.
Background: Amyoplasia is the most common arthrogrypotic disorder, characterized by contractures of multiple joints and symmetrical positioning of the limbs. In most patients the upper extremities present adduction and internal rotation of the shoulders, extension contracture of the elbows, pronation of the forearms, flexion of the wrist, adduction of the thumb and flexion of the PIP joints. Most authors refer to stiffness as extra articular and recomend passive range motion exercises (PROM) starting early in life; the importance of daily therapy; home programs and splinting at night to maintain the achieved position. But that therapy, by itself, rarely achieves significant improvement.
Methods: S.A.S, 24 months, arthrogriposis detected before birth by Ultrasound. She was evalueted and had characteristic contractures of Amyoplasia in the lower and upper extremities. Hand Therapy treatment started on first week of life. At first, positioning splints were made for the hands, PROM iniciated and were performed by parents at home. The splints were remolded every week. At three months of age, elbow extension pattern was present and a 30 degree angle of flexion dorsal elbow splint was worn during the day, for short periods, to control the extension movement. Within two weeks, active elbow flexion was observed and an early developmental stimulation program was performed.
Results: At 24 months of age, S has active flexion of the elbows, free movements of the sholder, active extension of the wrists and the thumb is abducted. She is also able to bring her hands actively to her mouth and eats independently. Authors believe that, during the elbow extension , co-contrations occur and not only the extensor is activated, but some activity of weak flexors is present and, by inhibitting extension, contraction of the flexors is more effective and active movement is possible.
Conclusion: A hand therapist while treating and splinting Amyoplasia must observe PROM, positioning and also the effects of them during muscle activity. This case report shows that we can go further since active elbow flexion in this pathology is not described. This case report intends to show the result of early intervention in a child diagnosed with Amyoplasia, treated with a splinting program that included day and night use, early developmental stimulation and parents orientation
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