Restoration of Elbow Flexion with Functioning Free Muscle Transfer in Arthrogryposis

Abstract

Arthrogryposis is a collective term applied to a variety of different syndromes characterized by nonprogressive, multiple joint contractures present at birth due to neurogenic, myopathic (amyoplasia), or connective-tissue disorders1-6. The most serious impairment of amyoplasia in the upper extremity is an inability to flex the elbow due to extension contracture of the elbow. The ability to flex one elbow is essential to enable a child to become functionally independent with self-feeding and self-care of the face and hair; extension of the other elbow is necessary for independent toileting7. Active elbow flexion is the definitive goal of surgical intervention. Multiple procedures6, including triceps transfer8-11, pectoralis major transfer11-14, latissimus dorsi transfer15,16, and the Steindler flexorplasty12,16,17, have been used to provide active elbow flexion in patients with arthrogryposis. However, these conventional procedures do not always provide satisfactory long-term function and may result in a decreased arc of elbow flexion and in flexion contracture because of weakness of the donor muscles. Free muscle transfer is a reliable procedure for restoration of elbow flexion following brachial plexus injury18-22. We present the long-term results of a functioning free muscle transfer with use of the gracilis muscle for restoration of elbow flexion in two patients with arthrogryposis. The patients and their parents were informed that data concerning the cases would be submitted for publication, and they provided consent. Case 1. A three-month-old boy with amyoplasia of both upper extremities without lower-extremity involvement was referred to our clinic for treatment (Fig. 1-A). The pectoralis major and latissimus dorsi muscles were hypoplastic and thus could not be used for transfer bilaterally. The patient underwent preoperative physiotherapy to improve passive …