Upper limbs are commonly involved in Arthrogyposis Multiplex Congenita. They may be 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, pro-nation 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

Introduction. We performed humeral head replacement (HHR) with smaller head for closing the cuff defect in patients of cuff tear arthropathy (CTA). And also, if the cuff defect could not close by decreasing the head size, we add muscle tendon transfer such as latissimus dorsi transfer for posterosuperior defect and pectoralis major transfer for anterosuperior defect. Aim. The purpose of this study was to investigate clinical and functional outcomes of this procedure for CTA according to Hamada-Fukuda classification. Methods. 76 shoulders in 77 patients with CTA underwent HHR based on our strategy at average age of 74 years. Hamada-Fukuda classification was classified into five categories. There were 13 type 1, 24 type 2, 26 type 3, 9 type 4, 5 type 5. Clinical outcomes (JOA score) were evaluated at an average of 25 months. Results. The average Japanese Orthopaedic Association shoulder score all improved significantly. Forward elevation improved from 57° to 146.5° in type 1, 65.5° to 132.1° in type 2, 82° to 123.9° in type 3, 90.6° to 122.1° in type 4 and 91° to 130° in type 5. Improvement of External rotation 25° in type 1, 23.1° in type 2, 10.89°in type 3, 11.3° in type 4 and −7.3° in type 5. Internal rotation similarly improved between Hamada-Fukuda classification. Conclusion. In patients of type 1 and 2, our procedure could get a good ER recovery. However, in patients of type 3, 4, and 5, functional outcomes were partly inferior to type 1 and 2. Our procedure for type 1 and 2 CTA is one of useful methods

Introduction. In recently, Reverse shoulder arthroplasty (RSA) in patients with irreparable rotator cuff tear has been worldwidely performed. Many studies on RSA reported a good improvement in flexion of the sholulder, however, no improvement in external rotation (ER)and internal rotation motion (IR). Additionally, RSA has some risks to perform especially in younger patients, because high rates of complications such as deltoid stretching and loosening, infection, neurologic injury, dislocation, acromial fracture, and breakage of the prosthesis after long-term use were reported. Favard et al noted a 72% survival with a Constant-Murley score of <30 at 10 years with a marked break occurring at 8 years. Boileau et al noted caution is required, as such patients are often younger, and informed consent must obviously cover the high complication rate in this group, as well as the unknown longer-term outcome. Its use should be limited to elderly patients, arguably those aged over 70 years, with poor function and severe pain related to cuff deficiency. We developed a novel strategy in 2001, in which we used the humeral head to close the cuff defect and move the center of rotation medially and distally to increase the lever arm of the deltoid muscle. Aim. The aim of this study was to investigate clinical outcome of our strategy for younger patients with an irreparable rotator cuff tear. Materials and Methods. Eighteen shoulders (9 of male patients, 9 of female patients) of patients under 70 years old with an irreparable cuff tears and who were treated with Humeral Head Replacement (HHR) and cuff reconstruction were followed up for more than 12 months. The average age was 63.9 years (range, 58–69 years). The average follow-up period was 27.3 months (range, 12–76 months). The cuff defect was successfully closed in 8 shoulders, whereas 8 shoulders required a Latissimus Dorsi transfer; one other shoulder required a Pectralis Major transfer, and one required both Latissimus dorsi and pectoralis major transfers. Range of motion (flexion, ER), the shoulder score of Japanese Orthopaedic Association (JOA score), and complications were evaluated. Results. Shoulder pain decreased in all patients after surgery. JOA score was improved from 41.1 to 82.6 points after surgery, Flexion motion improved from 72.5 to 145.6 degrees postoperatively and ER increased from 17.5 to 37.8 degrees postoperatively. There were no complications. Conclusion. In our study, HHR using the small head of the humerus and cuff reconstruction for patients under 70 years old with an irreparable rotator cuff tear yielded favorable results as compared to RSA, especially in terms of the ER Furthermore, the advantages of our strategy is able to keep bone stock of the glenoid after surgery. If revision surgery is required, RSA can be performed. Since the patients included in our study were relatively active, long-term follow-up will be required to assess their progress

Introduction: The purpose of this study was to evaluate the results 9 to 12 years after the transfer of 3 distal parts of pectoralis major muscle to restore active elbow flexion in patient with arthrogryposis. Material and methods: From 1996 to 1999, elbow flexion was reconstructed in 9 upper extremities by 5 patient aged 4.3–9 years. The patients were clinically evaluated according to the subjective and objective assessment and examined electromyographically before the surgery and during the follow up. Last clinical examination was provided 8 to12 years after the surgery in patients aged 15 to 18 years. Results: 3–4 years after pectoralis major transfer, 6 very good and good results were achieved, the average active ROM of elbow ranged 15 to 95 degrees. The power of elbow flexion was graded as 4 and 4+. This method was unsuccessful in the remaining 3 cases, the patients were not able to reach the mouth with the hands because of limited elbow flexion. 9 to12 years after the surgery, the results were similar. 5 extremities remain very good and good. One deterioration was obvious. The ROM of elbow changed, namely the extension was mostly reduced even in very good and good evaluations (the average decrease was 13.8 degrees, the range −20 to +10 deg.). The active flexion was not changed in 5 elbows, or it was increased (the average increase 4,4 deg., the range 0–15 deg.). The final limitation of extension (30 – 50 degrees) does not restrict using the hands for perineal hygiene and the final active flexion (85–100 degrees) allows elevating the hands to the head for feeding and toilet. Discussion and Conclusions: From the analysis of unsuccessful results 3–4 years after the surgery it was obvious that all cases were related to very limited preoperative passive flexion of the elbow; restricted movement of the shoulder and failed distal fixation of the transferred muscle. After 9–12 years, the active elbow flexion and extension for raising the hands to the mouth and for toilet needs, respectively, remain in majority previously successful cases. Because most children reached the skeletal maturity, no further shortening of the transferred muscle and limitation of extension is expected. These findings do not concur with the literature reports. According to our results, the transfer of the pectoralis major represents the efficient method for permanent restoring of bilateral active elbow flexion with the remaining functional extension. The ROM does not change significantly after having reached the plateau 2 years postoperatively. The prerequisites for successful results are a minimum of 90 degrees of passive flexion of the elbow before the surgery, the active shoulder abduction of 80–90 degrees, long-term rehabilitation and successful fixation of the transferred muscle to the forearm