Lower extremity deformities of patients with arthrogryposis multiplex congenita present a wide spectrum of severity and deformity combinations. Treatment goals range from merely ensuring comfortable seating and shoe wear, to fully independent and active ambulation, but the overarching intention is to help realize the patient's greatest potential for independence and function. Treatment of hip and knee contractures and dislocations has become more interventional, whereas treatment of foot deformities has paradoxically become much less surgical. This article synopsizes the treatment strategies presented in September 2014 in Saint Petersburg, Russia at the second international symposium on arthrogryposis.

Background. Patients with amyoplasia and hip dislocation have different variants of hip contractures and deformities, but there is no difference in the selection of the method. Aim. To justify the algorithm of rational selection of surgery methods in children under 3 years of age with hip dislocation in amyoplasia Materials and methods. Level of Evidence II. 70 patients were examined, including 21 children under the age of 12 months (main group) after 25 hip open reduction, 19 children under the age of 3 years (main group) after hip open reduction, Salter innominate osteotomy, femoral osteotomy and 30 patients aged 3 to 7 years (control group) who had not previously received conservative and surgical treatment. All patients were divided into two subgroups depending on the variant of hip contracture: flexion-extension-abduction-external rotation (frog-like) (subgroup 1) and flexion-extension-adduction-external rotation (subgroup 2). We used clinical, radiological and statistical methods. Results. In children of subgroup 1, after hip open reduction, good results were noted in 17% of cases, satisfactory in 50%, unsatisfactory in 33%, severe complications class III, IV according to the modified Clavien-Dindo-Sink classification were obtained in 83%. After hip open reduction, Salter innominate osteotomy, and femoral osteotomy performed in patients of subgroup 1 under the age of 3 years, good results were noted in 50% of cases, satisfactory and unsatisfactory in 25% with less severe complications (50%) (p=0.041). In children of subgroup 2, after hip open reduction, good results were obtained in 90% of cases, satisfactory in 10% with a rate of severe complications of 10%, and when this surgery was combined with Salter innominate osteotomy, femoral osteotomy, good results were noted in 75% of cases, satisfactory in 19% and unsatisfactory in 6%, with a rate of severe complications of 25% (p=0.05). Conclusion. A differentiated approach to the treatment of children with hip dislocation in amyoplasia, in our opinion, will increase the effectiveness of the treatment methods, and its introduction into clinical practice will help to improve the outcomes

BACKGROUND: The absence of active elbow flexion is the most common problem in children with amyoplasia, leading to daily living difficulties. Many variants of muscle transfer are used for the restoration of active elbow flexion. The pectoralis major and latissimus dorsi muscles are the most used muscles for this purpose; however, the optimal age for these operations is not reported in the literature. AIM: This study aimed to determine the optimal age of children with amyoplasia for the restoration of active elbow flexion. MATERIALS AND METHODS: The retrospective study involved 61 patients (90 upper limbs) with amyoplasia (30 [49%] girls and 31 [51%] boys) who were examined and treated between 2011 and 2020. In 46 (51.1%) cases, we used major muscles, and in 44 (48.9%) cases, the latissimus dorsi muscle was used as a donor muscle. All patients were divided into four groups: group 1 included children aged 13 years (n = 17, 27.9%); group 2, 37 years (n = 30, 49.2%); group 3, 711 years (n = 8, 13.1%), and group 4, 1218 years (n = 6, 9.8%). The clinical examination of the patients was conducted before and after the operation (6 months). Statistical data processing was performed using Statistica 10 and SAS JMP 11. To describe the numerical scales, the average value and standard deviation (M SD) were used. RESULTS: The age of the patients at the time of surgery was 5.16 3.72 years, and the postoperative follow-up period was 41.93 30.13 months. Elbow flexion contractures were observed mainly in groups 13 (p 0.05). The greatest changes in indicators such as the strength of forearm flexor muscles, active elbow flexion, and function of the elbow were noted in group 1 (p 0.05). The same postoperative indicators were worse in group 4 than in younger patients (p 0.05). Groups 3 and 4 had less strength of the donor muscles than groups 1 and 2 (p 0.05). CONCLUSIONS: The retrospective analysis of the results of the restoration of active elbow flexion in children with amyoplasia allowed us to recommend these operations in children aged 13 years. The prevention of elbow flexion contractures and the formation of a new stereotype of movement help improve the self-ability of these patients and the treatment results.

Introduction Amyoplasia is the most common type of arthrogryposis multiplex congenita. Some patients may lack active elbow flexion due to aplasia of the forearm flexor muscles. The objective was to identify the optimal age for pectoralis major muscle transfer to improve elbow flexion in children with amyoplasia and estimate outcomes of the procedure depending on the level of spinal cord injury. Material and methods Restoration of active elbow flexion was performed for 34 children with amyoplasia (39 upper limbs) between 2011 and 2020 using partial monopolar pectoralis major muscle transfer. The age of patients ranged between 1.5 and 15.5 years (6.24 ± 4.24 years). The patients were divided into 3 groups depending on the level of spinal cord injury: C6–C7 (n = 4; 11.8 %), C5–C7 (n = 24; 70.6 %), C5–Th1 (n = 6; 17.6 %). The outcomes were estimated at 6 to 99 months (44.53 ± 31.72 months). The patients underwent preoperative and postoperative clinical and neurological examination. The results were statistically analyzed. Results Active elbow flexion improved by 56.8 degrees (p < 0.0001), forearm flexor muscles strengthened by 2.0 points (p < 0.0001) and extension deficit improved by 14.5 degrees (p < 0.0001) were postoperatively statistically significant. Results were rated as good in 15 (38.5 %); as fair, in 8 (20.5 %) and poor in 16 (41 %) cases. Greater differences were found between the group of patients with the level of C6-C7 spinal cord injury in relation to the group of patients with the level of C5-Th1 (p < 0.05). There were no statistically significant differences between patients with C6–C7 and C5–C7 lesion levels (p > 0.05). Children of different age groups showed no differences in the results of treatment (p > 0.05). Conclusion Pectoralis major muscle could be used for active elbow flexion restoration in patients with amyoplasia. The best results were observed in patients with C6–C7, C5–C7 segmental lesions of the spinal cord. There was no correlation between age of patient at the time of surgery and the effectiveness of operation.

BACKGROUND: The absence of active forearm flexion in children with amyoplasia leads to severe functional disorders. Muscle transfer can potentially restore active elbow flexion and the patients daily living. AIM: This study compares the results of the transposition of the latissimus dorsi and pectoralis major to the biceps brachii and identifies the optimal donor area for restoring active elbow flexion in children with amyoplasia. MATERIALS AND METHODS: The retrospective study involved 61 patients with amyoplasia (30 (49%) girls and 31 (51%) boys) who were examined and treated from 2011 to 2020. Restoration of elbow flexion was performed in 90 cases. In 46 cases (51.1%), we used the pectoralis major, and in 44 (48.9%), the latissimus dorsi as donor muscles. In both groups, we performed monopolar muscle transfers. The clinical examination of the patients was conducted before and after the operation. Statistical data processing was performed using Statistica 10 and SAS JMP 11. RESULTS: The age of patients at the time of surgery was from 1.5 to 15.5 years (6.24 4.24 years), the follow-up period after surgery was from 6 to 99 months (41.25 30.19 months). After surgery, all patients had elbow flexion contractures. However, when the latissimus dorsi was used as a donor muscle, the degree of contracture was less than after pectoralis major transfer (15.19 13.04 and 23.24 15.37, respectively, p = 0.0483). In addition, after the latissimus dorsi transfer, the strength of the forearm flexors was on average 1 point greater than after the pectoralis major transfer (2.85 1.08 and 4.00 0.62 points, respectively, p 0.0001). After the latissimus dorsi transfer, the active elbow amplitude flexion was bigger than that of the pectoralis major transfer (75.37 17.86 and 55.88 24.60, respectively, p = 0.0022). CONCLUSIONS: The study demonstrated the effectiveness of using the latissimus dorsi and the pectoralis major to restore elbow flexion in children with amyoplasia. However, if it is possible to choose a donor muscle, it should be the latissimus dorsi.