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Background. Arthrogryposis is severe congenital musculoskeletal disease with contractures of two or more joints of the lower and/or upper limbs and usually in combination with muscular hypo- or atrophy.Clinical Case Description. Child F. was hospitalized in the department of newborns and premature babies’ pathology on 6th day of life in critical condition. Spontaneous motor activity was severely decreased. He responds to the examination with little increase in motor activity and weak painful scream. Muscle tone is dystonic: it is diffusely reduced in the proximal parts of the upper and lower limbs, in the distal parts of the upper limbs it is significantly increased, there are flexion contractures of the fingers on both hands (mostly on the left one). Passive fingers contractures reversal is difficult. There were no feet deformities or craniofacial anomalies. Hereditary history is burdened: child’s father (1984 year of birth), great-grandfather and cousin uncle (on the paternal line) have finger deformities on both hands. Father and relatives have not been diagnosed before. The clinical diagnosis was established after consultation of geneticist and orthopedist: «Distal arthrogryposis, type 2A, autosomal dominant type of inheritance with incomplete penetrance». The molecular genetic testing was not performed due to the refusal of the child's parents. Therapeutic gymnastics, stage plaster correction of finger contractures were performed in the department during the child hospitalization (21 days). Positive dynamics was noted: finger extension amplitude has increased.Conclusion. Early conservative treatment of infants with arthrogryposis allows to correct musculoskeletal deformities. Early initiation of treatment is expected to increase the amplitude of both passive and active movements in the hand joints, whereas, it will improve the function of hand grasping and self-care capacities of patients.
Background. Arthrogryposis is severe congenital musculoskeletal disease with contractures of two or more joints of the lower and/or upper limbs and usually in combination with muscular hypo- or atrophy.Clinical Case Description. Child F. was hospitalized in the department of newborns and premature babies’ pathology on 6th day of life in critical condition. Spontaneous motor activity was severely decreased. He responds to the examination with little increase in motor activity and weak painful scream. Muscle tone is dystonic: it is diffusely reduced in the proximal parts of the upper and lower limbs, in the distal parts of the upper limbs it is significantly increased, there are flexion contractures of the fingers on both hands (mostly on the left one). Passive fingers contractures reversal is difficult. There were no feet deformities or craniofacial anomalies. Hereditary history is burdened: child’s father (1984 year of birth), great-grandfather and cousin uncle (on the paternal line) have finger deformities on both hands. Father and relatives have not been diagnosed before. The clinical diagnosis was established after consultation of geneticist and orthopedist: «Distal arthrogryposis, type 2A, autosomal dominant type of inheritance with incomplete penetrance». The molecular genetic testing was not performed due to the refusal of the child's parents. Therapeutic gymnastics, stage plaster correction of finger contractures were performed in the department during the child hospitalization (21 days). Positive dynamics was noted: finger extension amplitude has increased.Conclusion. Early conservative treatment of infants with arthrogryposis allows to correct musculoskeletal deformities. Early initiation of treatment is expected to increase the amplitude of both passive and active movements in the hand joints, whereas, it will improve the function of hand grasping and self-care capacities of patients.
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
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