The multiple phenotypes of Arthrogryposis multiplex congenita with reference to the neurogenic variant
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Arthrogryposis multiplex congenita
Muscle contracture
Arthrogryposis multiplex congenita
Muscle contracture
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Summary: The functional, radiological, and ambulatory status of 30 patients (58 knees) with arthrogryposis multiplex congenita (AMC) were analyzed after a follow-up of 1-36 years. The muscular imbalance presented in many of these patients corresponded well to the type of deformity. The primary treatment was nonoperative in most cases. In spite of the initial promising results, a significant number of patients underwent operation for residual symptoms. Treatment of flexion contractures seems to be more discouraging than the treatment of extension contractures. On the other hand, the risk for degenerative arthritis seemed to be elevated, especially in the extension-contracture group.
Arthrogryposis multiplex congenita
Muscle contracture
Degenerative arthritis
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Summary: The functional, radiological, and ambulatory status of 30 patients (58 knees) with arthrogryposis multiplex congenita (AMC) were analyzed after a follow-up of 1-36 years. The muscular imbalance presented in many of these patients corresponded well to the type of deformity. The primary treatment was nonoperative in most cases. In spite of the initial promising results, a significant number of patients underwent operation for residual symptoms. Treatment of flexion contractures seems to be more discouraging than the treatment of extension contractures. On the other hand, the risk for degenerative arthritis seemed to be elevated, especially in the extension-contracture group.
Arthrogryposis multiplex congenita
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To identify all patients with arthrogryposis multiplex congenita presenting to a specialized pediatric orthopedic institution over a 28-year period; classify them into three groups (general arthrogryposis not otherwise specified [NOS], amyoplasia, or distal arthrogryposis); report the frequency of various musculoskeletal features; and determine the rate of operative treatment.Patients with arthrogryposis under the age of 18 years who presented between 1990 and 2017 were included. Patients were placed into one of three groups based on clinical features and family history when available. Age of presentation, joint involvement, and utilization of surgical treatment were recorded.There were 417 patients in total (184 females, 233 males); 235 patients (56.4%) had general arthrogryposis NOS, 107 (25.7%) had amyoplasia, and 75 (18.0%) had distal arthrogryposis. Patients with amyoplasia presented at a younger age (median 4mo) than those with general arthrogryposis NOS (median 1y 3mo, p=0.005), and had a lower rate of spine involvement than patients with general arthrogryposis NOS (p=0.004) and distal arthrogryposis (p=0.023). The average number of surgeries across all patients was 1.9 (SD 2.2).Pediatric patients with amyoplasia present to orthopedic care earlier and are less likely to have spine involvement than other forms of arthrogryposis. Multiple surgeries are common among all patients with arthrogryposis.
Arthrogryposis multiplex congenita
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We studied 61 patients with Arthrogryposis Multiplex Congenita during the period of 1926 to 1990. Joint contractures of the lower extremity at first visit were reviewed.Of 122 extremities; 98 foot deformities, 84 knee contractures and 63 hip contractures (40 dislocations and 23 contractures only) were seen showing the frequency of joint contractures to be higher distally than proximally.The main cause of the gait disturbances are multiple joint contractures. The combination of hip dislocation-knee extension contrature (dislocation)-pes equinovarus is the most important factor in the development of the gait disturbance.
Arthrogryposis multiplex congenita
Muscle contracture
Joint Contracture
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Arthrogryposis multiplex congenita is a syndrome characterized by the presence of congenital contractures involving multiple joints usually with flexion deformities, with or without pterygia or webbing at the joints. The aim of this case report was to highlight the challenges of management of severe knee contractures in AMC and possible solutions in a resource limited setting like ours. We presented a one-year-old child with bilateral severe knee contractures secondary to arthrogryposes. The involvement of knee is present in about 70% of cases with arthrogryposis, with flexion deformity more common than extension deformity. Knee flexion contractures, as seen in our patient, is one of the most disabling deformities. The surgical option adopted in the case presentedwere met with various challenges including abnormal muscles insertions, shortening and fibosis of the muscles, abnormal position and shortening of the neurovascular structures in the popliteal fossa.
Arthrogryposis multiplex congenita
Muscle contracture
Neurovascular bundle
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Arthrogryposis multiplex congenita (AMC), or arthrogryposis, and multiple congenital contractures (MCC) are equivalent terms used to describe a phenotypic condition associated with multiple congenital non-progressive contractures in three or more body sites. Arthrogryposis is not a single disease but rather a clinical feature associated with more than 300 conditions [1]. The contractures develop secondary to fetal akinesia, i.e., in utero movement limitation, of varying etiology: muscle and/or neuronal pathology, connective tissue abnormalities, external limitations of in utero space, maternal diseases or exposures, compromised blood supply of placenta and/or fetus, metabolic disorders, and epigenetic influence. Children are born immobilized with fixed and thickened joints and decreased muscle mass. Early medical care may improve the condition over time [1, 2].
Arthrogryposis multiplex congenita
Muscle contracture
Etiology
Fetal movement
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In part I the authors deal with the present possibilities of the diagnostics and therapy of arthrogryposis multiplex congenita. They proceed from the classification after Hall of 1982 (classification of the so-called distal arthrogryposis) and of 1983 where congenital contractures are divided into three groups according to associated system anomalies. On the basis of their own experience they deal in details with distal arthrogryposis - amyoplasia which differs from spinal degenerative diseases by 3 symptoms: 1. it does not deteriorate after the birth, 2. nerves are not affected, 3. intelligence is normal. Differential diagnostics is carried out on the basis of clinical-genetic examination. Dermatoglyphic examination contributes to an early diagnosis mainly in distal arthrogryposis. The seriousness of neurogenic or myogenetic affection is determined by EMG examination. The authors compare therapeutical literary aspects with their own methods of treatment. The evaluation of diagnostic and therapeutical results is presented in part II of the communication. Key words: arthrogryposis multiplex congenita, amyoplasia, distal arhtrogryposis, electromyography, dermatoglyphes, surgical treatment.
Arthrogryposis multiplex congenita
Muscle contracture
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Objective To study the treatment in arthrogryposis multiplex congenita(AMC).Methods Thirty five patients with AMC (knee 42, feet 66), treated from 1980 to 1997 were studied and evaluated according to the Sodergard grading.Results The overall prognosis for extension contracture of the knees appeared to be better than that of flexion contracture. Recurrence of the deformity occurred in 44 feet ( 73.3% ). Some of these patients required three operations.Conclusions Treatment of flexion contractures of the knees is discouraging. The patients with foot deformity can be treated primarily with soft tissue release before 1 year of age.
Arthrogryposis multiplex congenita
Muscle contracture
Foot (prosody)
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Distal arthrogryposis as a special form of arthrogryposis multiplex congenita is a rare malformation syndrome associated with contractures of the hands. A special case demonstrates the possibilities of treating flexion contractures of the hand. This procedure is compared with the treatment of other contractures of the hand. One possibility is the shortening of the metacarpals by osteotomy and shortening of extensor tendons.
Arthrogryposis multiplex congenita
Muscle contracture
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