Arthrogryposis Multiplex Congenita

Friedlander, Harvey Lee; Westin, G W; Wood, W. Burton

doi:10.2106/00004623-196850010-00006

Cited by 113 publications

(24 citation statements)

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“…Descrita oficialmente por primera vez en 1841 por Otto como miodisplasia (3,4,12,15), la AMC comprende un grupo de patologías cuya etiología aún es desconocida; sin embargo, hay varias teorías encaminadas a explicar la hipomobilidad fetal como rasgo principal de este tipo de pacientes (4,14), lo que a su vez hace que haya proliferación de tejido conectivo sobre las articulaciones, explicando así las contracturas presentes.…”

Section: Discussionunclassified

“…Entre las cirugías más usadas en el miembro superior están la liberación de la placa palmar en los dedos, colgajos o plastias de piel; el alargamiento musculotendinoso; las capsulotomias, y las osteotomías periarticulares para completar rangos de movilidad en muñeca o codo (8,(10)(11)(12). Las transferencias tendinosas pueden mejorar escasos grados de movilidad, mientras que las transferencias musculares pueden ser de ayuda para el codo y el hombro (11,13).…”

Section: Introductionunclassified

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Artrogriposis múltiple congénita: espectro de deformidades en el miembro superior, a propósito de una serie de casos

Vergara-Amador

Acosta

2017

Rev. Fac. Med.

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INVESTIGACIÓN ORIGINAL | Resumen |Introducción. La artrogriposis múltiple comprende un grupo complejo de patologías que producen disminución de la movilidad articular. Su tratamiento está enfocado en mejorar la movilidad con rehabilitación y cirugía en algunos casos.Objetivo. Mostrar las deformidades por artrogriposis en el miembro superior, los tipos de tratamiento y sus resultados. Materiales y métodos.Se estudiaron 27 casos (19 operados) que fueron agrupados de acuerdo a compromiso distal en dedos o muñeca o proximal en codo y hombro Resultados. El compromiso principal fue de muñecas y dedos, solo se presentaron dos casos con afectación única del codo. Las cirugías comprendieron z-plastias, liberación de placa palmar y fijación de articulaciones; en la muñeca se realizó liberación de partes blandas, fijación provisional y artrodesis. Se dio un caso de liberación del tríceps, otro de liberación de un pterigio y otro de transferencia muscular para flexión de codo. Conclusión.En miembros superiores hay disminución o ausencia de pliegues en zonas de flexión, rotación interna del hombro, limitación marcada de flexión de codo y de la muñeca en flexión y desviación cubital. En los dedos se encontró camptodactilia y aducción del pulgar. Las cirugías mejoraron la función para las actividades diarias. El tratamiento es individualizado y de acuerdo al grado de afectación. | Abstract |Introduction: Arthrogryposis multiplex comprises a complex group of pathologies that cause decreased joint mobility. Its treatment is focused on improving mobility through physical rehabilitation and surgery in some cases.

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Section: Discussionunclassified

Section: Introductionunclassified

Artrogriposis múltiple congénita: espectro de deformidades en el miembro superior, a propósito de una serie de casos

Vergara-Amador

Acosta

2017

Rev. Fac. Med.

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“…Deformities are characteristically bilateral and symmetric, with quadrimellic involvement in approximately 50%, lower bimellic involvement in 40% and upper bimellic involvement in 5% to 10%.3. 21 Axial skeletal anomalies such as pes excuvutum and scoliosis also commonly occur. Additional findings are of smooth, shiny periarticular skin due to loss of skin creases, dimples around affected joints, and webbing across joints in up to 30% of patients.…”

Section: Discussionmentioning

confidence: 99%

Arthrogryposis multiplex congenita: a report of two cases

Brooks

Coster

1994

Australian and New Zealand Journal of Ophthalmology

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Arthrogryposis multiplex congenita refers to a group of birth defects characterised by multiple joint contractures. The syndrome is caused by neuropathic disease, myopathic disease, or any other cause of decreased fetal joint mobility. Multiple and usually symmetric joint abnormalities with associated muscular and soft tissue hypoplasia are noted at birth. A variety of associated congenital anomalies affecting many organ systems are frequently found. Ocular and orbital findings have been described in patients with arthrogryposis but corneal disease has only rarely been reported. We present corneal findings in two patients with arthrogryposis and discuss the implications for disease pathogenesis. Previous ophthalmologic reports are also reviewed.

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“…There are approximately 150 conditions or syndromes, which are clinically presented by joint contractures 4 . AMC is a non-progressive disorder in which the patient suffers from joint contractures and muscle defects, while the organ sensations remain normal 5,6 . Arthrogryposis is multifactorial in etiology and involves factors such as myopathic processes, connective tissue abnormalities, teratogenic factors, and genetic factors, which may all decrease fetal movements 7 .…”

Section: Introductionmentioning

confidence: 99%

Assessment of outcome of Quadricepsplasty in Knee contractures of patients with arthrogryposis

Moghadam

Birjandinejad

Ghoreishi

2015

Bangladesh J Med Sci

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Arthrogryposis multiplex congenita (AMC) refers to a group of conditions, unrelated to one another, which are similarly characterized by the contracture of multiple joints in the body. One of the common therapies in patients with AMC and knee extension contracture is quadricepsplasty. However, no comprehensive studies have been conducted on the results of the application of this method. This prospective study included 14 patients (9 females and 5 males) with 23 hyperextension contractures of the knee. Before surgery, all patients had underwent non-surgical treatment (manipulation and serial casting) for at least 6 months, but none had responded to the treatment. Then patients underwent a Quadricepsplasty. Range of motion (ROM) of the knee and patients ambulation based on modified form of Functional Mobility Scale (FMS) before and after surgery were evaluated in a follow up 3 years. The mean passive knee ROM before surgery was 7.17° (0° to 15°), which increased to 65.65° (35° to 105°) after the operation. According to t-test results, this increase was statistically significant (P<0.005). The mean ambulation score (modified FMS) of the patients before surgery was 1.57, which increased to 3.71. According to t-test results, this increase was statistically significant (P<0.005). Quadricepsplasty is the method of choice for the surgical treatment of knee extension contracture in AMC patients, who do not respond to non-surgical treatments. This method leads to a significant improvement in knee ROM. On the other hand, it preserves quadriceps strength at 4/5 level, and finally, along with other therapeutic interventions for other lower limb deformities, leads to ambulation improvement in these patients.

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Arthrogryposis Multiplex Congenita

Cited by 113 publications

References 0 publications

Artrogriposis múltiple congénita: espectro de deformidades en el miembro superior, a propósito de una serie de casos

Artrogriposis múltiple congénita: espectro de deformidades en el miembro superior, a propósito de una serie de casos

Arthrogryposis multiplex congenita: a report of two cases

Assessment of outcome of Quadricepsplasty in Knee contractures of patients with arthrogryposis

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