Resumen

PILA PEREZ, Rafael et al. Arthrogryposis multiplex congenita: presentation of two cases. AMC [online]. 2010, vol.14, n.4. ISSN 1025-0255.

Background: arthrogryposis multiplex congenita is a clinical syndrome characterized by the presence of contractures and rigidity of several articulations, not progressive and of appearance in the prenatal period, being present in the birth moment. Objective: to contribute to the existent literature, two new cases of arthrogryposis multiplex congenita. Clinical cases: Case 1: a forty-nine years old patient, because of a pregnancy and a normal childbirth, she was born with deformity of the four members and of both knees. Case 2: a fifteen years old patient, the previous patient's only daughter who was studied by skeletal deformities of superior and inferior limbs. It was product of a pregnancy and a normal childbirth, attracting the attention the absence of fetal movements during her intra-uterine life. Conclusions: the real prevalence of arthrogryposis multiplex congenita is variable. Within the heterogeneous group of arthrogryposis, there is a series of differentiated illnesses, being the most frequent the classic arthrogryposis multiplex congenita that includes more than 40-50% of children suffering from arthrogryposis. Causes are numerous and it may be due to the action of extrinsic mechanical forces upon a normal fetus or to fetal intrinsic problems. In the classic form of arthrogryposis multiplex congenita the prognosis is usually determined only by the functional limitation that articular contractures produce. The management and child's treatment with arthrogryposis multiplex congenita should be carried out by a multidisciplinary team. The genetic council will be in connection with the specific cause of the affection.

Palabras clave : ARTHROGRYPOSIS; MUSCULOSKELETAL ABNORMALITIES; DISEASE MANAGEMENT; ADOLESCENT; CASE STUDIES.

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