Arthrogryposis multiplex congenita

Abstract

Introduction: arthrogryposis multiplex congenita is a clinical condition characterized by multiple non-progressive articular contractures in two or more articulations, that can be detected at birth. It’s a disease with a complex therapeutic approach requiring the atten­tion of a multiprofessional team. The treatment’s success depends on a care network that includes not only monitoring by medical and fisioterapheutical professionals but the orien­tation of parents to maintain the exercises and stimulation at home. Objective: report a case of a preschool admitted in a puericulture and pediatrics ambulatory of the greater Rio de Janeiro’s university hospital in a clinical condition of multiple muscular contractures and to discuss its clinical evolution. Case description: the patient is three years old girl, that had arrived belatedly, since 15 months old, under monitoring on the puericulture and pediatrics ambulatory with an AMC diagnostic. She showed, at birth, malformations on her upper and lower limbs, characterized by congenital contractures associated to facial hemangioma, as well as echocardiographic changes described by interatrial communica­tion, patent oval foramen and pulmonary artery branch stenosis. The mother has a regular prenatal history from a basic health care site showing: gestational diabetes, chronic arte­rial hypertension and urinary infection. Discussion: it is known that the sooner AMC’s therapy is applied the bigger are the betterments on the articular functions of the patient, giving them bigger autonomy and life quality. Therefore, the diffusion of knowledge on this rare disorder is important, so both the precocious diagnostic and treatment are established.