Multidisciplinary management of arthrogryposis multiplex congenita type 2A case

Abstract

Arthrogryposis multiplex congenita (AMC) is a congenital disorder characterized by nonprogressive multiple joint contractures affecting one or more areas of the body, muscle weakness, and fibrosis. This term includes a heterogeneous group of diseases, neurological, neuromuscular, and genetic or mechanical origin. Two types of classification have been developed: A clinical one (types I, II, and III) and an etiological one. A multidisciplinary approach is needed for better care and appropriate follow-up. It is a case of AMC type 2A. A girl, with an antecedent of low levels of amniotic fluid, presented at birth with multiple malformations and stiffness of several articulations: bilateral clubfoot, bilateral clubhand, temporomandibular joint involvement, postural torticollis, and congenital hip dislocation. She had also a small persistence of arterial canal and ambiguous external genitalia. The care was performed by a multidisciplinary team including a physiatrist, pediatric surgeon, physiotherapist, prosthetist, and orthotist. In infants with arthrogryposis, joint stiffness is maximal at birth. The first step is passive mobilizations before surgical joint treatment. Azbell et al. found in their study that at 9 months of age, many of this infant's impairments of body structure and function, functional activity limitations, and participation restrictions improved. The program of stretching, muscle strengthening, facilitation of motor skills, orthopedic intervention, and parent education may have contributed to this infant's progress. Prospective intervention studies exploring specific intervention strategies are needed to establish the plan of care for these patients. Arthrogryposis describes a set of joint contractures present from birth and nonprogressive. The common physiopathological mechanism is fetal immobility syndrome. Multidisciplinary care is necessary and should be early and continued to gain maximum autonomy and facilitate social integration.