Pediatric Arthrogryposis: An Introduction

Arthrogryposis is a group of disorders characterized by multiple joint contractures, muscle atrophy, and muscle weakness present at birth. The word “arthrogryposis” is derived from the Greek language. Arthro, which means joint, and gryp, which means curved, describe the multiple congenital contractures. Most individuals with arthrogryposis have normal or above and. Arthrogryposis can be seen in isolation or in association with other congenital abnormalities as part of a syndrome with or without central nervous system involvement. There are over 300 different syndromes associated with congenital contractures, the most common being. This term translates to “no muscle growth” (a = no, myo = muscle, plasia = growth). It is the most common form of arthrogryposis and represents one third of all cases. Most affected individuals have all four limbs involved, including the feet (most common site affected), knees, and hips. The contractures are usually most severe at birth. The goals of treatment are to improve the function of these children specifically regarding mobility, self-care, and daily activities and to render them as independent as possible. Management of these children should be in . Treatment should start immediately after birth and includes intensive physiotherapy and bracing for both upper and lower limb deformities. Aggressive surgery is warranted as many of these have the potential to improve their function, even if the contractures have a known tendency for. In general, mild contractures can be addressed with intense physiotherapy and bracing. Moderate deformities can be addressed with soft tissue releases and growth modulation, especially around the knees. Severe deformities are best corrected with bony procedures. Very severe and recurrent deformities may also be addressed with gradual correction with external fixators (Table 1).