gms | German Medical Science

Objectives/Interrogation: Arthrogryposis is characterized congenital contractures of two or more joints [1], [2]. The term arthrogryposis multiplex congenita is used to describe a large heterogeneous group of disorders, and it has been subdivided into syndromic arthrogryposis, distal arthrogryposis, and classic arthrogryposis (amyoplasia). The degree of stiffness and weakness ranges from mild to severe and is not progressive. Elbow dysfunction poses a substantial functional limitation for these children [2], [9-11]. With the elbow stiff in extension, they cannot bring the hand to the mouth to feed them or attend to facial hygiene. The goal of treatment for children with arthrogryposis is to improve their quality of life by facilitating functional independence.

Methods: 1.5yr old female child born out of non consanguineous marriage came to our OPD with parental concerns of bilateral upper limb deformity. Though she had almost 90° of range of motion in her right shoulder but no movement was possible at elbow. The X-Ray findings revealed normal elbow joint and MRI showed Normal alignment of bilateral elbow joint with complete atrophy of muscle in anterior compartment both arm. Posterior capsulotomy with triceps lengthening (v-y) was performed for both elbow joints.

Results and Conclusions: Posterior capsulotomy with triceps lengthening (v-y) was performed first on right side and followed by similar sugery on left side after 3 months. The duration of follow-up was 3 years in this case. The arc of motion of elbows improved from 0° preoperatively to 110° at the time of final follow-up. The child was able to reach the mouth without passive assistance and is able to feed herself independently. The child do not underwent any subsequent tendon transfer surgery.

Elbow capsulotomy with triceps lengthening successfully increases elbow flexion and the arc of elbow motion of children with arthrogryposis, enabling hand-to-mouth activities. From this our main emphasis is that one can be benefitted to very extent with elbow capsulotomy and triceps lengthening and one should be little careful while doing or selecting tendon transfer for example in this case Latissimus Dorsi was not working, and we could not use pectoralis major, while performing triceps to biceps could result in flexion contracture deformity which is more cumbersome for patient as it restrict free out reach of hand and table top activity such as use of computer which has become a necessity in today's world.