summary Arthrogryposis is a non-progressive congenital disorder of unknown etiology that presents with multiple rigid joints leading to stiffness and severe limitation in motion in all 4 limbs. Diagnosis is made clinically with presence of concomitant elbow and knee hyperextension contractures, shoulder internal rotation contractures, hands with intrinsic plus deformity and severe limitation in range of motion of all 4 limbs. Genetic and enzyme testing can be helpful in supplementing diagnosis. Treatment is a multidisciplinary approach to address joint stiffness in all 4 limbs with a trial of bracing and casting followed by surgical soft tissue release or osteotomies as necessary. Epidemiology Incidence 1:3000 live births Etiology Mechanism symmetry of contractures due to immobilization in utero neurogenic (90%) myopathic (10%) Pathophysiology exact mechanism unknown some mothers have serum antibodies inhibiting the fetal acetylcholine receptors leading to a decreased number of anterior horn cells Associated conditions orthopaedic manifestations upper extremity deformity teratologic hip subluxation and dislocation knee contractures foot conditions login to view 2 more bullets neuromuscular C-shaped scoliosis (33%) fractures (25%) Prognosis Nonambulatory (25%) Classification Arthrogryposis classification Type I Single localized deformity (e.g., forearm pronation) Type II Full expression (absence of shoulder muscles, thin limbs, elbows extended, wrists flexed and ulnarly deviated, intrinsic plus deformity of hands, adducted thumbs, no flexion creases) Type III Full expression (type II) with polydactyly and involvement of non-neuromuscular systems Presentation Physical exam inspection & palpation shoulders adducted and internally rotated (absense of shoulder muscles) elbows extended (no flexion creases) wrists flexed and ulnarly deviated hands with intrinsic plus deformity thumb adducted hips flexed, abducted, and externally rotated login to view 1 more bullet knees extended (classical), most of the time flexed clubfeet normal intelligence, facies, sensation, and viscera range of motion severely limited usually involving all four extremities Studies Perform at 3-4 months of age neurologic studies enzyme tests muscle biopsies Upper Extremity Deformity Treatment goals allow optimal function to increase ability to drive an electric chair and use computer assisted devices one elbow in extension for positioning and perianal care and one elbow in flexion for feeding nonoperative passive manipulation and serial casting login to view 2 more bullets operative soft tissue releases, tendon transfers, osteotomies login to view 3 more bullets Teratologic Hip Subluxation & Dislocation Introduction present in 68-80% of patients with arthrogryposis Treatment nonoperative observation alone login to view 4 more bullets Pavlik harness and rigid abduction brace are unlikely to succeed operative closed reduction login to view 2 more bullets medial open reduction with possible femoral shortening login to view 4 more bullets Knee Contractures Treatment operative soft tissue releases (especially hamstrings) login to view 4 more bullets femoral angulation through guided growth (epiphysiodesis) login to view 4 more bullets supracondylar femoral osteotomy login to view 2 more bullets Foot Conditions Clubfoot treatment nonoperative login to view 3 more bullets operative login to view 8 more bullets Vertical Talus treatment operative login to view 6 more bullets