summary Congenital Radial Ulnar Synostosis is a congenital condition caused by failure of differentiation that leads to the presence of a bony bridge between the proximal radius and ulna. Diagnosis is made radiographically with the presence of a proximal radial ulnar bony connection. Treatment is usually observation in patients who are asymptomatic. Operative management is indicated in patients with deformity limiting ability to participate in specific activities or perform activities of daily living. Epidemiology Demographics male > female (3:2) 60% bilateral Etiology Pathophysiology forearm begins as a single cartilaginous anlage and divides from distal to proximal into the radius and ulna in the 7th week in utero failure of differentiation results in synostosis in proximal aspect of the forearm Genetics familial cases with autosomal dominant inheritance associated with chromosomal abnormalities, particularly duplication of sex chromosomes 20% with positive family history Associated syndromes (30%) Apert syndrome (acrocephalosyndactyly) Carpenter's syndrome (acropolysyndactlyly) Arthrogryposis Mandibulofacial dysostosis Klinefelter's syndrome (XXY) and other sex chromosome abnormalities Presentation Symptoms painless most commonly asymptomatic, noticed by parents and teachers difficulty with specific tasks keyboard, tabletop activities - deficient pronation eating, washing face, catching a ball - deficit supination Physical Exam average age of diagnosis is 6 years of age can go unnoticed until early adolescence, especially in unilateral cases elbow flexion usually preserved fixed forearm pronation average position is 30° of pronation compensatory motion shoulder abduction - compensates for loss of active pronation shoulder adduction - compensates for loss of active supination wrist hypermobility Imaging Radiographs recommended views AP and lateral of forearm and elbow findings can see proximal synostosis radius is wide and bowed ulna is narrow and straight radial head may be dislocated and/or malformed Classification Cleary Classification based on appearance of the synostosis and radial head reduction Cleary Classification of Congenital Proximal Radioulnar Synostosis Type 1 No osseous synostosis, radial head reduced Type 2 Osseous synostosis, radial head reduced Type 3 Long osseous synostosis, radial head hypoplastic and posteriorly dislocated Type 4 Short osseous synostosis, radial head mushroom-shaped and anteriorly dislocated Treatment Nonoperative observation indications usually the preferred treatment, particularly when asymptomatic and unilateral Operative indications absolute deformity is limiting ability to participate in specific activities (sports, hygiene, eating) relative severe pronation deformity > 60° bilateral deformities general options mobilization of the synostosis - to restore active forearm rotation rotational osteotomy - to improve static forearm and hand position synostosis excision with soft tissue interposition goal restore active forearm rotation technique excise synostosis and interpose vascularized fascio-fat graft vascularized fat better than free fat graft interposed anconeus muscle did not prevent reossification excision alone without graft interposition results in nearly 100% recurrence of synostosis outcomes gain in active forearm motion is usually slight unsatisfactory results in most studies forearm derotational osteotomy goal place the forearm in more functional resting position technique perform between 3-6 years of age (average age ~5 years) osteotomy location radius and ulna proximal diaphysis at synostosis rotation takes place over narrow space - risks soft tissue tightness, loss of correction and neurovascular compromise radius and ulna diaphysis distal to synostosis, at different levels osteotomies at different levels distributes rotational correction - less soft tissue tightness and risk of neurovascular complications radius distal diaphysis alone timing of correction immediate correction at time of osteotomy delayed correction 10 days following osteotomy gradual correction with circular external fixator frame (Ilizarov) lowest rate of neurovascular complications (compartment syndrome, nerve palsies) positioning unilateral - fix the forearm in 0-30° pronation bilateral - fix dominant forearm in 0-15° pronation and nondominant forearm in neutral older studies state the nondominant forearm should be placed in 10-15° of supination; however, this was at a time when keyboards and mobile devices were not as ubiquitous and is no longer recommended stabilization casting alone (no fixation) circular external fixator frame (Ilizarov) percutaneous pins outcomes most techniques result in improved forearm position and patient function with low rate of deformity recurrence Complications Recurrence of synostosis nearly 100% recurrence of synostosis with excision alone or with interposition of anconeus muscle interposition of vascularized fascio-fat graft has 0% recurrence Recurrence of malrotation casting after derotational osteotomy associated with 15-20° loss of correction Compartment syndrome up to 36% associated with large rotational corrections > 60° close observation post-operatively some authors advocate for prophylactic forearm fasciotomies in acute and/or large deformity corrections Neurologic deficit PIN palsy - particularly with proximal (synostosis) osteotomy AIN palsy radial nerve palsy higher risk with acute/large deformity correction most resolve within 3 months