Congenital radioulnar synostosis: symptom complex and surgical treatment

Abstract

Background. Congenital radioulnar synostosis (CRUS) is a rare musculoskeletal disease with a wide-ranging symptom complex. Attitudes toward surgical treatment of the disease is very diverse, ranging from complete negation to acceptance. When choosing a treatment method, high recurrence and complication rates should be taken into account.Aims. To analyze the clinical implications of CRUS and to identify optimal treatment options.Materials and methods. From 2008 to 2015, 54 patients (31 boys and 23 girls; aged 1–14 years) with CRUS were examined and treated. Presenting complaints and the possible factors leading to disease development were investigated; orthopedic examination, roentgenography, electromyography, and computed tomography were performed. The treatment approach was determined on the basis of the clinicoroentgenological presentation.Results. All cases of CRUS were sporadic. In 43.7% patients, risk factors resulting in disease development were detected. Unilateral lesions were observed in 30 patients, whereas bilateral lesions were observed in 24 patients. According to the Cleary and Omer classification, the first type is the rarest; it is distinguished by the absence of bony fusion and close to average forearm positioning. In such cases, operative treatment is not necessary. For the second and third types, pronounced pronation forearm realignment requiring corrective derotational osteotomy of the radial bone is the main factor. For the fourth type, the main functional disorder is the restriction of the forearm flexion; treatment for this type involves resection of the radius head. We attempted to divide the synostosis (to achieve active movements) in five patients; however, we were unsuccessful. In three patients, synostosis recurrence occurred; and in two patients, active movements were not obtained after surgery. In four patients, radial nerve neuropathy was detected in the postoperative period after conservative therapy. In two patients, ulnar fractures occurred as a result of a fall; in one of these patients, fragment apposition was required.Conclusions. Clinicoroentgenological manifestations of CRUS determine the treatment options. The most typical and important of these manifestations is the pronation positioning of the forearm. In such cases, it is reasonable to start operative CRUS treatment after 3 years. All variants of deformation are indicators for operation, and treatment options are determined by the degree of severity of the deformation. Attempts to form the forearm bone neoarthrosis in order to get rotational movements is not effective and can result in deformation recurrence.

Congenital Bilateral Proximal Radio-Ulnar Synostosis in a Nigerian Child

“…Since our patient is still actively growing, her skeletal deformity and functional limitation may get worse, requiring orthopaedic specialist care in later life. Some authors consider it reasonable to start CRUS corrective surgery at the age of 3 years [8]. The radiological findings in our patient are in keeping with Cleary and Omer Type III bilaterally.…”

“…The functional deficits associated with CRUS depend on the severity of the malformation and whether or not it is bilateral; and the management may be conservative or surgical [8].…”

“…The frequency of CRUS among all congenital upper limb deformities ranges between 1.12% and 9.35%. Among other musculoskeletal deformities, the frequency of CRUS ranges between 0.11% and 0.61% [8]. Although its exact aetiology is not clear, usually cases of CRUS are sporadic, but in a number of instances, inherited synostosis has been reported [6] [8].…”

“…Among other musculoskeletal deformities, the frequency of CRUS ranges between 0.11% and 0.61% [8]. Although its exact aetiology is not clear, usually cases of CRUS are sporadic, but in a number of instances, inherited synostosis has been reported [6] [8]. A number of authors have reported a male preponderance of the disease while some reports have indicated that the disease has no sex predilection [1].…”

“…Currently, the attitude towards surgical intervention in patients with CRUS is controversial among orthopaedic surgeons. Indications for surgical treatment are related to bilaterality, the degree of deformity and the severity of the limitation of functions at the elbow joint(s) [1] [8]. Therefore, indications for surgery are based on individual functional limitations than on absolute forearm position [1] [8].…”