Congenital Radioulnar Synostosis

Abstract

Congenital radioulnar synostosis is a rare condition resulting in fusion of the proximal portions of the radius and ulna. Patients commonly present in early childhood with functional deficits because of limited forearm rotation and fixed positioning of the forearm. Compensatory motion and hypermobility are frequently observed at the wrist and shoulder, which may predispose these joints to overuse injury. Plain radiographs are used for diagnosis and classification. The most commonly used classification does not aid in treatment decisions. Limited high-level evidence exists to guide management because the literature primarily consists of case reports and case series. Nonsurgical management is often used, yet surgical management is most commonly reported in the literature. Resection of the synostosis has been shown to have high-complication rates and lead to subpar outcomes. Currently, surgical management most commonly involves one or more derotational osteotomies.

Derotational Osteotomy and Plate Fixation of the Radius and Ulna for the Treatment of Congenital Proximal Radioulnar Synostosis

“…CRUS is an uncommon congenital forearm deformity considered to be caused by the failure of longitudinal segmentation and formation of the proximal radius and ulna during the seventh week of development ( 1 , 2 , 10 – 12 ). The clinical characteristics of CRUS are highly diverse and the majority of patients present bilateral, yet asymmetric, affection.…”

“…The clinical characteristics of CRUS are highly diverse and the majority of patients present bilateral, yet asymmetric, affection. The noticeable range of motion will be limited in severe persistent pronation deformities with pathologic fusion ( 1 , 2 ).…”

“…Congenital radioulnar synostosis (CRUS) is one of the uncommon congenital upper limb deformities of the forearm that refers to an aberrant stiff connection between the proximal radius and ulna as a result of chromosomal disorders and other abnormalities of the upper limb ( 1 ). The majority of patients show bilateral involvement with the deformity ( 2 ).…”

“…With mild deformity, the ipsilateral shoulder and wrist joints can effectively compensate for any functional impairment ( 3 ). However, daily activities may be significantly restricted in cases with a severe permanent pronation deformity or whenever bilateral involvement is present ( 1 , 2 ).…”

“…Several surgical options, such as restoration of rotation and acute or staged derotation, have been described in the literature. Nevertheless, there is no common agreement on the surgical technique ( 1 – 27 ). A commonly accepted surgical management approach to treating CRUS is derotational osteotomy ( 3 , 10 – 17 , 19 – 26 ).…”