Results after treatment of congenital radioulnar synostosis: a systematic review and pooled data analysis

Abstract

Congenital radioulnar synostosis (CRUS) is one of the most common congenital disorders affecting the elbow and forearm, with the forearm being fixed in a range of positions usually varying from neutral rotation to severe pronation. The aim of this study, apart from a systematic review of all surgical procedures described for CRUS, is to derive any correlation between various influencing factors, outcomes and complications. This review was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses format by an electronic literature search of Ovid , MEDLINE and the Cochrane Library databases. Grading was according to the Newcastle-Ottawa scale and the Modified Coleman Methodology Score. Demographic data, surgical procedures, outcomes and complications were analyzed. Outcome data were pooled to establish means and ranges across all studies. Spearman correlations were performed. A total of 23 articles, showing a poor overall study quality (all Level of Evidence IV), met the inclusion criteria. A total of 374 forearms with a mean age of 6.7 years (2.0–18.8) were analyzed. Derotational surgeries were more commonly performed (91%) than motion-preserving surgeries (9%). The mean deformity improved from 64.8° pronation (−75° to 110°) to a mean of 2.8° pronation (−50° to 80°). In total, 17.9% of patients presented with complications. A significant correlation was noted between age and major complications, proximal osteotomies and complications, and postoperative loss of reduction and double level osteotomies as the primary treatment modality. Most of the complications occurred above the threshold of 65–70° of correction and in children 7 years and above. Surgery is essential to improve the quality of life of children with CRUS. However, each type of surgery is associated with complications, along with the respective hardware being used in rotation osteotomies. Caution is, nevertheless, warranted in interpreting these results in view of the inherent limitations of the included studies.

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.…”

“…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 ). Generally, the forearm is fixed in the pronation position, which is the most noticeable characteristic of this deformity.…”

“…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 ).…”

“…The treatment of CRUS is widely debated. Although It has been reported in the literature that surgery is essential to improve the quality of life of children with CRUS, there are no specific guidelines available to help the surgeon decide between nonsurgical and surgical treatment options for CRUS since each patient’s situation is different ( 2 ). As a result, surgical considerations should be based on functional impairment in addition to a significant pronation deformity.…”

MECOM-related disorder: Radioulnar synostosis without hematological aberration due to unique variants

“…[4][5][6] In contrast, orthopedic surgery is the primary focus for patients with isolated RUS. 7,8 Genetic differences also exist between isolated RUS and RUSAT. RUSAT is usually caused by heterozygous HOXA11 or MECOM variants.…”