Congenital Radioulnar Synostosis Research Articles

Letter regarding 'Results after treatment of congenital radioulnar synostosis: a systematic review and pooled data analysis'.

Letter regarding 'Results after treatment of congenital radioulnar synostosis: a systematic review and pooled data analysis'.

Congenital Radioulnar Synostosis

Introduction: congenital radioulnar synostosis is an uncommon congenital anomaly, thatcauses a limitation of the forearm supination, often bilateral. The origin of this anomaly occurs when the differentiation of the cartilaginous contour of the radius and ulna does not take place during the embryonic period.
 Objective:to show two congenital radioulnar sinostosis cases analyzing variable about the topic.
 Case report:there are two different types of synostosis. In these cases the symptoms are mild because the compensate thein limitation in pronosupinationwith hypermobility of other joints of the upper limb. X-rays of upper limbs are the diagnostic procedures. The management will be conservative, but the treatment depends on the degree of functionality of the forearm.
 Conclusions:proximal radioulnarsynostosis is anon frequent affection, that brings about difficulties carrying out actions with the hands, Its diagnosis is relatively simple by means of the clinical findings andplain X-rays of the elbow.

Congenital Radioulnar Synostosis

Introduction: congenital radioulnar synostosis is an uncommon congenital anomaly, that causes a limitation of the forearm supination, often bilateral. The origin of this anomaly occurs when the differentiation of the cartilaginous contour of the radius and ulna does not take place during the embryonic period. Objective: to show two congenital radioulnar synostosis cases analyzing variable about the topic. Case report: there are two different types of synostosis. In these cases the symptoms are mild because the compensate thein limitation in pronosupination with hypermobility of other joints of the upper limb. X-rays of upper limbs are the diagnostic procedures. The management will be conservative, but the treatment depends on the degree of functionality of the forearm. Conclusions: proximal radioulnar synostosis is a non frequent affection, that brings about difficulties carrying out actions with the hands, Its diagnosis is relatively simple by means of the clinical findings andplain X-rays of the elbow.

Measurement of compensatory wrist joint rotation using three-dimensional motion analysis in patients with unilateral proximal congenital radioulnar synostosis.

This study aims to investigate compensatory rotational movements of the wrist joint in patients with proximal congenital radioulnar synostosis (CRUS), using a valid and reliable three-dimensional (3D) motion analysis technique. A total of 26 patients (6 females, 14 males; mean age=15.3 years; and age range=6-32 years) who were diagnosed with unilateral proximal CRUS but were not operated were enrolled in this study. Patients were then categorized into 2 groups: Group I included 5 patients younger than 10 years, and Group II included 15 patients older than 10 years. Eighteen light-reflective skin markers were placed on the bony landmarks of both upper limbs, and both distal forearms were fixed using a U-shaped device to minimize forearm rotation. Each patient grasped the handle of an instrument that used a goniometer to measure wrist rotation; maximal passive pronation and supination angles of the wrist were measured in this manner and also using 3D motion analysis. There was a significant correlation between measurements by the goniometer and 3D motion analysis (r=0.985, p<0.001). The test-retest reliability of the 3D motion analysis was acceptable for both the affected side (ICC=0.992) and the contralateral normal side (ICC=0.997) with low standard measurement errors (1.3° and 0.8°, respectively). Although no significant difference was observed in the range of the wrist rotation between the affected and contralateral sides in Group I (p=0.686), there was a significant difference in the wrist rotation between the affected and contralateral sides in Group II (p=0.001). Further, the pronation angle of the wrist joint was significantly larger in the affected side than that in the contralateral normal side in Group II (p=0.001). The 3D motion analysis technique seems to be a valid and reliable method to measure the rotation of the wrist joint. Unilateral proximal CRUS patients older than 10 years of age may develop rotational hypermobility of the wrist joint compared to the contralateral normal side as a compensatory phenomenon. Level III, Diagnostic Study.

Congenital Radioulnar Synostosis.

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 in the treatment of congenital radioulnar synostosis: A report of 11 cases

Introduction: Congenital proximal radio-ulnar synostosis is a rare congenital anomaly of the upper extremity which is severely disabling as it causes severe pronation deformity. So the aim of this study was to assess the efficacy of proximal radioulnar derotational osteotomy followed by internal fixation for the treatment of congenital radioulnar synostosis.Materials and Method: Between June 2018 and April 2020, 11 patients with CRUS who underwent derotational osteotomy at the proximal radioulnar synostosis site were evaluated. There were 7 boys and 4 girls. The mean age at the time of surgery was 10.5 years (range, 2 to 13) years. The forearm was derotated to the goal position (20 degrees of supination to 10 degrees of pronation) using plates for internal fixation and plaster splints for external immobilization. Pre- and postoperative positions of the forearm were recorded; forearm function was evaluated based on the classification system proposed by Failla et al.Results: The mean follow-up duration was 6 months. The mean initial pronation deformity was 65.0 ± 5.2 (55 to 80) degrees. The mean correction achieved was 75.27 ± 5.25 (50 to 90) degrees, resulting in a mean final position of 9.45 ± 3.25 degrees of supination (20 degrees of supination to 10 degrees of pronation). Based on the Failla classification system, 7 forearms were rated as good, 4 were rated as fair preoperatively. At the final follow-up, 9 forearms were rated as excellent and 2 were rated as good. All patients achieved bone union after 10 weeks. Post-operative period was uneventful and no complications were seen.Conclusion: Proximal radioulnar derotational osteotomy followed by plate fixation is a safe and feasible procedure with a low complication rate. The technique can effectively improve the function of the forearm.

Application of 3-Dimensional Printing Technology in Rotational Osteotomy of Congenital Radioulnar Synostosis

To investigate whether computer-aided design (CAD) and 3-dimensional printing technology can assist in accurate completion of ulna-radius proximal rotational osteotomy in congenital radioulnar synostosis (CRUS). We treated 1 right arm and 4 left arms of 4 boys with a mean age of 5.2 years (range, 4.3-6.0 years) between July 2018 and April 2019. Computed tomography (CT) scans of the forearm were performed on the children before surgery. Using the CT data, the pronation angle of the forearm was measured. An individualized dial with a matching forearm diameter was designed before surgery to control the angle of the rotational osteotomy. Ulna and radius models, and individualized dials, were prepared for each patient using 3-dimensional printing technology. Preoperative simulated surgery was performed using the 3-dimensional printed models. During the surgery, 2 Kirschner wires were used as operating levers for rotation, and rotational angle correction was precisely controlled using the dial. The cast and internal fixation were removed after x-ray examination showed that the osteotomy had healed, about 5 weeks after surgery. The rotational osteotomies were completed in a single operation, and the correction angle was 60°. No complications occurred after the operation. All patients showed functional improvements in activities of daily living. For CRUS patients, models and dials made with CAD and 3-dimensional printing technology can assist in precise rotational osteotomy. Therapeutic V.

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

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.

A Three-Step Method for the Treatment of Radioulnar Synostosis with Posterior Radial Head Dislocation

Congenital radioulnar synostosis with posterior dislocation of the radial head remains challenging to treat. We describe a three-step treatment method that combines radial shaft osteotomy with a custom-made device, ulnar shaft osteotomy, and local adipofascial flap elevation procedures. For posterior radial head dislocation treatment, osteotomy near the proximal radius cannot recover physiological rotation of the radial head. Thus, we chose a precise radial shaft osteotomy with a custom-made device according to preoperative planning based on three-dimensional evaluation of the bone deformation. Performing radial shaft osteotomy alone, however, may not be enough to achieve sufficient supination range of motion. We, therefore, also performed ulnar shaft osteotomy. Finally, we elevated the local adipofascial flap to prevent re-adhesion. In three patients, the range of motion of the elbow improved postoperatively. In conclusion, our three-step method does not require a microsurgical technique and is easy to perform.

Case report: an adult presenting with congenital radio-ulnar synostosis

Case report: an adult presenting with congenital radio-ulnar synostosis

Treatment of congenital radioulnar synostosis with radial derotational osteotomy and local subcutaneous pedicled fat flap filling in fusion area

To investigate the effectiveness of local subcutaneous pedicled fat flap filling and separation of the synostosis area associated with radial shortening osteotomy and plate internal fixation for the treatment of congenital radioulnar synostosis. Between February 2014 and June 2018, 36 patients (41 sides) with congenital radioulnar synostosis were analyzed retrospectively, including 21 males and 15 females, aged 2.5-4.5 years with an average of 3.1 years. The fixed pronation deformity of the forearm ranged from 30° to 90° with an average of 71.6°, and the range of motion of the elbow flexion was 120°-135° with an average of 128.2°. According to the Cleary-Omer classification, there were 8 sides of type Ⅱ, 17 sides of type Ⅲ, and 16 sides of type Ⅳ. All patients were treated by local subcutaneous pedicled fat flap filling and separation of the synostosis area associated with radial shortening osteotomy and plate internal fixation. The range of motion of the elbow, muscle strength, joint stability, and patient discomfort were evaluated by using the Broberg and Morrey elbow scoring system preoperatively and postoperatively. In addition, the ability for daily living of the affected limb was evaluated by using the Failla grading standard. Radial nerve palsy occurred in 3 cases, and nerve function recovered at 2-4 weeks after operation. All the 36 cases were followed up 6-52 months, with an average of 38 months. All osteotomy sites healed, the healing time was 5-12 weeks (mean, 6.3 weeks), and the pedicled fat flap between the radius and ulna survived when the internal fixation was taken. At last follow-up, the flexion range of motion of elbow joint was not decreased, and the pronation and supination range of motion of forearm were improved. The elbow flexion range of motion was 125°-135° with an average of 132.4°. The pronation range of motion of forearm was 15°-45° with an average of 30.1°, and the supination range of motion of forearm was 10°-40° with an average of 22.6°. At last follow-up, the Broberg and Morrey elbow scores increased from the preoperative 85.6±1.0 to 91.8±1.8, showing significant difference ( t=25.593, P=0.000). Moreover, the results were good in 3 sides, fair in 9 sides, and poor in 29 sides according to the Failla grading standard before operation, with an excellent and good rate of 7.3%. At last follow-up, the results were excellent in 6 sides, good in 28 sides, and fair in 7 sides, with an excellent and good rate of 82.9%, showing significant difference when compared with preoperative value ( Z=-5.781, P=0.000). The application of local subcutaneous pedicled fat flap filling and separation of the synostosis area associated with radial shortening osteotomy and plate internal fixation is an effective surgical method for the treatment of congenital radioulnar synostosis. It can restore the partial rotation function of the forearm and improve the quality of life of children.

What's New in Congenital Hand Surgery.

Congenital conditions of the hand and upper extremity are a frequent source of consultation among pediatric orthopaedists and hand surgeons. Advances in the fields of molecular biology and genetics have helped to better understand some of these conditions and redefine previous classification systems. New outcome measurement tools have been used to assess surgical results and have brought into focus a different aspect of the patients' experience. We searched PubMed database for papers related to the treatment of congenital hand anomalies published from January 1, 2015 to October 31, 2018. The search was limited to English articles yielding 207 papers. Three pediatric hand surgeons selected the articles based upon the criteria that the topic was germane, the article fell under the subheadings within the manuscript, and the conclusions were meaningful. A total of 40 papers were selected for review, based upon their quality and new findings. Research articles with significant findings were included for syndactyly, symbrachydactyly, cleft hand, polydactyly, radial longitudinal deficiency, congenital radio-ulnar synostosis, and macrodactyly. Our knowledge of the embryology and pathophysiology of congenital upper extremity conditions continues to evolve. Functional assessments combined with patient and parent-reported outcomes have our understanding of the results following surgical procedures. Further research and standardization of our scientific data will provide better answers and higher quality of evidence. Level V-literature review and expert opinion.

Surgical outcome of both bone de-rotation osteotomy in congenital radioulnar synostosis: A case report and literature review

Radioulnar synostosis is found as a congenital deformity in children or as a post-traumatic deformity in adolescents and adults. Congenital variety is more commonly located in the proximal part of the radius and ulna and restricts the range of motion of the forearm leading to some functional disabilities in the child such as using daily objects and carrying out basic hand to mouth activities like eating. The pronation deformity occurring as a result of this entity can be effectively dealt with single stage both bone forearm de-rotation osteotomy done at different levels in radius and ulna. We report our experience using this procedure in a 4-year old child with unilateral proximal congenital radioulnar synostosis in the non-dominant side. This procedure is relatively simple and we were able to achieve a good functional outcome at one year of follow-up with no associated complications.

Application of fusion site osteotomy combined with ulna and radius osteotomy for the treatment of congenital proximal radioulnar synostosis

Objective To explore the clinical features and surgical treatment of congenital proximal radioulnar synostosis. Methods From May 2014 to May 2017, 9 cases of congenital proximal radioulnar synostosis were treated in our hospital. By preoperative X-ray examination, forearm shape, forearm rotation function, elbow flexion and extension function, the fusion and development of radial head, ulna and radius were observed and classified. The operations including fusion site osteotomy, ulna osteotomy, radius osteotomy, interosseous membrane release and circular ligament reconstruction were performed. Results Nine patients performed operation on 14 sides were follow-up for 3 to 38 months with an average of 17 months. No re-synostosis occurred, and the shape and rotation function of the forearm were improved. The elbow function was evaluated by Mayo score, and the results were excellent in 7 sides, good in 5 sides and fair in 2 sides. Conclusion Congenital proximal radioulnar synostosis seriously affects the quality of life of children, and the fusion site osteotomy combined with ulna and radius osteotomy can achieve good treatment outcomes. Key words: Congenital abnormalities; Surgical procedures, operative; Proximal radioulnar synostosis; Osteotomy

Efficacy and feasibility of proximal radioulnar derotational osteotomy and internal fixation for the treatment of congenital radioulnar synostosis

BackgroundThe aim of this study was to assess the feasibility and efficacy of proximal radioulnar derotational osteotomy followed by internal fixation for the treatment of congenital radioulnar synostosis (CRUS).MethodsBetween May 2008 and August 2016, 31 patients (36 forearms) with CRUS who underwent derotational osteotomy at the proximal radioulnar synostosis site were evaluated. There were 20 boys and 11 girls. The mean age at the time of surgery was 4.87 ± 3.06 (range, 2 to 13) years. The forearm was derotated to the goal position (20 degrees of supination to 10 degrees of pronation) using plates for internal fixation and plaster splints for external immobilization. Pre- and postoperative positions of the forearm were recorded; forearm function was evaluated based on the classification system proposed by Failla et al.ResultsThe mean follow-up duration was 55.19 ± 27.10 (24 to 123) months. The mean initial pronation deformity was 62.92 ± 7.11 (55 to 80) degrees. The mean correction achieved was 70.86 ± 9.58 (50 to 90) degrees, resulting in a mean final position of 7.94 ± 7.25 degrees of supination (20 degrees of supination to 10 degrees of pronation). Based on the Failla classification system, 2 forearms were rated as good, 30 were rated as fair, and 4 were rated as poor preoperatively. At the final follow-up, 34 forearms were rated as excellent and 2 were rated as good. All patients achieved bone union after 2 months. Complications occurred in three patients (two transient nerve palsies and one compartment syndrome), and the overall complication rate was 9.7%.ConclusionsProximal radioulnar derotational osteotomy followed by plate fixation is a safe and feasible procedure with a low complication rate. The technique can effectively improve the function of the forearm.Level of evidence IVRetrospective case series

Surgical correction of congenital radioulnar synostosis: a protocol of treatment

Purpose The aim of this study is to establish a protocol of treatment with clear indications of surgical intervention in congenital radioulnar synostosis (CRUS) and to assess the results of this protocol by performing the osteotomy through the synostosis mass. Patients and methods A total of 20 forearms with CRUS in 15 patients underwent surgical correction through an osteotomy of the synostosis mass according to our protocol. The mean age of the patients at surgery was 6.5 years. The synostosis mass was divided, and the osteotomy was fixed using an intramedullary wire. The average duration of follow-up was 4.2 years (3–6 years). Results The desired correction was achieved intraoperatively in all patients. The mean preoperative fixed pronation was 80.7° in the dominant limb, with a mean improvement of 40.7°, and 29.1° in the nondominant limb, with a mean improvement of 49.1°. All patients were extremely satisfied with the result of surgery regarding the cosmetic improvement as well as performing activities of daily living comfortably. Conclusion Correction of CRUS by osteotomy through the synostosis mass is a safe, easy, and efficient technique that markedly improves the child’s function. The clear guidelines set in this study on when to intervene, which forearm to correct, and how much derotation to perform help clarify confusion in literature. Because of loss of correction observed in all forearms, we recommend overcorrection by 10° than initially planned according to the protocol.

A case report of congenital bilateral proximal radioulnar synostosis in a 22-month-old child

Congenital radioulnar synostosis is a skeletal abnormality present at birth which may appear clinically as a mild abnormality in the early years. The diagnosis is usually delayed until functional abnormality is noticed. This is a typical case of congenital bilateral proximal radioulnar synostosis diagnosed at about 2 years despite mild upper limb abnormality noticed at birth. The rarity of this condition prompted the report of this case.

Outcomes of derotation osteotomy at synostosis site for congenital radioulnar synostosis

Congenital radioulnar synostosis (CRUS) is rare upper limb malformation in which proximal portions of the radius and ulna fuse with each other and restricts the rotation of the forearm. In cases with severe deformity, performing activities of daily living is difficult, and a surgical intervention is recommended. We present a retrospective study including patient operated for CRUS between January 2009 and December 2017 by derotation in synostosis site. Ten patients were operated. Five was unilateral and five bilateral (only one patient was operated for both sides. Mean age was 5 years. Preoperative assessment revealed limitation of the activities of daily life in all patients with an average pronation deformity of 62o. Type III of Cleary and Omer classification was the most frequent.. The average correction of the forearm angle was 54o. Nine patients were satisfied and showed improvements in the ability to perform the activity of daily life. We report one case of complication (compartmental syndrome with nonunion). In summary, CRUS is a rare deformity, frequently bilateral and more commonly seen in male patients. Indications for surgery are pronation deformity of 60o or more, bilaterality and pronation of 20o to 60o with disability. It has been reported that complications are more common in osteotomy in synostosis site.

Long-term results after mobilization with a free vascularized adipofascial graft for congenital radioulnar synostosis

Long-term results after mobilization with a free vascularized adipofascial graft for congenital radioulnar synostosis

Derotational shortening osteotomy at proximal 1/3 and middle 1/3 of the ulna and radius for the treatment of congenital proximal radio-ulnar synostosis

Objective To explore the clinical efficacy and safety of derotational shortening osteotomy at proximal 1/3 and middle 1/3 of the ulna and radius for the treatment of congenital proximal radio-ulnar synostosis, and to determine the optimal forearm position. Methods From February 2010 to April 2016, 36 cases (44 forearms) with congenital proximal radio-ulnar synostosis were treated by derotational shortening osteotomy at proximal 1/3 and middle 1/3 of the ulna and radius. The forearm was fixed at 30° to 110° of pronation before the operation. The forearm was placed in a certain supination position during the operation, and the osteotomy end was fixed with plate and screw. The forearm position was measured at the time of internal fixator removal and last follow-up, and the forearm rotation range after shoulder compensation was measured before and after operation. Results All the patients were follow-up for 18 to 72 months, with an average of 31 months. The forearm was fixed at -10° to 80° of supination, with an average of (33. 64±19. 66)° on the left side and (21. 36±14. 51)° on the right side. The degree of correction was 60° to 140°. The range of pronation after shoulder compensation was (188. 18±10. 44)° and (122. 58±20. 43)° before and after the operation respectively, while the range of supination was (-33. 64±33. 52)° and (60. 30±27. 07)°. The difference was statistically significant before and after operation (P 0. 05). The results from the right side were the same as those from the left side. The plate of radius was broken in 4 cases. No complications such as fracture of ulnar plate, nonunion of fracture, deformity recurrence, forearm ischemic contracture and neurovascular injury were observed in all patients. Conclusion The derotational shortening osteotomy at proximal 1/3 and middle 1/3 of the ulna and radius is safe and effective for the treatment of congenital proximal radio-ulnar synostosis. Placing forearm at 30° to 50° of supination is better for the function of forearm and hand. Key words: Osteotomy; Treatment outcome; Congenital proximal radio-ulnar synostosis; Deformity