Congenital Proximal Radioulnar Synostosis Research Articles

Congenital Radioulnar Synostosis – A Case Report

Congenital radio-ulnar synostosis (CRS) is a rare congenital disorder characterized by the fusion of the radius and ulna bones in the forearm. This results in a lack of mobility and flexibility in the forearm, and can cause functional limitations, including difficulty with grasping and holding objects. A 4-year-old child with pain and restricted mobility of the elbow joint was admitted to the hospital. Plain film radiography and CT examination was performed. Radiological examinations showed a congenital proximal radioulnar synostosis. Diagnostic imaging including computed tomography with three-dimentional (3D) reconstructions, preceding surgery enables planning of the surgical treatment.

Type 4 congenital proximal radio-ulnar synostosis mimicking as malunited radial neck and deformed radial head with insidious onset extension block: a case report and results after excision

<p class="abstract">Proximal radio ulnar synostosis is a rare entity presenting with restriction of supination and pronation. Among the various types, type 4 variety is even more-rare and its presentation, can pose a diagnostic challenge specially, if with history of trauma. Radiologically, it is usually overlooked as a malunited radial neck and therefore needs thorough evaluation. We presented a case of a 15-year-old male who complains of new onset extension block following trauma, with chronically restricted supination and pronation. History suggested the restriction in supination and pronation since birth, and the extension block occurring de novo. Radiographs and CT scan of the elbow revealed proximal radio-ulnar synostosis and anteriorly dislocated, mushroom-shaped radial head, misleading and mimicking like an old, neglected and malunited radial neck fracture with a deformed radial head. The family insisted to not address the restriction of supination pronation but, asked for solution to more disabling extension block. Thus, patient was managed with open procedure, involving radial head-neck excision and removal of malformed radial head. At 1-year follow-up, the child has a well-reduced and stable elbow joint with a functional range of flexion and extension movements with restricted supination and pronation. Type 4 congenital radio ulnar synostosis not only leads to conventionally known restriction of supination pronation but also give rise to extension block de novo as presented in this case. Excision of the deformed head and neck can treat the restriction in extension, however the synostosis will require various other augmented procedures.</p>

An anatomical classification of congenital proximal radioulnar synostosis based on retrospective MRI measurement combined with radiography

Existed classifications of congenital proximal radioulnar synostosis (PRUS) mainly focus on osseous changes and do not cover all types of congenital PRUS, ignoring the role and developing status of the supinator. This study aims to explore the correlation between supinator development and radiographic deformity of congenital PRUS. Pediatric patients diagnosed with congenital PRUS in two pediatric Orthopedic centers were evaluated retrospectively. MRI and radiographic images of their bilateral forearms (including normal ones) were collected. The area of supinator, extensor carpi radialis longus (ECRL), extensor carpi radialis brevis (ECRB), brachioradialis (BRAR) muscle and extensor indicis (EI) muscle were measured on each forearm. The ratios of these muscles were calculated and regarded as an indicator of the developing status of supinator muscle. Twenty-seven congenital PRUS forearms of 16 patients (average 3.45 years) were included. A new MRI & X-ray classification system was proposed to cover all types of radiographic deformity and provide a comprehensive description of supinator development. This study revealed the relation between MRI measured supinator volume and radiographic deformity of congenital PRUS. Supinator muscles were observed in all congenital PRUS cases. A novel classification was proposed, providing a more comprehensive understanding of congenital PRUS.

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

PurposeTo evaluate the clinical outcomes of derotational osteotomy followed by plate fixation at the radius and ulna for the treatment of congenital proximal radioulnar synostosis.MethodsA total of 10 eligible patients (12 forearms) with congenital proximal radioulnar synostosis were admitted to our institution from January 2013 to January 2016 and treated by radioulnar derotational osteotomy followed by plate fixation. There were 5 males and 5 females with an average age of 5.4 ± 2.0 (3–9) years old. The average forearm position was 56.67 ± 14.36° (range, 40°–80° pronation) in pronation before surgery. According to the classification system of Cleary and Omer, 3 forearms were categorized as type II, 7 as type III, and 2 as type IV. The pre- and postoperative forearm function was recorded and evaluated by the Failla scoring system.ResultsAll included patients were successfully followed up for an average time of 73.90 ± 8.24 months (range, 61–84 months). The mean achieved correction of the forearm was 53.33° ± 12.67° (range, 35°–70°). The average final position was 3.33° ± 14.98° (range, 20° of supination to 25° of pronation) in pronation. Bony union was achieved in a mean of 10.38 ± 1.25 weeks (range, 8.4–12.3 weeks) with no loss of correction. There were no incidences of nonunion, osteomyelitis, or neurologic or circulatory complications. The preoperative functional results were good in 1 forearm, fair in 8 forearms and poor in 3 forearms. In terms of final follow-up functional evaluations, 3 forearms were excellent, 6 forearms were good, and 3 forearms were fair.ConclusionsCongenital proximal radioulnar synostosis can be successfully treated using derotational osteotomy and plate fixation of the radius and ulna, which is an effective method with fewer postoperative complications and expected clinical outcomes.

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.

Radial Pronation Angle: A Novel Radiological Evaluation Index of Congenital Proximal Radioulnar Synostosis.

Congenital proximal radioulnar synostosis (CPRUS) is a relatively rare forearm deformity that is characterized by a fixed pronated forearm of varying severity. The osseous synostosis between the proximal part of the ulna and the radius can be seen on the X-ray images in most cases. Many researchers have attempted to identify methods to measure the disease severity to guide in the treatment of CPRUS. However, to describe the overall deformity, the use of multiple indicators is essential, and some of these measurements require special software or need to be conducted on cadavers. The aim of the current study was to introduce the radius pronation angle (RPA), a novel radiological evaluation index of CPRUS, and analyze the relationship between the RPA and the severity of the deformity. Three-dimensional models of 43 CPRUS forearms (19 left forearms and 24 right forearms) of 32 patients (23 males and 9 females; average age was 6 years 8 months; range, from 1.5 to 27 years) treated at Beijing Ji Shui Tan Hospital during 2016 to 2019 were reconstructed using a computer-assisted technique. The special flexed posterior-anterior views of the X-ray image (the f-PA view) of the forearms were obtained, and the forearm rotation angle and the ulnar inner rotation angle were measured on each forearm. The RPA was measured on the f-PA view, and the lengths of the osseous synostosis, ulna, and the radial head were measured on the computed tomography scan images using the multiplanar reconstruction function. The Pearson index was analyzed between the RPA and the other measurements. The RPAs were correlated with the forearm rotation angle, ulnar inner rotation angle, relative length of the osseous synostosis, and the relative length of the radial head (P < 0.05). The RPA can be measured quickly and easily on the f-PA view of the X-ray image and can be used as a reliable indicator of the severity of CPRUS.

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

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.

The experience of treatment of locking elbow with congenital proximal radioulnar synostosis

The experience of treatment of locking elbow with congenital proximal 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

Three-dimensional analysis of deformities of the radius and ulna in congenital proximal radioulnar synostosis.

We reconstructed three-dimensional images of radius and ulna in 38 forearms of 25 patients with congenital proximal radioulnar synostosis from their computed tomographic studies. We also analysed correlations between the deformities of radius and ulna and degrees of fixed pronation of these forearms. The average ulnar deviation, flexion and internal rotation deformities of the radius were 6°, 3° and 18°, respectively. The average radial deviation, extension and internal rotation deformities of the ulna were 3°, 4° and 30°, respectively. The flexion deformity of the radius and the internal rotation deformity of the radius and ulna were correlated significantly with degree of fixed pronation. We conclude that the patients with congenital proximal radioulnar synostosis have remarkable flexion deformity of the radius and internal rotation deformity of the radius and ulna, which might impede forearm rotation after corrective surgery in the proximal part of the forearm.

Evaluation of ADL Before and After Mobilization of a Congenital Proximal Radio-Ulnar Synostosis

Evaluation of ADL Before and After Mobilization of a Congenital Proximal Radio-Ulnar Synostosis

Changes of Supination and Pronation Range during Elbow Flextion-extention Motion in Post Separation Operative Congenital Proximal Radio-ulnar Synostosis

Changes of Supination and Pronation Range during Elbow Flextion-extention Motion in Post Separation Operative Congenital Proximal Radio-ulnar Synostosis

Snapping elbow with congenital proximal radioulnar synostosis

Snapping elbow with congenital proximal radioulnar synostosis

Postoperative Results of Ulnar Rotational Osteotomy with Separation Mobilization Surgery for Congenital Proximal Radioulnar Synostosis

Postoperative Results of Ulnar Rotational Osteotomy with Separation Mobilization Surgery for Congenital Proximal Radioulnar Synostosis

Mobilization of a Congenital Proximal Radioulnar Synostosis with a Vascularized Fascio-fat Graft in 104 Forearms of 87 Patients: Level 4 Evidence

There is no financial information to disclose. Congenital proximal radioulnar synostosis is a rare congenital anomaly characterized by a fixed forearm rotation. It has the high tendency toward re-ankylosis after separation without interposition between separated radius and ulna. Can the congenital radioulnar synostosis be mobilized? If so, what is the safe and simple procedure for mobilization? We performed mobilization procedures for 104 forearms in 87 patients. Through a posterior incision, the synostosis was exposed and separated with a high-speed burr. After separation, osteotomy of the radius was performed to reduce the dislocated radius head. After that, a vascularized fascio-fat was interposed between separated radius and ulna. Four different procedures were used to interpose the vascularized fat. A free vascularized fascio-fat graft (FVFG) was used in 26 forearms, a pedicle forearm fascio-fat graft with one incision (PVFG-1) in 25, a pedicle forearm fascio-fat graft with both anterior and posterior incisions (PVFG-2) in 26, and a pedicle upperarm fascio-fat graft in two incisions (PVFG-3) in 27. The mean age at the surgery was 8.1 years (range, 5.1 to 13 years), 7.4 years (range, 4.3 to 10.9 years), 7.8 years (range, 4 to 14 years) and 6.0 years (range 4.5 to 6.0) respectively. Preoperative forearm ankylosis was 34.8 degrees of pronation in FVFG, 39.3 degrees in PVFG-1, 40.2 degrees in PVFG-2, and 37.4 degrees in PVFG-3. They were followed-up more than 2 years after surgery. All patients reported improvements in performing some activities, such as catching a ball, accepting objects such as coins, holding a bowl of soup, and performing gymnastics. Four re-ankyloses were observed among 104 mobilizations. The mean range of active forearm rotation (mean ± SD) after mobilization was 81.6 ± 15.8 degrees in FVFG, 75.4 ± 23 degrees in PVFG-1 excluding 3 re-ankyloses, 71.1 ± 27.4 degrees in PVFG-2, and 70.2 ± 25.4 degrees in PVFG-3 excluding 1 re-ankylosis. The average surgery time was 9.8 ± 0.79 hours in FVFG, 4.3 ± 2.0 hours in PVFG-1, 3.9 ± 1.3 hours in PVFG-2, and 4.2 ± 1.7 hours in PVFG-3. •This mobilization procedure prevented re-ankylosis after separation of the synostosis and provided some forearm rotation that improved a child's daily activities.•Mobilization with a pedicle fascio-fat graft shortened the surgery time than those with free vascularized fascia-fat graft.•Two-incision technique could securely fasten PVFG to the space between separated radius and ulna and may prevent re-ankylosis.

Minimally invasive single-session double-level rotational osteotomy of the forearm bones to correct fixed pronation deformity in congenital proximal radioulnar synostosis.

BackgroundCongenital proximal radioulnar synostosis is the most common congenital disease of the elbow joints and forearms.MethodsThis was a prospective study of 12 consecutive children (14 forearms) who presented to the National Institute of Neuromotor System in Egypt between September 2012 and September 2013 with severe congenital proximal radioulnar synostosis, having a mean pronation deformity of 70.7° (range 60°–85°), and who underwent operative correction by single-session double-level rotational osteotomy and percutaneous intramedullary K-wires of both the radius and ulna. Ten forearms were type III, and four were type II according to Cleary and Omer classification. The mean age at the time of surgery was 5 years and 2 months (range 4 years and 10 months to 6 years and 5 months). They were evaluated for functional results after rotational corrective osteotomy at a mean interval of 30.4 months (range 24–36 months) by physical examination and radiographs.ResultsAll children had a mean pronation deformity correction of 59.8° (range 30°–90°) reaching a final position of 20°–30° of pronation in the affected dominant extremities and 20° of supination in the affected non-dominant extremities after osteotomy. All children showed improvement in functional activities, with no loss of correction or non-union in any child, and no circulatory disturbances, neuropathies, or hypertrophic scars.ConclusionMinimally invasive single-session double-level rotation osteotomy of the proximal ulna and distal radius with percutaneous intramedullary K-wire fixation is a safe, technically simple and efficient procedure which corrects pronation deformity.

Long-term results after a free vascularized adipofascial graft for congenital proximal radioulnar synostosis with an average follow-up of 10 years: a series of four cases

The aim of this study was to observe the long-term and chronologic changes in clinical and radiologic findings after a free vascularized adipofascial graft interposition with radial osteotomy for congenital proximal radioulnar synostosis (PRUS). Six forearms in 4 patients with congenital PRUS who underwent a free vascularized adipofascial graft interposition combined with radial osteotomy were followed up for an average of 10 years (8-12 years) postoperatively. Extension and flexion angles of the elbow and pronation and supination angles of the forearm as well as radiographs were evaluated preoperatively and throughout the postoperative follow-up period. The average extension/flexion angles of the elbow and the average pronation/supination angles of the forearm were 3°/130° and 14°/- before surgery, -4°/135° and 39°/23° at 1 year after surgery, 6°/138° and 44°/30° at 3 years after surgery, and -2°/139° and 35°/7° at the time of the final follow-up, respectively. Final radiographs showed hypertrophy of the radial head in 4 patients, dislocation of the radial head in 2 patients, and deformity of the radial head in 1 patient. Extension and flexion angles of the elbow in patients with congenital PRUS were constant and the average range of pronation was relatively well maintained throughout the postoperative period, but the average range of supination decreased by 16° from 1 year postoperatively to the time of the final follow-up.

Surgical outcome of delayed presentation of congenital proximal radioulnar synostosis.

Background: Presentation of proximal radioulnar synostosis varies from cosmetic concerns with no functional limitations to significant pronation deformity which hampers activities of daily living. Surgical management must be considered based on the position of the forearm and functional limitations. We describe the surgical technique, results, and complications of excision of the radial head along with the proximal radius up to the distal extent of the synostosis site and securing the osteotomized radial shaft with a tensor fascia lata graft. Materials and methods: Four patients having six affected elbows with delayed presentation of congenital proximal radioulnar synostosis with dislocated radial head managed surgically were included in the study. There were three males and one female with an average age of 20.25 years (ranging from 16 to 25 years). Preoperatively wrists were locked in the mean pronation position of 51.6° (ranging from 30° to 70°). The indications for surgery were limitation in activities of daily living and an obvious cosmetic deformity. Results: All patients were satisfied with the surgery and showed significant improvement in functional status. Mean active supination was 15° (ranging from 5 to 32°) with passive supination was a mean of 24.8° (ranging from 11° to 44°). Similarly, mean active pronation was 58.5° (ranging from 50° to 71°) with further passive correction up to a mean of 64.16° (ranging from 57° to 87°) at last follow up. Conclusions: This procedure is simple, cost effective, and a reasonable option for treatment of proximal radioulnar synostosis with a dislocated radial head in adult patients. The operation does not require any specialized team or implants, and can be performed in a moderately equipped hospital.

Results of Single-staged Rotational Osteotomy in a Child With Congenital Proximal Radioulnar Synostosis

For congenital proximal radioulnar synostosis, both conservative and operative treatments have been described. Most of the studies describing surgical interventions are based on subjective evaluation of the forearm function and have used severe degree of forearm pronation as an indication for surgery. This study describes a single-staged rotational osteotomy of the proximal third ulna and distal third radius. The aim of the study was to assess the utility of the described surgical procedure by subjective and objective evaluations of the forearm function. Forty-eight children with congenital proximal radioulnar synostosis were evaluated by subjective and objective assessments and were followed up prospectively. Subjective evaluation consisted of a set of 12 questions regarding the basic activities of life. Objective evaluation was made using the Jebsen-Taylor hand-function test and a classification system used by Failla and colleagues for 15 tasks described by Morrey and colleagues. Eleven children were treated conservatively. Thirty-six children underwent a single-staged rotational osteotomy of the proximal third ulna and distal third radius. After surgery, the evaluations were repeated. The mean age at surgery was 8.6±3.7 years, and the mean postoperative follow-up period was 54±13 months. All operated forearms showed a statistically significant improvement in functioning after surgery as per the subjective and objective evaluations. The mean time taken to carry out all activities before surgery was 47.7+10.0 seconds, which significantly reduced to 33.3+6.6 seconds after surgery (P=0.0001) as per the results of the Jebsen-Taylor hand-function test. All good (n=19) and fair (n=11) results were converted to excellent (n=30) after surgery as per the modified Failla classification. There were no neurovascular injuries as compared with other published techniques. Only 1 child had delayed union, and 1 had persistent dorsal angulation at the radial osteotomy site. For patients with congenital radioulnar synostosis and pronation deformity interfering with function and quality of life, the single-staged rotational osteotomy of the radius and ulna is a good alternative procedure. Level IV--therapeutic.