UNASSIGNED: Radioulnar synostosis is an uncommon complication of forearm fractures and presents with varying degrees of restricted forearm movement. The diaphysial distal third synostosis is less common and excision of the synostosis is fraught with risk of re-ossification. Use of inert or biological interposing material has thus been accompanied with the synostosis excision and various methods have been described. There is still no consensus on the ideal treatment method.
UNASSIGNED: We, hereby, report a case of a long-standing radioulnar synostosis with rotational restriction of movement. Despite the movement restriction, the patient could perform basic activities of daily living and wanted to improve the movements. The presence of diaphyseal radioulnar synostosis was conformed on the radiographs and computerized tomography scan. A volar forearm approach was used and the bony bridge was excised. The ipsilateral native palmaris longus (PL) tendon was extracted from distal wrist crease and with its proximal attachment intact, circumferentially wrapped around the ulnar raw surface as an interposing material. Apart from this, free fat was also placed at the synostosis site. In the long-term follow-up of 10 years, there was no radiological evidence of re-ossification noted. The clinical improvement was not much but the patient was performing activities of daily living with no discomfort.
UNASSIGNED: The use of an encircling loop of the native PL tendon, over the raw surface of one of the forearm bones, may be another useful method to decrease the chances of recurrence following the excision of the synostosis.

暂无摘要。

BACKGROUND: The Boyd approach is a single-incision posterior approach to the proximal radius and ulna based on a lateral anconeus muscle reflection and release of the lateral collateral ligamentous complex. This approach remains a lesser-used technique following early reports of proximal radioulnar synostosis and postoperative elbow instability. Although limited by small case series, recent literature does not support these early reported complications. This study presents a single surgeon\'s outcomes using the Boyd approach for the treatment of simple to complex elbow injuries.
METHODS: Following institutional review board approval, a retrospective review of all patients with simple to complex elbow injuries treated consecutively using a Boyd approach by a shoulder and elbow surgeon was conducted from 2016 to 2020. All patients with at least 1 postoperative clinic visit were included. Data collected included patient demographics, injury description, postoperative complications, elbow range of motion, and radiographic findings including heterotopic ossification and proximal radioulnar synostosis. Categorical and continuous variables were reported using descriptive statistics.
RESULTS: A total of 44 patients were included with an average age of 49 years (range 13-82 years). The most commonly treated injuries were Monteggia fracture-dislocations (32%) and terrible triad injuries (18%). Average follow-up was 8 months (range 1-24 months). Final average elbow active arc of motion was from 20° (range 0°-70°) of extension to 124° (range 75°-150°) of flexion. Final supination and pronation were 53° (range 0°-80°) and 66° (range 0°-90°), respectively. There were no cases of proximal radioulnar synostosis. Heterotopic ossification contributing to less than functional elbow range of motion occurred in 2 (5%) patients who elected conservative management. There was 1 (2%) case of early postoperative posterolateral instability due to repair failure of injured ligaments that required revision using a ligament augmentation procedure. Five (11%) patients experienced postoperative neuropathy, including 4 (9%) with ulnar neuropathy. Of these, 1 underwent ulnar nerve transposition, 2 were improving, and 1 had persistent symptoms at final follow-up.
CONCLUSIONS: This is the largest case series available demonstrating the safe utilization of the Boyd approach for the treatment of simple to complex elbow injuries. Postoperative complications including synostosis and elbow instability may not be as common as previously understood.

Background and Objectives: Only nine patients with interstitial de novo 8q22.2q22.3 microdeletions have been reported to date. The objective of this report is to present clinical features of a new patient with an 8q22.2q22.3 microdeletion, to compare her phenotype to other previously reported patients, and to further expand the phenotype associated with this microdeletion. Materials and Methods: We describe an 8½-year-old girl with developmental delay, congenital hip dysplasia, a bilateral foot deformity, bilateral congenital radioulnar synostosis, a congenital heart defect, and minor facial anomalies. Results: Chromosomal microarray analysis revealed a 4.9 Mb deletion in the 8q22.2q22.3 region. De novo origin was confirmed by real-time PCR analysis. Conclusions: Microdeletions in the 8q22.2q22.3 region are characterized by moderate to severe intellectual disability, seizures, distinct facial features and skeletal abnormalities. In addition to one already reported individual with an 8q22.2q22.3 microdeletion and unilateral radioulnar synostosis, this report of a child with bilateral radioulnar synostosis provides additional evidence, that radioulnar synostosis is not an incidental finding in individuals with an 8q22.2q22.3 microdeletion. Additional patients with similar microdeletions would be of a great importance for more accurate phenotypic description and further analysis of the genotypic-phenotypic relationship.

The MECOM gene encodes multiple protein isoforms that are essential for hematopoietic stem cell self-renewal and maintenance. Germline MECOM variants have been associated with congenital thrombocytopenia, radioulnar synostosis and bone marrow failure; however, the phenotypic spectrum of MECOM-associated syndromes continues to expand and novel pathogenic variants continue to be identified. We describe eight unrelated patients who add to the previously known phenotypes and genetic defects of MECOM-associated syndromes. As each subject presented with unique MECOM variants, the series failed to demonstrate clear genotype-to-phenotype correlation but may suggest a role for additional modifiers that affect gene expression and subsequent phenotype. Recognition of the expanded hematologic and non-hematologic clinical features allows for rapid molecular diagnosis, early identification of life-threatening complications, and improved genetic counseling for families. A centralized international publicly accessible database to share annotated MECOM variants would advance their clinical interpretation and provide a foundation to perform functional MECOM studies.

OBJECTIVE: The main objectives of this case report are to discuss prenatal ultrasound findings of congenital radioulnar synostosis and to review the literature.
METHODS: A patient was diagnosed with congenital radioulnar synostosis at eight months old when parents noticed limited motions in the child\'s left forearm. The parent denied any traumatic or family history of bony malformations. Physical examination by a pediatric orthopedics specialist and digital radiography revealed proximal radioulnar synostosis. The case report includes perinatal course, comparison between the postnatal radiography and fetal ultrasound images.
CONCLUSIONS: Congenital radioulnar synostosis is often associated with sex chromosome abnormalities and congenital musculoskeletal disorders or syndromes affecting limbs. Isolated congenital radioulnar synostosis is hardly diagnosed before birth, in some cases even have been neglected postnatally. Knowing the developmental milestones of the forearm and specified high-risk groups might help develop a targeted screening strategy to increase the possibility of early detection and intervention.

OBJECTIVE: The deformity of congenital radioulnar synostosis is quite complicated and difficult. This study aims to find out the related factors of the \"forearm rotation angle\" (FR) which relate to the severity of congenital radioulnar synostosis (CRUS), and try to quantify the internal relations of each deformity and help to understand the reconstruction method in surgery treatment of this disease.
METHODS: This study is case series research. We established 48 digital three-dimensional forearm bone models of 48 patients with congenital radioulnar synostosis classified as Cleary and Omer type 3. All the patients were treated at our institution from January 2010 to June 2016. In total, 10 independent deformities (the rotation angle of forearm; the internal rotation, radial, and dorsal angulation of radius and ulna; the relative length of osseous fusion at PRUJ; the relative dislocation distance of distal radioulnar joint; the relative area of proximal radial epiphysis) involved in the CRUS complex deformity were measured. Pearson correlation analysis for each deformity which was mentioned above was performed, and multivariate linear regression analysis was also performed with FR as the dependent variable and the other deformities as the influential factors.
RESULTS: The \"dorsal angle of radius\" (DAR, 21.69° ± 21.55°) had the strongest correlation with the FR (79.72° ± 40.39°), the Pearson correlation coefficient was 0.601 (p < 0.01), the internal rotation angle of the radius (IRAR, 82.69° ± 54.98°) had a moderate correlation with FR, the Pearson correlation coefficient was 0.552 (p < 0.01). A forearm deformity equation was established: FR = 35.896 + 0.271 DAR + 0.989 IRAR.
CONCLUSIONS: The dorsal angulation deformity of radius may be the most important deformity that effects the severity of CRUS and should be correct in the first place during reconstruction operation.

We read the article \"Rare complication of open reduction and internal fixation of fracture distal radius: A case report of distal radioulnar synostosis\" by Ahmed Elmahdi [1], with a lot of interest. We commend the authors efforts in describing a rare case of distal radioulnar synostosis after open reduction and internal fixation for distal radius fracture. It is the purpose of this letter-to-the-editor to express our opinion, which is based on the research that has been published, which indicates that our opinion is supported by the research.

Congenital radioulnar synostosis is a rare musculoskeletal disorder of the elbow, occurring as a result of variable degree and length of the congenital fusion of the proximal radioulnar joint. Patients presents early to the hospital depending on the severity of the synostosis and its effect on elbow function. It may have psychosocial effects on the affected individuals as they grow older especially when the deformity is dramatic. Treatment may be conservative, surgical (which may have a variable degree of success) and psychotherapy.

UNASSIGNED: To evaluate the clinical outcomes of derotational osteotomy followed by plate fixation at the radius and ulna for the treatment of congenital proximal radioulnar synostosis.
UNASSIGNED: A 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.
UNASSIGNED: All 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.
UNASSIGNED: Congenital 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.