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      Treatment of Congenital Radioulnar Synostosis Using a Free Vascularized Fascia Lata Graft

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          Abstract

          Objective

          To describe the modified mobilization surgery technique that uses a free vascularized fascia lata graft as the interposition graft, and to evaluate the outcome of this procedure in treating congenital radioulnar synostosis (CRUS).

          Methods

          Eleven patients (eight boys and three girls with an average age of 6.0 years) were treated using this procedure between 2012 and 2017 in our institution. Five bilateral cases (four left forearms and one right forearm were treated), and six unilateral cases (three left forearms and three right forearms) were included. All 11 cases were treated with mobilization procedure with free vascularized fascia lata as the interposition graft, and were followed‐up for an average of 2.2 years (range, 2–4 years). The parental satisfaction, postoperative ankylosis at proximal radioulnar joint, and active range of forearm rotation motion (measured by physical examination) were evaluated at the last follow‐up.

          Results

          The average preoperative fixed pronation angle was 67.3° (range, 20°–90°). Ipsilateral thumb hypoplasia was noted in one case, and cleft palate and bilateral thumb hypoplasia were noted in one case; none of the patients had a family history of congenital radioulnar synostosis. Pronation and supination splints were used 3 days after the operation and were worn every night for 4–6 months postoperatively. Active and passive rehabilitation for elbow flexion and forearm rotation was initiated 4 weeks postoperatively. All patients were followed up for at least 2 years (average, 26 months; range, 24–48 months). The average forearm pronation range was 39° (range, 20°–60°), and the average forearm supination range was 33.2° (range, 10°–60°) at the latest follow‐up. Re‐ankylosis occurred in one case. An osseous bridge developed between the radius and ulna at the osteotomy site in one case. Radial nerve paralysis developed in two cases and spontaneously resolved 2 months later. Plate breakage was noted in one case 9 weeks postoperatively; however, union was achieved 7 months later.

          Conclusion

          Mobilization of proximal radioulnar synostosis using a free vascularized fascia lata graft as the interposition graft may prevent re‐ankylosis and restore the forearm rotation function, making it a good option for the surgical treatment of CRUS.

          Abstract

          Fascia lata can be used as interposition in mobilization procedure to avoid postoperative re‐ankylosis in congenital radioulnar synostosis patients, we tried this novel technique in 11 children in our clinic from 2012 to 2017, the parental satisfaction, radiological findings, and active forearm rotation function were satisfying after 2 years follow‐up.

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          Most cited references16

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          Congenital proximal radio-ulnar synostosis. Natural history and functional assessment.

          We evaluated the cases of twenty-three patients with thirty-six congenital proximal radio-ulnar synostoses who had been followed between 1938 and 1984. None of the patients had had any attempt at surgical correction or any ipsilateral congenital anomalies. Emphasis was placed on analyzing the natural history of the lesion in these patients who had not been operated on, and on determining the effect of a fixed position of the forearm on function. Ten patients had unilateral and thirteen, bilateral synostosis. Their mean age when last examined was twenty-two years (range, three to fifty years). Eight patients were female and fifteen, male. The forearms were fixed in an average of 30 degrees of pronation. The position of the forearm was not found to be related to subjective functional limitations, employment status, or the results of the hand-function test of Jebsen et al. Most patients had few or no functional limitations, and were employed in jobs that demanded extensive use of the forearm. Contrary to the findings of other studies, we observed four distinct radiographic patterns based on the presence of an osseous synostosis and the position of the radial head. However, we noted no relationship between any of these patterns and function. We concluded that operative treatment of congenital radio-ulnar synostosis is rarely indicated, that less emphasis should be placed on the single factor of the position of the forearm, and that objective functional tests should be included in the assessment of these patients.
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            Mutations in MECOM, Encoding Oncoprotein EVI1, Cause Radioulnar Synostosis with Amegakaryocytic Thrombocytopenia.

            Radioulnar synostosis with amegakaryocytic thrombocytopenia (RUSAT) is an inherited bone marrow failure syndrome, characterized by thrombocytopenia and congenital fusion of the radius and ulna. A heterozygous HOXA11 mutation has been identified in two unrelated families as a cause of RUSAT. However, HOXA11 mutations are absent in a number of individuals with RUSAT, which suggests that other genetic loci contribute to RUSAT. In the current study, we performed whole exome sequencing in an individual with RUSAT and her healthy parents and identified a de novo missense mutation in MECOM, encoding EVI1, in the individual with RUSAT. Subsequent analysis of MECOM in two other individuals with RUSAT revealed two additional missense mutations. These three mutations were clustered within the 8(th) zinc finger motif of the C-terminal zinc finger domain of EVI1. Chromatin immunoprecipitation and qPCR assays of the regions harboring the ETS-like motif that is known as an EVI1 binding site showed a reduction in immunoprecipitated DNA for two EVI1 mutants compared with wild-type EVI1. Furthermore, reporter assays showed that MECOM mutations led to alterations in both AP-1- and TGF-β-mediated transcriptional responses. These functional assays suggest that transcriptional dysregulation by mutant EVI1 could be associated with the development of RUSAT. We report missense mutations in MECOM resulting in a Mendelian disorder that provide compelling evidence for the critical role of EVI1 in normal hematopoiesis and in the development of forelimbs and fingers in humans.
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              Congenital radioulnar synostosis.

              Congenital radioulnar synostosis can be severely disabling, especially if it is bilateral or if severe hyperpronation exists. Functionally, patients with severe deformity have trouble getting a cup to the mouth, using eating utensils, or accepting objects in an open palm. Of 33 patients (17 bilateral and 7 unilateral) underwent derotational osteotomy, with the majority being performed through the synostosis held with, an intramedullary wire and secondary transfixing device. There were eight complications, four involving neurovascular compromise. In bilateral cases, the best end position appears to be 10% to 15% of pronation in the dominant extremity and neutral in the other. Eighty-two percent of the patients had good or excellent results.
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                Author and article information

                Contributors
                drcsl@qq.com
                Journal
                Orthop Surg
                Orthop Surg
                10.1111/(ISSN)1757-7861
                OS
                Orthopaedic Surgery
                John Wiley & Sons Australia, Ltd (Melbourne )
                1757-7853
                1757-7861
                07 May 2022
                June 2022
                : 14
                : 6 ( doiID: 10.1111/os.v14.6 )
                : 1229-1234
                Affiliations
                [ 1 ] Peking University Fourth School of Clinical Medicine Beijing China
                [ 2 ] Department of Hand Surgery Beijing Ji Shui Tan Hospital Beijing China
                Author notes
                [*] [* ] Address for correspondence Shanlin Chen, MD, Department of Hand Surgery, Beijing Ji Shui Tan Hospital, Beijing, China 100035. Tel: +8613910667213; Fax: +86‐010‐58516095; Email: drcsl@ 123456qq.com

                Author information
                https://orcid.org/0000-0002-3332-6352
                https://orcid.org/0000-0002-6524-6058
                https://orcid.org/0000-0002-9704-8275
                https://orcid.org/0000-0003-2551-5660
                https://orcid.org/0000-0002-7167-7444
                https://orcid.org/0000-0002-7481-2146
                https://orcid.org/0000-0003-0778-1017
                https://orcid.org/0000-0003-1341-6489
                https://orcid.org/0000-0002-0565-8050
                Article
                OS13226
                10.1111/os.13226
                9163976
                35524650
                5618ada8-1b5d-4b8e-bbe7-29f32624d68f
                © 2022 The Authors. Orthopaedic Surgery published by Tianjin Hospital and John Wiley & Sons Australia, Ltd.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.

                History
                : 10 January 2022
                : 23 August 2020
                : 19 January 2022
                Page count
                Figures: 5, Tables: 0, Pages: 6, Words: 4113
                Funding
                Funded by: Beijing JST Research Funding
                Award ID: 2019‐YJ14
                Funded by: Beijing Natural Science Foundation , doi 10.13039/501100004826;
                Award ID: 7192082
                Categories
                Operative Technique
                Operative Technique
                Custom metadata
                2.0
                June 2022
                Converter:WILEY_ML3GV2_TO_JATSPMC version:6.1.6 mode:remove_FC converted:03.06.2022

                congenital radioulnar synostosis,fascia lata,forearm,operative procedures,osteotomy

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