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Congenital Radioulnar Synostosis

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Congenital Anomalies of the Upper Extremity

Abstract

Congenital radioulnar synostosis is a rare but important congenital condition. Although most children will be able to compensate for the deformity, severe and bilateral deformities can be quite disabling. Most children will present to clinic after their parents or teachers at school notice their deformity and any associated compensatory movements. Only some of these children will have significant functional deficit. Surgical intervention is indicated for severe pronation deformities of more than 60° or significant bilateral deformities that are disabling for the child. The surgical procedures broadly involve either mobilization of the synostosis to restore forearm rotation or an osteotomy to place the forearm in a more functional position. Separation of the synostosis has historically produced poor results with loss of correction and neurovascular complications. Recent attempts at using an interposition free vascularized fascio-fat graft have produced better results. Osteotomy has been the commonly performed procedure, and these can be performed at the level of the synostosis, distal radius, or both radius and ulna. The Ilizarov technique has been successfully used for a gradual correction. The most significant complications of surgery are compartment syndrome and neurological deficit. Loss of correction and recurrence has also been reported.

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References

  1. Cleary JE, Omer GE. Congenital proximal radio-ulnar synostosis. Natural history and functional assessment. J Bone Joint Surg Am. 1985;67(4):539–45.

    Article  CAS  Google Scholar 

  2. Wilkie D. Congenital radio-ulnar synostosis. Br J Surg. 1914;1:366–75.

    Article  Google Scholar 

  3. Ogino T, Hikino K. Congenital radio-ulnar synostosis: compensatory rotation around the wrist and rotation osteotomy. J Hand Surg Br. 1987;12(2):173–8.

    Article  CAS  Google Scholar 

  4. Green WT, Mital MA. Congenital radio-ulnar synostosis: surgical treatment. J Bone Joint Surg Am. 1979;61(5):738–43.

    Article  CAS  Google Scholar 

  5. Bergsma D, editor. Birth defects compendium. For The National Foundation, March of Dimes. 2nd ed. New York: Alan R. Liss; 1979.

    Google Scholar 

  6. Hansen OH, Andersen NO. Congenital radio-ulnar synostosis. Report of 37 cases. Acta Orthop Scand. 1970;41(3):225–30.

    Article  CAS  Google Scholar 

  7. Burgemeister AL, Daumiller E, du Bois G. Clinical report of 8 patients with 49,XXXXY syndrome: Delineation of the facial gestalt and depiction of the clinical spectrum. Eur J Med Genet. 2019;62(3):210–6.

    Article  Google Scholar 

  8. James C, Robson L, Jackson J, Smith A. 46,XY/47,XYY/48,XYYY karyotype in a 3-year-old boy ascertained because of radioulnar synostosis. Am J Med Genet. 1995;56(4):389–92.

    Article  CAS  Google Scholar 

  9. Townes PL, Ziegler NA, Lenhard LW. A patient with 48 chromosomes (XYYY). Lancet. 1965;1(7394):1041–3.

    Article  CAS  Google Scholar 

  10. Dawson HG. A congenital deformity of the forearm and its operative treatment. Br Med J. 1912;2(2701):833–5.

    Article  CAS  Google Scholar 

  11. Germeshausen M, Ancliff P, Estrada J, Metzler M, Ponstingl E, Rütschle H, et al. MECOM-associated syndrome: a heterogeneous inherited bone marrow failure syndrome with amegakaryocytic thrombocytopenia. Blood Adv. 2018;2(6):586–96.

    Article  CAS  Google Scholar 

  12. Giuffrè L, Corsello G, Giuffrè M, Piccione M, Albanese A. New syndrome: autosomal dominant microcephaly and radio-ulnar synostosis. Am J Med Genet. 1994;51(3):266–9.

    Article  Google Scholar 

  13. Jaffer Z, Nelson M, Beighton P. Bone fusion in the foetal alcohol syndrome. J Bone Joint Surg Br. 1981;63B(4):569–71.

    Article  CAS  Google Scholar 

  14. Mital MA. Congenital radioulnar synostosis and congenital dislocation of the radial head. Orthop Clin North Am. 1976;7(2):375–83.

    CAS  PubMed  Google Scholar 

  15. Simmons BP, Southmayd WW, Riseborough EJ. Congenital radioulnar synostosis. J Hand Surg Am. 1983;8(6):829–38.

    Article  CAS  Google Scholar 

  16. Waters P, Simmons B. Congenital abnormalities: elbow region. In: Peimer CA, editor. Surgery of the hand and upper extremity. New York: McGraw Hill; 1996.

    Google Scholar 

  17. Ramachandran M, Lau K, Jones DHA. Rotational osteotomies for congenital radioulnar synostosis. J Bone Joint Surg Br. 2005;87(10):1406–10.

    Article  CAS  Google Scholar 

  18. Tachdjian’s pediatric orthopaedics: from the Texas Scottish Rite Hospital for Children – 5th edition [Internet]. [cited 2019 Jul 8]. Available from: https://www.elsevier.com/books/tachdjians-pediatric-orthopaedics-from-the-texas-scottish-rite-hospital-for-children/herring/978-1-4377-1549-1.

  19. Rubin G, Rozen N, Bor N. Gradual correction of congenital radioulnar synostosis by an osteotomy and Ilizarov external fixation. J Hand Surg Am. 2013;38(3):447–52.

    Article  Google Scholar 

  20. VanHeest AE, Lin TE, Bohn D. Treatment of blocked elbow flexion in congenital radioulnar synostosis with radial head excision: a case series. J Pediatr Orthop. 2013;33(5):540–3.

    Article  Google Scholar 

  21. Hung NN. Derotational osteotomy of the proximal radius and the distal ulna for congenital radioulnar synostosis. J Child Orthop. 2008;2(6):481–9.

    Article  Google Scholar 

  22. Griffet J, Berard J, Caton J, Michel C. Les synostoses congenital radio- cubitales supeiurse. Int Orthop. 1986;10:265–9.

    Google Scholar 

  23. Kanaya F, Ibaraki K. Mobilization of a congenital proximal radioulnar synostosis with use of a free vascularized fascio-fat graft. J Bone Joint Surg Am. 1998;80(8):1186–92.

    Article  CAS  Google Scholar 

  24. Garg G, Gupta SP. Surgical outcome of delayed presentation of congenital proximal radioulnar synostosis. SICOT J. 2015;1:33.

    Article  Google Scholar 

  25. Miura T, Nakamura R, Suzuki M, Kanie J. Congenital radio-ulnar synostosis. J Hand Surg Br. 1984;9(2):153–5.

    Article  CAS  Google Scholar 

  26. Funakoshi T, Kato H, Minami A, Suenaga N, Iwasaki N. The use of pedicled posterior interosseous fat graft for mobilization of congenital radioulnar synostosis: a case report. J Shoulder Elbow Surg. 2004;13(2):230–4.

    Article  Google Scholar 

  27. Kawaguchi S, Kitamura M, Usui M. Proximal radioulnar synostosis treated with a free vascularised fascio-fat graft – report of two cases. Hand Surg. 2000;5(2):161–4.

    Article  CAS  Google Scholar 

  28. Kelikian H. Congenital deformities of the hand and forearm. Philadelphia: WB Saunders Company; 1974.

    Google Scholar 

  29. Hankin FM, Smith PA, Kling TF, Louis DS. Ulnar nerve palsy following rotational osteotomy of congenital radioulnar synostosis. J Pediatr Orthop. 1987;7(1):103–6.

    Article  CAS  Google Scholar 

  30. Fujimoto M, Kato H, Minami A. Rotational osteotomy at the diaphysis of the radius in the treatment of congenital radioulnar synostosis. J Pediatr Orthop. 2005;25(5):676–9.

    Article  Google Scholar 

  31. Murase T, Tada K, Yoshida T, Moritomo H. Derotational osteotomy at the shafts of the radius and ulna for congenital radioulnar synostosis. J Hand Surg Am. 2003;28(1):133–7.

    Article  Google Scholar 

  32. Nakasone M, Nakasone S, Kinjo M, Murase T, Kanaya F. Three-dimensional analysis of deformities of the radius and ulna in congenital proximal radioulnar synostosis. J Hand Surg Eur Vol. 2018;43(7):739–43.

    Article  Google Scholar 

  33. Manske PR, McCarroll HR, Hale R. Biceps tendon rerouting and percutaneous osteoclasis in the treatment of supination deformity in obstetrical palsy. J Hand Surg Am. 1980;5(2):153–9.

    Article  CAS  Google Scholar 

  34. Lin HH, Strecker WB, Manske PR, Schoenecker PL, Seyer DM. A surgical technique of radioulnar osteoclasis to correct severe forearm rotation deformities. J Pediatr Orthop. 1995;15(1):53–8.

    Article  CAS  Google Scholar 

  35. Satake H, Kanauchi Y, Kashiwa H, Ishigaki D, Takahara M, Takagi M. Long-term results after simple rotational osteotomy of the radius shaft for congenital radioulnar synostosis. J Shoulder Elbow Surg. 2018;27(8):1373–9.

    Article  Google Scholar 

  36. Yammine K, Salon A, Pouliquen JC. Congenital radioulnar synostosis: study of a series of 37 children and adolescents. Ann Chir Main Memb Super. 1998;17(4):300–8.

    CAS  Google Scholar 

  37. Gill GG, Sakovich L, Thompson E. Pedicle fat grafts for the prevention of scar formation after laminectomy. An experimental study in dogs. Spine. 1979;4(2):176–86.

    Article  CAS  Google Scholar 

  38. Langenskiöld A, Valle M. Epidurally placed free fat grafts visualized by CT scanning 15-18 years after discectomy. Spine. 1985;10(1):97–8.

    Article  Google Scholar 

  39. Kanaya K, Iba K, Yamashita T. 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. J Shoulder Elbow Surg. 2016;25(8):1258–67.

    Article  Google Scholar 

  40. Bolano LE. Congenital proximal radioulnar synostosis: treatment with the Ilizarov method. J Hand Surg Am. 1994;19(6):977–8.

    Article  CAS  Google Scholar 

  41. Kadiyala RK, Waters PM. Upper extremity pediatric compartment syndromes. Hand Clin. 1998;14(3):467–75.

    CAS  PubMed  Google Scholar 

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Taneja, T., Shetty, V., Ramachandran, M. (2021). Congenital Radioulnar Synostosis. In: Laub Jr., D.R. (eds) Congenital Anomalies of the Upper Extremity. Springer, Cham. https://doi.org/10.1007/978-3-030-64159-7_10

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  • DOI: https://doi.org/10.1007/978-3-030-64159-7_10

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