Skip to main content

Synostosis and Coalitions of the Hand and Wrist

  • Chapter
  • First Online:
Congenital Anomalies of the Upper Extremity

Abstract

Congenital carpal coalitions are uncommon. The incidence is variable with respect to race, with African Americans having a higher percentage. These coalitions can be either isolated or associated with a syndrome. Isolated carpal coalitions most frequently occur between the lunate and triquetrum; however, coalitions have been described between almost all adjacent carpal bones. When carpal coalitions are associated with a syndrome, multiple carpal bones can be involved. Most inter-carpal coalitions are asymptomatic and are usually discovered as incidental findings during radiographic evaluation following a traumatic event. Patients with symptomatic carpal coalitions tend to have incomplete coalitions.

Metacarpal synostosis is a rare entity characterized by the coalescence of two adjacent metacarpals. The most common form is between the ring and little finger metacarpals and is associated with abduction of the little finger in an awkward position. Multiple treatment options exist to correct the deformity, but close follow-up in skeletally immature patients is required given the potential for recurrence.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 119.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 159.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Sledge CB. Some morphologic and experimental aspects of limb development. Clin Orthop Relat Res. 1966;44:241–64.

    Article  CAS  PubMed  Google Scholar 

  2. Resnick CS, Grizzard JD, Simmons BP, Yaghmai I. Incomplete carpal coalition. AJR Am J Roentgenol. 1986;147:301–4.

    Article  Google Scholar 

  3. Kennedy K, Waller CJ, Hartley RH. Congenital lunotriquetral and capitotrapezoid coalitions. J Hand Surg Eur Vol. 2010;35B:79–80.

    Article  Google Scholar 

  4. Garn SM, Burdi AR, Babler WJ. Prenatal origins of carpal fusions. Am J Phys Anthropol. 1976;45:203–8.

    Article  CAS  PubMed  Google Scholar 

  5. Simmons BP, Mckenzie WD. Symptomatic carpal coalition. J Hand Surg Am. 1985;10A:190–3.

    Article  Google Scholar 

  6. O’Rahilly R. A survey of carpal and tarsal anomalies. J Bone Joint Surg. 1953;35A:626–42.

    Article  Google Scholar 

  7. Richterman IE, Kozin SH. Symptomatic pisiform hamate synchondrosis: a case report and review of the literature. J Hand Surg Am. 1996;21A:311–3.

    Article  Google Scholar 

  8. Cockshott WP. Carpal fusions. Am J Roentgenol Radium Therapy, Nucl Med. 1963;89:1260–71.

    CAS  Google Scholar 

  9. Cockshott WP. Pisiform hamate fusion. J Bone Joint Surg. 1969;51A:778–80.

    Article  Google Scholar 

  10. Burnett SE. Hamate-pisiform coalition: morphology, clinical significance, and a simplified classification scheme for carpal coalition. Clin Anat. 2011;24:188–96.

    Article  PubMed  Google Scholar 

  11. Kawamura K, Yajima H, Takakura Y. Pisiform and hamate coalition: case report and review of literature. Hand Surg. 2005;10:101–4.

    Article  PubMed  Google Scholar 

  12. Gross SC, Watson HK, Strickland JW, Palmer AK, Brenner LH. Triquetral-lunate arthritis secondary to synostosis. J Hand Surg Am. 1989;14A:95–102.

    Article  Google Scholar 

  13. Garn SM, Frisancho AR, Poznanski AK, Schweitzer J, McCann MB. Analysis of tiquetral-lunate fusion. Am J Phys Anthropol. 1971;34:431–3.

    Article  CAS  PubMed  Google Scholar 

  14. Ritt MJ, Maas M, Bos KE. Minnaar type 1 symptomatic lunotriquetral coalition: a report of nine patients. J Hand Surg Am. 2001;26A:261–70.

    Article  Google Scholar 

  15. Szaboky GT. Anomalous fusion between the lunate and triquetrum. J Bone Joint Surg. 1969;51A:1001–3.

    Article  Google Scholar 

  16. DeVilliers Minnaar AB. Congenital fusion of the lunate and triquetral bones in the South African Bantu. J Bone Joint Surg. 1952;34B:45–8.

    Article  Google Scholar 

  17. Hughes PC, Tanner JM. The development of carpal bone fusion as seen in serial radiographs. Br J Radiol. 1966;39:943–9.

    Article  CAS  PubMed  Google Scholar 

  18. Carlson DH. Coalition of the carpal bones. Skelet Radiol. 1981;7:125–7.

    Article  CAS  Google Scholar 

  19. Burnett SE, Case DT. Naviculo-cuneiform I coalition: evidence of significant differences in tarsal coalition frequency. Foot. 2005;15:80–5.

    Article  Google Scholar 

  20. Van Schoonhoven J, Prommersberger KJ, Schmitt R. Traumatic disruption of a fibrocartilage lunate-triquetral coalition—a case report and review of the literature. Hand Surg. 2001;6:103–8.

    Article  PubMed  Google Scholar 

  21. Peters S, Colaris JW. Carpal coalitions: symptomatic incomplete bony coalition of the capitate and trapezoid—case report. J Hand Surg Am. 2011;36A:1313–5.

    Article  Google Scholar 

  22. Poznanski AK, Holt JF. The carpals in congenital malformation syndromes. Am J Roentgenol Radium Therapy, Nucl Med. 1971;112:443–6.

    Article  CAS  Google Scholar 

  23. Samir N, Al-Mahrezi A. Congenital fusion of the trapezium and trapezoid. Sultan Qaboos Univ Med J. 2010;10:405–6.

    PubMed Central  PubMed  Google Scholar 

  24. Wilson SM, Moreel P, Roulot E. Symptomatic congenital fusion of the scaphoid and the trapezium. J Hand Surg Am. 2006;31B:581.

    Article  Google Scholar 

  25. Moreel P, Wilson SM, Descamps S, Roulot E. Bilateral congenital fusion of the scaphoid and the trapezium. A case report. Rev Chir Orthop Reparatrice Appar Mot. 2008;94:84–6.

    Article  CAS  PubMed  Google Scholar 

  26. Weinzweig J, Watson HK, Herbert TJ, Shaer JA. Congenital synchondrosis of the scaphotrapezio-trapezoidal joint. J Hand Surg Am. 1997;22A:74–7.

    Article  Google Scholar 

  27. Ingram C, Hall RF, Gonzalez M. Congenital fusion of the scaphoid, trapezium, trapezoid and capitate. J Hand Surg Am. 1997;22B:167–8.

    Article  Google Scholar 

  28. Kahane S, Isaac SM, Wildin C. A new type of carpal coalition. J Hand Surg Eur Vol. 2012;37:581–2.

    Article  CAS  PubMed  Google Scholar 

  29. Delaney TJ, Eswar S. Carpal coalitions. J Hand Surg Am. 1992;17A:28–31.

    Article  Google Scholar 

  30. Ganos DL, Imbriglia JE. Symptomatic congenital coalition of the pisiform and hamate. J Hand Surg Am. 1991;16A:646–50.

    Article  Google Scholar 

  31. Berkowitz AR, Melone CP, Belsky MR. Pisiform-hamate coalition with ulnar neuropathy. J Hand Surg Am. 1992;17A:657–62.

    Article  Google Scholar 

  32. Gottschalk MB, Danilevich M, Gottschalk HP. Carpal coalitions and metacarpal synostoses: a review. J Hand. 2016;11(3):271–7.

    Article  Google Scholar 

  33. Nievergelt K. Positiver Vaterschaftsnachweis auf grund erblicher Missbildungen der Extremitaeten. Arch Klaus Stift Vererbunsforsch. 1944;19:157.

    Google Scholar 

  34. Pearlman HS, Edkin RE, Warren RF. Familial tarsal and carpal synostosis with radial-head subluxation (Nievergelt’s syndrome). J Bone Joint Surg. 1964;46:585–92.

    Article  CAS  PubMed  Google Scholar 

  35. Ellis RW, Van Creveld S. Syndrome characterized by ectodermal dysplasia, polydactyly, chondro-dysplasia and congenital morbus cordis; report of three cases. Arch Dis Child. 1940;15:65–84.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  36. Polymeropoulos MH, Ide SE, Wright M, Goodship J, Weissenbach J, Pyeritz RE, et al. The gene for the Ellis-van Creveld Syndrome is located on chromosome 4p16. Genomics. 1996;35:1–5.

    Article  CAS  PubMed  Google Scholar 

  37. Galdzicka M, Patnala S, Hirshman MG, Cai JF, Nitowsky H, Egeland JA, et al. A new gene, EVC2, is mutated in Ellis-van Creveld syndrome. Mol Genet Metab. 2002;77:291–5.

    Article  CAS  PubMed  Google Scholar 

  38. Muensterer OJ, Berdon W, McManus C, Oestreich A, Lachman RS, Cohen MM Jr, et al. Ellis-van Creveld syndrome: its history. Pediatr Radiol. 2013;43(8):1030–6.

    Article  PubMed  Google Scholar 

  39. Caffey J. Chondroectodermal dysplasia (Ellis-Van Creveld disease); report on three cases. Am J Roentgenol. 1952;68:875–86.

    CAS  Google Scholar 

  40. Stern AM, Gall JC Jr, Perry BL, Stimson CW, Weitkamp LR, Poznanski AK. The hand-foot-uterus syndrome. A new hereditary disorder characterized by hand and foot dysplasia, dermatoglyphic abnormalities, and partial duplication of female genital tract. J Pediatr. 1970;77:109–16.

    Article  CAS  PubMed  Google Scholar 

  41. Goodman FR, Bacchelli C, Brady AF, Brueton LA, Fryns JP, Mortlock DP. Novel HOXA13 mutations and the phenotypic spectrum of hand-foot-genital syndrome. Am J Hum Genet. 2000;67:197–202.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  42. Halal F. The hand-foot-genital (hand-foot-uterus) syndrome: family report and update. Am J Med Genet. 1988;30:793–803.

    Article  CAS  PubMed  Google Scholar 

  43. Innis JW. Hand-foot-genital syndrome. In: Pagon RA, Adam MP, Bird TD, editors. Gene reviews [internet]. Seattle: University of Washington; 1993–2013.

    Google Scholar 

  44. Letts M, Davidson D, Beaule P. Symphalangism in children: case report and review of the literature. Clin Orthop Relat Res. 1999;366:178–85.

    Article  Google Scholar 

  45. Tuncay I, Akpinar F, Tosun N. Congenital true complete symphalangism of all proximal interphalangeal joints of hands with carpal anomalies: a case report. Hand Surg. 2001;6:223–6.

    Article  CAS  PubMed  Google Scholar 

  46. Cushing H. Hereditary anchylosis of the proximal phalangeal joints (Symphalangism). J Nerv Ment Dis. 1916;43:445.

    Article  Google Scholar 

  47. Geelhoed GW, Neel JV, Davidson RT. Symphalangism and tarsal coalitions: a hereditary syndrome. J Bone Joint Surg. 1969;51B:278–89.

    Google Scholar 

  48. Flatt AE, Wood VE. Rigid digits or symphalangism. Hand. 1975;7:197–213.

    Article  CAS  PubMed  Google Scholar 

  49. Dellon AL, Gaylor R. Bilateral symphalangism of the index finger. J Bone Joint Surg. 1976;58A:270–1.

    Article  Google Scholar 

  50. Harle TS, Stevenson JR. Hereditary symphalangism associated with carpal and tarsal fusions. Radiology. 1967;89:91–4.

    Article  CAS  PubMed  Google Scholar 

  51. Kalampokas E, Kalampokas T, Sofoudis C, Deligeoroglou E, Botsis D. Diagnosing arthrogryposis multiplex congenital: a review. ISRN Obstet Gynecol. 2012;2012:264918.

    Article  PubMed Central  PubMed  Google Scholar 

  52. Bamshad M, Van Heest AE, Pleasure D. Arthrogryposis: a review and update. J Bone Joint Surg. 2009;91(Suppl 4):40–6.

    Article  PubMed  PubMed Central  Google Scholar 

  53. Bevan WP, Hall JG, Bamshad M, Staheli LT, Jaffe KM, Song K. Arthrogryposis multiplex congenital (Amyoplasia) an orthopaedic perspective. J Pediatr Orthop. 2007;27:594–600.

    Article  PubMed  Google Scholar 

  54. Swinyard CA, Bleck EE. The etiology of arthrogryposis (multiple congenital contracture). Clin Orthop Relat Res. 1985;194:15–29.

    Article  Google Scholar 

  55. Newcombe DS, Abbott JL, Munsie WJ, Keats TE. Arthrogryposis multiplex congenital and spontaneous carpal fusion. Arthritis Rheum. 1969;12:345–54.

    Article  CAS  PubMed  Google Scholar 

  56. Ezaki MB, Carter PR. Carpal wedge osteotomy for the arthrogrypotic wrist. Tech Hand Up Extrem Surg. 2004;8:224–8.

    Article  PubMed  Google Scholar 

  57. Langer LO Jr. The roentgenographic features of the oto-palato-digital (OPD) syndrome. Am J Roentgenol Radium Therapy, Nucl Med. 1967;100(1):90–106.

    Article  Google Scholar 

  58. Gottschalk HP, Bednar MS, Moor M, Light TR. Metacarpal synostosis: treatment with a longitudinal osteotomy and bone graft substitute interposition. J Hand Surg Am. 2012;37A:2074–81.

    Article  Google Scholar 

  59. Buck-Gramcko D, Wood VE. The treatment of metacarpal synostosis. J Hand Surg Am. 1993;18A:565–81.

    Article  Google Scholar 

  60. Foucher G, Navaro R, Medina J, Khouri RK. Metacarpal synostosis: a simple classification and a new treatment technique. Plast Reconstr Surg. 2001;108:1225–31.

    Article  CAS  PubMed  Google Scholar 

  61. Miura T. Congenital synostosis between the fourth and the fifth metacarpal bones. J Hand Surg Am. 1988;13A:83–8.

    Article  Google Scholar 

  62. Jamsheer A, Zemojtel T, Kolanczyk M, Stricker S, Hecht J, Krawitz P, et al. Whole exome sequencing identifies FGF16 nonsense mutations as the cause of X-linked recessive metacarpal 4/5 fusion. J Med Genet. 2013;50(9):579–84. https://doi.org/10.1136/jmedgenet-2013-101659.

    Article  CAS  PubMed  Google Scholar 

  63. Temtamy D, McKusick V. The genetics of hand malformations (Chapter 8). In: Birth defects, Original article series. Vol. 14, No. 3. New York: Alan R. Liss; 1978.

    Google Scholar 

  64. Horii E, Miura T, Nakamura R, Nakao E, Kato H. Surgical treatment of congenital metacarpal synostosis of the ring and little fingers. J Hand Surg Am. 1998;23B:691–4.

    Article  Google Scholar 

  65. Iwasawa M, Hayashi R, Matsuo K, Hirose T. The use of costal cartilage as a spacer in the treatment of congenital metacarpal fusion. Eur J Plast Surg. 1988;11:138–40.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Terry R. Light .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2021 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Gottschalk, H.P., Light, T.R. (2021). Synostosis and Coalitions of the Hand and Wrist. In: Laub Jr., D.R. (eds) Congenital Anomalies of the Upper Extremity. Springer, Cham. https://doi.org/10.1007/978-3-030-64159-7_18

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-64159-7_18

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-64158-0

  • Online ISBN: 978-3-030-64159-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics