Skip to main content
SpringerLink
Log in

Hajdu-Cheney syndrome

  • Current Problem Case
  • Published:
Archives of orthopaedic and traumatic surgery Aims and scope Submit manuscript

Summary

Hajdu-Cheney syndrome is a rare type of syndrome characterized by acro-osteolysis, dolichocephaly with multiple Wormian bones, absence of frontal sinuses and joint laxity. A case of this syndrome is presented. A histological study of the osteolytic lesion revealed destruction characterized by microfractures with a poor reparative process. It is postulated that an abnormality of osteoblast or osteoid function is the pathogenesis of this syndrome.

Zusammenfassung

Es wird über einen Fall von Hajdu-Cheney-Syndrom bei einem 16jährigen Knaben berichtet. Das Syndrom ist hauptsächlich charakterisiert durch Akroosteolysen, Dolichocephalie mit multiplen Wormschen Schaltknochen, offenen Schädelnähten, fehlenden Stirnhöhlen und Schlottergelenke. Histologisch ist die Destruktion im Bereiche der Akroosteolysezonen durch zahlreiche Mikrofrakturen und geringe reparative Vorgänge charakterisiert. Die Autoren diskutieren als Pathogenese des Syndroms eine Störung in der Funktion der Osteoblasten oder im Osteoid.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Brown, D. M., Gorlin, R. J., Densnick, R. J., Langer, L. O. Jr., Jowsey, J., Sauk, J. J. Jr.: The acro-osteolysis syndrome; morphologic and biochemical studies. J. Pediatrics 88, 573–580 (1976)

    Google Scholar 

  2. Chawla, S.: Cranio-skeletal dysplasia with acro-osteolysis. Br. J. Radiol. 37, 702–705 (1964)

    PubMed  Google Scholar 

  3. Cheney, W. D.: Acro-osteolysis. Am. J. Roentgenol. 94, 595–607 (1965)

    Google Scholar 

  4. Greenberg, B. E., Street, D. M.: Idiopathic non-familial acro-osteolysis. Report of a case observed for five years. Radiology 69, 259–262 (1957)

    PubMed  Google Scholar 

  5. Hajdu, N., Kauntze, R.: Cranio-skeletal dysplasia. Br. J. Radiol. 21, 42–48 (1948)

    Google Scholar 

  6. Harnasch, H.: Die Acroosteolysis, ein neues Krankheitsbild. Fortschr. Roentgenstr. 72, 352–359 (1950)

    Google Scholar 

  7. Herrmann, J., Zugibe, F. T., Gilbert, E. F., Opitz, J. M.: Arthro-dento-osteo dysplasia (Hajdu-Cheney syndrome). Z. Kinderheilk. 114, 93–110 (1973)

    PubMed  Google Scholar 

  8. Papavasiliou, C. G., Gargano, F. P., Walls, W. L.: Idiopathic nonfamilial acro-osteolysis associated with other bone abnormalities. Am. J. Roentgenol. 83, 687–691 (1960)

    Google Scholar 

  9. Schulze, R., Gulbin, O.: Beitrag zum Problem der Akroosteolyse (gleichzeitig ein Beitrag zur Kenntnis der Patella profunda). Fortschr. Roentgenstr. 109, 209–216 (1968)

    PubMed  Google Scholar 

  10. Silverman, F. N., Dorst, J. P., Hajdu, N.: Acro-osteolysis (Hajdu-Cheney syndrome). Birth defects; Original article series X, 106–123 (1974)

    Google Scholar 

  11. Spranger, J. W., Langer, L. O., Wiedmann, H. R.: Bone dysplasias, pp. 211–218. Stuttgart: G. Fischer 1974

    Google Scholar 

  12. Terashima, Y., Urist, M. R.: Chondrogenesis in outgrowth of muscle tissue modified bone onto matrix in tissue culture. Clin. Orthop. 127, 248–256 (1977)

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Dept. of Orthopedic Surgery, Shizuoka Children's Hospital, 860 Urushiyama, 420, Shizuoka-shi, Japan

    Tsutomu Iwaya M.D.

  2. Inariyama Institute for Handicapped Children, Koshoku-shi, Nagano-ken

    Kazuhiko Taniguchi

  3. Division of Genetics, Japan

    Junzo Watanabe

  4. Shizuoka Children's Hospital, Division of Pathology, Japan

    Kazuso Iinuma

  5. Institute of Clinical Medicine, The University of Tsukuba, 300-31, Niihari-gun, Ibaraki-ken, Japan

    Yutaka Hamazaki & Seizo Yoshikawa

Authors
  1. Tsutomu Iwaya M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kazuhiko Taniguchi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Junzo Watanabe
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Kazuso Iinuma
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Yutaka Hamazaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Seizo Yoshikawa
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Iwaya, T., Taniguchi, K., Watanabe, J. et al. Hajdu-Cheney syndrome. Arch. Orth. Traum. Surg. 95, 293–302 (1979). https://doi.org/10.1007/BF00389701

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00389701

Keywords

Search

Navigation