Skip to main content
SpringerLink
Log in

The proteus syndrome: the magnetic resonance and radiological features

  • Originals
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

The Proteus syndrome is a recently delineated group of skeletal and mesodermal malformations. Its characteristics include hemihypertrophy and fatty/lymphangiomatous masses. This description outlines the imaging sequences available to the radiologist. It is mainly concerned with the use of magnetic resonance imaging. This shows the extent of the mesodermal malformation with particular reference to the extent of intra abdominal infiltration.

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. Wiedemann HR, Burgio GR, Aldenhoff P, Kunze J, Kaufmann HJ, Schirg E (1983) The proteus syndrome. Eur J Pediatr 140: 5

    CAS  PubMed  Google Scholar 

  2. Cohen MM, Hayden PW et al. A newly recognized hamartomatous syndrome. In: Birth defects. Original article series XV (5B), p 291

  3. Fay JT, Schow SR (1968) A possible case of Maffucci's syndrome. J Oral Surg 26: 739

    CAS  PubMed  Google Scholar 

  4. Kontras SB (1974) Case report 19. Synd Ident II: 1

    Google Scholar 

  5. Pawlaczyk B, Sioda T (1976) Hypertrophied lumbar and muscular atrophy. Synd Ident IV: 4

    Google Scholar 

  6. Temtamy SA, Rogers JG (1976) Macrodactyly, hemihypertrophy, and connective tissue nevi: report of a new syndrome and review of the literature. J Pediatr 89: 924

    CAS  PubMed  Google Scholar 

  7. Burgio GR, Wiedemann HR (1984) Further and new details on the Proteus syndrome. Eur J Pediatr 143: 71

    Article  CAS  PubMed  Google Scholar 

  8. Gorlin RJ (1984) Proteus syndrome. J Clin Dysmorph 2: 8

    CAS  PubMed  Google Scholar 

  9. Lezama DB, Buyse ML (1984) The Proteus syndrome: The emergence of an entity. J Clin Dysmorph 2: 10

    CAS  PubMed  Google Scholar 

  10. Viljoen DL, Nelson MM, de Jong G, Beighton P (1987) Proteus syndrome in Southern Africa: 6 new cases. Am J Med Genet (in press)

  11. Costa T, Fitch N, Azouz EM (1985) Proteus syndrome: report of two cases with pelvic lipomatosis. Pediatrics 76: 984

    CAS  PubMed  Google Scholar 

  12. Tibbles JAR, Cohen MM (1986) The Proteus syndrome: the Elephant Man diagnosed. Br Med J 293: 683

    CAS  Google Scholar 

  13. Dooms GC, Hriacak H, Scotluto RA, Higgins CD (1985) Lipomatous tumours with fatty component: MR imaging potential and comparison of MR and CT results. Radiology 157: 479

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Human Genetics, University of Cape Town, Rondebosch, South Africa

    D. L. Viljoen & P. Beighton

  2. Medical Research Council, Tygerberg, South Africa

    S. Wynchank

  3. Department of Paediatric Radiology, Red Cross Children's Hospital, 7700, Rondebosch, South Africa

    B. J. Cremin

Authors
  1. B. J. Cremin
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. D. L. Viljoen
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. S. Wynchank
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. P. Beighton
    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

Cremin, B.J., Viljoen, D.L., Wynchank, S. et al. The proteus syndrome: the magnetic resonance and radiological features. Pediatr Radiol 17, 486–488 (1987). https://doi.org/10.1007/BF02388284

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02388284

Keywords

Search

Navigation