Advertisement

European Radiology

, Volume 14, Issue 11, pp 2025–2029 | Cite as

Parkes Weber or Klippel-Trenaunay syndrome? Non-invasive diagnosis with MR projection angiography

  • S. Ziyeh
  • J. Spreer
  • J. Rössler
  • R. Strecker
  • A. Hochmuth
  • M. Schumacher
  • J. Klisch
Vascular-Interventional

Abstract

Klippel-Trenaunay and Parkes Weber (Klippel-Trenaunay-Weber) syndromes consist of vascular malformations of the capillary, venous and lymphatic systems combined with soft tissue and bone hypertrophy of the affected extremity. Klippel-Trenaunay syndrome is a pure low-flow condition, while Parkes Weber syndrome is characterized by significant arteriovenous fistulas. The distinction of both entities is relevant, since the prognosis and therapeutic strategies differ significantly. Our purpose is to demonstrate that thick-slice dynamic magnetic resonance projection angiography (MRPA) is a non-invasive tool to detect arteriovenous shunting in Parkes Weber syndrome. Four patients underwent MR imaging and MRPA. MRPA demonstrated arteriovenous shunting in three patients. Arteriovenous shunting was characterized by early appearing draining veins. The time of arrival between normal arteries and pathological veins varied between less than 0.5 and 1.0 s. Therefore, the diagnosis in these cases could be specified as Parkes Weber syndrome. In all these cases, arteriovenous shunting was confirmed by intraarterial digital subtraction angiography. One patient showed normal results in MRPA and could be diagnosed as having Klippel-Trenaunay syndrome.

Keywords

Vascular malformation Parkes Weber syndrome Klippel-Trenaunay syndrome MR projection angiography 

Notes

Acknowledgements

All patients were presented and discussed at the interdisciplinary conference “Hemangiomas and Vascular Malformations” at the University Hospital Freiburg. We thank all persons who contributed to and participated in this conference.

References

  1. 1.
    Capraro PA, Fisher J, Hammond DC, Grossman JA (2002) Klippel-Trenaunay sydrome. Plast Reconstr Surg 109:2052–2060Google Scholar
  2. 2.
    Cohen MM (2000) Klippel-Trenaunay syndrome. Am J Med Genet 93:171–175CrossRefPubMedGoogle Scholar
  3. 3.
    Cohen MM (2002) Vasculogenesis, angiogenesis, hemangiomas, and vascular malformations. Am J Med Genet 108:265–274CrossRefPubMedGoogle Scholar
  4. 4.
    Orszagh M, Schulte DM, Korinthenberg R, Schumacher M (1999) Analysis of hemodynamics by MR-Angio and embolization of Klippel-Trenaunay-Weber syndrome: 5-year follow-up. Rivista di Neuroradiologia 12 [Suppl 2]:127–131Google Scholar
  5. 5.
    Kanterman RY (1997) Klippel-Trenaunay and Parkes-Weber syndromes. Am J Roentgenol 169:311–312Google Scholar
  6. 6.
    Zoppi MA, Ibba RM, Floris M, Putzolu M, Crisponi G, Monni G (2001) Prenatal sonographic diagnosis of Klippel-Trenaunay-Weber syndrome with cardiac failure. J Clin Ultrasound 422–426Google Scholar
  7. 7.
    Roebuck DJ (1997) Klippel-Trenaunay and Parkes-Weber syndromes. Am J Roentgenol 169:311Google Scholar
  8. 8.
    Noel AA, Gloviczki P, Cherry KJ, Rooke TW, Stanson AW, Driscoll DJ (2000) Surgical treatment of venous malformations in Klippel-Trenaunay syndrome. J Vasc Surg 32:840–847PubMedGoogle Scholar
  9. 9.
    Donelly LF, Adams DM, Bisset GS (2000) Vascular malformations and hemangiomas: a practical approach in a multidisciplinary clinic. Am J Roentgenol 174:597-608PubMedGoogle Scholar
  10. 10.
    Yakes WF, Rossi P, Odink H (1996) Arteriovenous malformation management. Cardiovasc Intervent Radiol 19:65–71CrossRefPubMedGoogle Scholar
  11. 11.
    Burrows PE, Mulliken JB, Fellows KE, Strand RD (1983) Childhood hemangiomas and vascular malformations: angiographic differentiation. Am J Roentgenol 141:483–488Google Scholar
  12. 12.
    Paltiel HJ, Burrows PE, Kozakewich HP, Zurakowski D, Mulliken JB (2000) Soft-tissue vascular anomalies: utility of US for diagnosis. Radiology 214:747–754PubMedGoogle Scholar
  13. 13.
    Fordham LA, Chung CJ, Donnelly LF (2000) Imaging of congenital vascular and lymphatic anomalies of the head and neck. Neuroimaging Clin N Am 10:117–136PubMedGoogle Scholar
  14. 14.
    Rak KM, Yakes WF, Ray R, Dreisbach JN, Parker SH, Luethke JM, Stavros AT, Slater DD, Burke BJ (1992) MR imaging of symptomatic peripheral vascular malformations. Am J Roentgenol 159:107–112Google Scholar
  15. 15.
    van Rijswijk CS, van der Linden E, van der Woude HJ, Bloem JL (2002) Value of dynamic contrast-enhanced MR imaging in diagnosing and classifying peripheral vascular malformations. Am J Roentgenol 178:1181–1187Google Scholar
  16. 16.
    Trop I, Dubois J, Guibaud L, Grignon A, Patriquin H, McCuaig C, Garel LA (1999) Soft-tissue venous malformations in pediatric and young adult patients: diagnosis with Doppler US. Radiology 212:841–845PubMedGoogle Scholar
  17. 17.
    Hennig J, Scheffler K, Laubenberger J, Strecker R (1997) Time-resolved projection angiography after bolus injection of contrast agent. Magn Reson Med 37:341–345Google Scholar
  18. 18.
    Sonnet S, Buitrago-Téllez CH, Scheffler K, Strecker R, Bongartz G, Bremerich J (2002) Dynamic time-resolved contrast-enhanced two-dimensional MR projection angiography of the pulmonary circulation: standard technique and clinical applications. Am J Roentgenol 179:159–165Google Scholar
  19. 19.
    Aoki S, Yoshikawa T, Hori M, Nanbu A, Kumagai H, Nishiyama Y, Nukui H, Araki T (2000) MR digital subtraction angiography for the assessment of cranial arteriovenous malformations and fistulas. Am J Roentgenol 175:451–453Google Scholar
  20. 20.
    Tsuchiya K, Katase S, Yoshino A, Hachiya J (2000) MR digital subtraction angiography of cerebral arteriovenous malformations. Am J Neuroradiol 21:707–711PubMedGoogle Scholar
  21. 21.
    Griffiths PD, Hoggard N, Warren DJ, Wilkinson ID, Anderson B, Romanowski CA (2000) Brain arteriovenous malformations: assessment with dynamic MR digital subtraction angiography. Am J Neuroradiol 21:1892–1899PubMedGoogle Scholar
  22. 22.
    Wetzel SG, Bilecen D, Lyrer P, Bongartz G, Seifritz E, Radue EW, Scheffler (2000) Cerebral dural arteriovenous fistulas: detection by dynamic MR projection angiography. Am J Roentgenol 174:1293–1295Google Scholar
  23. 23.
    Klisch J, Strecker R, Hennig J, Schumacher M (2000) Time-resolved projection MRA: clinical application in intracranial vascular malformations. Neuroradiology 42:104–107Google Scholar
  24. 24.
    Yoshikawa T, Aoki S, Hori M, Nambu A, Kumagai H, Araki T (2000) Time-resolved two-dimensional thick-slice magnetic resonance digital subtraction angiography in assessing brain tumors. Eur Radiol 10:736–744PubMedGoogle Scholar
  25. 25.
    Arnold SM, Strecker R, Scheffler K, Spreer J, Schipper J, Neumann HPH, Klisch J (2003) Dynamic contrast enhancement of paragangliomas of the head and neck: evaluation with time-resolved 2D MR projection angiography. Eur Radiol 13:1608–1611CrossRefPubMedGoogle Scholar
  26. 26.
    Goldman JP, Lookstein R, Marin M (2003) Rapid functional CE-MRA in determining direction of contrast flow: initial feasibility and clinical applications. Proc Int Soc Mag Reson Med 11:323Google Scholar

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • S. Ziyeh
    • 1
  • J. Spreer
    • 1
  • J. Rössler
    • 2
  • R. Strecker
    • 3
  • A. Hochmuth
    • 1
  • M. Schumacher
    • 1
  • J. Klisch
    • 1
  1. 1.Section of Neuroradiology, NeurocenterUniversity of FreiburgFreiburgGermany
  2. 2.Department of Pediatric Hematology and OncologyUniversity of FreiburgFreiburgGermany
  3. 3.Section of Medical Physics, Department of RadiologyUniversity of FreiburgFreiburgGermany

Personalised recommendations