A 9-month-old girl was brought by her parents because of swelling of the right leg since birth. On examination there was hemi-hypertrophy, venous varicosities and pinkish discoloration.

Plain radiographs showed marked soft tissue enlargement and mild bone enlargement.



Colour Doppler showed absent deep venous system and few varicose venous channels.















Angiography of right lower extremity showed increased little tortuous arterial branches and absent deep venous system

















What is the most likely diagnosis?


ANSWER

Klippel-Trénaunay Syndrome

Klippel-Trénaunay Syndrome  is a rare congenital malformation characterised by the triad of capillary malformations, atypical varicosities or venous malformations, and bony or soft tissue hypertrophy usually affecting one extremity.The most frequent clinical aspect of Klippel-Trenaunay Syndrome is a dermal capillary stain associated with dilated marginal vein of Servelle often identified in the subcutaneous fat of the lateral calf and thigh. It should be distinguished from Parkes-Weber syndrome by absence of underlying arteriovenous malformation.Prominent superficial varicose veins are present in majority of the patients.Extremity pain, spontaneous haemorrhages, venous insufficiency or thrombophlebitis are commonly encountered. Treatment is usually conservative and includes application of graded compressive stockings or pneumatic compression devices. Percutaneous sclerosis of varicosities and surgical treatment by epiphysiodesis to control leg length discrepancy, excision of hypertrophied soft tissue or less commonly reconstructive surgery of deep venous obstruction has been reported in some patients.

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15 comments

  1. Anonymous Says:
  2. Nevus varicosus osteo-hypertrophycus or Parks-Weber syndrome....

     
  3. Anonymous Says:
  4. Klippel-Trénaunay-Weber syndrome...

     
  5. Anonymous Says:
  6. klippel trenaunay syndrome

     
  7. Anonymous Says:
  8. ???

     
  9. Anonymous Says:
  10. may b elephantiasis

     
  11. Anonymous Says:
  12. please,I want to know the correct answer

     
  13. Erick Says:
  14. elephantiasis... XD

     
  15. Anonymous Says:
  16. Parks- Weber Syndrome

     
  17. drkhalil Says:
  18. Klippel-Trénaunay Syndrome

     
  19. Anonymous Says:
  20. please,I want to know the correct answer

     
  21. vein Says:
  22. what's that? i didn't see before this look very serious.

     
  23. Anonymous Says:
  24. Parks- Weber Syndrome

     
  25. Anonymous Says:
  26. parks-weber syndrome

     
  27. klippel trenaunay weber syndrome

     
  28. Anonymous Says:
  29. Klippel–Trénaunay–Weber syndrome

     

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