Klippel-Trénaunay-Weber syndrome

Case contributed by Dr Aneesh km


Right upper limb hypertrophy, capillary malformation

Patient Data

Age: 6 yrs
Gender: Female

Contrast enhancing hyperintense T2 and hypo to isointense T1 signals noted diffusely involving the entire muscular compartments of right forearm and upper arm without respecting facial planes, also involving skin and subcutaneous tissue.

Mid and distal shaft of humerus, radius and ulna show abnormal signals in the marrow. Multiple flow voids noted in the lesions indicating high flow component. CT showed multiple phleboliths.

Case Discussion

Features are suggestive of mixed vascular malformation involving the entire upper limb, involving skin, subcutaneous tissues, muscles and bone. Flow voids indicate high flow component, possibly AV fistulas.

Klippel-Trénaunay-Weber or Parkes-Weber syndrome.

PlayAdd to Share

Case information

rID: 18263
Published: 24th Jun 2012
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.