Klippel-Trénaunay-Weber syndrome

Case contributed by Dr Aneesh km


Right upper limb hypertrophy, capillary malformation

Patient Data

Age: 6 years
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.

Diagnosis: Klippel-Trénaunay-Weber or Parkes-Weber syndrome.

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