Sana Aslam, Hina Hanif Mughal, Umme Kalsoom, Aniqua Saleem, Nida Akram, Azalfa Shafi.
Spectrum of imaging findings in Klippel-trenaunay syndrome affecting lower limb: a case report.
Pak J Radiol Jan ;33(4):216-9.

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder featuring triad of vascular malformation, capillary malformations (port-wine stains), and limb hypertrophy. It usually manifests unilaterally in lower limbs. We discuss a case of a 2-year-old boy born with swelling of right lower limb with enlarged right leg. Physical examination showed a grossly enlarged right thigh. There were multiple venous varicosities along with diffuse port-wine stains on the right leg along anterior and lateral aspect. Doppler ultrasound revealed several venous varicosities. Plain radiographs confirmed limb hypertrophy. On the right lower limb MRI, multiple tortuous superficial veins were seen diffusely affecting the skin and subcutaneous tissues with circumferential soft tissue hypertrophy.. This massive venous and lymphatic malformation was seen extended inferiorly to the dorsum of the foot and superiorly to the hip joint. These radiological findings were concerning for the diagnosis of Klippel-Trenaunay syndrome .Medical imaging is the cornerstone in the diagnosis and assessment of severity and complications, follow-up and differentiation of KTS from other similar conditions.

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