J Korean Soc Radiol.  2012 Oct;67(4):289-292. 10.3348/jksr.2012.67.4.289.

Rectal and Splenic Vascular Malformation in Klippel-Trenaunay-Weber Syndrome: A Case Report

• Kim HY
• Chang YW
• Lee DH

• KMID: 1439543
• DOI: http://doi.org/10.3348/jksr.2012.67.4.289

Abstract

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital disorder, characterized by a cutaneous vascular nevus of the involved extremity, vascular malformations, bone and soft tissue hypertrophy of the extremity. We present the case of an 18-year-old female patient with KTWS, showing a marked rectosigmoid wall thickening and phlebolith, and also variable sized cystic masses in the spleen, as a result of vascular malformations.

MeSH Terms

Adolescent
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Extremities
Female
Humans
Hypertrophy
Klippel-Trenaunay-Weber Syndrome
Nevus
Spleen
Vascular Malformations

Figure

• 

  Fig. 1 An 18-year-old female patient diagnosed with Klippel-Trenaunay-Weber syndrome. A. Cutaneous hemangioma on the right ankle. B. Unilateral hypertrophy of soft tissue is noted in the right calf area. C. Venography of the right leg obtained 14-year-old shows dilated superficial veins in the subcutaneous tissue. D. Axial T2-weighted fat suppression magnetic resonance image obtained 14-year-old shows diffuse high signal intensity venous and lymphatic vascular malformations in the pelvic cavity, bilateral muscular compartment and subcutaneous tissue. E, F. Axial (E) and coronal reconstruction CT (F) images show diffuse recto-sigmoid wall thickening (arrow), phleboliths and tubular enhanced vascular engorgement as a result of vascular malformation. Also note numerous venous malformations in the right buttock. G. Axial CT scan shows multiple low density cystic masses in spleen without contrast enhancement, suggestive of lymphatic malformations of the spleen. H. Colonoscopy shows vascular malformation involved the recto-sigmoid colon.

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