Klippel-Trenaunay Syndrome Associated with Urinary Calculus Disease in a Child: Case Report and a Review of the Literature

Klippel-Trenaunay syndrome (KTS) is a rare disease composed of a triad of (a) malformed capillaries, (b) varicose veins and/or vascular malformation, and (c) hypertrophic soft or bone tissue. It is present at birth. The capillary malformations, also known as port-wine stain, are the most common skin abnormalities in KTS. Lymphoid hyperplasia is found in more than 50% of these patients. It may be located in any part of the body, but is most common in the limbs, in which case the increased size of the affected limb becomes more noticeable with age. Complications include secondary infection, thrombophlebitis, neuropathic pain, and bone involvement. Diagnosis is clinical and support from diagnostic imaging such as computed tomography angiography, NMR, or conventional angiography can be important for diagnosis and treatment. In general, treatment is conservative.