Abstract

GONTERO, R.; ORTIZ, A.; ROVERANO, S.  and  PAIRA, S.. Klippel-Trenaunay syndrome: communication of two cases. Rev. argent. reumatolg. [online]. 2017, vol.28, n.1, pp.42-47. ISSN 0327-4411.

Klippel-Trenaunay syndrome is characterized by a classic triad consisting of cutaneous capillary malformation, hypertrophy of the soft tissue and/or bone in extremities, and resulting venous malformations in varicose veins or other malformations of the venous system deep. It is diagnosed by the presence of any of two of the three characteristics mentioned above. It should be borne in mind that SKT may present atypical manifestations such as bleeding and ulcers caused by vascular malformations in the gastrointestinal tract, genitourinary tract, spleen, central nervous system. The differential diagnosis of a vascular injury associated with enlargement of the limbs should include other disorders such as Sturge-Weber syndrome, Parkes-Weber syndrome and Proteus syndrome among others. Klippel-Trenaunay syndrome with its multisystemic complications requires a multidisciplinary approach to comprehensive management. Two cases are reported that present with the three characteristics of the syndrome.