Capraro PA, Fisher J, Hammond DC, Grossman JA.
Klippel-Trenaunay syndrome.
Plast Recovery Surg 2002 May;109(6):2052-60; quiz 2061-2

Clinic for Plastic Surgery, Easton Hospital, Denver, CO 80220, USA.

The association of three physical findings including capillary malformation,
varicosities, and hypertrophy of bony and soft tissues corresponds to
Klippel-Trenaunay syndrome. This triad of findings, described by the two French
physicians Klippel and Trenaunay in 1900, differs from Parkes-Weber syndrome, in
that Klippel-Trenaunay syndrome does not incorporate significant hemodynamic
arteriovenous fistulas. Generally, management of this disease process should be
individualized. Surgery should be considered in cases where skin ulcerations lead
to persisting and recurrent bleeding, or where digital deformities lead to
functional disabilities or where significant limb overgrowth leads to both
functional and psychological impairment. Persistent hematochezia, hematuria, and
vaginal and esophageal bleeding are considered indications for surgical
intervention. Recurrent attacks of thrombophlebitis and cellulitis are treated
medically with antiinflammatory agents and antibiotics. Otherwise, management of
this syndrome is generally conservative, consisting of psychological
encouragement, reassurance, and the continued use of graduated compressive
stockings for varicosities and intermittent pneumatic compression pumps for
lymphatic edema.