Klippel-Trenaunay syndrome

Definition: The Klippel-Trenaunay syndrome (KTS) is a rare anomaly characterized by naevus, soft tissue hypertrophy, associated deep venous system abnormalities and varicosities. Vascular lesions associate hemangiomata, varicose veins, and both bony and soft tissue hemihypertrophy. Abdominal viscera affected by ipsilateral hemangiomata include colon, liver, spleen, jejunum, kidney, and liver.
Ent. Nom. 1900, Klippel and Trenaunay.

Synopsis

Vascular malformations

 nevus flammeus
 arteriovenous fistulas
 abnormalities of arteries
 capillary-venous malformation
 cutaneous hemangioma
 mediastinal cavernous hemangioma (12554906)
 lymphatic malformations (lymphangioma)
 associated deep venous system abnormalities
 varicosities
 naevus
 soft tissue hypertrophy
 deep venous system abnormalities
 vascular lesions

• hemangiomatosis (multiple hemangiomas)
  • ipsilateral visceral hemangiomas: colon, liver, spleen, jejunum, kidney, and liver.

• varicose veins
• bony hemihypertrophy and soft tissue hemihypertrophy

 asymmetric hemi-hypertrophy of limbs and trunk, macrodactyly, syndactyly, polydactyly, oligodactyly
 soft tissue hypertrophy, soft tissue overgrowth

 hyperpigmented nevi and streak

Complications

 stasis dermatitis
 thrombophlebitis
 cellulitis
 limb disparity
 thrombosis
 bleeding
 pulmonary embolism
 congestive heart failure

References

 Lie JT. Pathology of angiodysplasia in Klippel-Trenaunay syndrome.
Pathol Res Pract. 1988 Nov;183(6):747-55. PMID: 2851776