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Klippel-Trenaunay syndrome.
Philippe A Capraro1, Jay Fisher, Dennis C Hammond
1Clinic for Plastic Surgery, Easton Hospital, Denver, CO 80220, USA. pcapraro@clinic4plasticsurgery.com
Plastic and Reconstructive Surgery
|May 8, 2002
Summary
Klippel-Trenaunay syndrome is a vascular malformation characterized by capillary malformations, varicosities, and tissue hypertrophy. Management is individualized, with surgery for severe complications and conservative care for milder symptoms.
Area of Science:
- Vascular Malformations
- Genetics and Developmental Biology
Background:
- Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder.
- It presents as a triad of capillary malformations, venous varicosities, and lymphatic or bony/soft tissue hypertrophy.
Observation:
- KTS is distinct from Parkes-Weber syndrome due to the absence of significant arteriovenous fistulas.
- Clinical manifestations vary widely among affected individuals.
Findings:
- Surgical intervention is indicated for severe bleeding from skin ulcerations, functional impairment from digital deformities, or significant limb overgrowth.
- Recurrent thrombophlebitis and cellulitis necessitate medical treatment with anti-inflammatory agents and antibiotics.
Implications:
- Individualized management is crucial for KTS.
- Conservative measures include psychological support, compression stockings, and pneumatic pumps.
- Early diagnosis and tailored treatment can improve patient outcomes and quality of life.