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Klippel-Trénaunay syndrome: spectrum and management
A G Jacob1, D J Driscoll, W J Shaughnessy
1Section of Pediatric Cardiology, Mayo Clinic Rochester, Minnesota 55905, USA.
Mayo Clinic Proceedings
|January 27, 1998
Summary
Klippel-Trénaunay syndrome (KTS) is a rare congenital condition. Conservative management is often best, but surgery can help leg length discrepancies and symptomatic venous malformations.
Area of Science:
- Vascular Malformations
- Congenital Disorders
- Orthopedic Surgery
Background:
- Klippel-Trénaunay syndrome (KTS) is a rare congenital vascular malformation.
- It is characterized by capillary malformations, atypical varicosities/venous malformations, and limb hypertrophy.
Purpose of the Study:
- To describe the clinical characteristics and findings of 252 patients with Klippel-Trénaunay syndrome.
- To discuss management options for KTS.
Main Methods:
- Retrospective review of 252 KTS patients (136 female, 116 male) assessed between 1956 and 1995.
- Analysis of clinical features, malformations, and surgical interventions.
Main Results:
- Capillary malformations occurred in 98%, venous malformations in 72%, and limb hypertrophy in 67%.
- All three KTS features were present in 63% of patients.
- Surgical interventions included epiphysiodesis, vein stripping, excision, and amputations.
Conclusions:
- Conservative management is recommended for most KTS patients.
- Epiphysiodesis is indicated for leg length discrepancies >2.0 cm.
- Surgical removal of symptomatic varicosities/venous malformations can yield good results if deep venous system is functional.