From the Research
Klippel-Trenaunay syndrome (KTS) is diagnosed based on the presence of a triad of clinical features: capillary malformations (port-wine stains), venous malformations or varicosities, and soft tissue or bone hypertrophy affecting one or more limbs, as supported by the most recent study 1.
Diagnostic Criteria
The diagnostic criteria for KTS include:
- Capillary malformations (port-wine stains)
- Venous malformations or varicosities
- Soft tissue or bone hypertrophy affecting one or more limbs Imaging studies such as ultrasound, MRI, CT scans, or venography may be used to evaluate the extent of vascular malformations, as noted in 2 and 3.
Treatment Options
Treatment for KTS is primarily supportive and symptom-focused, as there is no cure for the syndrome. Conservative management includes:
- Compression garments to manage venous insufficiency and lymphedema
- Physical therapy to maintain mobility
- Pain management with analgesics as needed For more severe cases, interventional procedures may be necessary, including:
- Sclerotherapy for venous malformations
- Laser therapy for port-wine stains
- Endovenous laser or radiofrequency ablation for varicose veins Surgical options include debulking procedures for tissue overgrowth, vein stripping for varicosities, and orthopedic interventions for limb length discrepancies, as discussed in 4 and 5.
Management Approach
Patients with KTS should be managed by a multidisciplinary team including vascular specialists, dermatologists, orthopedic surgeons, and physical therapists. Regular follow-up is essential to monitor for complications such as thrombosis, bleeding, cellulitis, and lymphedema. The management approach should be individualized based on the specific manifestations and severity of the syndrome in each patient, as emphasized in 1.