Abstract
Background: In Klippel-Trenaunay syndrome (KTS), a congenital combined vascular (capillary, venous and lymphatic) malformation with localised disturbed growth, venous thromboembolisms (VTEs) are frequently reported in small cohorts.
Design and methods: We quantified the frequency of VTE by screening a large KTS-patient cohort with duplex compression ultrasonography. Additionally, we performed a case-control study to evaluate whether coagulation alterations were related to VTE and magnitude of vascular malformations as quantified by magnetic resonance imaging (MRI).
Results: Twenty-nine (39%) of 75 patients had signs of current or previous VTE, including superficial venous thrombosis, six (8%) of whom had a deep venous thrombosis or a pulmonary embolism. Compared with 105 controls, 54 adult patients (both: median age 33 years) had higher plasma levels of D-dimer, medians 266 (IQR 195-366) versus 457 (IQR 270-3840) mg/l (p
Conclusions: Otherwise healthy KTS patients had a very high rate of current or previous VTE. Very high D-dimer levels were observed and these were related to the extent of the vascular malformation. Based on these findings we advise appropriate patient education on the signs and symptoms of thromboembolic complications in these patients, and screening for VTE in case of complaints.
| Original language | English |
|---|---|
| Pages (from-to) | 246-252 |
| Number of pages | 7 |
| Journal | Netherlands Journal of Medicine |
| Volume | 71 |
| Issue number | 5 |
| Publication status | Published - Jun 2013 |
| Externally published | Yes |
Keywords
- Venous thromboembolism
- Klippel-Trenaunay syndrome
- vascular malformation
- duplex compression ultrasonography
- deep venous thrombosis
- D-dimer
- RECURRENT PULMONARY-EMBOLISM
- WEBER-SYNDROME
- MALFORMATIONS
- COAGULATION
- MANAGEMENT
- HYPERTENSION
- FEATURES
- PLASMA
- LIMBS