Abstract
Background: Klippel-Trenaunay syndrome (KTS) is characterized by vascular malformations and disturbed soft tissue or bony growth, involving one or more extremities. A high incidence of venous thromboembolism (VTE) has been reported in this disorder, along with cases of belated diagnosed chronic thromboembolic (CTE) pulmonary hypertension (CTEPH). We performed a cross-sectional study to investigate the prevalence of GTE in patients with KTS.
Methods: Those from our KTS patient cohort willing to participate were examined with a sequential diagnostic workup including perfusion scintigraphy, computed tomography, and echocardiography.
Results: Of 68 patients, 48 patients participated in the study (median age 43 years; 29 [60%] were female). Eleven patients (23%) had an abnormal perfusion scan result, of whom computed tomographic scanning showed signs of GTE in two patients (4.2%; 95% confidence interval [CI] 1.2%-14%); both patients had a history of VTE. Echocardiography showed no signs of CTEPH in these patients. In total, 23 patients (48%; 95% CI 35%-62%) had a history of superficial vein thrombosis and 8 patients (17%; 95% CI 8.7%-30%) had a history of deep Vein thrombosis or pulmonary embolism, which was associated with more shortness of breath.
Limitations: Echocardiography was only performed in patients with GTE.
Conclusion: A large proportion of patients with KTS had a history of VTE. The prevalence of GTE in the total KTS cohort, however, appeared less alarming than previously assumed. Based on these results, we suggest that there is only a limited indication for CTEPH screening among patients with KTS. Nevertheless, awareness for CTEPH remains appropriate, especially among patients presenting with shortness of breath and a history of VTE. (J Am Acad Dermatol 2012;66:71-7.)
Original language | English |
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Pages (from-to) | 71-77 |
Number of pages | 7 |
Journal | Journal of the American Academy of Dermatology |
Volume | 66 |
Issue number | 1 |
DOIs | |
Publication status | Published - Jan 2012 |
Keywords
- chronic thromboembolic pulmonary hypertension
- chronic thromboembolism
- Klippel-Trenaunay syndrome
- pulmonary embolism
- vascular malformations
- venous thromboembolism
- VENOUS MALFORMATIONS
- WEBER-SYNDROME
- FOLLOW-UP
- COAGULATION ABNORMALITIES
- THROMBOEMBOLISM
- HYPERTENSION
- ANGIOGRAPHY
- PREVALENCE
- MANAGEMENT
- CRITERIA