Baseline Quality of Life in patients with Klippel–Trenaunay syndrome

Oduber, Charlène E.U.; Khemlani, Kavita; Sillevis Smitt, J. Henk; Hennekam, Raoul C.M.; van der Horst, Chantal M.A.M.

doi:10.1016/j.bjps.2009.01.055

« Previous Next »Journal of Plastic, Reconstructive & Aesthetic Surgery
Volume 63, Issue 4 , Pages 603-609, April 2010

Baseline Quality of Life in patients with Klippel–Trenaunay syndrome

Charlène E.U. Oduber
- Department of Plastic, Reconstructive and Hand Surgery, Academic Medical Centre, Amsterdam, The Netherlands
- Corresponding author. Academic Medical Centre, University of Amsterdam, Department of Plastic, Reconstructive and Hand Surgery, P.O. Box 22700, 1100 DD Amsterdam, The Netherlands. Tel.: +31 20 566 2974; fax: +31 20 691 7549.
Kavita Khemlani
- Department of Plastic, Reconstructive and Hand Surgery, Academic Medical Centre, Amsterdam, The Netherlands
J. Henk Sillevis Smitt
- Department of Dermatology, Academic Medical Center, University of Amsterdam, The Netherlands
Raoul C.M. Hennekam
- Department of Paediatrics, Academic Medical Centre, Amsterdam, The Netherlands
- Clinical and Molecular Genetics Unit, Institute of Child Health, Great Ormond Street Hospital for Children, UCL, London, UK
Chantal M.A.M. van der Horst
- Department of Plastic, Reconstructive and Hand Surgery, Academic Medical Centre, Amsterdam, The Netherlands

Received 9 September 2008; accepted 30 January 2009. published online 16 March 2009.

Summary

Background

Klippel–Trenaunay syndrome (KTS) is a congenital group of disorders characterised by vascular malformations (capillary malformation (CM), venous malformation (VM), and lymphatic malformation (LM)) and disturbed growth regulation. The burden caused by KTS symptoms can be evaluated using Quality of Life (QoL)-measuring questionnaires. This study aimed to assess the QoL in KTS patients using the Short Form Health Survey Questionnaire (SF-36) and Skindex-29 questionnaires, and to determine three grades of severity (mild, moderate and severe) according to the scores obtained. In addition, we compared the SF-36 results to those of a general Dutch population sample and a selected group of other chronic conditions.

Methods

KTS patients of the Dutch KTS foundation and of two medical centres answered SF-36 and Skindex-29 questionnaires. Control data of validated Dutch population SF-36 scores and literature-acquired scores for other diseases were available.

Results

A total of 78 patients were enrolled, of whom 34 (43.6%) were male; the mean age was 39.3 years (SD: 17.1; range: 12–78 years). The Dutch KTS group scored significantly lower than the general Dutch population on all SF-36 scales except Mental Health and Role Emotional. Furthermore, they scored significantly lower than other medical conditions on the Physical Functioning and Bodily Pain scales. According to the Skindex-29 results, KTS patients fall in the categories – symptoms: severe to very severe; emotions: diminutive to mild and functions: mild. The total score is lower than 40, indicating a negligible negative impact on QoL; however, new cut-off values are being calculated.

Conclusions

Classification according to severity is important to educate patients accordingly, predict prognosis and set treatments. Especially in cases of severe KTS, physicians should not only be attentive to the physical aspects but also to the psychological and social aspects of KTS.

Keywords: Klippel–Trenaunay syndrome, Klippel–Trenaunay–Weber syndrome, Quality of Life, Severity, Sub-classification