JBM  Vol.4 No.11 , November 2016
Klippel-Trenaunay Syndrome: A Rare Cause of Vaginal Bleeding in the Prepubescent Girl
Abstract: Klippel-Trénaunay syndrome (KTS) is an uncommon congenital disorder with unknown etiology that consists of mixed vascular malformations. Visceral organs may also be involved leading to a variety of manifestations. Although seemingly uncommon, genital tract involvement can occur and be a source of significant morbidity. We hereby describe a case of KTS of a 7-year-old female patient presenting with genital bleeding, large venous and capillary malformations and soft tissue hypertrophy of right limb. Patient was treated conservatively and asked to follow-up regularly. KTS should be kept on mind as a cause of vaginal bleeding in preteen girls especially those with suggestive clinical features. Management of this complex condition is challenging because of its progressive nature and wide range of disease severity.
Cite this paper: Essabar, L. , Amenzouy, S. , Oudghiri, F. , Kerboubi, L. and Dakhama, B. (2016) Klippel-Trenaunay Syndrome: A Rare Cause of Vaginal Bleeding in the Prepubescent Girl. Journal of Biosciences and Medicines, 4, 1-7. doi: 10.4236/jbm.2016.411001.

[1]   Suman, S., Sinha, A.K., Kumari, S., et al. (2015) Klippel Trenaunay Syndrome: A Case Report. Journal of Evolution of Medical and Dental Sciences, 4, 5981-5985.

[2]   Sung, H.M., Chung, H.Y., Lee, S.J., Lee, J.M., et al. (2015) Clinical Experience of the Klippel-Trenaunay Syndrome. Archives of Plastic Surgery, 42, 552-558.

[3]   Baskerville, P.A., Ackroyd, J.S. and Browse, N.L. (1985) The Etiology of the Klippel- Trenaunay Syndrome. Annals of Surgery, 202, 624-627.

[4]   Jacob, A.G., Driscoll, D.J., Shaughnessy, W.J., Stanson, A.W., Clay, R.P. and Gloviczki, P. (1998) Klippel Trénaunay Syndrome: Spectrum and Management. Mayo Clinic Proceedings, 73, 28-36.

[5]   Husmann, D.A., Rathburn, S.R. and Driscoll, D.J. (2007) Klippel-Trenaunay Syndrome: Incidence and Treatment of Genitourinary Sequelae. The Journal of Urology, 177, 1244-1249.

[6]   Cha, S.H., Romeo, M.A. and Neutze, J.A. (2005) Visceral Manifestations of Klippel- Trénaunay Syndrome. Radiographics, 25, 1694-1697.

[7]   Samo, S., Sherid, M., Husein, H., Sulaiman, S., Yungbluth, M. and Vainder, J.A. (2013) Klippel–Trenaunay Syndrome Causing Life-Threatening GI Bleeding: A Case Report and Review of the Literature. Case Reports in Gastrointestinal Medicine, 2013, Article ID: 813653.

[8]   Wang, Z.K., Wang, F.Y., Zhu, R.M. and Liu, J. (2010) Klippel-Trenaunay Syndrome with Gastrointestinal Bleeding, Splenic Hemangiomas and Left Inferior Vena Cava. World Journal of Gastroenterology, 16, 1548-1552.

[9]   Schofield, D., Zaatari, G.S. and Gay, B.B. (1986) Klippel–Trenaunay and Sturge-Weber Syndromes with Renal Hemangioma and Double Inferior Vena Cava. The Journal of Urology, 136, 442-445.

[10]   Aggarwal, K., Jain, V.K., Gupta, S., Aggarwal, H.K., Sen, J. and Goyal, V. (2003) Klippel- Trenaunay Syndrome with a Life-Threatening Thromboembolic Event. The Journal of Dermatology, 30, 236-240.

[11]   Pedersen, R.S., Hedelund, L., Poulsen, L.H., Bach, A. and Keller, J. (2013) Fatal Klippel- Trénaunay Syndrome in a Child with Pulmonary Embolism. Ugeskr Laeger, 175, 2183- 2184.

[12]   Gloviczki, P., Stanson, A.W., Stickler, G.B., et al. (1991) Klippel-Trenaunay Syndrome: The Risks and Benefits of Vascular Interventions. Surgery, 110, 469-479.