International Urology and Nephrology

, Volume 42, Issue 3, pp 575–578 | Cite as

A girl of Klippel-Trenaunay Weber syndrome coexistence of recurrent bloody vaginal discharge

  • Mehmet Turkmen
  • Salih KavukçuEmail author
  • Handan Çakmakci
  • Alper Soylu
  • Sebnem Aktan
  • Yeliz Çağan
Urology - Case Report


Klippel-Trenaunay Weber syndrome (KTWS) is the coexistence of capillary vascular malformations, varicose veins, dilated arteries and arteriovenous fistulas, soft tissue and/or bone hypertrophy. We present a girl of KTWS associated with hypertrophied left kidney, enlargement in venous structures of the left kidney, recurrent bloody vaginal discharge and angiokeratomas. A 6-year-old girl was admitted to our department with complaints of recurrent bloody vaginal discharge and swelling in the left inguinal region. Physical examination revealed hypertrophy of the left lower extremity. Vaginoscopy and cystoscopy revealed normal findings. Abdominal ultrasound revealed an enlarged left kidney. Enlargement in the venous structures of the hypertrophied left kidney was detected by abdominal magnetic resonance imaging, and arteriovenous fistulas were revealed by conventional angiography. The patient was diagnosed KTWS. Ophthalmic examination was normal. Galactosidase A (GLA) level was found to be at the lower limit of the normal range, and mutation was not detected in the GLA gene. In conclusion, we have emphasized that the girls with recurrent vaginal discharge might be KTWS. Angiokeratoma may be considered as a dermatological finding of KTWS. KTWS may also have enlarged kidney and enlargement in venous structures of the kidney in hypertrophied side.


Klippel-Trenaunay Weber syndrome Angiokeratoma Enlarged kidney 



The authors would like to thank Gulgun Kavukcu, MD, for invaluable assistance in the preparation of this manuscript.


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Copyright information

© Springer Science+Business Media, B.V. 2009

Authors and Affiliations

  • Mehmet Turkmen
    • 1
  • Salih Kavukçu
    • 1
    Email author
  • Handan Çakmakci
    • 2
  • Alper Soylu
    • 1
  • Sebnem Aktan
    • 3
  • Yeliz Çağan
    • 1
  1. 1.Department of PediatricsDokuz Eylul University HospitalIzmirTurkey
  2. 2.Department of RadyologyDokuz Eylul University HospitalIzmirTurkey
  3. 3.Department of DermatologyDokuz Eylul University HospitalIzmirTurkey

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