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

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 t...

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Bibliographic Details
Published in:International urology and nephrology 2010-09, Vol.42 (3), p.575-578
Main Authors: Turkmen, Mehmet, Kavukçu, Salih, Çakmakci, Handan, Soylu, Alper, Aktan, Sebnem, Çağan, Yeliz
Format: Article
Language:English
Subjects:
Child
Female
Hemorrhage - etiology
Humans
Kidney - abnormalities
Klippel-Trenaunay-Weber Syndrome - complications
Klippel-Trenaunay-Weber Syndrome - diagnosis
Klippel-Trenaunay-Weber Syndrome - pathology
Medicine
Medicine & Public Health
Nephrology
Urology
Urology - Case Report
Vagina - blood supply
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Summary: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.
ISSN:0301-1623
1573-2584
DOI:10.1007/s11255-009-9640-z