Background: Klippel-Trenaunay syndrome is a rare disease characterized by capillary malformationsand soft tissue and bony hypertrophy and atypical varicosities. Management of this syndrome is focused primarily on treatment of the complications that arise from these malformations. Ascites and lymphedema are two of the more common complications in these patients.
Case: A 15-year-old female with Klippel-Trenaunay syndrome presented with chylous ascites, vaginal drainage, and unilateral lower extremity lymphedema. Treatment included dilation, hysteroscopy and curettage, and laparoscopic evacuation of abdomino-pelvic ascites with resolution of symptoms for 32 months. Repeat laparoscopic drainage was successful and remains symptom free after 12 months.
Conclusion: Vaginal drainage of chylous ascites is a rare complication from Klippel-Trenaunay syndrome and can be successfully managed by techniques to remove abdomino-pelvic ascites.
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