Congenital Venous Lymphatic Malformation as an Unusual Source of Premenarchal Vaginal Bleeding

Premenarchal vaginal bleeding can present a diagnostic challenge and has not been previously reported in association with congenital venous lymphatic malformation.

A 3-year-old girl presented with intermittent vaginal bleeding since birth. Evaluation showed premenarchal estradiol and gonadotropin hormones, a normal pelvic ultrasound and normal colonoscopy. Examination under anesthesia and vaginoscopy showed a congested appearance of the hymen and vaginal petechiae. Sudden development of a vulvar cyst led to a pelvic magnetic resonance imaging scan, which revealed a venous malformation of the pelvis and vulvar lymphatic cyst. Vaginal bleeding stopped for approximately 6 months after sclerotherapy with doxycycline and ethanol, however recurred and required repeat sclerotherapy.

Congenital venous lymphatic malformations can rarely be associated with premenarchal vaginal bleeding and should be considered when all more common etiologies have been ruled out. Diagnosis is made using magnetic resonance imaging, and treatment might include doxycycline and ethanol sclerotherapy.