Background
Lymphangiomatosis is a condition marked by the presence of cysts that result from an increase both in the size and number of thin-walled lymphatic channels that are abnormally interconnected and dilated. Although the condition is benign, these deranged lymphatics invade surrounding tissues and cause problems due to invasion and compression of adjacent structures. Pelvic lymphangiomatosis usually present with abdominal pain. We present here a case of a 7 year-old girl who presented with vaginal discharge as primary symptom of this rare condition.
Case
A 7 year-old girl consulted at the emergency room in September 2008 for vaginal bleeding. She experienced the bleeding for the first time 3 weeks earlier, and it happened three more times until her visit to the hospital. At each time, her underwear was soiled by a mix of wateous and bleeding secretions. She was in good health and had only a history of constipation. At exam, she was Tanner 1 in her puberty development and had no abdominal mass. At external gynecologic exam, she had an intact perforated hymen, a small angioma on the left labia and no anal stricture was found. Cultures were done and came back negative. She underwent a pelvic ultrasound that showed normal uterus and ovaries without sign of a mass, foreign body or sign of precocious puberty. An exam under anesthesia was planned and the vaginoscopy failed to explain the discharge. She came back to the emergency because of increasing discharge and another exam under anesthesia was performed with a cystoscopy without any result. A MRI was done and diagnosed an extensive abdominal, pelvic and retroperitoneal lymphatic anomaly. An exploratory laparoscopy was done and showed an confirmed the disseminated pelvic lymphangiomatosis.
Because of the worsening of the symptomatology of the patient (she had to wear a diaper to manage the vaginal discharge), four Doxycycline sclerotherapies were performed from July 2009 to February 2011.
Comments
To our knowledge, this is the first case published in the literature of pelvic lymphangiomatosis that presented initially with vaginal discharge in a child.
Article info
Publication history
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Identification
Copyright
© 2012 Published by Elsevier Inc.
