MULTI-MODAL IMAGING FEATURES AND LYMPHO-VENOUS SHUNT FOR VAGINAL CHYLOUS FISTULA IN LYMPHANGIOLEIOMYOMATOSIS: CASE REPORT AND REVIEW

Abstract

Vaginal chylous fistula is a very rare lymphatic disorder and lymphangioleiomyomatosis (LAM) is also a rare multisystemic disease. LAM with the development of a vaginal chylous fistula has rarely been reported. Here we report a case of vaginal chylous fistula accompanied with LAM. A 61-year-old woman occasionally experiencing milky vaginal discharge for the last 3 months underwent CT lymphangiography (CTL) after direct lymphangiography (DLG) and lymphoscintigraphy. DLG showed lymph flowing backwards toward the pelvic cavity. CTL revealed abnormal distribution of contrast agent in the pelvic cavity, including uterus, vagina, perineum, bladder, and muscles in the bilateral pelvis. Multiple small thinwalled cystic lesions were present in both lungs. Lymphoscintigraphy demonstrated lymphangiectasia in the pelvic cavity bilaterally with radionuclide tracer flowing back abnormally into the pelvic walls. The vaginal exudate was positive for chyle. According to LAM guidelines, the patient was diagnosed with LAM. This case presents multimodal images of a rare case of vaginal chylous fistula accompanied with LAM and highlights the value of CT lymphangiography after DLG in establishing the diagnosis and providing important guidance for sequential therapy