폐와 후복강에 발생한 림프관평활근종증 1예

A Case of Pulmonary and Retroperitoneal Lymphangioleiomyomatosis

저지들은 캐나다 방문당시 복부 팽만감을 주소로 그곳 병원을 림프관평활근 종증을 진단받고 귀국 후 본 병원에서 매달 Depo provera를 근주하고 매달 폐기능 검사등을 실시하여 추적관찰하고 있는 폐와 후복강에 발생한 림프관평활근종증의 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Lymphangioleiomyomatosis, a rare disease in women of childbearing age, is the result of benign nodular hypertrophy of the smooth muscle of the lypmhatics and other tissues of the abdomen and thorax. We report a 36-years-old woman with pulmonary and retroperitoneal lymphangioleiomyomat.osis who responded with hormone treatment. She developed vaginal pruritis and a pelvic ultraound was done given her significant past medical history. Ultrasound examination demonstrated a large mass in the right side of her pelvis. Therefore she was admitted to St. Michael's Hospital in Toronto for laparoscopy. Result of cytology was to be consistent with the diagnosis of retroperitoneal lymphangioleiomyomatosis. High resolution CT sacn of the thorax demonstrated multiple small cystic lesions, without associated nodularity compatible with a diagnosis of pulmonary lymphangioleiomyomatosis. She has been taking Provera tablets 100mg po tid since Dec. 15, 1993. We have given her a prescription for Depo provera 500mg IM monthly since she came back to Korea. and made arrangements for regular follow up monthly. We performed chest X-ray, CT of chest(high resolution), abdomen and pelvis, pulmonary function tests and arterial blood gas analysis. Chest X-ray and CT findings showed no significant change since July. 20, 1993.