Abstract
We had expierienced pulmonary lymphangioleiomyomatosis(LAM) with bilateral chylothorax and chylous ascites. A twenty-one-year-old lass with chief complaint of abdominal pain was admitted through the emergency room. She received emergent pelvicoscopic surgery for the rupture of the right corpus luteum. We aspirated 1000ml of the uncoagulated blood. The bleeding point was cautherized electrically. LAM was diagnosed with tissue from the retroperitoneum. Chylous ascites and bilateral chylothorax were occurred despite of various treatments. On thoracotomy, bullous changed lung and lymphatic leakage from visceral and parietal pleura were observed. She died of respiratory insufficiency and general weakness after 6 months from admission.
림프관평활근종증은 드문 질환이며, 임상경과상 악성으로 분류할 수 있다. 복통을 주소로 입원한 21세 여자 환자가 골반 내시경을 통해 난소 황체 출혈을 지혈하였고, 그 후 유미성 복수로 개복하여 후 복막에 위치한 낭성 종양을 절제하여 림프관평활근종증으로 조직 진단 받았다. 홀몬 치료 중 유미흉이 발생하여 흉부외과에서 개흉하여 림프액 누출부위를 봉합결찰하였고, 10% 포타딘 관주로 유미흉은 호전되었으나 유미성 복수가 재발하였으며 진단 6개월에 전신 쇠약과 호흡 부전으로 사망하였다.
