폐림프관 평활근종증 환자에서 이식편 기능부전에 의한 반대쪽 단측폐 재이식수술

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease in women of childbearing ages that eventually leads to respiratory failure. Lung transplantation is the only conclusive therapeutic modality in end-stage LAM. While single-lung transplantation is the preferred operation, the graft failure or recurrence of LAM was reported. We performed a single lung transplantation on a 36-year-old woman suffering from respiratory failure due to lymphangioleiomyomatosis. After a 1-year follow up, the patient was readmitted because of graft failure with collapsed transplanted lung. The lung volume reduction surgery (LVRS), tracheostomy and ventilator care were performed. However, neither the medical nor surgical treatment had any effect. Subsequently, we performed a contralateral single lung re-transplantation and had a good postoperative results.