Pulmonary lymphangiomyomatosis treated by single lung transplantation.

Pulmonary lymphangiomyomatosis is a rare disease resistant to almost all medical treatments to date. We describe the case of a 44-yr-old woman with end-stage pulmonary lymphangiomyomatosis who was treated by single-lung transplantation. The patient is doing well in her sixteenth post-transplantation month and has a marked improvement in her pulmonary function tests and walking distance as compared with preoperative values, and she is enjoying an unrestricted life-style.