Abstract
The patient was a 31-year-old woman with intractable and recurrent pneumothorax. She had been diagnosed with tuberous sclerosis and renal angiomyolipoma at 21 years old. She underwent bullectomy for the right pneumothorax by thoracoscopic surgery at 26 years old, and LAM was diagnosed histopathologically. In spite of anti-hormone therapy for LAM, she was admitted to our hospital because of recurrence of the left pneumothorax pooly controlled by tube drainage. Multiple air cysts and three apparent thin-walled bullae were recognized on the surface of the lung. Bullectomy was performed, however, left pneumothorax recurred three months later. Although lung transplan-tation was considered, chemical pleurodesis was performed along with the direct closure of the pulmonary fistula. She has remained well with no evidence of recurrence of pneumothorax for 18 months. Pleural adhesion has been regarded as a relative contraindication for lung transplantation in Japan, but chemical pleurodesis may be acceptable before transplantation to maintain the patient's QOL or to prevent life-threatening respiratory failure due to intractable and recurrent pneumothorax associated with LAM.
