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Previous Volume 335:1275-1280 October 24, 1996 Number 17 Next

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ABSTRACT

Background Lymphangioleiomyomatosis is a rare disease of unknown origin that usually leads to progressive deterioration of lung function and eventual death from respiratory failure. It occurs in women of reproductive age and people with tuberous sclerosis. Lung transplantation is a recent therapeutic approach.

Methods We conducted a retrospective study by questionnaire of 34 patients, treated at 16 transplantation centers, who underwent lung transplantation for end-stage lymphangioleiomyomatosis between 1983 and 1995.

Results Of the 34 patients, 27 received single-lung transplants; 6, bilateral transplants; and 1, a heart–lung transplant. As of August 31, 1995, the actuarial survival calculated by the Kaplan–Meier method was 69 percent after one year and 58 percent after two years. Eighteen patients were alive a mean (±SD) of 33±20 months (range, 3 to 74) after transplantation. Forced expiratory volume in one second increased from 24±12 percent of the predicted value before transplantation to 48±16 percent six months after transplantation. Five early deaths (within one month) were due to hemorrhage (in one patient), acute lung injury (in three), and dehiscence of the bronchial anastomosis (in one). Eleven late deaths (after one month) were due to infections (in eight patients), bronchiolitis obliterans (in two), and metastatic nephroblastoma (in one). Disease-associated problems were extensive pleural adhesions in 18 patients, leading to moderate-to-severe intraoperative hemorrhage in 4; pneumothorax in the native lung after single-lung transplantation in 6 patients; postoperative chylothorax in 3; and recurrent lymphangioleiomyomatosis in the allograft in 1 patient, who died of disseminated aspergillosis.

Conclusions Although disease-related complications are frequent, lung transplantation can be a valuable therapy for patients with end-stage lymphangioleiomyomatosis.

Source Information

From the Department of Internal Medicine (A.B., R.S., E.W.R.) and the Department of Surgery (W.W.), University Hospital of Zurich, Zurich, Switzerland; and the St. Louis International Lung Transplant Registry, St. Louis, and other participating institutions.

Address reprint requests to Dr. Boehler c/o Dr. Speich, Department of Internal Medicine, University Hospital, 8091 Zurich, Switzerland.

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