Lymphangioléiomyomatose

Abstract

Lymphangioleiomyomatosis is a rare disease in women leading to respiratory failure. We describe a single-institution experience with lung transplantation for end-stage lymphangioleiomyomatosis.We retrospectively reviewed records of patients transplanted for lymphangioleiomyomatosis between 1989 and 2001. Follow-up was complete on all patients (median 3.5 years).Seven single and 7 bilateral transplants were performed on 14 recipients (mean age 41.8 years). Eleven patients suffered the following intra-operative complications: dysrhythmia (1); blood loss > 1,000 ml (7); extensive pleural adhesions (10); hypothermia (1); phrenic nerve injury (1); and graft dysfunction (2). The following post-operative complications occurred in 11 recipients: dysrhythmia (7); respiratory failure (5); sepsis (3); airway dehiscence (2); vocal cord dysfunction (1); cholecystitis (1); deep vein thrombosis (1); acute renal failure (1); and pericarditis (1). Post-operative chylous fistulas necessitated thoracic duct ligation (1); sclerosis (6); and drainage of ascites (1). There were no peri-operative deaths. Late deaths occurred due to sepsis in 2 patients and obliterative bronchiolitis in 1 patient. Survival rates were 100%, 90% and 69% at (1, 2 and 5 years, respectively.) Mean FEV1 (1.77 ± 1.06 vs 0.60 ± 0.91) and 6-minute walk (1,519 ± 379 vs 826 ± 293 feet) improved at 1 year as compared with pre-transplant evaluation. Five patients reached criteria for bronchiolitis obliterans syndrome. One recipient has had a documented recurrence of lymphangioleiomyomatosis in the transplanted lung.Early and late survival after lung transplant are comparable in lymphangioleiomyomatosis patients versus patients with other diseases. Morbidity is common after transplant for lymphangioleiomyomatosis and is usually due to lymphangioleiomyomatosis-related complications. Lymphangioleiomyomatosis recurrence in the allograft does not pose a substantial clinical problem.