PURPOSE: Lymphangioleiomyomatosis (LAM) is a progressive pulmonary disease which may ultimately require lung transplantation. Many patients develop pneumothorax (70%) or chylothorax (30%) requiring pleural interventions which can complicate lung transplantation and affect candidacy. Management strategies for pleural complications of LAM remain controversial.
METHODS: A questionnaire exploring management recommendations for LAM-related pleural complications was sent to the 52 active lung transplant centers in the United States. Twenty-two centers responded.
RESULTS: For treatment of the first pneumothorax, 95% (21/22) recommended non-operative procedures (observation, thoracentesis, or tube thoracostomy). For treatment of first recurrence, 18% (4/22) selected non-operative procedures, 23% (5/22) chemical pleurodesis via chest tube, 45% (10/22) VATS procedure (with chemical, mechanical, or chemical plus mechanical pleurodesis), and 14% (3/22) open intervention (thoracotomy). For subsequent recurrences, 9% (2/22) recommended non-operative procedures, 14% (3/22) chemical pleurodesis via chest tube, 41% (9/22) VATS procedure, and 36% (8/22) open intervention. For treatment of first chylothorax, 91% (20/22) selected non-operative therapy (thoracentesis, dietary modification, tube thoracostomy, octreotide). For treatment of first recurrence, 23% (5/22) selected non-operative therapy, 18% (4/22) chemical pleurodesis via chest tube, 50% (11/22) VATS procedure, and 9% (2/22) open procedures. For subsequent recurrences, 5% (1/22) selected non-operative procedures, 18% (4/22) chemical pleurodesis via chest tube, 41% (9/22) VATS procedure, and 36% (8/22) open interventions.
CONCLUSION: Non-operative management of initial chylothorax and initial pneumothorax was the preferred strategy recommended by most transplant centers. This challenges the convention that patients with secondary spontaneous pneumothorax should undergo pleural symphysis. With subsequent recurrences, more invasive interventions were recommended, including talc pleurodesis and pleurectomy. However, these more aggressive pleural symphysis procedures are considered a contraindication to transplantation at many centers.
CLINICAL IMPLICATIONS: When managing pleural complications in LAM patients, it is imperative to take into consideration the durability of the treatment, as well as the effect of the treatment on future lung transplant candidacy.
DISCLOSURE: Christopher Lyons, No Financial Disclosure Information; No Product/Research Disclosure Information