Jeffrey D. Edelman

LUNG TRANSPLANTATION: A Disease-Specific Approach

Lung transplantation has emerged as a viable option for the treatment of end-stage disease attributable to a wide spectrum of primary disorders. Although many aspects of patient management are indifferent to the underlying indication, important differences related to timing of transplantation, selection of candidates, choice of procedure, and post-transplant complications exist among the various primary disease groups. Optimal utilization of transplantation for these challenging patient populations with advanced lung disease mandates a thorough appreciation of those differences.

Lymphangioleiomyomatosis: The surgeon’s role in diagnosis ☆ ☆☆

with lymphangioleiomyomatosis. These patients may have spontaneous pneumothorax or chylothorax or may require evaluation of interstitial lung disease. Because of the rarity of this disease, the diagnosis of lymphangioleiomyomatosis may not always be considered by referring clinicians. Proper diagnosis may lead to significant alterations in patient management, and it is therefore important for thoracic surgeons to be familiar with this rare disease. We recently established a previously missed diagnosis of lymphangioleiomyomatosis in a 59-year-old woman who had a spontaneous chylothorax. Case report. The patient is a 59-year-old nonsmoking woman who had undergone hysterectomy and oophorectomy for uterine fibroid tumors at the age of 45 years and had since been receiving estrogen supplements. Six years before presentation to us she was admitted to an outside hospital with shortness of breath attributed to a significant left pleural effusion. The fluid was drained and reported to be chylous. A computed tomogram (CT) of the chest did not show any parenchymal abnormalities. The effusion did not recur, and further work-up was not pursued. In the interim, the patient had progressive dyspnea and was told she had emphysema. Pulmonary function tests showed severe obstruction unresponsive to bronchodilators, with normal lung volumes and a diffusion impairment. The patient became more symptomatic over the 3 weeks before admission to our thoracic surgery service. Chest x-ray films demonstrated a significant right pleural effusion and a diffuse interstitial pattern. A tube inserted in the right side of the chest drained 800 mL of milky fluid. A trial of thoracic duct embolization failed, and the patient continued to have 300 to 500 mL of chylous drainage daily. She remained dyspneic and hypoxemic. In the setting of a chylous effusion, increased interstitial markings, and airflow obstruction, the attending surgeon considered a diagnosis of lymphangioleiomyomatosis. A high-resolution CT scan showed diffuse cystic disease in both lung fields consistent with lymphangioleiomyomatosis (Fig 1). Talc pleurodesis was performed successfully. The estrogen supplement that she had been receiving was discontinued and progesterone therapy was initiated. After 1 year’s follow-up, the patient has stable lung function and no recurrence of the chylothorax. Discussion. Pulmonary lymphangioleiomyomatosis is a rare, idiopathic disease that most often affects women of reproductive age. The average age at presentation is 33 years.1 Postmenopausal presentation, as in this case, is rare 622 Brief communications The Journal of Thoracic and Cardiovascular Surgery March 2000