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J Thorac Cardiovasc Surg 2000;119:622-623
© 2000 Mosby, Inc.
BRIEF COMMUNICATIONS |
From the Divisions of Cardio-thoracic Surgery, Department of Surgery,a and Pulmonary and Critical Care Medicine,b University of Pennsylvania School of Medicine, Philadelphia, Pa.
Address for reprints: Maher Deeb, MD, 6 Silverstein, Hospital of the University of Pennsylvania, 3400 Spruce St, Philadelphia, PA 19104 (E-mail: MDEEB1{at}hotmail.com).
Thoracic surgeons are often involved in the care of patients 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.
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1 Postmenopausal presentation, as in this case, is rare and is usually reported in association with exogenous estrogen administration. 2 Dyspnea, occurring in more than 90% of patients, and pneumothorax, reported in more than 80% of patients, are the most common clinical manifestations. 3 Chylothorax is uncommon at presentation (0%-7%) 1-3 but will develop in 28% at some time in the course of the disease. 3 Other symptoms include chest pain, cough, and hemoptysis. 3 Extrapulmonary manifestations may include chylous ascites, abdominal or pelvic adenopathy or masses, and renal angiomyolipomas. 2
Smooth muscle proliferation within the airways, lymphatics, and blood vessels is responsible for the various clinical presentations of lymphangioleiomyomatosis. 2,3 Lymphatic obstruction may produce chylothorax or chyloperitoneum. Involvement of blood vessels may result in interstitial bleeding and hemosiderosis. Smooth muscle proliferation in the airways leads to the development of airflow obstruction, diffuse cystic changes, and frequently pneumothorax.
Pulmonary function testing reveals airflow obstruction with normal or increased lung volumes and a diffusion impairment. 4 Plain chest radiographs usually show abnormalities. The most common findings are a reticular interstitial pattern, cysts or bullae, pneumothorax, or pleural effusion. 4,5 High-resolution CT scans demonstrate a distinctive pattern with discrete thin-walled cysts 3 to 40 mm in diameter, uniformly distributed throughout the parenchyma. 5
In the setting of cystic changes and airflow obstruction, the differential diagnosis includes eosinophilic granuloma and emphysema. If additional findings such as chylous pleural effusion, chylous ascites, or renal angiomyolipomas are present, the CT scan may be considered diagnostic. However, some patients with findings limited to the lung parenchyma may require biopsy. Pathologic findings include diffuse cystic changes and the proliferation of atypical smooth muscle cells involving the air spaces, bronchioles, pleura, vasculature, and lymphatics. 2
These changes may be difficult to detect when tissue is obtained by transbronchial rather than surgical biopsy. Immunohistochemical staining with HMB-45, a monoclonal antibody that binds to lymphangioleiomyomatosis cells, may increase the diagnostic yield in this setting. 4
The natural history of the disease is that of progressive deterioration of pulmonary function. In two recent case series, survival 8.5 years after the onset of symptoms ranged from 38% to 78%. 1,3 The administration of medroxyprogesterone has been associated with improvement or stabilization of the clinical course of the disease. 3
Lung transplantation is an additional therapeutic option for patients with advanced disease.
Given the rarity of lymphangioleiomyomatosis and the observation that clinical symptoms may predate radiographic findings, delays in diagnosis as in our case are common. The clinical sequelae of lymphangioleiomyomatosis often require intervention by thoracic surgeons. Familiarity with this rare disease is therefore essential to assure prompt diagnosis and optimum management.
References
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