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Chest Imaging

Lymphangiomyomatosis

(LAM), a rare disease characterized by progressive proliferation of spindle cells, resembling immature smooth muscle, in the lung parenchyma and along lymphatic vessels in the chest and abdomen. Proliferation of spindle cells along the bronchioles leads to air trapping and the development of thin-walled cysts. Rupture of these cysts can result in pneumothorax. The spindle cell proliferation can also involve the hilar, mediastinal and extrathoracic lymph nodes, sometimes resulting in dilatation of intrapulmonary lymphatics. Involvement of the lymphatics can lead to chylous pleural effusion (see chylothorax).

Lymphangiomyomatosis occurs only in women, usually of child-bearing age. The majority present with dyspnoea. Sixty per cent develop chylous pleural effusions, and 40% develop pneumothorax. Identical clinical, radiological and pathological pulmonary changes may be seen in about 1% of patients with tuberous sclerosis. Although tuberous sclerosis affects both sexes equally, the pulmonary changes have been described almost exclusively in women. Almost all patients die within 10 years of the onset of symptoms. Recently, improved prognosis has been reported following treatment with progesterone or oophorectomy.

The plain radiological manifestations of LAM include reticular, reticulonodular, miliary and honeycomb patterns. Lung volumes can be increased in patients with this disease. On HRCT, patients with LAM characteristically show numerous thin-walled lung cysts, surrounded by relatively normal lung parenchyma (Fig.1). These cysts usually range from 2 mm to 5 cm in diameter, but can be larger. Their size tends to increase with progression of the disease. In the majority of patients the cysts are distributed diffusely throughout the lungs, and no lung zone is spared; diffuse lung involvement is seen even in patients with mild disease. In some cases, a slight increase in linear interstitial markings, interlobular septal thickening, or patchy areas of ground glass opacity are also seen. Other features of LAM that can be seen on CT include hilar, mediastinal, and retrocrural adenopathy. Pleural effusion can also be seen, and can be helpful in distinguishing LAM from Langerhans cell histiocytosis.

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Fig.1

HRCT scan in a patient with lymphangiomyomatosis (LAM) showing multiple rounded, thin-walled lung cysts.
Lymphangiomyomatosis, Fig.1