대한흉부영상의학회 Weekly Chest Cases

Case No : 40 Date 1998-08-03

Courtesy of Jae-Woo Song, M.D., Jung-Gi Im, M.D. / Boramae Hospital, Seoul National University Hospital
Age/Sex 34 / F
Chief Complaintmild dyspnea for several months, history of pneumothorax (+)

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CT1

Diagnosis With Brief Discussion

Diagnosis: Lymphangioleiomyomatosis (LAM)
Radiologic Findings: LAM is a rare disease characterized by the progressive proliferation of spindle cells, resembling immature smooth muscle, in the lung parenchyma and along the lymphatic vessels in the chest and abdomen. Proliferation of spindle cells along the bronchioles leads to air-trapping and development of emphysema and thin-walled lung cysts. Rupture of these cysts can result in pneumothorax. Involvement of lymphatic system leads to lymphadenopathy, distention of thoracic duct, and chylous pleural effusion or ascites. Proliferation of spindle cells along the venous wall causes venous obstruction, leading to pulmonary edema, hemorrhage, and hemosiderosis.
LAM occurs only in the women of childbearing age, usually between 17 and 50 years. Identical clinical, radiological, and pathologic pulmonary changes may be seen in about 1% of the patients with tuberous sclerosis. Although tuberous sclerosis affects both sex equally, the pulmonary changes have been described almost exclusively in women.
The plain radiographic findings of LAM include reticular, reticulonodular, miliary, honeycomb patterns, and hyperinflation, may precede, accompany, or postdate other manifestation od the disease such as pneumothorax, and chylous pleural effusion. On HRCT, patients with LAM characteristically show numerous thin-walled lung cysts, surrounded by relatively normal lung parenchyma. The walls of the lung cysts usually range from being faintly perceptible to 4 mm in thickness. In the majority of patients the cysts are distributed diffusely throughout the lungs, and no lung zone is spared.
Brief Review
References
Keywords: Lung, Interstitial lung disease, LAM, ILD,

No. of Applicants : 52

▶ Correct Answer : 51/52, 98.1%

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대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 40 Date 1998-08-03

Discussion

No. of Applicants : 52