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Pulmonary Lymphangioleiomyomatosis: Correlation of Ventilation-Perfusion Scintigraphy, Chest Radiography, and CT with Pulmonary Function Tests¹

¹ From the Departments of Diagnostic Radiology, Warren Grant Magnuson Clinical Center (N.A.A., E.C.J.) and Nuclear Medicine (C.C.C., R.D.N.), the Pulmonary-Critical Care Medicine Branch (S.C.C., J.M.), and the Office of Biostatistics Research, Division of Epidemiology and Clinical Applications (M.W.), National Heart, Lung, and Blood Institute, National Institutes of Health, Bldg 10, Rm 1C-660, 10 Center Dr, MSC 1182, Bethesda, MD 20892-1182. From the 1998 RSNA scientific assembly. Received December 9, 1998; revision requested December 30; final revision received May 20, 1999; accepted June 7. Address reprint requests to N.A.A. (e-mail: navila@nih.gov).

View larger version (128K): [in a new window]   Figure 1a. Lymphangioleiomyomatosis in a 35-year-old woman. (a) Transverse thin-section CT scan and (b) posterior ventilation (left) and perfusion (right) images demonstrate minimal extent of disease visible on the CT scan, which consists of pulmonary cysts (arrows in a) involving less than 30% of the lungs, normal ventilation, minimally abnormal perfusion, and minimal speckling (arrow in b).

View larger version (93K): [in a new window]   Figure 1b. Lymphangioleiomyomatosis in a 35-year-old woman. (a) Transverse thin-section CT scan and (b) posterior ventilation (left) and perfusion (right) images demonstrate minimal extent of disease visible on the CT scan, which consists of pulmonary cysts (arrows in a) involving less than 30% of the lungs, normal ventilation, minimally abnormal perfusion, and minimal speckling (arrow in b).

View larger version (99K): [in a new window]   Figure 2a. Lymphangioleiomyomatosis in a 54-year-old woman. (a) Transverse thin-section CT scan and (b) posterior ventilation (left) and perfusion (right) images show the moderate extent of disease visible on the CT scan, which consists of pulmonary cysts (arrow in a) involving 30%-60% of the lungs; mild ventilatory abnormalities, aside from the defect caused by pleural effusion; moderate perfusion defects (arrow in b); and moderate speckling consisting of focal areas of increased uptake of radionuclide in the lungs.

View larger version (80K): [in a new window]   Figure 2b. Lymphangioleiomyomatosis in a 54-year-old woman. (a) Transverse thin-section CT scan and (b) posterior ventilation (left) and perfusion (right) images show the moderate extent of disease visible on the CT scan, which consists of pulmonary cysts (arrow in a) involving 30%-60% of the lungs; mild ventilatory abnormalities, aside from the defect caused by pleural effusion; moderate perfusion defects (arrow in b); and moderate speckling consisting of focal areas of increased uptake of radionuclide in the lungs.

View larger version (129K): [in a new window]   Figure 3a. Lymphangioleiomyomatosis in a 52-year-old woman. (a) Transverse thin-section CT scan and (b) posterior ventilation (left) and perfusion (right) images show the severe extent of disease visible on the CT scan, which consists of pulmonary cysts (arrow) involving more than 60% of the lungs, severe ventilation and perfusion abnormalities, and marked speckling consisting of multiple areas of increased uptake of radionuclide that extend to the periphery throughout both lungs.

View larger version (67K): [in a new window]   Figure 3b. Lymphangioleiomyomatosis in a 52-year-old woman. (a) Transverse thin-section CT scan and (b) posterior ventilation (left) and perfusion (right) images show the severe extent of disease visible on the CT scan, which consists of pulmonary cysts (arrow) involving more than 60% of the lungs, severe ventilation and perfusion abnormalities, and marked speckling consisting of multiple areas of increased uptake of radionuclide that extend to the periphery throughout both lungs.