LAUSR

At Gifu University School of Medicine, we identified four cases of generalized lymphatic anomaly (GLA) and three cases of kaposiform lymphangiomatosis (KLA) treated between October 2004 and September 2016. The differentiation of GLA from KLA was done on pathology. All patients were examined using multidetector-row computed tomography (CT), and those patients who had chest CT abnormalities were further examined on magnetic resonance imaging (MRI). An experienced radiologist, blinded to patient information, reviewed all imaging. CT was evaluated for the presence of pleural effusion, pericardial effusion, pulmonary nodules, ground-glass opacity, and thickening of interlobular septa. Thickening of bronchovascular bundles, mediastinal soft-tissue proliferation (STP), and extrapleural STP were assessed on both CT and MRI. Signal intensity (SI) on phase-shift imaging (PSI) was measured in regions of interest (Fig. 1e,f), and SI index (SII) was calculated using the formula [(SI on in-phase imaging SI on opposed-phase imaging)/(SI on in-phase imaging)] 9 100%. The presence of fatty deposition was defined as SII >10%. The chest symptoms were also reviewed. Of the seven patients, one (25%) with GLA (male; age, 7 years) and three (100%) with KLA (all male; mean age, 11 years; range, 7–18 years) had chest CT abnormalities (Table S1). Pleural effusion was seen bilaterally in one patient (25%) with GLA and unilaterally in two (67%) with KLA. In the KLA group, pericardial effusion was observed in one patient (33%), pulmonary nodules in one (33%), ground-glass opacity in two (67%), and thickening of interlobular septa in three (100%). In the three patients (100%) with KLA, thickening of bronchovascular bundles and mediastinal and extrapleural STP was observed with T2 hyperintensity and diffuse enhancement (Fig. 1) (Table S2). In the GLA group, extrapleural STP was observed in one patient (25%), but pericardial effusion, pulmonary nodules, ground-glass opacity, thickening of bronchovascular bundles and interlobular septa, and mediastinal STP were not seen in any of the GLA patients. In the three patients with KLA, SII of mediastinal STP ranged from 15.0% to 37.1% (mean, 26.0%) and that of extrapleural STP ranged from 34.5% to 50.8% (mean, 40.0%). In one patient with GLA, SII of extrapleural STP was 16.0%. Chest pain, cough, and dyspnea were seen in one patient (33%) with KLA, but were not observed in any patient with GLA.