PMC6679868_01
Patient with multiple cutaneous neurofibromatosis and cafe au lait macules.
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She had multiple both cutaneous neurofibromas and cafe au lait macules and freckling in her back and the axilla (Figure 1).
High resolution CT of the chest shows emphysema of the upper lobe (a) and bilateral mosaic pattern of the lower lobe (b).
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High-resolution computed tomography (HRCT) showed markedly enlarged central pulmonary arteries, upper-lobe centrilobular emphysema with multiple bullae, and bilateral ground-glass nodules with multiple cystic lesions in the lower lobe (Figure 2).
Transthoracic echocardiography shows that the left ventricle was compressed due to the enlargement of the right ventricle during end-diastole in deteriorating pulmonary hypertension, parasternal long-axis view (a), and parasternal short axis view (b).
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Echocardiography showed enlargement of the right ventricle with ventricular septal flattening during systole (Figure 3).