PMC3420428_01
Computed tomography (CT) of the thorax. (a) CT of the thorax of patient 1 at the age of 3.5 months. Small cystic, emphysematous transformations ubiquitously involving both lungs and consolidations in the dorsal parts of the lungs were found. (b) CT of the thorax of patient 2 at the age of 4 months. Consolidations in both lungs with some cystic areas, a relatively small left lung, and enlarged central pulmonary arteries with rarefication of the branches in the periphery are shown. Please note the more focal involvement of the lungs compared to patient 1.
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fig1
Computed tomography (CT) of the thorax demonstrated small cystic, emphysematous transformations of both lungs (Figure 1(a)). While the initial chest X-ray after birth was normal, chest radiography and CT of the thorax at the age of 4 months showed consolidations in both lungs with some cystic areas, a relatively small left lung, and enlarged central pulmonary arteries with rarefication of the branches in the periphery (Figure 1(b)).
Lung biopsies of patient 1 (a and b), patient 2 (c and d), patient 3 (e), and control (f). (a) Altered lung architecture with slight emphysema and slight to moderate thickening of alveolar walls due to proliferation of capillaries. Note venous sclerosis ( ) and the capillary duplications in some alveolar walls. Changes reflect pulmonary arterial hypertension (Hematoxylin and Eosin, x100), (b) High-power view of thickened alveolar septa due to proliferation and tortuosity of capillaries. Endothelial cells lining the inner surface of capillaries are marked by CD-34 staining (Immunopoeroxidase, x200), (c) Irregular emphysematous changes and focal capillary proliferation in thickend alveolar walls. Changes are less prominent than in patient 1 (Hematoxylin and Eosin, x100), (d) Capillary proliferation and protrusion of tufts of capillaries is found in some alveolar septa ( ) and in the wall of a vein ( ). Endothelial cells lining the inner surface of capillaries are marked by CD-31 staining (Imminoperoxidase, x200), (e and f) Post mortem lung histology of patient 3 (e) versus control (f). pseudoglandular stage of lung development at 15 weeks of gestational age. Endothelial cells express CD-31. (Immunoperoxidase, x200), (e) Broadened space between pseudoglands with proliferation of capillaries in lung tissue of patient 3. Regressive changes are due to advanced autolysis following intrauterine death, (f) Normal lung architecture at 15 weeks of gestational age.
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fig2
Histological examination of an open lung biopsy performed at the age of 14 weeks showed ectatic alveoli (Figures 2(a) and 2(b)). While the first biopsy showed only minor changes suggestive of PCH, the second biopsy confirmed this diagnosis (Figures 2(c) and 2(d)).  Histological examination of the fetal lung showed abnormal capillaries (Figures 2(e) and 2(f)).
Chest X-ray of patient 2 in anterior-posterior condition, (a) Chest X-ray at the age of 3 years: there are signs of hyperlucent and hyperinflation with cystic-imaging areas ( ) and laterally ascending ribs as a sign of lung inflation. In some areas of the lungs, there are consolidations. The heart silhouette is enlarged and clips are present in typical location after surgical closure of a patent ductus arteriosus, (b) Chest X-ray at the age of 5.5 years: persistent signs of bronchial obstruction. Chronic consolidations are found perihilar at the right side and behind the heart.
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fig3
Except for elective surgery, the girl was never on mechanical ventilation.  Figure 3(a) shows the chest X-ray at the age of three years. A recent X-ray of the thorax is shown in Figure 3(b) demonstrating focal chronic consolidations and bronchial obstruction.