DOI: 10.1148/rg.254055019
Lung Disease in Premature Neonates: Radiologic-Pathologic Correlation1
Geoffrey A. Agrons, MD,
Sherry E. Courtney, MD,
J. Thomas Stocker, COL, MC, USA and
Richard I. Markowitz, MD
1 From the Department of Radiologic Pathology, Armed Forces Institute of Pathology, 6825 16th St NW, Washington, DC 20306 (G.A.A.); Department of Radiology, Pennsylvania Hospital, Philadelphia, Pa (G.A.A.); Division of Neonatology, Schneider Children’s Hospital, North Shore Long Island Jewish Health System, New Hyde Park, NY (S.E.C.); Department of Pathology, Uniformed Services University of the Health Sciences, Bethesda, Md (J.T.S.); and Department of Radiology, Children’s Hospital of Philadelphia, Pa (R.I.M.). Received February 4, 2005; accepted March 7. S.E.C. participated in a speakers’ bureau for iNO Therapeutics, Clinton, NJ, and was a sponsored speaker for Viasys, Yorba Linda, Calif; all remaining authors have no financial relationships to disclose.
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Figure 1. Normal development of the major airways. Schematic shbows induction of dichotomous branching of the lung bud (arrow) by contact with primitive mesenchyme (arrowhead). (Illustration by Aletta Ann Frazier, MD, Department of Radiologic Pathology, Armed Forces Institute of Pathology.)
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Figure 2. Embryonic phase of normal respiratory tract development. Photomicrograph (original magnification, x100; hematoxylineosin [H-E] stain) of the developing lungs of a 4-mm embryo at approximately 30 days gestation shows the primary bronchial buds (arrows) surrounded by primitive mesenchyme.
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Figure 3. Immature lung in the pseudoglandular phase. Photomicrograph (original magnification, x15; H-E stain) of the lungs at 13 weeks gestation shows blind-ending terminal bronchioles (arrows) surrounded by immature lung parenchyma, which has begun to organize into lobules and clusters of primitive acini (arrowheads).
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Figure 4. Immature lung in the canalicular (acinar) phase. Photomicrograph (original magnification, x425; H-E stain) of the lungs shows blood-filled capillaries (arrow) that lie immediately beneath the surface of alveolar duct structures lined by cuboidal epithelium (early type II pneumocytes (arrowhead). (Reprinted, with permission, from reference 6.)
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Figure 5. Saccular phase of lung development. Photomicrograph (original magnification, x350; H-E stain) of the lungs reveals saccules subdivided by secondary crests (arrows) composed of thinning type I cells immediately adjacent to capillary beds.
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Figure 6. Alveolar phase of lung development. Photomicrograph (original magnification, x25; H-E stain) of the normal lung at 38 weeks gestation shows mature alveolar ducts and alveolar saccules with delicate septa, resulting in a thin air-blood barrier.
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Figure 7. Endogenous surfactant delivery. Diagram of a type II pneumocyte demonstrates migration of lamellar bodies (arrow) from the nucleus to the apical cell surface, where surfactant (in pink) is released into the alveolus by exocytosis. (Diagram by Aletta Ann Frazier, MD, Department of Radiologic Pathology, Amred Forces Institute of Pathology.)
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Figure 8. Clinical features of neonatal respiratory distress. Drawing depicts a representative preterm newborn with RDS exhibiting substernal and intercostal retractions, nasal flaring, and circumoral cyanosis. (Illustration by Aletta Ann Frazier, MD, Department of Radiologic Pathology, Armed Forces Institute of Pathology.)
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Figure 9. Surfactant deficiency in a premature infant. Histologic features. Photomicrograph (original magnification, x75; H-E stain) shows collapsed acini surrounding dilated alveolar ducts lined by smooth homogeneous hyaline membranes (arrows).
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Figure 10. Surfactant deficiency in a premature infant. Gross pathologic features. Photograph of an autopsy specimen demonstrates small atelectatic lungs with focal hemorrhage (arrow) visible on the pleural surface.
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Figure 11. Mild RDS. Magnified radiograph of the right lung of a preterm neonate shows effacement of vascular definition by diffuse reticulogranular opacities. Peripheral air bronchograms (arrows) are visible at the medial lung base. The minor fissure (arrowheads) is slightly thickened.
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Figure 12a. Symmetric surfactant effect in a 36-week-gestationalage infant of a diabetic mother. (a) Pretreatment radiograph shows diminished lung expansion, diffuse bilateral reticulogranular opacities, and air bronchograms, findings consistent with severe RDS. (b) Repeat radiograph, obtained 6 hours after endotracheal administration of one dose of surfactant, reveals marked improvement in lung aeration and vascular definition.
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Figure 12b. Symmetric surfactant effect in a 36-week-gestationalage infant of a diabetic mother. (a) Pretreatment radiograph shows diminished lung expansion, diffuse bilateral reticulogranular opacities, and air bronchograms, findings consistent with severe RDS. (b) Repeat radiograph, obtained 6 hours after endotracheal administration of one dose of surfactant, reveals marked improvement in lung aeration and vascular definition.
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Figure 13. Asymmetric surfactant effect in a 2-day-old, 32-week-gestational age newborn with RDS. Frontal chest radiograph demonstrates multifocal residual consolidations that mimic pneumonia or meconium aspiration syndrome.
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Figure 14. Asymmetric distribution of endotracheal surfactant into the right mainstem bronchus in a 1-day-old preterm neonate with RDS. Frontal radiograph of the chest shows a clear hyperexpanded right lung, shift of mediastinal structures to the left, and persistence of diffuse left lung opacification.
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Figure 15a. Pulmonary hemorrhage in a 26-week-gestationalage neonate following prophylactic surfactant therapy. (a) Frontal chest radiograph obtained after one dose of surfactant and during treatment with nasal CPAP shows hyperinflated lungs with faint symmetric residual opacities. (b) Repeat radiograph, obtained after 24 hours for evaluation of sudden respiratory decompensation and bloody endotracheal aspirates, shows dense bilateral airspace consolidation.
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Figure 15b. Pulmonary hemorrhage in a 26-week-gestationalage neonate following prophylactic surfactant therapy. (a) Frontal chest radiograph obtained after one dose of surfactant and during treatment with nasal CPAP shows hyperinflated lungs with faint symmetric residual opacities. (b) Repeat radiograph, obtained after 24 hours for evaluation of sudden respiratory decompensation and bloody endotracheal aspirates, shows dense bilateral airspace consolidation.
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Figure 16. Immature lungs in a profoundly premature neonate delivered at a gestational age of 22 weeks and 6 days. Anteroposterior chest radiograph obtained on the first postnatal day shows diffuse fine interstitial thickening.
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Figure 17a. Immature lungs in a 24-week-gestational-age neonate with a birth weight of 530 g. (a) Anteroposterior radiograph obtained on the second postnatal day shows clear lungs. (b) Repeat radiograph, obtained at 48 days, shows coarse parenchymal opacities with no regions of disordered aeration or cystic lucency, findings consistent with uniform fibrosis in "new" BPD.
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Figure 17b. Immature lungs in a 24-week-gestational-age neonate with a birth weight of 530 g. (a) Anteroposterior radiograph obtained on the second postnatal day shows clear lungs. (b) Repeat radiograph, obtained at 48 days, shows coarse parenchymal opacities with no regions of disordered aeration or cystic lucency, findings consistent with uniform fibrosis in "new" BPD.
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Figure 18. Proposed model for the pathogenesis of long-standing healed BPD. Schematic 1 represents three normal acini (a, b, c), each supplied by a terminal bronchiole. As shown in schematic 2, during positive-pressure ventilation and oxygen delivery for treatment of surfactant deficiency, hyaline membranes or occlusive debris from necrotizing bronchiolitis (arrow) "protects" acinus a from alveolar septal injury, whereas varying degrees of partial occlusion of the bronchioles to acini b and c permit alveolar septal necrosis from barotrauma-volutrauma and oxygen toxicity. Schematic 3 depicts the phase following resolution of bronchiolar obstruction, in which acinus a hyper-expands and continues to develop new alveoli, acinus b has undergone septal fibrosis and is inhibited from further alveolar development, and acinus c has atrophied. (Illustration by Aletta Ann Frazier, MD, Department of Radiologic Pathology, Armed Forces Institute of Pathology.) (Adapted, with permission, from reference 85.)
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Figure 19a. Long-standing "healed" BPD, pathologic features. (a) Photomicrograph (original magnification, x30; H-E stain) demonstrates diffuse alveolar septal fibrosis within an acinus (bottom) adjacent to another acinus (top) with hyperexpanded but otherwise normal alveoli. (b) Photomicrograph (original magnification, x100; H-E stain) depicts a continuous band of hyperplastic smooth muscle (arrow) surrounding a bronchiole. (c) Photograph of the pleural surface of a lung at 1 month of age shows alternating populations of hyperexpanded and collapsed acini de-fined by irregular pseudofissures (arrows).
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Figure 19b. Long-standing "healed" BPD, pathologic features. (a) Photomicrograph (original magnification, x30; H-E stain) demonstrates diffuse alveolar septal fibrosis within an acinus (bottom) adjacent to another acinus (top) with hyperexpanded but otherwise normal alveoli. (b) Photomicrograph (original magnification, x100; H-E stain) depicts a continuous band of hyperplastic smooth muscle (arrow) surrounding a bronchiole. (c) Photograph of the pleural surface of a lung at 1 month of age shows alternating populations of hyperexpanded and collapsed acini de-fined by irregular pseudofissures (arrows).
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Figure 19c. Long-standing "healed" BPD, pathologic features. (a) Photomicrograph (original magnification, x30; H-E stain) demonstrates diffuse alveolar septal fibrosis within an acinus (bottom) adjacent to another acinus (top) with hyperexpanded but otherwise normal alveoli. (b) Photomicrograph (original magnification, x100; H-E stain) depicts a continuous band of hyperplastic smooth muscle (arrow) surrounding a bronchiole. (c) Photograph of the pleural surface of a lung at 1 month of age shows alternating populations of hyperexpanded and collapsed acini de-fined by irregular pseudofissures (arrows).
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Figure 20. New BPD in very low-birth-weight infants. (Reprinted, with permission, from reference 9.)
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Figure 21. Arrested acinar development in post-surfactant BPD. Schematics of a trio of acini show oversimplified anatomy due to alveolar paucity with either thin alveolar septa (center) or uniform mild fibrosis (right), compared with normal alveolar growth and development (left). (Illustration by Aletta Ann Frazier, MD, Department of Radiologic Pathology, Armed Forces Institute of Pathology.) (Adapted, with permission, from reference 85.)
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Figure 22. BPD in a surfactant-treated infant. Photomicrograph (original magnification, x50; H-E stain) shows expanded, simplified alveolar ducts, saccules, and alveoli with little or no alveolar septal fibrosis.
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Figure 23a. "Classic" severe BPD in a 3-month-old premature infant. (a) Frontal radiograph shows heterogeneous aeration, coarse strandlike areas of opacity, and intervening cystic lucencies. (b) Axial CT scan demonstrates right upper lobe regional air trapping anteriorly, architectural distortion with fibrotic subpleural parenchymal bands (arrows) and subsegmental atelectasis posteriorly, and diffuse coarse reticular opacities in the left lung.
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Figure 23b. "Classic" severe BPD in a 3-month-old premature infant. (a) Frontal radiograph shows heterogeneous aeration, coarse strandlike areas of opacity, and intervening cystic lucencies. (b) Axial CT scan demonstrates right upper lobe regional air trapping anteriorly, architectural distortion with fibrotic subpleural parenchymal bands (arrows) and subsegmental atelectasis posteriorly, and diffuse coarse reticular opacities in the left lung.
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Figure 24. BPD in a 33-day-old preterm infant. Frontal chest radiograph demonstrates uniform distribution of reticular opacities and small cystic lucencies.
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Figure 25a. Acute PIE. (a) Photograph of a sectioned lung specimen shows linear (arrow) and irregular (arrowhead) collections of interstitial gas adjacent to compressed bronchovascular bundles. (Reprinted, with permission, from reference 6.) (b) Cut specimen shows that following centrifugal migration of interstitial gas, subpleural blebs (arrow) collect within interlobular septa.
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Figure 25b. Acute PIE. (a) Photograph of a sectioned lung specimen shows linear (arrow) and irregular (arrowhead) collections of interstitial gas adjacent to compressed bronchovascular bundles. (Reprinted, with permission, from reference 6.) (b) Cut specimen shows that following centrifugal migration of interstitial gas, subpleural blebs (arrow) collect within interlobular septa.
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Figure 26. Pneumomediastinum, gross pathologic features. Photograph of an autopsy specimen with the lungs removed shows multiple collections of gas (arrow) within loose mediastinal connective tissue.
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Figure 27. Unilateral acute PIE in a premature infant who underwent positive-pressure ventilation for RDS. Frontal radiograph shows a profusion of irregular cystic lucencies within the left lung. Granular consolidation of the right lung is consistent with uncomplicated RDS.
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Figure 28. "Pseudocyst" in a premature infant who underwent mechanical ventilation. Frontal chest radiograph shows a large, rounded, right juxtahilar gas collection with a smooth thin wall (arrowheads).
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Figure 29a. Persistent PIE, pathologic features. (a) High-power photomicrograph (original magnification, x300; H-E stain) shows a cyst wall (arrowheads) composed of fibrotic connective tissue lined by multinucleated foreign body giant cells (arrow). (Reprinted, with permission, from reference 6.) (b) Photograph of the sectioned surgical specimen shows the lung parenchyma compressed by multiple intercommunicating air-filled cysts delimited by smooth membranes.
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Figure 29b. Persistent PIE, pathologic features. (a) High-power photomicrograph (original magnification, x300; H-E stain) shows a cyst wall (arrowheads) composed of fibrotic connective tissue lined by multinucleated foreign body giant cells (arrow). (Reprinted, with permission, from reference 6.) (b) Photograph of the sectioned surgical specimen shows the lung parenchyma compressed by multiple intercommunicating air-filled cysts delimited by smooth membranes.
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Figure 30a. Diffuse persistent PIE in a 3-week-old girl with a history of positive-pressure mechanical ventilation. (a) Frontal chest radiograph shows overexpansion of most of the left lung by multiple cystic lucencies, producing contralateral mediastinal displacement and left retrocardiac compressive atelectasis. (b) Axial CT scan shows multiple collections of interstitial gas in the left upper lobe that surround lines (arrows) and dots (arrowheads) of soft-tissue attenuation. (c) Photograph of the resected left upper lobe shows the surface of the lung blistered by multiple subpleural cysts (arrows).
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Figure 30b. Diffuse persistent PIE in a 3-week-old girl with a history of positive-pressure mechanical ventilation. (a) Frontal chest radiograph shows overexpansion of most of the left lung by multiple cystic lucencies, producing contralateral mediastinal displacement and left retrocardiac compressive atelectasis. (b) Axial CT scan shows multiple collections of interstitial gas in the left upper lobe that surround lines (arrows) and dots (arrowheads) of soft-tissue attenuation. (c) Photograph of the resected left upper lobe shows the surface of the lung blistered by multiple subpleural cysts (arrows).
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Figure 30c. Diffuse persistent PIE in a 3-week-old girl with a history of positive-pressure mechanical ventilation. (a) Frontal chest radiograph shows overexpansion of most of the left lung by multiple cystic lucencies, producing contralateral mediastinal displacement and left retrocardiac compressive atelectasis. (b) Axial CT scan shows multiple collections of interstitial gas in the left upper lobe that surround lines (arrows) and dots (arrowheads) of soft-tissue attenuation. (c) Photograph of the resected left upper lobe shows the surface of the lung blistered by multiple subpleural cysts (arrows).
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Figure 31a. Localized persistent PIE in a 1-year-old boy delivered at 27 weeks gestational age. (a) Frontal chest radiograph shows a circumscribed lucent lesion at the medial right lung base with a smooth, lobulated, thin wall (arrows). (b) Axial CT scan shows an aggregate of thin-walled, gas-containing cysts in the right lower lobe that contains lines (arrows) and dots (arrowheads) of soft-tissue attenuation. (c) Photograph of the cut surface of the resected specimen shows a broncho-vascular bundle (arrows) surrounded by gas within a multiloculated cystic mass.
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Figure 31b. Localized persistent PIE in a 1-year-old boy delivered at 27 weeks gestational age. (a) Frontal chest radiograph shows a circumscribed lucent lesion at the medial right lung base with a smooth, lobulated, thin wall (arrows). (b) Axial CT scan shows an aggregate of thin-walled, gas-containing cysts in the right lower lobe that contains lines (arrows) and dots (arrowheads) of soft-tissue attenuation. (c) Photograph of the cut surface of the resected specimen shows a broncho-vascular bundle (arrows) surrounded by gas within a multiloculated cystic mass.
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Figure 31c. Localized persistent PIE in a 1-year-old boy delivered at 27 weeks gestational age. (a) Frontal chest radiograph shows a circumscribed lucent lesion at the medial right lung base with a smooth, lobulated, thin wall (arrows). (b) Axial CT scan shows an aggregate of thin-walled, gas-containing cysts in the right lower lobe that contains lines (arrows) and dots (arrowheads) of soft-tissue attenuation. (c) Photograph of the cut surface of the resected specimen shows a broncho-vascular bundle (arrows) surrounded by gas within a multiloculated cystic mass.
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Figure 32a. Bilateral pneumothoraces. (a) Anteroposterior chest radiograph shows juxtamediastinal lucencies and bisagittal compression of the lobes of the thymus (arrows), producing a "figure 8" configuration contour. A "deep sulcus sign" (arrowhead) is seen in the right lung. (b) On a radiograph obtained after spontaneous resolution of air leak, the mediastinal contour appears normal.
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Figure 32b. Bilateral pneumothoraces. (a) Anteroposterior chest radiograph shows juxtamediastinal lucencies and bisagittal compression of the lobes of the thymus (arrows), producing a "figure 8" configuration contour. A "deep sulcus sign" (arrowhead) is seen in the right lung. (b) On a radiograph obtained after spontaneous resolution of air leak, the mediastinal contour appears normal.
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Figure 33. Bilateral pneumothoraces. Frontal chest radiograph obtained with the infant rotated toward the right reveals the anterior junction line (arrowheads) outlined by pleural gas. Both diaphragm leaflets and the right aspect of the cardiothymic silhouette are abnormally well defined.
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Figure 34. Pneumomediastinum. Frontal chest radiograph shows the lobes of the thymus (arrows), which are displaced superolaterally by a large central lucent area.
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Figure 35. Extensive air leak in a premature neonate who received positive-pressure assisted ventilation for treatment of RDS. Frontal chest radiograph demonstrates a thin pericardial membrane (straight white arrows), which is defined medially by intrapericardial gas and laterally by pleural or mediastinal gas. Extrapleural gas outlines the medial aspect of the left diaphragm (black arrows). Bilateral pneumothoraces produce deep sulcus signs (curved arrows). Mediastinal gas tracks into the cervical soft tissues and right lateral chest wall (arrowheads). The tip of the endotracheal tube enters the right mainstem bronchus.
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Figure 36. Systemic air embolism in the setting of diffuse bilateral PIE. Frontal radiograph of the chest and abdomen shows elevation and compression of the base of the heart (white arrowheads) by tension pneumopericardium. The cardiac chambers are filled with gas, and intraluminal gas is demonstrated in the inferior vena cava (straight arrow), hepatic veins (black arrowhead), and abdominal aorta surrounding the tip of the umbilical artery catheter (curved arrow). Both lungs are overexpanded by innumerable cystic lucencies representing PIE. (Courtesy of Gael J. Lonergan, MD, Austin Radiological Association, Austin, Tex.)
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Copyright © 2005 by the Radiological Society of North America.
