The fetal lung 2: pulmonary hypoplasia

Laudy, J. A. M.; Wladimiroff, J. W.

doi:10.1046/j.1469-0705.2000.00252.x

Cited by 139 publications

(109 citation statements)

References 133 publications

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“…Pulmonary hypoplasia occurs in 9-11 per 10 000 live births 1 . Most cases of pulmonary hypoplasia occur secondary to conditions such as congenital diaphragmatic hernia, oligohydramnios or skeletal deformities.…”

Section: Discussionmentioning

confidence: 99%

“…Various modalities have been proposed to assess the presence of pulmonary hypoplasia in utero, including biometric indices and fetal breathing movements 1 . Sokol et al found that branch pulmonary artery diameter measured by fetal echocardiography correlated strongly with lung weight, and that for diaphragmatic hernia correlated with postnatal respiratory morbidity 19 .…”

Section: Discussionmentioning

confidence: 99%

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Prenatal diagnosis of primary pulmonary hypoplasia in fraternal twins

Chen

Ursell

Adatia

et al. 2009

Ultrasound in Obstet & Gyne

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CASE REPORTA 38-year-old woman, gravida 2 para 1, was referred for fetal echocardiography for evaluation of abnormal cardiac axes in a set of twins. The woman had previously delivered a term male infant who died at 1 month of age from severe respiratory distress despite aggressive mechanical support. Autopsy revealed bilateral lung hypoplasia with arrested acinar development, small pulmonary arteries and a radial alveolar count of four. The couple was not consanguineous.The mother presented to our program at 18 weeks' gestation with dizygotic twins (female and male), products of in-vitro fertilization. General fetal ultrasound examination revealed a right-sided subependymal hemorrhage in the female, and mildly decreased thoracic circumference and normal amniotic fluid levels in both twins. Fetal echocardiography in both demonstrated normal cardiac anatomy and function. Both had a leftward cardiac axis (female, 90 • ; male, 80 • ) and an increased cardiothoracic diameter ratio (female, 60%; male, 50%), and the female had a small pericardial effusion (Figure 1). Both fetuses had hypoplastic branch pulmonary arteries (0.9-1.2 mm in female and 1.3-1.5 mm in male vs. 1.8 mm expected). Flow could not be demonstrated in the pulmonary arteries or pulmonary veins by color or pulsed Doppler imaging in either twin. The findings of hypoplastic branch pulmonary arteries with decreased flow and increased cardiothoracic diameter ratios suggested pulmonary hypoplasia. The parents requested elective termination of the pregnancy.The twins were delivered at 19 weeks' gestation, weighing 169 g (male) and 127 g (female). In both, the diaphragms were intact and there were no cardiac malformations, skeletal dysplasia or renal malformations. The lungs were normally configured but extremely small. In the male, the lungs weighed 1.6 g (expected 5.6 g). In the female, the lungs weighed 1.4 g. Both had markedly hypoplastic extrapulmonary branch pulmonary arteries.The lungs of each fetus showed similar histopathology: there was a lobular architecture with thickened connective tissue septa and prominent lymphatics. In contrast to cuboidal epithelium expected at this developmental stage, the distal sac epithelium of the twins appeared intermediate between columnar and cuboidal epithelium, with low columnar epithelium containing dark nuclei. There was a striking paucity of terminal airspaces within each lobule, and the mesenchymal interstitium was thicker than normal. Peripheral airways were larger than those observed in controls. The findings suggested persistent pseudoglandular stage. The cartilaginous airways were found closer to the pleura than normal, suggesting failure

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis of primary pulmonary hypoplasia in fraternal twins

Chen

Ursell

Adatia

et al. 2009

Ultrasound in Obstet & Gyne

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“…Tracheal obstruction in animal models has been shown to accelerate fetal lung growth (Alcorn and Mendelson, 1993;Piedboeuf et al, 1997). Conditions that are associated with lung liquid drainage are characterized by pulmonary hypoplasia and an excess of type II cells (Flecknoe et al, 2003;Laudy and Wladimiroff, 2000). Hypoplasia is reversible with tracheal ligation, where presumed accumulation of lung fluid induces rapid lung growth and changes in alveolar structure (Flecknoe et al, 2000).…”

Section: Discussionmentioning

confidence: 99%

Differentiation of xenografted human fetal lung parenchyma

Pavlović

Floros

Phelps

et al. 2008

Early Human Development

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The goal of this study was to characterize xenografted human fetal lung tissue with respect to developmental stage-specific cytodifferentiation. Human fetal lung tissue (pseudoglandular stage) was grafted either beneath the renal capsule or the skin of athymic mice (NCr-nu). Tissues were analyzed from 3 to 42 days post-engraftment for morphological alterations by light and electron microscopy (EM), and for surfactant protein mRNA and protein by reverse transcription-polymerase chain reaction (RT-PCR) and immunocytochemistry (ICC), respectively. The changes observed resemble those seen in human lung development in utero in many respects, including the differentiation of epithelium to the saccular stage. Each stage occurred over approximately one week in the graft in contrast to the eight weeks of normal in utero development. At all time points examined, all four surfactant proteins (SP-A, SP-B, SP-C, and SP-D) were detected in the epithelium by ICC. Lamellar bodies were first identified by EM in 14 day xenografts. By day 21, a significant increase in lamellar body expression was observed. Cellular proliferation, as marked by PCNA ICC and elastic fiber deposition resembled those of canalicular and saccular in utero development. This model in which xenografted lung tissue in different stages of development is available may facilitate the study of human fetal lung development and the impact of various pharmacological agents on this process.

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“…It can be unilateral or bilateral and it usually leads to severe respiratory distress immediately after birth, while it represents a common cause of perinatal mortality [1].…”

Section: Introductionmentioning

confidence: 99%

Unilateral pulmonary hypoplasia in an adult patient

Papadopoulos

Misthos

Chorti

et al. 2018

Monaldi Arch Chest Dis

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Pulmonary hypoplasia (PH) is a developmental anomaly of the lung parenchyma, characterized by a decrease in the number and size of airways, alveoli and vessels. We present a case of a 31-year-old patient with a history of chronic productive cough and frequent respiratory infections, who was referred for investigation of abnormal chest x-ray. The combination of chest computed tomography (CT) and bronchoscopy set the diagnosis of left pulmonary hypoplasia and the patient was treated surgically with a left pneumonectomy. PH is usually diagnosed immediately after birth, causing severe respiratory failure with high mortality. The less severe, unilateral forms can possibly survive by causing compensatory hyperinflation of the other lung and remain undiagnosed until adulthood, presenting either asymptomatic or with symptoms of chronic bronchitis and recurrent respiratory infections. Chest CT is considered the imaging technique of choice for the diagnosis and for the differential diagnosis from other congenital or acquired conditions. The treatment is usually conservative, although surgical resection is indicated in cases of severe cystic changes and intense symptomatology.

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The fetal lung 2: pulmonary hypoplasia

Cited by 139 publications

References 133 publications

Prenatal diagnosis of primary pulmonary hypoplasia in fraternal twins

Prenatal diagnosis of primary pulmonary hypoplasia in fraternal twins

Differentiation of xenografted human fetal lung parenchyma

Unilateral pulmonary hypoplasia in an adult patient

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