Hypoplasia and Immaturity of the Terminal Lung Unit (Acinus) in Congenital Diaphragmatic Hernia

Abstract

We assessed the maturity of the lungs and the radial count in 10 infants with congenital diaphragmatic hernia who died in the immediate perinatal period. The lungs were all immature, with a histologic appearance of less than stated gestational age. The ipsilateral lungs were less mature in appearance than were the contralateral ones. The radial count, an assessment of acinar complexity, was reduced in all cases, and once again the ipsilateral lung was more affected. We present evidence that, in 4 of 6 lungs, volumes were more reduced than predicted from the radial count, and this may be due to loss of (airway) units. We have discussed the controversy about the appearance of the acinus in diaphragmatic hernia and present reasons to explain this. Hyaline membrane disease often occurs in diaphragmatic hernia, even at full term. Intra-alveolar hemorrhage is a common complication of diaphragmatic hernia.

Antenatal Glucocorticoid Corrects Pulmonary Immaturity in Experimentally Induced Congenital Diaphragmatic Hernia in Rats

“…Nitrofen Fed respond to glucocorticoid because their lungs reflect the biochemical and morphologic patterns characteristic of prematurity (9,10,13) and fall within the well-known window of responsiveness to glucocorticoid therapy. Although glucocorticoid therapy in this study inhibited both somatic and pulmonary growth, long-term follow-up in other studies of both animals (43) and human beings (44.…”

“…George 1.f nl. (13) documented that lungs from 10 neonates dying of CDH (seven of them full term) were morphologically immature for their gestational age.…”

Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies

“…1 The combination of pathophysiologic derangements associated with CDH, including lung hypoplasia, lung dysmaturity and pulmonary hypertension (PPHN), requires a multidisciplinary approach to care. 2,3 The complex nature of clinical management and the relatively infrequent presentation of infants with this rare malformation to single centers has lead to difficulty identifying consistent strategies for CDH management. A recent report from the Canadian Neonatal Research Network indicates better than predicted survival in Network centers, with significant improvement in the higher mortality odds strata, and 90% survival in the three centers that cared for at least 12 infants over a 22-month period.…”

Impaired VEGF and nitric oxide signaling after nitrofen exposure in rat fetal lung explants

“…Despite many advances in neonatal care, CDH often remains refractory to diverse medical interventions, such as inhaled nitric oxide (NO) therapy, and persists as a common indication for extracorporeal membrane oxygenation therapy (1). Although many factors contribute to survival of infants with CDH, the overall severity of CDH is largely due to an underlying disruption of normal lung growth and development, which leads to striking decreases in airway branches, alveolarization, and vascular growth and development (20,40). Recent studies have suggested a potential genetic basis for CDH in some cases, but mechanisms that contribute to impaired lung growth in CDH remain poorly understood (4,5).…”