Hyaline membrane disease (HMD): the role of the perinatal pathologist

Abstract

Hyaline membrane disease (HMD), the pathologic correlate of respiratory distress syndrome (RDS) of the newborn, is an acute lung disease of premature infant caused by inadequate amounts of surfactant. Decreased surfactant results in insufficient surface tension in the alveolus during expiration, leading to atelectasis, decreased gas exchange, severe hypoxia and acidosis. HMD predominantly occurs in infants younger than 32 weeks of gestation and weighing less than 1,200 g. In the interpretation of perinatal lung pathology, it is necessary to consider the development of the immature lung, particulary in the third trimester. Microscopically HMD is characterized by the occurrence of dilated terminal and respiratory bronchioles and of alveolar ducts lined by acellular eosinophilic hyaline membranes. The membranes are composed of necrotic alveolar lining cells, amniotic fluid constituents and fibrin. Retinopathy of prematurity and bronchopulmonary dysplasia are late complications of RDS that usually occur in infants who weigh less than 1,500 g and were maintained on a mechanical respiration more than 6 days. Here a pratical approach to a microscopic analysis of the lung in newborns died with the clinical setting of RDS is presented. The most important pathological findings for a complete clinical pathological diagnosis are: the evaluation of the architectural lung development; the endothelial cell lesions; the interstitial edema; the occurrence of disseminated intravascular coagulation; the presence of associated inflammatory lesions. The usefulness of some immunohistochemical stains is also underlined, including anti-surfactant, anti-smooth muscle actin and anti-CD31 to better evaluate surfactant production, pulmonary artery maturation and endothelial cell damage, respectively. Finally, the prevalent role of endothelial dysfunction and endothelial barrier loss is underlined, representing a major pathological event in the deposition of HMD. Proceedings of the International Course on Perinatal Pathology (part of the 10th International Workshop on Neonatology · October 22nd-25th, 2014) · Cagliari (Italy) · October 25th, 2014 · The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken