4 - Developmental and Pediatric Lung Disease

Abstract

Abstract This chapter provides an overview of the major forms of lung disease affecting neonates, infants, children, and adolescents; where appropriate, it highlights the distinctions from diseases more commonly seen in adults. A standard approach for tissue handling is presented to maximize the diagnostic yield for pediatric lung biopsies and lobectomies. Congenital lung malformations and acquired cystic lesions are discussed, followed by a review of diffuse disorders of lung development, including pulmonary hypoplasia, pulmonary hyperplasia, acinar dysplasia, and congenital alveolar dysplasia. Developmental disorders of the vasculature are also reviewed, including alveolar capillary dysplasia, primary lymphangiectasia, and lymphangiomatosis. Acute and chronic complications of premature birth remain a significant cause of morbidity and mortality in neonates. Pathologic features of hyaline membrane disease, bronchopulmonary dysplasia, and pulmonary interstitial emphysema are summarized. Diffuse lung disease in children includes a variety of patterns of alveolar and interstitial disease including some diseases unique to this age group, such as pulmonary interstitial glycogenosis, neuroendocrine cell hyperplasia of infancy, and the genetic disorders of surfactant metabolism. The differential diagnosis of diffuse lung disease in children also includes entities such as hypersensitivity pneumonitis, eosinophilic pneumonia, aspiration, and obliterative bronchiolitis. These diseases are reviewed briefly and discussed in more detail in other chapters. A practical approach to pathologic diagnosis is emphasized, by way of integration of clinical features, gross pathology, microscopic findings, and use of special studies when necessary.