Clinical Picture

Pneumatocoele and conjunctivitis in a newborn

This chapter discusses the transgenerational, antenatal, and early childhood factors that affect lung development across the life course. The consequences of these adverse influences may be direct, or secondary to an intermediate disease state such as prematurity and obesity. Many of these adverse effects cannot be remedied; we cannot stop atopic women bearing children, for example! There are two important take-home messages. The first is that we must identify and focus on what is preventable. This means legislation to protect vulnerable lungs against tobacco and nicotine (cigarettes and e-cigarettes) and traffic pollution; the institution of affordable and practical measures to minimize or eliminate exposure to biomass fuel; and being sure that due weight is given to the longer term consequences of Caesarean section by women and obstetricians when planning delivery. The second is to convince the community, especially adult thoracic physicians, that many lung diseases would disappear if our vulnerable fetuses, babies, and infants were better protected from the consequences of adult behavior.

This chapter discusses the spectrum of congenital lung disease from the upper airway down to the lung parenchyma and microvasculature, and associated relevant malformations in the chest wall and mediastinum, and systemically. A systematic way of describing an individual malformation is proposed, using clear words to delineate the components of the malformation before planning treatment. Congenital lung disease may present in utero right up to old age. Many large malformations diagnosed antenatally largely regress in the third trimester of pregnancy, and are only detectable postnatally on computed tomography (CT) scanning. Management of asymptomatic congenital cystic malformations is controversial; many remain symptom free for a long time, whereas others become the seat of infection or malignancy, or result in other complications, such as air embolism. Histological overlap between what were once thought of as discrete entities, such as congenital cystic adenomatoid malformation and sequestration, are common, and attempting to determine histology from clinical images is fraught with difficulty. Newer imaging techniques, such as magnetic resonance and CT angiography, are increasingly used to image congenital lung malformations, with conventional angiography reserved for situations when therapeutic embolization of the malformation is being considered. Advances in surgical techniques, in particular for congenital diaphragmatic hernia, mean that there are more survivors into childhood and adult life. The optimal follow-up for patients with congenital lung malformations, whether surgically treated or not, remains to be determined.