Fetal Lung Growth in Congenital Laryngeal Atresia

Abstract

Morphometric and biochemical indexes of lung growth were measured in 2 cases of uncomplicated laryngeal atresia at 27 and 30 weeks gestation and in 1 case of cryptophthalmos syndrome with anomalies including laryngeal atresia and renal agenesis. Findings were compared with those in normally formed fetuses and newborn infants. The cases of pure laryngeal atresia showed a marked increase in surface area and lung volume for age, associated with an increase in alveolar number and apparent advance in elastin maturation, but little increase in cell population as measured by lung DNA content. Alveolar walls were thin but there was no increase in disaturated phosphatidylcholine (DSPC) content. Similar features were observed in the case of cryptophthalmos in marked contrast to the lung hypoplasia expected to result from renal agenesis. The results give further support to the importance of lung liquid retention for normal fetal lung growth. Overdistention with lung liquid appears to promote alveolar development by redistribution of cells rather than increase in cell population.

Key Words:

• pulmonary development
• human lung
• laryngeal atresia
• cryptophthalmos syndrome
• renal agenesis
• elastin