Primary pulmonary hypoplasia: report of a case with polyhydramnios
G. Mendelsohn and G. M. Hutchins
We describe a case of extreme primary pulmonary hypoplasia. No other
congenital anomalies and none of the conditions known to be associated with
pulmonary hypoplasia were present. Pregnancy had been complicated by
substantial polyhydramnios. The hypoplasia was due to a marked deficiency
of the respiratory parenchyma in the presence of normal upper airways and
bronchi. Virtually no parenchymal development had occurred and there were
very few bronchioles, alveolar ducts, and alveoli. The changes differ from
those seen in pulmonary hypoplasia secondary to congenital diaphragmatic
hernia, bilateral renal agenesis, anomalies of the urinary outflow tracts,
and malformations of the thoracic cavity, in which the pulmonary hypoplasia
appears to be compressive in nature. While neither the etiology or
pathogenesis of the pulmonary hypoplasia are apparent in this case, the
presence of substantial polyhydramnios during pregnancy suggests the
possibility that the developing lungs may offer an important surface area
for reabsorption and recycling of constituents of amniotic fluid.
