Pathology
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Fetopathology and developmental pathology of the embryo and fetus
Marta Ježová, Josef Feit et al.
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Pulmonary hypoplasia
Etiology

Variable etiology:

  • compression of the lung in congenital diaphragmatic hernia
  • prolonged oligohydramnion in congenital renal malformations of urinary tract obstructions
  • prolonged oligohydramnion in chronic leakage of amniotic fluid
  • pleural effusions usually associated with hydrops fetus universalis
  • abnormalities of the thoracic cage in lethal osteochochondrodysplasias e.g. thanatophoric dysplasia, short rib polydactyly, osteogenesis imperfecta
  • anencephaly, neuromuscular disorders, fetal akinesis
  • trisomy 13,18,21
  • large defects of the abdominal wall
Clinical signs

Newborn infant with severe pulmonary hypoplasia presents shorly after birth with respiratory distress and need of ventilatory support. The distress is often worsened by pneumothorax.

Macroscopic appearance

The lungs are small with decreased number of alveoli, bronchi and bronchioli. Pulmonary hypoplasia is evaluated autoptically; gestational age, weight of the lungs and body weight are taken into account. Normal values of lung to body weight ratio matches 0.012 or more in infants of 28 weeks gestation and 0.015 for those of lower gestational age.