INTRODUCTION TO PULMONARY HYPOPLASIA  

 

DEFINITION
  1. Absolute decrease in lung volume and weight for gestational age (1).
  2. Decrease ratio of lung weight to body weight (2).

Normal growth and development of the fetal lung requires:

  1. Adequate intrathoracic space.
  2. Adequate intrauterine space.
  3. Sufficient amount of amniotic fluid.
  4. Normal fetal breathing movement.

Disturbances in one of the above factors may result in pulmonary hypoplasia. The degree of pulmonary hypoplasia depends on the nature of the insult as well as the gestational age when the insult occurred as well as the severity of the insult. This results in a wide spectrum of clinical manifestations of pulmonary hypoplasia that ranges from acute respiratory insufficiency that results in death to milder degrees of respiratory insufficiency leading to pulmonary hemorrhage or bronchopulmonary dysplasia.
 

INCIDENCE

1.4% of all live births increasing to 6.7% of all stillborn infants and constitutes about 10-15% of all neonatal autopsies (3-5).
 

 

 

REFERENCES
  1. Schinzel A, Savodelli G, Briner J et.al. Prenatal sonographic diagnosis of Jeune syndrome. Radiology 1985;154:777-778.
  2. Swischuk LE, Richardson CJ, Nichols MM et.al. Primary pulmonary hypoplasia in the neonate. J Pediatr 1979;95:573-577.
  3. Knox WF, Barson AJ. Pulmonary hypoplasia in a regional perinatal unit. Early Hum Dev 1986;14:33-42.
  4. Reale FR, Esterly JR. Pulmonary hypoplasia: A Morphometric study of the lungs of infants with diaphragmatic hernia, anencephaly, and renal malformations. Pediatrics 1973;51:91-96.
  5. Wigglesworth JS, Desia R. Use of DNA for growth assessment in normal and hypoplastic fetal lungs. Arch Dis Child 1981;56:601-605.