FLASHPATHH A Z E M A L I

PULMONARY HYPOPLASIA

H A Z E M A L I

CLINICAL• Incomplete development of one or both lungs, leading to

decreased size and weight for gestational age and body weight

• Present with respiratory distress– Usually fatal in bilateral cases

• Commonly associated with other anomalies in Heart, GIT, Genitourinary, and Musculoskeletal system

CLINICAL• For lung development to proceed normally:

– Adequate physical space of the fetal thorax– Adequate amniotic fluid volume– Adequate fetal breathing movements

• To bring amniotic fluid to the developing lungs, leading to their distension

• IF any of these factors is inadequate incomplete lung development

CLINICAL Causes of Pulmonary Hypoplasia

Oligohydramnios

Renal agenesis

Fetal membrane rupture

Decreased intrathoracic

space

Renal cystic disease

Diaphragmatic hernia

Reduced breathing

Anencephaly

Musculoskeletal disorders

Brainstem Hypodevelopme

nt

CLINICAL• Remember Potter’s syndrome:

– Renal agenesis– Oligohydramnios– Pulmonary hypoplasia– Deformed limbs– Potter's facies:

• Flattened 'parrot-beaked' nose• Recessed chin and micrognathia• Prominent infraorbital folds• Low-set ears

GROSS• Small lungs, relative to the thoracic cavity and to the heart

– With a normal-sized heart, the diaphragmatic surface of the lungs should be close to the heart apex

• Decreased lung weight– Left lung weight (at term) is 18 g– Right lung weight ( at term) is 21 g

• Decreased lung/body weight ratio– < 0.015 in fetuses/neonates < 28 weeks gestation– < 0.012 in fetuses/neonates > 28 weeks gestation

MICROSCOPY• Decreased alveolar number and size, reduction in

airway generations

• Decreased radial alveolar counts– Counting the alveoli or alveolar walls transected by an imaginary line

from the mid-point of a respiratory bronchiole to the nearest pleural or interlobular septum

Normal Hypoplasia

DIFFERENTIAL DIAGNOSIS

Lung Atelectasis• Commonly Acquired• Collapsed alveoli (no decrease in number)

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