FlashPath - Lung - Pulmonary Hypoplasia
Transcript of FlashPath - Lung - Pulmonary Hypoplasia
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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