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Congenital pulmonary airway malformation in a fetus, ultrasound at 19 weeks - transverse. Stomach on left image; heart on right image: displaced to right by cystic mass. The earliest point at which a CPAM can be detected is by prenatal ultrasound. The classic description is of an echogenic lung mass that gradually disappears over subsequent ...
Chest x-ray of infant showing CPAM in the left lung causing a mediastinal shift towards the right. The cysts appear as bubbles in the left lung. Congenital pulmonary airway malformation (CPAM) is a rare disease in which the lung airways develop abnormally in the fetus. This leads to infants having pockets of air and cystic masses in their lungs.
All patients with situs ambiguus lack lateralization and symmetry of organs in the abdominal and thoracic cavities and are clinically considered to have a form of heterotaxy syndrome. Heterotaxy syndrome with atrial isomerism occurs in 1 out of every 10,000 live births and is associated with approximately 3% of congenital heart disease cases. [3]
There is still much debate to whether pulmonary sequestration is a congenital problem or acquired through recurrent pulmonary infection. It is widely believed that extralobar pulmonary sequestrations are a result of prenatal pulmonary malformation while intralobar pulmonary sequestrations can develop due to recurrent pulmonary infections in adolescents and young adults.
Hydrops Fetalis resulting from fetal CPAM can be treated using either a fetal needle drainage of effusion or placement of thoracoamniotic shunt or a maternal administration of corticosteroids, betamethasone 12.5 mg IM q24 h × 2 doses or dexamethasone 6.25 mg IM q12 h × 4 doses. [15]
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Congenital diaphragmatic hernia (CDH), in this case, the upward displacement of the diaphragm and abdominal organs, is a possible clinical outcome detectable before birth. [7] The displacement is caused by organ herniation occupying the empty space in the chest wall, while this space is created by the absence of lung tissue. [ 7 ]
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