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Virtual Pediatric Hospital: Paediapaedia: Pulmonary Hypoplasia Due to Fetal Anuria Syndrome Paediapaedia: Neonatal Chest Diseases

Pulmonary Hypoplasia Due to Fetal Anuria Syndrome

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Respiratory distress in full term infant with oligohydramnios during pregnancy.

Etiology/Pathophysiology:
Causes of fetal anuria and oligohydramnios include renal agenesis, renal cystic disease, and obstructive uropathy in the form of posterior urethral valves. The decreased amniotic fluid causes extrathoracic compression of the thorax and leads to development of pulmonary hypoplasia. Pulmonary hypoplasia is frequently complicated by spontaneous pneumothorax or pneumomediastinum due to overdistension of small lungs with deficient alveoli that leads to rupture of terminal airways and dissection of air into the mediastinum or pleural space.

Pathology:
Have a diminished number of peripheral alveoli and pulmonary vessels and the muscular layers of the pulmonary arteries are thickened resulting in pulmonary hypertension and persistent fetal circulation.

Imaging Findings:
Small, hypoplastic lungs. Narrowed and constricted thoracic cage with short, dysplastic ribs. Pneumothorax and related air leak complications are common. icon gif Renal ultrasound will demonstrate the renal anomaly causing the oligohydramnios.

DDX:

References:
See References Chapter.

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