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Pulmonary hypoplasia – Potter sequence — RACP Paediatrics MCQ

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HardRespiratoryPulmonary hypoplasia – Potter sequenceRACP Paediatrics

A term neonate with severe oligohydramnios (due to bilateral renal agenesis) develops immediate respiratory failure at birth with a bell-shaped chest and limb deformities. Ventilation is extremely difficult despite adequate ETT positioning. What is the most likely respiratory diagnosis?

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Correct answer: A — Pulmonary hypoplasia

Severe oligohydramnios from any cause (bilateral renal agenesis/Potter sequence, prolonged PPROM) leads to pulmonary hypoplasia due to absent fetal breathing movements and lung compression. The bell-shaped chest reflects underdeveloped lungs. Bilateral renal agenesis with pulmonary hypoplasia (Potter sequence) is incompatible with life.

Reference: RACP Paediatric Curriculum – Neonatology/Respiratory; RCH Melbourne – 2023