Introduction
Bronchial atresia is a rare condition, typically affecting the
subsegmental bronchi1, and often noted on prenatal
ultrasounds as a hyperexpanded fluid filled lung segment. The more
proximal the atresia, the more severe and life-threatening the
presentation. Only ten cases of mainstem bronchial atresia (MBA) have
been reported to date, with universally poor
outcomes2-6. Unlike peripheral bronchial atresia, MBA
presents a unique challenge with expansion of the involved lung,
mediastinal compression and contralateral shift, hydrops and fetal or
infant death. This represents a form of unilateral Congenital High
Airway Obstructive Syndrome (CHAOS), with laryngeal or tracheal atresia
causing bilateral lung hyperexpansion and diaphragmatic
eversion7.