Brachiocephalic Artery Compression Syndrome

Brachiocephalic artery compression syndrome occurs when the brachiocephalic artery takes an anomalous course, causing anterior compression of the trachea at the thoracic inlet. It is a syndrome of childhood due to the smaller size of the thoracic inlet. Affected patients most commonly present with stridor, recurrent respiratory tract infections, and severe respiratory distress. Other signs and symptoms include a croup-like “barking” cough, episodic apnea, difficulty feeding, and failure to thrive. CT with intravenous contrast is the preferred imaging modality and will often reveal tracheal compression at the level of the brachiocephalic artery. Treatment is centered on supportive care in patients with mild-to-moderate symptoms and aortopexy or brachiocephalic artery reimplantation in severe cases.