Innominate artery compression syndrome of the trachea in a 12-month old

Abstract

Presentation: A 12-month-old female presents after an episode of difficulty breathing with apnea and cyanosis. Parents performed chest compressions for 20 seconds until she woke up. This is her third episode of apnea over the last 10 months. Parents also describe chronic biphasic stridor at baseline since she was 2 months old. The stridor is not positional or associated with eating, but worsens when ill. She does not spit up after feeding and there is no stertor.

Physical Exam: T 36.2 C, HR 140 beats/min, BP 119/77 mmHg, RR 30 breaths/min, SpO2 98% on room air. She is well-appearing with mild congestion and biphasic stridor that is audible without a stethoscope.

Evaluation: Respiratory panel positive for rhinovirus, which was positive 3 weeks ago after a previous apneic event. Chest x-ray unremarkable. No signs of aspiration noted on evaluation by speech language pathologist. Video EEG was normal. EKG showed sinus tachycardia. CTA of the chest revealed significant compression of the trachea where the right brachiocephalic (innominate) artery passes over the trachea.

Diagnosis: She underwent bronchoscopy which showed tracheomalacia with tracheal compression to approximately 90% obstruction, consistent with a diagnosis of innominate artery compression syndrome (IACS). This patient underwent reimplantation of the innominate artery with post-operative bronchoscopy that showed significant improvement in tracheal compression. She presented one month later with apneic events and repeat bronchoscopy showed tracheomalacia with posterior wall prolapse. She then underwent posterior tracheopexy and subsequently had a bronchoscopy which showed no collapse or dynamic compression.

Discussion: Innominate artery compression of the trachea can cause biphasic stridor, respiratory arrest, and growth faltering. The degree of compression can range from asymptomatic to severe obstruction. Respiratory illnesses often cause stridor and can influence the index of suspicion for a rare condition like IACS. Surgical intervention is warranted in children who present with life-threatening apneic events.

Cite this article as: Taylor I, Van Meurs A, Tam R. Innominate artery compression syndrome of the trachea in a 12-month old. Pediatr Acad Case Rep. 2026;5(1):15-8.

The parents’ of this patient consent was obtained for this study.

The authors declared no conflicts of interest with respect to authorship and/or publication of the article.

The authors received no financial support for the research and/or publication of this article.