Introduction
IACS is a rare vascular cause of paediatric airway obstruction resulting from anterior compression of the trachea by the innominate artery. Clinical presentation is variable and may include stridor, recurrent respiratory infections and respiratory distress, often making diagnosis challenging. Most cases are managed conservatively, with surgical intervention reserved for severe airway compromise. We present a series of four infants with severe IACS requiring aortopexy within the first year of life.
Methods
A retrospective review was undertaken of four infants diagnosed with IACS at Bristol Royal Hospital for Children. Clinical presentation, laryngotracheobronchoscopy findings, radiological investigations, management and outcomes were reviewed. All patients underwent multidisciplinary airway assessment and were subsequently referred to Great Ormond Street Hospital (GOSH) for surgical management with aortopexy.
Results
Between January 2018 and January 2026, four infants were referred for surgical management of IACS. All patients were male; two of which were twins. All infants had laryngotracheobronchoscopy and 4D CT imaging demonstrating distal tracheal compression. Tracheal stenosis ranged from 58% to 84%, with dynamic calibre change of 39% to 52%. All patients proceeded to aortopexy with postoperative improvement in airway symptoms and no major complications.
Conclusion
IACS is an uncommon but important cause of severe airway obstruction in infancy. This case series highlights the variable presentation of the condition and the value of combined endoscopic and radiological assessment in identifying those requiring surgical intervention. The clustering of cases in siblings may suggest a possible underlying genetic contribution, which could be a potential area for further research.
References
Donuru A, Kandula V, Madueme P. Innominate Artery Compression Syndrome. Aorta (Stamford). 2022 Jun;10(3):145-146. doi: 10.1055/s-0042-1742697. Epub 2022 Nov 1. PMID: 36318937; PMCID: PMC9626248.
Wenig BL, Abramson AL. Innominate artery compression of the trachea. Bull N Y Acad Med. 1984 Jun;60(5):525-31. PMID: 6375793; PMCID: PMC1911753.
Jones DT, Jonas RA, Healy GB. Innominate artery compression of the trachea in infants. Ann Otol Rhinol Laryngol. 1994 May;103(5 Pt 1):347-50. doi: 10.1177/000348949410300502. PMID: 8179249.