Congenital Airway Anomalies and Surgical Management

Congenital airway anomalies encompass a spectrum of malformations in the upper respiratory tract that arise during embryonic development. Among the most severe are tracheal agenesis and tracheal atresia, in which the tracheal lumen is either entirely absent or occluded by a fibromuscular band. Other anomalies include laryngeal atresia, complete rings of cartilage encircling the trachea and bronchial tree, and aberrant vascular rings that constrict the airway. Prenatal diagnosis may be suggested by polyhydramnios, lung hyper-echogenicity or features of congenital high airway obstruction syndrome, yet definitive identification often requires foetal MRI or ex utero intrapartum treatment planning. At birth, affected infants present with immediate respiratory distress, inability to intubate and profound cyanosis. Initial resuscitation strategies range from mask ventilation and oesophageal intubation to emergency tracheostomy or extracorporeal membrane oxygenation, each with variable success. Surgical management has evolved from straightforward tracheostomy to complex reconstruction: creation of a neo-trachea using oesophageal tissue, staged autologous grafting and, more recently, application of bioresorbable three-dimensional printed stents. Advances in imaging, multidisciplinary collaboration and regenerative materials have improved survival in selected centres. Global efforts now emphasise early recognition, standardised protocols for delivery-room management and innovation in scaffolds and tissue engineering to restore airway continuity and function.

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Congenital Airway Anomalies and Surgical Management publication trend

The graph below shows the total number of articles in congenital airway anomalies and surgical management across all publications each year (not limited to Nature Index journals).

Technical terms

Tracheal agenesis: Complete absence of the tracheal lumen below the larynx, often associated with broncho-oesophageal fistula.

Tracheal atresia: Partial or total failure of tracheal canal formation, characterised by a fibromuscular obstacle to airflow.

Tracheoesophageal fistula (TOF): Abnormal communication between the trachea and oesophagus allowing passage of air into the digestive tract.

EXIT procedure: Ex utero intrapartum treatment that maintains placental circulation while securing the neonatal airway before complete delivery.

Bioresorbable stent: A temporary scaffold, typically manufactured from degradable polymer, that supports airway patency and is absorbed over time.

References

1. Tracheal agenesis versus tracheal atresia: anatomical conditions, pathomechanisms and causes with a possible link to a novel MAPK11 variant in one case. Orphanet Journal of Rare Diseases (2024).
2. External Esophageal Stenting Technique in Palliation for Tracheal Agenesis in a Case of Esophageal Lung: A Lesson Learned from the Experience for Tracheomalacia. Children (2023).
3. Successful application of the innovation process to a case of Floyd Type I tracheal agenesis. Surgery Open Science (2022).
4. Unexpected tracheal agenesis with prenatal diagnosis of aortic coarctation, lung hyperecogenicity and polyhydramnios: a case report. Italian Journal of Pediatrics (2020).
5. Tracheal Agenesis: A Challenging Prenatal Diagnosis—Contribution of Fetal MRI. Case Reports in Obstetrics and Gynecology (2015).
6. A case report of type II Floyd tracheal agenesis with staged tracheal and alimentary reconstructions. Surgical Case Reports (2024).