Tracheal agenesis: management of the first 10 months of life

• Published in: Pediatric anesthesia 2004-09, Vol.14 (9), p.774-777
• Main Authors: Baroncini‐Cornea, S., Fae, M., Gargiulo, G., Gentili, A., Lima, M., Pigna, A., Pilu, G., Tancredi, S., Turci, G.
• Format: Article
• Language: English
• Subjects: Abnormalities, Multiple; Anesthesia; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Biological and medical sciences; Cardiac Catheterization; Cardiac Surgical Procedures; Catheterization; congenital malformations: tracheal agenesis; difficult intubation; Digestive System Abnormalities - diagnosis; Digestive System Abnormalities - surgery; Digestive System Surgical Procedures - methods; Esophagus - abnormalities; Esophagus - surgery; Female; Follow-Up Studies; Heart Defects, Congenital - diagnostic imaging; Heart Defects, Congenital - surgery; Humans; Imaging, Three-Dimensional; Infant; Larynx - diagnostic imaging; Medical sciences; newborn: respiratory distress; Rare Diseases; Respiratory System Abnormalities - diagnosis; Respiratory System Abnormalities - surgery; Stents; Tomography, X-Ray Computed; Trachea - abnormalities; Tracheoesophageal Fistula - diagnosis; Tracheoesophageal Fistula - surgery; Tracheostomy - methods; Ultrasonography
• ISSN: 1155-5645; 1460-9592
• DOI: 10.1111/j.1460-9592.2004.01281.x

Summary Tracheal agenesis is a potentially lethal congenital anomaly, appearing only at birth. We describe a newborn preterm infant who presented with immediate respiratory distress and no audible cry. There was almost complete tracheal agenesis with a very short segment of distal trachea (only two tracheal rings) arising from the anterior wall of the esophagus, before dividing into the mainstem bronchi. The anomaly was unsuspected prenatally, as the scan showed pyloric atresia and complex congenital cardiac disease. Despite the patient's difficult course, with correction of the rare‐associated malformations (cardiac and gastrointestinal tract anomalies), the fact that the child is lively and neurologically normal for her age, requires that we now consider the patency of the airway and the possibility of surgical correction, in accordance with a good quality of life.