Clinical presentation and airway management of tracheal atresia: A systematic review

• Published in: International journal of pediatric otorhinolaryngology Vol. 101; pp. 57 - 64
• Main Authors: Smith, Mariana M., Huang, Amy, Labbé, Mathilde, Lubov, Joshua, Nguyen, Lily H.P.
• Format: Journal Article
• Language: English
• Published: Ireland Elsevier B.V 01.10.2017
• Subjects: Airway Management - methods; Atresia; Congenital abnormalities; Constriction, Pathologic - diagnosis; Constriction, Pathologic - mortality; Constriction, Pathologic - surgery; Female; Humans; Infant, Newborn; Male; Newborn; Pregnancy; Resuscitation; Survival Rate; Trachea; Trachea - abnormalities; Trachea - surgery; Tracheal agenesis; Tracheal agenesis; Atresia; Congenital abnormalities; Newborn; Trachea; Resuscitation
• ISSN: 0165-5876; 1872-8464
• DOI: 10.1016/j.ijporl.2017.07.028

Tracheal atresia (TA) is a rare congenital condition that typically requires an unexpected and emergent resuscitation in the delivery room. The mortality rate associated is very high, with only a few long-term survival cases reported. We describe the findings of a systematic review on the clinical presentation and airway management of TA. Using the keywords “tracheal atresia”, “tracheal agenesis” and “tracheal hypoplasia” a search through Embase and Pubmed databases was performed following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocol. Articles published from 1950 to 2015 in English, French, Italian, Portuguese and Spanish were included. Exclusion criteria were cases of stillborn, and unclear diagnosis or outcome. 149 cases of TA were identified after reviewing 1125 initial references. There was a male preponderance (65%), and associated malformations were described in 94.2% of patients. Prenatal ultrasound was abnormal in 56.3% of cases, with polyhydramnios being the most common finding. The most frequent type of TA was Faro Type C. 94 (41.3%) patients did not survive beyond the first 24 h of life. Only 13 (8.4%) patients survived more than three months of life, after undergoing a variety of surgical approaches. This review, which to our knowledge is the largest one to date, confirms that TA is a rare malformation, occurs more frequently in males, and has a very high mortality rate. Depending on the presence and type of concomitant malformation, as well of the length of the remaining trachea, different surgical management options are described.