Congenital tracheal stenosis managed conservatively: systematic review of the literature.

OBJECTIVE

Congenital tracheal stenosis (CTS) is challenging. Its natural history and epidemiology are poorly described. Information is scarce on cases not undergoing surgery.

METHODS

A retrospective review of patients with CTS in one pediatric otolaryngology practice was performed. A Medline search of English publications from 1966 to 2008 was conducted using the terms "CTS," "management of CTS," and "slide tracheoplasty."

RESULTS

Four patients were diagnosed over 5.5 years. Diagnosis was achieved within 4 months of life in three and at 13 years of age in one. Three children presented with difficulty to ventilate in the neonatal intensive care unit. One was diagnosed with asthma early in life. Other congenital abnormalities were encountered in three patients. One child required a tracheostomy due to respiratory failure, for reasons other than CTS. None required surgical repair, and conservative management was pursued. Three patients were followed for 5 years. Our search yielded 20 relevant reports from 1996 to 2008. All were retrospective case series. A total of 310 patients with a mean age of 17.14 months (± 24.10) were used in our analysis. The male to female ratio of those reported was 1.7:1. The mean follow-up was 5.1 years. Thirty-five to 43% of patients had no qualified indications for their surgical management. Mortality and complication rates make tracheal repair a significant undertaking. Little is reported on the long-term outcomes, swallowing functions, and quality of life of patients repaired who did not require immediate interventions.

CONCLUSION

There is a need to identify specific indications for surgery and a stratification of successful candidates by various disease-related factors. If the difficulties weaning from assisted ventilation or coping with growth and activity requirements are circumvented, children may be managed conservatively.