Prevalence of Laryngeal Cleft in Pediatric Patients With Esophageal Atresia.

Importance: Esophageal atresia (EA), with or without tracheoesophageal fistula (TEF), and laryngeal cleft are rare congenital anomalies that often occur together. Previous reports have established a link between EA/TEF and laryngeal cleft, but there have been no large case series to further characterize this relationship.

Objectives: To assess the prevalence of laryngeal cleft among patients with EA/TEF, identify associations between types of laryngeal cleft and EA/TEF, and identify factors associated with the timing of diagnosis for both conditions.

Design, Setting, and Participants: Retrospective analysis of 270 patients. The Esophageal Atresia database was used to identify patients seen at the Esophageal and Airway Treatment (EAT) Center at Boston Children's Hospital, Boston, Massachusetts, a tertiary referral hospital, from August 1, 2009, to August 1, 2016. Patients were included if they were younger than 18 years at the time they were diagnosed with EA, TEF, or both and had undergone a procedure or examination by a surgeon from the EAT Center. Patients with acquired airway or esophageal problems were excluded.

Main Outcomes and Measures: Data regarding patient demographics, primary diagnoses, dates of diagnosis, comorbidities, and airway/esophageal surgical interventions were collected and analyzed. Association between type of EA/TEF and laryngeal cleft type was evaluated. Secondary outcomes were age at diagnosis between patients who resided in Massachusetts and those who resided outside the state.

Results: Of 270 patients diagnosed with EA/TEF during the 7-year period, 138 (51.1%) were male, and the median age at diagnosis of EA/TEF was 1.0 years (IQR, 0.4-2.0) and at diagnosis of laryngeal cleft was 1.1 years (interquartile range [IQR], 0.6-2.8). Laryngeal cleft was diagnosed in 53 of the 270 patients (19.6%) (95% CI, 14.9%-24.4%). No apparent difference was found in the distribution of types of laryngeal cleft by type of EA/TEF. Among patients with EA/TEF type A or B, 9 patients (56%) had a type I cleft, 6 (38%) had a type II cleft, and 1 (6%) had a type III cleft. Among those with EA/TEF type C or D, 20 (57%) had a type I cleft, 11 (31%) had a type II cleft, and 3 (9%) had a type III cleft. Out-of-state patients were more likely to be diagnosed with EA/TEF at an older age than in-state patients (mean difference, 1.5 years; 95% CI, 0-2.9 years).

Conclusions and Relevance: Pediatric patients with EA/TEF have a much greater prevalence of laryngeal cleft than the general population. Multidisciplinary esophageal and airway programs serve as an ideal clinical setting for management of EA/TEF.