Prevalence of Laryngeal Cleft in Pediatric Patients With Esophageal Atresia | Congenital Defects | JAMA Otolaryngology–Head & Neck Surgery | JAMA Network
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Original Investigation
February 2018

Prevalence of Laryngeal Cleft in Pediatric Patients With Esophageal Atresia

Author Affiliations
  • 1Department of Otolaryngology and Communication Enhancement, Boston Children’s Hospital, Boston, Massachusetts
  • 2Department of Otolaryngology, Harvard Medical School, Boston, Massachusetts
  • 3Department of Surgery, Boston Children’s Hospital, Boston, Massachusetts
  • 4Department of Surgery, Harvard Medical School, Boston, Massachusetts
JAMA Otolaryngol Head Neck Surg. 2018;144(2):164-168. doi:10.1001/jamaoto.2017.2682
Key Points

Question  What is the association between esophageal atresia/tracheoesophageal fistula and laryngeal cleft, and are there significant associations by type?

Findings  In this analysis of 270 patients diagnosed with esophageal atresia/tracheoesophageal fistula, laryngeal cleft was diagnosed in 53 (19.6%), and there was no difference in the distribution of types of laryngeal cleft by type of esophageal atresia/tracheoesophageal fistula.

Meaning  Because children with esophageal atresia/tracheoesophageal fistula have a much higher prevalence of laryngeal cleft than the general population, multidisciplinary esophageal and airway programs serve as an ideal clinical setting for treatment of these patients.

Abstract

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.

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