The association between laryngeal cleft and tracheoesophageal fistula: Myth or reality?

Abstract

Objectives/Hypothesis: Laryngeal cleft (LC) associated with tracheoesophageal fistula (TEF) with or without esophageal atresia (EA) has rarely been described. The purpose of this study is to review our experience, clinical features, management, delay in diagnosis, and complications in children with these anomalies.Study Design: Retrospective chart review at pediatric tertiary referral center. Methods: Patients diagnosed with LC alone or LC and TEF over a 10-year period were included. Data including demographics, type of TEF and LC, comorbidities, symptoms, management, complications and swallowing outcomes were analyzed.Results: There were 161 pediatric patients diagnosed with LC alone and 22 with LC and TEF. In patients with LC and TEF, aspiration was the most common presenting symptom (n 5 11, 50%). Seventeen patients (77%, mean age 4 years 7 months) underwent endoscopic repair and five patients (23%) with type I clefts did not require surgery. Two patients required revision surgery. For patients with LC alone, the mean age at repair was 3.70 years (4 months-19.9 years) compared to 4.69 years (8 months-17.83 years) for patients with LC and TEF (P 5 0.0187). The postoperative swallowing studies from 15 patients showed no aspiration. Mean follow-up was 4 years and 6 months.Conclusion: The diagnosis and management of LC in patients with TEF is often delayed. If a child presents with persistent aspiration after TEF repair, a complete airway endoscopy should be performed to evaluate for vocal fold mobility and cleft. Endoscopic repair is the recommended approach for those patients requiring surgical intervention.

Respiratory Care of Infants and Children with Congenital Tracheo-Oesophageal Fistula and Oesophageal Atresia

“…43,44 In one study of 88 infants with OA/TOF who underwent direct laryngotracheobronchoscopy prior to surgical repair, 20% were found to have additional airway defects. Defects included a second TOF site (17%), fistula from a main pulmonary bronchus (42%), laryngeal cleft (11%), and vallecular cyst (6%).…”

“…43 However, prospective endoscopic data is lacking, and the prevalence of laryngeal cleft associated with OA/TOF is not known. Diagnosis of a laryngeal cleft may be delayed by months to years after the surgical repair of TOF and thus requires a high index of suspicion; endoscopic evaluation for a laryngeal cleft should be considered in patients with a history of OA/TOF and recurrent wheezing, dysphagia/aspiration, or pneumonia.…”

“…Diagnosis of a laryngeal cleft may be delayed by months to years after the surgical repair of TOF and thus requires a high index of suspicion; endoscopic evaluation for a laryngeal cleft should be considered in patients with a history of OA/TOF and recurrent wheezing, dysphagia/aspiration, or pneumonia. 43 Alternatively, if a laryngeal cleft is diagnosed endoscopically around the time of primary OA/TOF surgery, early cleft repair may prevent subsequent lung damage and respiratory morbidity. 43 …”

“…43 Alternatively, if a laryngeal cleft is diagnosed endoscopically around the time of primary OA/TOF surgery, early cleft repair may prevent subsequent lung damage and respiratory morbidity. 43 …”

Aspiration Risk and Respiratory Complications in Patients with Esophageal Atresia

“…Recurrent TEF may arise in about 9% of EA patients. Laryngeal clefts (particularly types 1 and 2) and unilateral or bilateral vocal cord paresis or paralysis appears to be frequent in EA/TEF patients, and often leads to aspiration (5, 6). Vocal cord paresis or paralysis may be, in at least some cases related to EA/TEF corrective surgery (7).…”