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.
We recommend feeding and swallowing therapy for children with normal upper airway anatomy. Brain magnetic resonance imaging should be considered for patients with suspected brainstem or posterior fossa lesion based on neurologic examination findings. Most patients who aspirate thin and thickened liquids will have resolution of their swallowing dysfunction within 1 year of beginning therapy.
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