In Search of the Elusive Laryngotracheal Cleft

Abstract

Laryngotracheal clefts can be a cause of chronic aspiration and cough in children. While there are 4 subtypes, they can be grouped into 2 separate clinical entities. Type 3 and 4 clefts, in which tracheoesophageal communication can extend from the larynx into the trachea and down to the carina, are very rare with early presentation such as dramatic aspiration, bronchial flooding, and respiratory distress. Surgical repair for these anomalies is challenging with a nearly 50% mortality rate. In contrast, type 1 and 2 clefts, which extend to or through the cricoid, can be more difficult to identify. The incidence of type 1 cleft ranges from 0.2% in cadaveric studies to 7.6% in children undergoing direct laryngoscopy for chronic cough or recurrent pneumonia. Patients with type 1 or 2 clefts may go undiagnosed for years with recurrent aspiration, pneumonia, chronic cough, and swallowing difficulties. However, identification and effective management is critical as these patients can go on to develop chronic lung disease from the repeated aspiration events. Careful examination in the operating room with palpation of the interarytenoid region is essential to the diagnosis of type 1 and 2 laryngotracheal clefts. Treatment should include thickened feeds and swallowing therapy. If conservative management fails, endoscopic surgery can also be a minimally invasive way of managing these patients. A high clinical level of suspicion and effective communication between the pulmonologist, gastroenterologist, and otolaryngologist must be maintained to help identify this clinical entity.