Laryngeal clefts occur when the larynx fails to fuse the posterior cricoid lamina, affecting laryngeal and esophageal function. Common presentations include difficulty feeding, cough, dysphagia, dyspnea, failure to thrive, stertor or stridor, abnormal lung examination, and/or a weak cry.
Associated congenital abnormalities include tracheoesophageal fistula, esophageal atresia, and subglottic stenosis. Gastroesophageal reflux disease (GERD), recurrent pneumonia, and intellectual disability have also been associated.
Laryngeal clefts are classified as types I-IV based on their characteristics and severity. Types II-IV typically require corrective surgery.
- Type I cleft – Interarytenoid cleft present between the larynx and esophagus that does not extend below the vocal cords. As this is the mildest form of the disease, diagnosis may be delayed, and symptoms may be mild or remain occult.
- Type II cleft – Cleft extends partially into cricoid cartilage, which is completely below the vocal cords.
- Type III cleft – Cleft extends throughout the cricoid cartilage and possibly involves the cervical portion of the trachea. Often recognized within the first few days of life, this requires surgical correction.
- Type IV cleft – Cleft extends throughout the cervical trachea and into the thoracic trachea. Often recognized within the first few days of life, this requires surgical correction.