What is laryngotracheal cleft?

Laryngotracheal cleft (LTC) is a rare congenital defect in which there is a gap between the upper airway passage (larynx and possibly trachea) and the food passage (esophagus). In normal development, the airway and esophagus come from a common tube called the foregut. Early in embryologic development, the airway separates from the esophagus with the formation of the tracheoesophageal septum.

LTC is thought to occur in children whose septum does not completely develop, resulting in variable degrees of communication between the airway and esophagus. Because of the defect, the child can inadvertently inhale food or even secretions into the lungs. Children with LTC often also have other midline defects such as tracheoesophageal fistula, cleft lip/palate and heart defects.

Testing and diagnosis for laryngotracheal cleft

If doctors suspect your child has LTC, the diagnoses may be confirmed with a microlaryngoscopy in the operating room, during which the back of the larynx is visually inspected and gently palpated with a microinstrument. If a split is found between the larynx and esophagus, the doctor will then determine the severity of your child's defect.

• Type 1 clefts extend through the interarytenoid muscles but spare the cricoid cartilage.
• Type 2 clefts extend through the cricoid.
• Type 3 clefts extend into the upper trachea.
• Type 4 clefts are the most severe with extension into the thoracic portion of the trachea. This lesion may be life-threatening.

Aero-digestive evaluation

Infants with LTC need to be evaluated for both airway and feeding issues. Management of the airway often requires a combination of supportive, medical and surgical care. Feeding and swallowing issues are very common in children with these conditions and often need to be addressed by speech pathologists and GI specialists. Children born with laryngotracheal cleft often need other subspecialty evaluations (cardiology, ophthalmology, etc.) and benefit greatly from the coordinated care provided in the multidisciplinary setting of the Center for Pediatric Airway Disorders.

Treatment for laryngotracheal cleft

Treatment depends on the severity of the anomaly, how much it affects the child's breathing and eating, as well as what other medical conditions the child has. Most type 1 and type 2 clefts can be repaired endoscopically. Type 3 and 4 clefts require an open surgical repair. Despite surgical intervention, type 4 laryngeal clefts that extend all the way to the carina are often incompatible with long-term survival.

Treatment for mild cases

These include type 1 cases where the child is not having respiratory distress. These patients may initially be managed with close observation, thickened feeds and reflux management. In cases where these strategies are not effective, a temporary feeding tube and endoscopic repair is considered.

Treatment for severe cases

These include all patients with type 2, 3 or 4 clefts. Surgery should be performed as soon as the patient is stable and has been evaluated for other anomalies. The major goal of the surgical repair is to separate the airway from the esophagus in order to prevent aspiration. This is most often accomplished using an open (incision through the neck) approach and occasionally requires placement of a tracheostomy tube. Despite treatment, type 4 clefts are often incompatible with long-term survival.