Laryngeal Cleft

When a child’s larynx (voice box) develops normally, it is completely separate from the esophagus, so swallowed foods go directly into the stomach. A laryngeal cleft (or laryngotracheal cleft) is an abnormal opening between the larynx and the esophagus through which food and liquid can pass through the larynx into the lungs. This causes a number of eating and breathing problems.

Laryngeal clefts are classified in one of four ways:

• Type I is the mildest form of laryngeal cleft. The gap between the larynx and the esophagus is located above the vocal cords.
• Type II laryngeal cleft extends into the lower cartilage of the voice box, below the vocal chords.
• Type III laryngeal cleft extends beyond the voice box and into the trachea (windpipe).
• Type IV is the most severe form laryngeal cleft. The gap extends even further down into the windpipe, and it may go all the way to the bottom of the trachea.

If your child has a laryngeal cleft, you will most likely notice that he or she has trouble swallowing. When eating or drinking, for instance, liquids or food can go into your child’s lungs, which can make him or her inhale deeply and sometimes choke.

Other symptoms of the disorder include:

• Coughing
• Gagging
• Frequent respiratory infections

Laryngeal cleft is congenital, meaning it is present at birth. The cleft occurs during the early months of pregnancy, but we do not know why.

While present at birth, sometimes this disorder is sometimes not diagnosed until later childhood or adolescence. At Boston Children’s, laryngeal cleft is diagnosed through a comprehensive aero-digestive evaluation that may include one or more of the following tests:

• Bronchoscopy
• CAT scan (CT) with 3D reconstruction
• Echocardiogram
• Functional endoscopic evaluation of swallowing (FEES)
• Laryngoscopy
• Rigid bronchoscopy
• Swallow study

Your child may also require an airway evaluation in which a surgeon closely examines a region of the larynx (voice box). During this procedure, the surgeon will evaluate the length and depth of the gap and determine the type of cleft. Your child will be asleep for this procedure.

Our team of experts will discuss your child’s specific symptoms and suggest a treatment plan to address his or her condition.

Non-surgical treatments

Your child’s care team will work closely with other specialties to manage laryngeal clefts medically (without surgery). This includes customizing a feeding trial that ensures your child is able to eat and swallow safely and comfortably. Your child will also be treated for other problems he or she may be experiencing, such as gastroesophageal reflux disease (GERD), reactive airway disease, or a food allergy. Different treatments can help improve airway edema or irritation, which in turn may help swallowing function.

Surgical treatments

Patients with a type I cleft for whom medical management strategies have not been successful will likely need surgical repair. Patients with a type II, III, or IV laryngeal cleft will require a surgical repair, due to the severity of the disorder. After surgery, your child will continue to receive comprehensive follow-up care.

The Center for Airway Disorders at Boston Children's Hospital is designed to care for children with rare conditions of the airway, including laryngeal cleft. Our team provides the most advanced testing and airway treatments available for this condition.