Children with laryngeal clefts need care from a team of experts with experience diagnosing and treating these complex congenital defects. Laryngeal clefts are rare, occurring in one in 10,000 births, are often underdiagnosed, and can be fatal without treatment. Our team in the Esophageal and Airway Treatment Program at Johns Hopkins All Children’s Hospital in St. Petersburg, Florida, has developed treatment methods that incorporate advanced surgical techniques and technologies to minimize complications and provide better long-term results for our patients.

Laryngeal clefts typically involve the space between the vocal cords and sometimes the back of the larynx (voice box), allowing saliva and swallowed food to go into the airway and lungs. These are a shorter version of laryngo-tracheal esophageal clefts that are longer and involve the airway (trachea) itself with a slit-like connection to the esophagus. Sometimes this connection is relatively short in the neck region, while sometimes going longer all the way to the bottom of the trachea, and rarely going even longer into the bronchus, usually the left side.

Food and liquids enter the opening and go into the lungs instead of the stomach. In babies with a laryngeal cleft, they can appear as if they are drowning when they try to eat, and they may also have trouble breathing because of the associated tracheomalacia (a condition in which the airway collapses), which is frequent in these children.

Another problem is gastroesophageal reflux – which is very common – due to the stomach contents going up the esophagus and into the lungs where the acid and bile from the stomach and the food in the stomach injures the lungs. The cleft may become apparent if doctors try to insert a tube into the trachea or the esophagus, because the slit allows the tube to enter the wrong area of the body – a tracheal tube may end up in the esophagus, and vice versa.

Why Choose Johns Hopkins All Children’s

Experienced Team

Our team of pediatric thoracic surgeons is experienced in treating this rare congenital defect, including in its most severe forms. Our team includes pediatric anesthesiologists with expertise in meeting the unique anesthesia needs of children needing surgery for airway conditions, and we also work closely with pediatric gastroenterology specialists to address esophageal and swallowing function in our patients. The hospital’s radiology specialists and nutrition teams also provide additional expertise in caring for our patients. Learn more about our team.

Advanced Diagnostic Techniques

For simplicity’s sake, laryngeal clefts are often classified according to how far down the trachea the slit occurs. Because laryngeal clefts can vary significantly in presentation and severity, we have developed a more refined classification system that recognizes further degrees of difference among the four types. This enhances our diagnostic accuracy and ensures each patient receives the personalized care that best addresses their individual condition.

Our team has also developed a complete dynamic three-phase airway exam that allows us to do a full assessment of your child’s airway before surgery. Using various methods, we examine the structure and function of the vocal cords, larynx (or voice box), trachea, esophagus and into the stomach if needed, to establish a complete picture of your child’s current condition. This thorough testing allows us to carefully plan your child’s treatment.

Innovative Care

Historically, treatment for laryngeal clefts has carried risk of recurrence and continued issues with recurrent tracheoesophageal fistulas (an abnormal connection between the esophagus and trachea), severe tracheomalacia (a condition in which the airway collapses), and swallowing problems. Our team has developed treatment strategies to more effectively repair the cleft and better prevent recurrence of tracheo-esophageal fistulas and correct the tracheomalacia. We also monitor the nerves of the vocal cords during surgery to protect these nerves. Additionally, we have developed methods of better treating Type IV, in which the slit can extend all the way to the bottom of the trachea, and those that extend down into the bronchus, the most severe forms.

Our Approach to Treatment

Proper treatment of laryngeal cleft depends on accurate diagnosis and understanding of the length of the gap and how the airway is impacted. We perform a complete evaluation of a patient’s trachea and esophagus and do a CT scan with IV contrast to achieve a complete picture of your child’s condition and their anatomy so we can customize a surgical plan to meet their needs.

Testing includes:

Flexible laryngoscopy: A thin, flexible camera is used to examine the nasal passages, throat, and vocal cords, allowing us to look at the structure of the vocal cords and how they work when your child is breathing normally and when coughing collapses the airway.

Bronchoscopy: Done with anesthesia under the close care of our expert anesthesia team, a thin tube with a camera is used to examine the trachea, bronchi (the passages between the trachea and the lungs), and smaller airways. We can examine things like the anatomy of the trachea and the shape of the cartilage of the trachea and identify secretions that can be sent to the lab for testing, all of which may indicate the extent of the compression and help uncover related issues.

Positive pressure testing: Under anesthesia, we use positive pressure in the airway to open the posterior membrane. This allows us to see things like the extent of the cleft, additional holes which sometimes occur, connections to abnormal lung tissue, or fistulas, and aids us in identifying any rare anomalies that may be contributing to your child’s airway issues before surgery such as strictures and aberrant bronchi, etc.

During surgery, we monitor the trachea continually using a bronchoscope as we repair the airway and then monitor the esophagus with endoscopy. We separate the esophagus and the trachea, use endoscopic visualization to repair the trachea perfectly, and by supporting the back wall of the trachea onto the spine we prevent airway collapse and recurrent tracheo-esophageal fistulas. We then repair the esophagus in two layers and repair the cartilage of the larynx, avoiding fistulas.

What to Expect

We follow all our patients long-term after surgery. Typically, we’ll have a phone or video chat follow up with you about a week after your child’s discharge home, and then they will have an appointment with us in our clinic about a month after the surgery. We understand that travel is often difficult for our patients from other states and other countries, and we will do our best to work with you. We’ll determine appropriate follow up from there depending on your child’s condition.