Laryngeal Cleft

What is the definition of a laryngeal cleft?

A laryngeal cleft is a gap in the tissues between your larynx (voice box) and esophagus (food pipe). This abnormal gap creates an opening that connects your larynx and esophagus, allowing swallowed foods and liquids to enter the opening and travel into your lungs. A laryngeal cleft forms early in pregnancy while your baby is developing.

Is a laryngeal cleft a birth defect?

A laryngeal cleft is a rare congenital defect (present at birth). It can happen on its own, with other malformations or as part of an underlying syndrome such as:

• Opitz-Frias.
• VACTERL.
• Pallister-Hall.
• CHARGE.

In about 50% of cases, laryngeal clefts are associated with other abnormalities such as:

• Tracheobronchomalacia.
• Cleft lip and/or palate.
• Heart and blood vessel anomalies.
• Pulmonary agenesis.
• Bronchoesophageal fistula.
• Tracheoesophageal fistula.

What are the types of laryngeal cleft?

The type of laryngeal cleft depends on the size and location. There are four main types:

• Type I: The mildest form of laryngeal cleft often doesn’t cause significant symptoms and isn’t diagnosed right away. The opening between your larynx and esophagus is above your vocal cords.
• Type II: The opening extends below your vocal cords.
• Type III: The cleft is larger and extends beyond your larynx and into your trachea (windpipe).
• Type IV: This is the most severe form of laryngeal cleft. The opening extends further down your trachea. In some cases, the opening goes all the way down your trachea.

How common is laryngeal cleft?

A laryngeal cleft is rare. It affects 1 in 10,000 to 20,000 live births. It’s more common in boys than girls.

What causes a laryngeal cleft?

Researchers don’t know the exact cause of a laryngeal cleft. Because it’s congenital (present at birth), it develops spontaneously while your baby is in the womb, usually within the first few months of pregnancy.

What are the symptoms of a laryngeal cleft?

When your child has a laryngeal cleft, you may notice:

Eating problems

When your baby has a hole between their larynx and esophagus, food can enter their lungs rather than their esophagus. This is uncomfortable and can cause:

• Aspiration, getting food or drink in their lungs.
• Behavior and sleeping issues due to constantly feeling hungry.
• Failure to thrive, not gaining weight or growing appropriately.
• Feeding difficulties.
• Reflux.

Respiratory problems

When breast milk or formula travels into your child’s lungs, it increases the risk of infection and makes it difficult for your baby to breathe. Your baby may have:

• Coughing (especially during feeding).
• Cyanosis, or bluish skin tone. (In people with darker skin tone, cyanosis is easier to notice in their gums, lips, nails and around their eyes.)
• Pneumonia.
• Recurrent lung infections.
• Respiratory distress (depending on the depth of the cleft).
• Stridor, a harsh, raspy breathing sound.
• Wheezing.

Do laryngeal cleft symptoms differ depending on the type of cleft?

You may notice different symptoms depending on the type of laryngeal cleft. Milder forms, such as types I and II, often have milder symptoms and may not get diagnosed for years.

Types III and IV, however, usually have more severe respiratory symptoms like mucus in the lungs. Types III and IV are usually diagnosed within the first few days of your baby’s life because of the severity of the symptoms.

How is a laryngeal cleft diagnosed?

If your baby has many laryngeal cleft symptoms, their healthcare provider may decide to perform an endoscopic evaluation. The most common method and “gold standard” for laryngeal cleft diagnosis is a microlaryngoscopy and bronchoscopy.

Your child receives anesthesia. Their healthcare provider then inserts a camera into your child’s windpipe and uses an instrument to feel for a cleft.

Who treats a laryngeal cleft?

A team of doctors and specialists works together to create a treatment plan for your child. Members of this team usually include a:

• Pediatrician to assist with the initial symptoms and specialist referral.
• Speech pathologist to assess speech and feeding problems.
• Otolaryngologist (an ear, nose and throat doctor) to perform testing and diagnosis.
• Surgeon to perform any treatment procedures.
• Nurse coordinator to provide ongoing supervision of your child’s health.
• Social worker/psychologist to support your family and help with adjustment issues.
• Geneticist to help parents and adults with laryngeal cleft understand the chances of having more children with these conditions.
• Gastroenterologist to manage any reflux symptoms, as this improves the rate of success with cleft repair.

How is laryngeal cleft treated?

Laryngeal cleft typically requires surgical correction called laryngeal cleft repair, especially for types II, III and IV. Sometimes, type I resolves itself as your child grows. People with type I may only require medication to prevent reflux and aspiration.

The timing and type of surgery vary depending on the specific laryngeal cleft. Surgeons may repair them using:

• Minimally invasive injection placement: Your surgeon will place a temporary filler in the area of the cleft. This generally lasts one to three months. This helps identify the impact of the laryngeal cleft in swallowing. In some cases, no additional treatment is needed.
• Minimally invasive endoscopic surgery: Surgery usually takes about 60 to 90 minutes. Your surgeon typically uses a laser to remove abnormal tissues in the cleft, then closes the cleft opening with sutures (stitches). Your child is under anesthesia (asleep) throughout the procedure.
• Open surgery: Your surgeon repairs the cleft through an incision in your child’s neck. It’s usually the typical treatment option for children with a type IV laryngeal cleft.

Are there complications or side effects of laryngeal cleft treatment?

Risks involved with laryngeal cleft surgery include:

• Abnormal tightening of your child’s esophagus.
• Breaking or opening of the sutures.
• Injury to your child’s nerves attached to their larynx.
• Mediastinitis (swelling of your child’s chest area between their lungs).
• Surgical emphysema (presence of gas under your child’s skin).
• Dysphagia (difficulty swallowing).
• Tracheomalacia (collapse of your child’s airway while breathing).
• Noisy breathing.

How long will it take my child to recover from laryngeal cleft treatment?

After surgery, your child will stay in the hospital for at least one to two days. Because surgery involves sutures to close the cleft, it takes a few weeks or months to heal completely. In about 11% to 50% of cases, secondary surgeries or procedures are needed.

How can I prevent a laryngeal cleft in my baby?

Researchers don’t know what causes a laryngeal cleft, so they don’t know how to prevent it. The best thing you can do is follow your healthcare provider’s recommendations for prenatal care:

• Take a prenatal vitamin.
• Avoid drugs, alcohol and smoking.
• Eat a nutritious diet.
• Exercise regularly.

What is the outlook for a laryngeal cleft?

With early diagnosis and proper treatment, the outcome for children with a laryngeal cleft is good. Those with type IV have a higher chance of needing more than one surgery and longer hospitalization. Your child’s healthcare provider will talk to you about your child’s prognosis and follow-up care.

What questions should I ask my child’s healthcare provider about laryngeal cleft and its treatment?

If your child is diagnosed with a laryngeal cleft, you probably have questions. Having answers can help set your mind at ease and prepare for the road ahead. Questions to ask your child’s healthcare provider include:

• Why are you recommending this treatment or procedure?
• What can I do to make my child more comfortable before and after surgery?
• How long will my child need to stay in the hospital?
• What type of incision will be used, if any?
• What medications will my child need, if any?
• What are the risks of my child’s surgery and the anesthesia?
• How long will it take for my child to recover after surgery?
• What can I expect for my child’s long-term health?

A note from Cleveland Clinic

Laryngeal cleft is a rare defect that happens spontaneously in the uterus in the early months of pregnancy. This condition is present at birth. Early diagnosis and prompt treatment offer the best chance for success and a lower risk of complications. If you think your child might have a laryngeal cleft, see your child’s healthcare provider right away.