Laryngeal Cleft

What is a laryngeal cleft?

Babies born with a laryngeal cleft have a gap in the tissues between their larynx (voice box) and esophagus (food tube). The abnormal gap creates an opening between their larynx, which sits atop their trachea (windpipe), and esophagus. This allows food and liquids to enter the opening and travel into your baby’s lungs instead of their stomach.

Laryngeal cleft is a rare birth defect (congenital condition). It’s only present in 1 in 10,000 to 20,000 live births. It’s slightly more common in male babies.

If your child is born with this condition, it’s important that they get diagnosed as soon as possible. Food and liquids traveling down the wrong pipe can cause all sorts of issues. But how serious a laryngeal cleft is depends on the size and location of the gap.

Types of laryngeal cleft

There are four main types of laryngeal cleft:

• Type I. With the mildest form of laryngeal cleft, the opening is to your child’s vocal cords. This type often doesn’t cause significant symptoms and isn’t diagnosed right away.
• Type II. The opening extends below your child’s vocal cords.
• Type III. The cleft is larger and extends beyond your child’s larynx and into their windpipe.
• Type IV. The most severe form of laryngeal cleft extends further down your child’s windpipe. Sometimes, the opening goes all the way down your child’s windpipe.

What are the symptoms of a laryngeal cleft?

A laryngeal cleft can make it harder for your child to eat and breathe. Symptoms include:

• Choking or coughing during feedings
• Trouble swallowing
• Bluish skin tone (in babies with darker skin tones, cyanosis is easier to notice in their gums, lips, nails and around their eyes)
• A harsh, raspy, wet, breathing sound
• Behavior and sleeping issues due to constantly feeling hungry
• Not gaining weight or growing on schedule
• Aspiration (when food or drink goes down your child’s windpipe, which may lead to lung infections)
• Frequent bouts of pneumonia

What causes a laryngeal cleft?

Researchers don’t know the exact cause of a laryngeal cleft. But it likely develops during the first few months of fetal development. It can happen on its own or as part of an underlying syndrome like:

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

More than half of children with laryngeal clefts have other congenital conditions, such as:

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

How is a laryngeal cleft diagnosed?

Milder forms of laryngeal cleft, like types I and II, may not get diagnosed for years. Healthcare providers usually diagnose types III and IV within the first few days of a baby’s life because of how severe their symptoms are.

If your child has laryngeal cleft symptoms, an otolaryngologist (ear, nose and throat doctor) may perform an endoscopic evaluation. The most common method for laryngeal cleft diagnosis is a microlaryngoscopy and bronchoscopy. Your child will receive anesthesia that puts them to sleep for the procedure. The otolaryngologist will then insert a camera into your child’s windpipe. They’ll use an instrument to feel for a cleft.

Your child may need to meet with a speech-language pathologist (SLP). This specialist will check how their condition impacts their ability to speak and swallow.

How is laryngeal cleft treated?

Laryngeal cleft typically requires surgery called laryngeal cleft repair. This is especially the case for types II, III and IV. Sometimes, children compensate for type I as they grow. Children with type I may only need medicines to prevent reflux and food going down the wrong tube (aspiration).

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

• Minimally invasive injection placement. Your child’s surgeon will place a temporary filler in the cleft. It can provide symptom relief (typically up to three months) until your child is a candidate for surgery. Sometimes, it’s the only treatment needed.
• Minimally invasive endoscopic surgery. The surgeon typically uses a laser to remove abnormal tissues in the cleft. Then, they close the cleft opening with sutures (stitches).
• Open surgery. The surgeon repairs the cleft through an incision in your child’s neck. It’s the typical treatment option for children with a type IV laryngeal cleft.

If your child needs surgery, their healthcare provider will give them anesthesia to put them to sleep beforehand. They won’t feel any pain.

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
• Noisy breathing or laryngomalacia
• Injury to the nerves attached to your child’s larynx
• Mediastinitis (swelling of your child’s chest area between their lungs)
• Surgical emphysema (presence of gas under your child’s skin)
• Tracheomalacia (collapse of your child’s airway while breathing)

The chances of a complication depend on lots of factors, including the type of procedure and cleft, and your child’s overall health. Your child’s healthcare provider will discuss this with you beforehand.

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 a day or two. Because surgery involves sutures to close the cleft, it takes a few weeks or months to heal completely.

What is the outlook for this condition?

With early diagnosis and treatment, the outcome for children with a laryngeal cleft is good. According to a recent study, 9 out of 10 children who receive laryngeal cleft repair experience improvements within six weeks. But much depends on how advanced the cleft is. 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 is the survival rate for laryngeal cleft?

For most children, having a laryngeal cleft doesn’t impact how long they’ll live. But some babies who have advanced type IV laryngeal cleft and other severe congenital conditions may not survive. But fatalities related to laryngeal cleft — even when it’s advanced — are extremely rare.

With proper treatment and follow-up care, a laryngeal cleft doesn’t have to shorten your child’s life expectancy or reduce their quality of life.

When should I see my child’s healthcare provider?

After surgery, your child’s provider may recommend follow-up appointments every six months up to a year. How many visits your child needs within that time depends on how they’re doing.

During these appointments, your child’s provider may do imaging tests and ask about their symptoms. Come prepared to speak for your child. Discuss any changes you’ve noticed related to their symptoms and overall health.

What questions should I ask my child’s healthcare provider?

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?
• What are the risks of my child’s surgery?
• How long will it take for my child to recover?
• What can I expect for my child’s long-term health?