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Laryngeal Cleft: The Basics

Author Julia Casazza covers Laryngeal Cleft: The Basics on BackTable ENT

Julia Casazza • Jun 18, 2024 • 40 hits

At the beginning of embryonic development, the larynx and esophagus develop from one tube. When the larynx and the esophagus fail to split completely into two separate tubes, laryngeal cleft results. Workup of a laryngeal cleft includes assessment of the cleft severity (graded 1-4), management of comorbid conditions (such as GERD), and in select patients, investigation of potential genetic causes. Pediatric airway surgeon Dr. Hamdy El-Hakim of the University of Alberta, shares his insight into this condition on the BackTable ENT podcast.

This article features excerpts from the BackTable ENT Podcast. We’ve provided the highlight reel in this article, but you can listen to the full podcast below.

The BackTable ENT Brief

• The esophagus and larynx split off from each other during weeks 4-8 of embryonic development. Laryngeal cleft results when the esophagus and larynx fail to divide completely.

• Laryngeal clefts are graded on a scale of 1-4, with 4 being the most severe. Clefts graded 2-4 typically require surgical repair.

• Symptoms of laryngeal cleft include dysphagia, choking episodes, and difficulty handling secretions. Recurrent aspiration pneumonias are also common.

• Laryngeal cleft can occur as an isolated condition or as part of a genetic syndrome. Children with dysmorphic features benefit from a medical genetics workup.

• Fiberoptic endoscopic evaluation of swallowing (FEES) is the gold-standard test for assessing airway anatomy in a child with suspected laryngeal cleft.

Laryngeal Cleft: The Basics

Table of Contents

(1) What is Laryngeal Cleft?

(2) Laryngeal Cleft Symptoms

(3) Examining Laryngeal Cleft

What is Laryngeal Cleft?

During embryonic development, the esophagus and trachea develop from one tube. Around weeks 4-8 of embryonic development, a septation begins to split the two structures [1]. Laryngeal cleft results when the connection between the esophagus and trachea remains patent at some level. Grading of laryngeal clefts encompasses four types, with type 4 being the most severe. Type 1 clefts are supraglottic, type 2 clefts extend below the true vocal folds, type 3 clefts involve the cervical trachea, and type 4 involve the thoracic trachea. Type 1 clefts are by far the most common.

[Dr. Hamdy El-Hakim]
I usually start by saying that the esophagus and the trachea or the windpipe and the gullet initially were one project, and they usually decide to split and go their own ways in terms of the role for swallowing and breathing. Laryngeal cleft is essentially a low ramp in between the two entries, and it can get deeper and deeper, and the lesser fortunate are the ones who have a deeper one.

In terms of swallowing, I always like to explain to them that swallowing is a sequential domino-like process that basically starts in the mouth and ends up hopefully in the stomach safely. It has many factors that affect it, and we really need to take a holistic approach to the child. We need to make sure that breathing and swallowing are taken for granted and smooth as vision and hearing and a heartbeat. In terms of the structural abnormality, I don't talk about the structural abnormality until I find one.

[Dr. Gopi Shah]
In terms of classification, if you're teaching the residents or your fellow, how do you describe the classification?

[Dr. Hamdy El-Hakim]
I think the classic classification into four grades. I know that there are sub-classifications, but at the end of the day, the golden rule is never to depend on a flexible endoscopy. You have to do a rigid endoscopy. You have to instrument. It takes really no skill at all to pick grades two, three, and four, and lucky enough, they are pretty rare. If somebody talks about vast experience, I think they'd be lucky or maybe unfortunate. In terms of laryngeal cleft type one, it always reminds me of the myth of Loch Ness Monster to some extent. Some people say that they have seen it for certain, and others say it was the effect of the Scottish whiskey.

Listen to the Full Podcast

Navigating Type I Laryngeal Clefts in Children with Dr. Hamdy El-Hakim on the BackTable ENT Podcast)
Ep 169 Navigating Type I Laryngeal Clefts in Children with Dr. Hamdy El-Hakim
00:00 / 01:04

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Laryngeal Cleft Symptoms

Most commonly, laryngeal cleft presents in 6-9 month old babies. Occasionally, more subtle presentations are detected in younger school-age children. Dysphagia is the primary symptom, which can trigger choking episodes, difficulty clearing secretions, and aspiration pneumonia. Less specific symptoms include frequent throat clearing and post-nasal drip. Owing to their unique aerodigestive anatomy, many of these children also experience sleep-disordered breathing.

[Dr. Gopi Shah]
Going back to symptoms, we think of swallowing problems, we think of, as you mentioned, coughing, choking, sort of watery eyes reflective of a choking episode. Do you ever have other symptoms, like something like throat clearing or, sleep-disordered breathing? Any other symptoms that aren't necessarily "swallowing" or there's more to swallowing than choking as well? Maybe they make a funny face. What else do you think of in terms of symptoms?

[Dr. Hamdy El-Hakim]
That definitely is an interesting one because you've mentioned snoring. Half of my first 140 children snored, and I don't touch their tonsils and their adenoids. Granted, a good number of them, about 65% of this cohort had an airway abnormality. They had either tracheal stenosis, believe it or not, or malacia or subglottic stenosis. After I work also on the swallowing, quite often without an airway abnormality being corrected, the parents tell me the child has stopped snoring. The lesson is that sleep-disordered breathing can actually be mediated, not necessarily by an obstructive lesion, but by a compromise to respiratory reserve.

In terms of the other symptoms like throat clearing, now that is a nebulous symptom. It can be because of many reasons. Most people will also look at post-nasal drip, try and identify another Loch Ness monster, which is gastroesophageal reflux disease, and that would lead you to really a cochlea, an endless cochlea of discussions, especially with gastroenterologists who are, obviously, the masters of the art and the benchmark creators for it. I'm careful a little bit about those. It usually is more in an older child, so the story will have been forgotten where was the trigger.

What I worry about, and maybe in context, is the constantly rattly child. This is a child who cannot clear probably saliva. That needs a combination of inquiries. Part of them may relate to esophagitis, definitely. I quite often see it in association with a child who have atypical croup, has a good number of these are associated with eosinophilic esophagitis or motility problems in the esophagus. I must admit our center has not really invested enough in motility investigations, partly because the age group does not really lend itself to a motility investigation and a motility test. There are centers in the United States who are far ahead in that respect.

Examining Laryngeal Cleft

Physical examination of the child with suspected laryngeal cleft can determine cleft severity and associated abnormalities. Laryngeal cleft can occur as a part of genetic conditions, including VACTERL association, CHARGE syndrome, Optiz syndrome, and Pallister-Hall syndrome. Accordingly, clinicians should assess for muscle tone and dysmorphic features; abnormal findings may warrant a genetics referral. Chest auscultation can clue the clinician in to potential lower airway involvement.

Functional endoscopic examination of swallowing (FEES) provides a dynamic view of aerodigestive anatomy and secretion handling. If FEES is unavailable, a modified barium swallow study (MBSS) can be used in these patients, though MBSS provides a less nuanced view and requires radiation exposure.

[Dr. Hamdy El-Hakim]
A general exam is a good idea, and I think pediatric otolaryngologists need to always understand that they are dealing with children who need some form of developmental assessment, and their communication, their general demeanor tells them a lot. A child who is not feeding properly is a child that is not generally very happy, not very relaxed. Subtle dysmorphic features are pretty important. You may be dealing with a syndromic child. You're not going to diagnose the syndrome quite often, but you can tell if the child has some dysmorphic features related to your area.

The other feature is testing for tone, because if the child has a low tone in the end, you can expect a few problems. The second issue is after you ensure that you have an oral cavity that is within average, that you have gone through the nose and you expected patency before you leave the nose you should, I feel that whereas the young child, you cannot always tell a submucous cleft trans-orally you can actually tell that the child has a submucous cleft with a telescope a lot better. Because you're going to see the nasal septum rather than inserting itself at right angle onto the floor of the nose, it will be flying in the breeze and creating a bit of an acute angle. You will see that at the nasal side of the soft palate.

FEES to me is the queen. Not biased towards the kings and males. The queen of all examinations. You really need to see whether you have an open and patent velopharynx or not, and competent one. Sometimes, the first thing that is secretions inside the nose, saliva as a matter of fact forming, not discharge. Certainly if a little bit of in fact secretions in the nose especially in an infant, this is not a usual finding. This is telling you that there might be some nasopharyngeal escape. Then when you look at the larynx, in an infant in particular, we all know that the larynx is a bit high and the epiglottis usually gives you the high five.

Definitely before you even notice the base of tongue, you should see the epiglottis. If the epiglottis being hugged by the tongue base and the tongue base is slightly higher, your odds of finding an anterior larynx is pretty high. That particular risk factor, if you ask about risk factors, that is almost, in my opinion, a slam dunk. You're going to find a problem that needs a bit of rehabilitation there. The way the larynx and the hypopharynx handles salivary secretions is very important. There are no benchmark classifications. There is one from the adult world, purely subjective as any other endoscopy, and then you need to draw on your experience in laryngology.

The FEESer needs to be really an accomplished laryngologist. The child is not always incredibly happy, although we have advanced. Initially, we never tried with toddlers, but now, we do it with them and we have our own ways of entertaining them and convincing them diplomatically that we're doing well and we mean well. You determine whether you have malacia or not, you determine whether you have a movement disorder or not. I use that word in particular rather than talking about paralysis. Then you start your show with the feed, which the child really needs to play ball with it, and some of them definitely will say, "Not today. Don't try."

I do not forget to auscultate. That's something that we forget as an art. It tells us a few things. Certainly tells us whether the lower airway is affected or not, it tells us whether it's purely upper airway, not purely of course, but it definitely does help. A good proportion of my patients also were dysphonic, and that's the other thing, that the parents will quite often tell you, upon asking, "The child's voice is weak."

Podcast Contributors

Dr. Hamdy El-Hakim discusses Navigating Type I Laryngeal Clefts in Children on the BackTable 169 Podcast

Dr. Hamdy El-Hakim

Dr. Hamdy El-Hakim is the director of pediatric otolaryngology fellowship at the University of Alberta in Canada.

Dr. Gopi Shah discusses Navigating Type I Laryngeal Clefts in Children on the BackTable 169 Podcast

Dr. Gopi Shah

Dr. Gopi Shah is a pediatric otolaryngologist and the co-host of BackTable ENT.

Cite This Podcast

BackTable, LLC (Producer). (2024, April 30). Ep. 169 – Navigating Type I Laryngeal Clefts in Children [Audio podcast]. Retrieved from https://www.backtable.com

Disclaimer: The Materials available on BackTable.com are for informational and educational purposes only and are not a substitute for the professional judgment of a healthcare professional in diagnosing and treating patients. The opinions expressed by participants of the BackTable Podcast belong solely to the participants, and do not necessarily reflect the views of BackTable.

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