Podcast Transcript: Navigating Type I Laryngeal Clefts in Children

[Dr. Gopi Shah]
When patients come to you, how do they usually present? What symptoms will these patients have?

[Dr. Hamdy El-Hakim]
That's probably the very important starting point in even science and research because it's a no-brainer usually to tell if there is an ear problem or a nose problem because, at the end of the day, it's symptom-specific. The symptom leads you quite comfortably to the organ unless there is a referred sensation, which we all get taught in our basic years. With swallowing and breathing, you have a common, let's call it cavity, which is the hypopharynx and the larynx. They are so intertwined anatomically and neurally, and you cannot always, especially in the classic age of presentation, tell which is affected and which is not.

Plus there is a little bit of a myth more or let's call it culture and education-specific, culture-specific interpretation of symptoms of a child who is maybe choking, maybe having problems during mealtime, between pickiness, refusal, behavior difficulties, sensory problems. You can get a child who has simple problems like coughing and watery eyes up to blue spells, limp episodes, or recurrent pneumonias. Even with the latter, you find that many people ascribe all of this to infections, run-of-the-mill.

It's the period of infancy and toddlerhood where you expect a bit of immune development and an immune education. People accept to some extent that there could be an RSV there or an adenovirus here, but it's the frequency. A healthy child should sit at mealtime and get funny photographs of smudging everybody and everything, but it ends up with a long drama, a bit of ritual in order to get this little one fed adequately and the mealtime over.

[Dr. Gopi Shah]
It's interesting. I think of the younger newborns and infants, when I think of patients that might present with a blue spell or they come into the ER with having had to call the EMS because they turned blue at home, and now they're in the ER, and now we're getting into some of those questions about how they feed. Do you find that some of the symptoms differ by age? What do you find in your toddlers or even your older kids?

[Dr. Hamdy El-Hakim]
This is a tricky one because from research across the board, you'll find that the age of presentation is within the first probably six to nine months, and that also gets adjusted when you extrapolate it to the date of surgery. The date of surgery or bronchoscopy is usually around year one, which means essentially that there is time lag and delay. The older ones usually will have some form of a relationship to a lucky respirologist, and preschool asthma is a common tag for those children.

As a matter of fact, my first successful laryngeal cleft repair was a little girl who was five at the time, and she spent her first five years with monthly visits to the Stollery Emergency Department. After we did that repair, and I must admit that I skeptically has performed it, the family reported a dramatic improvement, and that was my conversion.

[Dr. Gopi Shah]
That's amazing. Have you ever found a laryngeal cleft unidentified in like a older child, like 8 or 10, 6 year old? Or is that just at this point now we know enough about it, we have enough studies, better understanding of swallow, so we tend to catch it earlier?

[Dr. Hamdy El-Hakim]
I think we're too early to say that we have established a knowledge base and the practice base across even in North America, which is a little bit more ahead, further ahead of the rest of the world in terms of aerodigestive practices. The argument is always, you sometimes see laryngeal clefts without symptoms related to it. That means that you have brought the child from the street and put them under anesthetic and did the bronchoscopy because they didn't have any symptoms. To some extent that narrative is hard to buy.

[Dr. Gopi Shah]
Do these kids have risk factors? Do these babies, have you found that it's more common in our term babies or, hey, this is actually more common in our preemies or?

[Dr. Hamdy El-Hakim]
I like to draw the parallel with sensorineural hearing loss. We basically have a swath of patients who have sensory hearing loss and they do not have any other neurological problem. We have a good drill in terms of saying whether they were preemies, cardiac, had meningitis, et cetera. The same applies for the patients with swallowing problem. Again, we have to understand that swallowing problems are not as clearly classified and identified as sensory versus neural and even conductive hearing loss.

To lump and start to control and identify risk factors that are directly responsible aside from famous midline abnormalities, like I, yesterday, was in the OR looking after one patient and the general surgeon was going to examine a TE fistula patient and operate on them. They said, "As long as you're here, why not perform a bronchoscopy?" I had a look, which in many situations and in many centers, pediatric general surgeons will actually perform the examination.

I located the TE fistula for him, but there was a type 1 cleft. Now, that is so subtle and outside the scope of their practice, no disrespect, because they are not laryngologists, and they will miss many of those. In terms of other risk factors, I can't really tell that a laryngeal cleft in particular is my main focus. My focus is more into what we call swallowing problem and dysphagia. I don't think I am that as obsessed by finding and detecting the laryngeal cleft as much as I am trying to explain why can't that child swallow.

[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.

[Dr. Gopi Shah]
Just to recap, in terms of risk factors, we're always thinking of possible associated tracheoesophageal fistula, potentially other secondary airway abnormalities. You said half your cohort had tracheal stenosis as well as subglottic stenosis. Then in terms of other GI comorbidities, we think of EoE plus minus GERD and esophageal motility disorders as well.

[Dr. Hamdy El-Hakim]
Correct. Again, I'm going to flip the argument again. By that I mean, how am I supposed to manage the child who has an oropharyngeal swallowing issue? Having taken care of oral problems, put them on the right track with the right professionals, speech and language pathologists, dietitians, and inquired to the best of my ability on esophageal problems. If I am dealing, and I know that I'm dealing primarily with an oropharyngeal problem, then a FEES or a modified barium swallow or a combination will identify for me the pattern of the swallowing problem. I know that we have the homing on whether it's penetration or aspiration.

For some purists, it's the penetration or aspiration scale. The brilliant idea that was proposed, if memory serves me, from Seattle, about the physiological cleft. There are children during FEES who can show premature spill only, who can show residues after a pharyngeal squeeze and swallow. There are others who may show penetration or aspiration from all borders of the larynx, be it an epiglottic undercoating or a spill-over aryepiglottic folds, or actually the posterior penetration or aspiration again, question mark, silent versus non-silent.

I think that the augmentation procedure, being an injection or a repair, should be directed at those primarily who have a posterior penetration or aspiration at the discretion of the surgeon. It depends on your aims, on your achievable aims. We don't always, in a child with cerebral palsy or a hypoxic-ischemic encephalopathy, achieve full oral FEES with surgery because it's too multifactorial. It makes sense to have an appreciation that you want to advance the FEES, you want to come close as possible to oral feeding. Even if it stays for some enjoyment, some degree of rehabilitation, a gradual one can actually flow.

Generally speaking, I think that most children, you will reach a ceiling with them by age around the age of two, and then if there is anything else that will come later, it really is a guess that is 50-50. You can't really tell which direction is it going to go through. I generally like to tell the parents that one of the main objectives is to protect their lungs, particularly in the first two years of life, so that we don't have a respiratory cripple.