top of page

BackTable / ENT / Article

Type 1 Laryngeal Cleft Repair

Author Julia Casazza covers Type 1 Laryngeal Cleft Repair on BackTable ENT

Julia Casazza • May 22, 2024 • 31 hits

Though uncommon, laryngeal cleft disposes patients to dysphagia, difficulty handling secretions, aspiration pneumonia, and in severe cases, airway emergencies. Laryngeal cleft is graded on a scale of 1-4, with 4 being the most severe. While type 2-4 clefts typically require surgery, symptoms caused by type 1 clefts sometimes improve with conservative management. If conservative management fails, injection laryngoplasty or laryngeal cleft repair surgery may fix the underlying abnormality.

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

• Children with type 1 laryngeal cleft may respond to conservative treatment, which consists of thickened feeds and optimization of comorbid conditions. If symptom relief is insufficient, diagnostic laryngoscopy with bronchoscopy (DLB) may be necessary.

• Coordination with colleagues from GI, pulmonary care, and speech language pathology is mandatory before any DLB.

• Injection laryngoplasty for laryngeal cleft involves insertion of hyaluronic acid or hydroxyapatite into the interarytenoid space to fill the cleft.

• Surgical repair of laryngeal cleft is the only definitive treatment for this abnormality.

• Children who undergo surgical repair of laryngeal cleft should be treated post-operatively with PPIs, inhaled steroids, and bronchodilators.

Type 1 Laryngeal Cleft Repair

Table of Contents

(1) Conservative Management of Laryngeal Cleft

(2) Laryngeal Cleft Repair

(3) Post-Operative Care Following Laryngeal Cleft Repair

Conservative Management of Laryngeal Cleft

Initial management of type 1 laryngeal cleft is non-operative. Assessing for cardiac, respiratory, and developmental risk factors can identify comorbid conditions, such as GERD, where comorbidity management can improve swallow function. Conservative treatment includes thickened feeds, which allow many patients to achieve optimized growth and functioning. Patients who present after an airway emergency or experience inadequate symptom relief after a trial of thickened feeds should undergo direct laryngoscopy with bronchoscopy (DLB) to illuminate airway pathology.

[Dr. Hamdy El-Hakim]
Clinical examination, on its own, is never enough, and that is an established benchmark of practice. Then we meet together in the aspiration clinic, no less than half an hour consultation, going through a standard set of questions and elimination of risk factors, inquiry about cardiac, respiratory, developmental, the rest of it. If we can get away after the FEES by introducing a thickened diet, a modified diet, or some advice in terms of pacing and so forth, then that's good, we're going to re-evaluate back again and see how does the child do.

That includes preliminary assessment and treatment sometimes of gastric esophageal reflux disease, bearing in mind that our glorious PPIs are now uncovering their ugly face in terms of side effects and so forth. The next step, I never really stage endoscopy and surgery. If I am failing with conservative or medical treatment, I will go and scope. I will never delay an endoscopy if the child has already dangerous symptoms like blue spells or apparent life-threatening episodes, or they failed within a short period of time if you were getting recurrent pneumonias and chest infections, or if they have an alternate route of feeding.

Because one of the main objectives of any clinic or any program is to reduce and shorten as much as possible the period of non-oral feeding. Then I will go and tell the parents, depending on my findings, "Look, I've seen a mobility disorder, I'll see what I can do on the day, of reasons." Or, "I am suspecting that we have a posterior problem over here, and I need to augment that area." I give them an overall view of the two main methods, whether it's a surgical repair or an injection. I quite often will say, "There is a possibility of a GI specialist coming in the same time."

I quite often will take bronchoalveolar lavage as well in the same time. The jury are out in terms of the utility of the yield, whether you really depend on the identification of lipid-laden macrophages or not, whether you have a child who has oropharyngeal flora and way more than contamination in the lower airway. Trust me, the interpretation of this in the literature and experience is at least heterogeneous. At the end of the day, I try and limit trips. I try and combine the endoscopic examination. I will do the airway examination to start with first with agreement with the respirologist. In follow-ups, should the child have adequate examination and treatment by otolaryngology and the remainder is a substantial plan for respriology, then they go and do subsequent lower airway examination. That, in my opinion, is more economical in terms of time, effort, and grouping of people together.

[Dr. Gopi Shah]
Yes, every once in a while I'll have a young newborn that's admitted within the first three to six weeks of life with, a blue spell. They get admitted. It's noted that they're having feeding difficulties. They see speech and have a feeding evaluation and, there's a concern for aspiration. We get consulted on the inpatient side and then NICU usually. If we're not making progress, we don't have a good feeding plan, then we end up doing these DLB evaluations while they're inpatient. At that time, do you usually also go ahead and talk to your GI colleagues if you are planning a DLB? It makes sense to me on the outpatient side, with a structured aspiration and feeding clinic because that partnership and that system is set up for it. Every once in a while, the consult is outside of that system.

[Dr. Hamdy El-Hakim]
No, it makes sense. Most definitely. Particularly the age range that you're talking about, Gopi, is tricky because you don't have enough history to tell whether the child has esophageal problems. There are some that will do. I remember a CMV-infected child, congenital CMV child, who basically had raging reflux and that was one of the very few slam-dunk situations where a fundo has made a world of difference without any problem. That is the other factor that you occasionally may see on a VFSS. You don't see an oropharyngeal abnormality, meaning aspiration from above, but an aspiration from below with regurgitation of the bolus and entry in the airway.

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

Earn CME

BackTable CMEfy button

Stay Up To Date

Follow:

Subscribe:

Sign Up:

Laryngeal Cleft Repair

Children whose symptoms do not improve with conservative management should be referred for operative repair of their laryngeal cleft. Of note, children with clefts graded 2-4 will require surgical repair. Children with grade 1 clefts often benefit from gel injection or surgery. Since a pre-operative DLB is required to plan for either procedure, Dr. El-Hakim often consents his patients for laryngeal cleft repair when they go in for their DLB to minimize the number of OR trips.

During gel injection, the surgeon injects hydroxyapatite or hyaluronic acid into the interarytenoid space, thus closing the cleft. In his experience with gel injection, Dr. El-Hakim finds that half of patients need no further treatment and the other half require subsequent injections or surgery. During surgical repair of a laryngeal cleft, the larynx is incised using cold steel or laser, and then the larynx and the esophagus are each sutured closed.

[Dr. Gopi Shah]
How do you decide when you're going to do a formal repair or when you're going to do an augmentation? For augmentation, I think I always think of the gel injection augmentation. Is that what you use as well?

[Dr. Hamdy El-Hakim]
I have started certainly with injections and the real reason is that historically we were all skeptical about the role of the laryngeal cleft type one or the deep inter-arytenoid notch in the whole process. Material has evolved I used once gel foam and I discovered soon that it's close to a waste of time because it oozes out and it doesn't really get retained in the tissue for too long. Then I started to use hyaluronic acid essentially availability and this was available because the urologists use it. It's smooth, it's quick, it's present.

Its history of biocompatibility is there but I soon discovered that 1 in 10 of those children will have croup-like symptoms afterwards and they may have it for along with strident for a good few days. Two or three are memorable, one of them ended up with a seroma. When I looked into the literature, I usually say to myself these materials have been used or that suture have been used or that instrument have been used in other specialties let's see what others have found and you can see in the cosmetic literature how hyaluronic acid can because seromas and reactions in tissues facial tissues and other places where they where it gets used.

Now my colleagues who use other materials including hydroxyapatite say that they do not encounter that problem. The advantage remains in terms of speed and simplicity but the problem of injection is that it's imprecise. You don't know exactly where and how much. It's not like a pocket of space that will retain it and you pump it up. It's not like your car tire, it can go in different areas. 1 in 10 problems that I just mentioned the evolution of any single surgeon and evolution of the specialty I think have impacted our approach and our attitude towards repair.

Repair in the olden times was done in the absence of enough choice of intravenous agents of anesthesia so people used inhalational anesthesia or situations or conditions where there is no spontaneous respiration and that lengthened the procedure. The instruments were definitely less precise at the time. Many of those children ended up in intensive care and intubated. We all forget that the end of the '80s and the '90s have seen the introduction of intravenous agents and flexible and even rigid scopes that are way more precise than that.

The next hurdle is really about technique and I was not really-- I was properly trained in six children's hospitals but we didn't do repairs over there at the time and the interest in swallowing had not caught up so I self-taught and I learned about ergonomics. I learned about which suture is going to be easier for me to use slippery versus non-slippery wetting my instruments wetting, the larynx that's going to dry up during your procedure. My first one took about maybe a couple of hours until I took two or three sutures, now, it's 20 minutes and 70% of my children go home on the same day, and zero end up intubated unless obviously, we're talking about a larger cleft or a deeper cleft.

I tell the parents, look, if I inject you're going to come back again and across the board, it's 50 to 60% response rate, 50% of them temporary and the rest permanent, whereas, all the evidence is coming confirming somewhere between 75 to 85% good success with repairs. Do some of them break? 100% but we don't know how many we don't know, which technique.

Post-Operative Care Following Laryngeal Cleft Repair

Post-operative management following surgical repair of laryngeal cleft is highly personalized. Patients continue on a thickened diet in the recovery period. Dr. El-Hakim places all of his patients on a two week course of PPIs, so that reflux cannot impair wound healing. He also prescribes bronchodilators and inhaled steroids on an individual basis. He sees all patients back 8-10 weeks post-surgery. At this appointment, he assesses symptom burden and transitions them to thinned feeds.

[Dr. Gopi Shah]
Then what is part of your postoperative management? Are these patients on PPIs? Do you send them home with a steroid taper, or a modified diet, or anything like that?

[Dr. Hamdy El-Hakim]
The diet depends on the result of the FEES, and it doesn't get changed immediately. Postoperatively, if they are on PPIs, they will continue on PPIs. I make sure that if the child has had pneumonias and wheezing, that they basically have been, let's call it, cleaned enough. Some bronchodilators and inhaled steroids are given beforehand, and they continue afterwards, and that varies, that gets individualized.

If they have not been on PPIs before, I only give them two weeks' worth of it. This is a tiny wound at the end of the day. your tonsillectomy heals completely after two weeks. That will probably heal in a handful of days. I don't really give a whole host of instructions to the parents. What is what you get in the immediate postoperative period? I never really repeat the assessment before eight to ten weeks. I used to try and be over-optimistic, and I think most surgeons actually have confirmed that. At 8 to 10 weeks, they come back and see you. What happens in your clinic? How do you assess them? What's a good outcome? What outcomes are you looking at? What's your metrics?

[Dr. Hamdy El-Hakim]
Good question. I tell the guys in training that I am autistic about how I assess and how I do it back again. I have a drill of questions. It's a smart phrase that I put in our electronic medical record system, and there is an equivalent ask for all when they come back, in addition to asking whether they had problems afterwards, including pain, readmission, and so forth.

We basically go and say, okay, how do you feel right now? If the child is still on a modified diet, then we really need to thin that during the examination and repeat the FEES or the modified period as well. If the child is on a non-modified diet, sometimes the parents would come and say the choking has gone down and so forth. I would caution, I always went along with, or initially, I went along with what the parents have seen, because at the end of the day, you don't treat a test, you treat the child.

I must admit that the resolution sometimes of the symptoms precedes the resolution of the instrumentals. One has to be skeptic also about the placebo effect. Parents are always hopeful that something is going to happen, child is going to be better. It also depends on the season. You might be doing it in the summer or the spring and the winter has gone, the number of infections are less. By the same token, they might have been ill because you've done it in the beginning of the fall and they have siblings and they've had a few colds and there is an interpretation that it's a doom and gloom.

8 to 10 weeks, I'll say that, and again, I need to remeasure that, it's probably, a response related to your surgery directly within the first six months in most of your successful patients. You get rid of the thickening or you thin it even more, or you work towards a thinner consistency, water type of consistency. The question that you pose is also very important.

If I have a neurologically affected child, and I'm very precise about what's a neurologically affected child, because there are neurologically affected children that their neurologic problem doesn't have anything to do with dysphagia. There's no need for lumping that, but you want as much as possible an oral component to those children. You need to start working it so that the plasticity of the brain doesn't actually get lost in terms of swallowing function.

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.

backtable-earn-free-cme.jpg
backtable-plus-vi-cta.jpg

Podcasts

Navigating Type I Laryngeal Clefts in Children with Dr. Hamdy El-Hakim on the BackTable ENT Podcast)

Articles

Laryngeal Cleft: The Basics

Laryngeal Cleft: The Basics

Topics

Get in touch!

We want to hear from you. Let us know if you’re interested in partnering with BackTable as a Podcast guest, a sponsor, or as a member of the BackTable Team.

Select which show(s) you would like to subscribe to:

Thanks! Message sent.

bottom of page