BackTable / ENT / Article
Mandibular Distraction to Treat Micrognathia: Indications & Techniques
Megan Saltsgaver • Updated Aug 30, 2024 • 50 hits
Micrognathia (small jaw) in infants often requires surgical intervention, particularly if the condition interferes with the child's breathing or feeding. Mandibular distraction is a common surgical procedure for the treatment of micrognathia in which hardware is placed to lengthen the mandible, thereby alleviating micrognathia symptoms. A trip to the operating room for mandibular distraction requires careful planning, coordination with the family, and significant time after the procedure for the distraction process to take place.
Pediatric otolaryngologist Dr. Brianne Roby explains her approach to mandibular distraction in the pediatric micrognathia patient, including indications, airway management, and the post-operative distraction process. This article features excerpts from the BackTable ENT Podcast. We’ve provided the highlight reel in this article, and you can listen to the full podcast below.
The BackTable ENT Brief
• Mandibular distraction, also called jaw distraction, is a surgical procedure that lengthens the jaw to improve breathing and/or feeding of an infant. Mandibular distraction should not be used for cosmetic reasons.
• Difficult airways and intubation should be assumed in micrognathic children. There must be multiple intubation options available before starting to intubate.
• External or internal hardware must be placed before the mandibular distraction process can begin. Both options have their pros and cons. Internal hardware is more discreet and leaves less of a scar, but requires a trip back to the operating room for removal, while external fixation has more hardware sticking out, but can be removed in the office.
• With either distraction method, children should be distracted a total of 12-14 millimeters after surgery, at a rate of 2 millimeters per day.
• The success rate for mandibular distraction can be high, especially in non-syndromic or mild syndromic cases, however more complex syndromic children require careful consideration, clear communication with families, and a willingness to adapt the surgical plan based on the child's unique anatomy and post-operative progress.
Table of Contents
(1) Indications for Mandibular Distraction
(2) How to Approach Intubation in Infants with a Small Jaw
(3) Surgical Management of Micrognathia
(4) After Micrognathia Surgery: The Mandibular Distraction Process
(5) Mandibular Distraction Complications in Children with Micrognathia
Indications for Mandibular Distraction
Mandibular distraction is a surgical procedure that can help to lengthen the jaw in a child that has micrognathia. Distraction is indicated in cases where it is suspected to improve breathing and/or feeding in an infant. Most will not do a mandibular distraction on an infant without breathing or feeding issues due to the risk of complications. Cosmetic concerns are not an indication for mandibular distraction. Due to the small surgical area that’s inherent when operating on an infant, Dr. Roby likes to wait until the patient is at least 37 weeks gestational age and ideally 2.45 kilograms or heavier.
[Dr. Gopi Shah]
Now we have a baby, let's say, who does need a distraction. What are the indications? We said feeding, breathing, sleeping. If they don't have one of those and they're not able to get temporized?
[Dr. Brianne Roby]
Yes. Those are it. It's really airway obstruction and feeding for a newborn. I've had kids that have done beautifully and they have mild Robin sequence symptoms, and they're doing great. I've had parents say, can we just do their jaw surgery now so that they won't remember it and we don't have to do jaw surgery when they're older?
The answer is no. I'm not going to do it other than for feeding and breathing symptoms because we don't know what's going to happen long-term with their jaw. There is complications related to doing distraction. Again, it's not a cosmetic procedure. It is an airway procedure in a newborn and trying to make the jaw look pretty symmetric is not one of my indications for doing it.
[Dr. Gopi Shah]
Do you have an age or weight minimum or requirement?
[Dr. Brianne Roby]
At the very least, they should be gestationally full-term. I've done one-- Not one. I've done a number of them that, if you do the 37 weeks is considered full term. They haven't been much older than that, but I've not done any that would be gestationally less than 37 or 38 weeks.
Then in terms of weight, I generally say I would never do anyone less than two kilograms, but the reality of it is usually slightly more than that. We actually just are publishing this myself and a couple other people looking at how small could we go safely. We had a number of kids that were 2.3, 2.35 kilograms. They did great without any additional complications.
I think that, for me, that's about as small as I would go. When you get much smaller than that, there's just no real estate to put the plates for the distraction. You can't find the room to put the plates on anyway. I tend to say roughly at least 2, but probably closer to 2.45 kilograms.
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How to Approach Intubation in Infants with a Small Jaw
Dr. Roby recommends a stepwise approach to intubation of micrognathia patients in the OR. The first step typically involves using a standard laryngoscope, such as the Parsons laryngoscope, which is preferred by Dr. Roby due to its wider blade that better controls the tongue in cases of glossoptosis. Direct laryngoscopy and bronchoscopy is then used to examine the rest of the anatomy to rule out laryngomalacia or laryngotracheal stenosis. If the rest of the exam is normal, then nasal tracheal intubation can be done and is preferred with jaw distraction, or if the child is desaturating, they can be orally intubated.
A GlideScope set should always be handy when working with these infants. It can aid in visualizing the anatomy and be particularly useful if extubation occurs and the NICU doctor needs to re-intubate. Telescopes and flexible fiberoptic nasal intubation can also be employed.
[Dr. Gopi Shah]
So now you have a baby scheduled for the OR. This is where I feel like whether you're doing the distraction or not, as an otolaryngologist, especially pediatrics, you're going to be part of that airway intubation, DLB, ours are always DLB distraction.
Usually, nobody's done-- Most of the time, not usually. Most of the time the baby's never been intubated. We don't know what's going to happen at induction. We know that, eventually, most distraction surgeons want a nasal intubation, I would say most of the time.
We do want to try to look at the rest of the airway before the distraction to check for subglottic stenosis, tracheomalacia, any other secondary lesions. It's very important to have your airway plan before the baby's in the room.
What's your airway plan? What instruments do you like? What's your conversation with your team, your OR team, anesthesia? How does that-- I feel like that's where, as an otolaryngologist, that's a baby we could lose. That's a baby where the airway can go bad, but that's our job there. Tell me what the airway plan is for you.
[Dr. Brianne Roby]
Yes. I would also say, and the first distraction case each fellow does with me, I say, you always assume that this kid is going to have a nightmare airway. It doesn't matter if on the spectrum of severity of Robin sequence, they don't look as bad. You really don't know how they're going to act until they've gotten some anesthesia. The first thing I will say is that this is not a case where you want an anesthesia resident, a first year anesthesia resident to be doing the anesthesia. This is where you want an experienced anesthesiologist, so probably not even one that just started. You want someone who you've worked enough with that you feel comfortable together managing airway stuff.
I've talked to the head of anesthesias before and been like, "I need you to be in this case and not the other person. Not because I don't love the other person, but they're new and I haven't done a bad airway with them yet. They can be in there, but I need you to be in there." I can't emphasize that part enough. Number two, this is not a case where you say, "Well, we'll let anesthesia give it a try. If it doesn't go well, then we'll take over." I think as an otolaryngologist, you should be ready to be in control of the airway from start to finish. Going there, I will say stepwise with our fellows.
Step one, normal, just using a normal laryngoscope, whether it's the Miller-- I always do a straight one, be either a Miller laryngoscope or a Parsons. I do like the Parsons, not for any other DL bronch, except for with kids that have a cleft because the Parsons is a little bit wider. With the tongue and the glossoptosis, I feel like you have a little bit better control with a wider laryngoscope. This is like the one time where I actually will use the Parsons. It's basically, you do the DL.
What do see with that, and a little bit of cricoid pressure, you have a view. I'll have the fellow take the telescope and quick just show me the rest of the anatomy and show me that everything else is normal. Now, for what it's worth, I get really annoyed when people say that babies with glossoptosis also have laryngomalacia. If you want to say that the definition of laryngomalacia is a floppy epiglottis, well, there's no way to have glossoptosis and not have a floppy epiglottis.
After the distraction, if they still have a floppy epiglottis, you can tell me that there's laryngomalacia. At the time of the DL bronch, you can't say that because it's more likely the glossoptosis causing the retroflex epiglottis. Anyway, that's an annoying end on my part because you'll look at data, and it's like, "All these babies had laryngomalacia." Maybe.
If with a normal DL and cricoid pressure, they can get the view, fantastic, great. Do the telescope, do the exam, put the breathing tube in and we'll just put it through the nose and do a nasal trachea intubation. Sometimes if the kid is not doing great, they're starting to desat and we have a great view, I actually will just orally intubate the kid. For me, the nasal tube is nice, but it's not necessary during surgery. If I feel the need to just get the oral tube in, I just do. If that goes great, great.
My next step is I always have the GlideScope set up for these kids, always. The reason why, it's amazing how often that you don't have a good view with just a normal laryngoscope, but you get the GlideScope in there and you can see how anterior it is and all of a sudden you have a really good view. There've been a number of these kids that I've intubated just with the GlideScope.
The reason that matters to me is when you take these babies up to the NICU and you're like, "If this baby suddenly extubates--" By the way, they are easy to intubate with the GlideScope. It is our job to be educating anyone else who might have to deal with this baby's airway. If you can just use the GlideScope, and many of the ICU settings now have them, it's just get the GlideScope, it should be an easy intubation with that. I do always have the GlideScope and I like that a lot.
If that doesn't work, but I can see the arytenoids with a DL, a lot of times these kids I've intubated over a telescope. I always have a telescope loaded with an endotracheal tube ready to go because I honestly think out of all things as an otolaryngologist that will help you get the airway, it's that. If you can do a DL and you can see the arytenoids and get your telescope up the anterior, you can slide in over the telescope and that's a great skill to have. It's what sets you apart, again, from anesthesia and things like that, is the ability to know how to use a telescope and intubate over a telescope.
Last but not least is the flexible fiber optic intubation nasally. This is from my standpoint in a perfect setting. I like this, again, because it's the easiest way to do a nasal intubation. I also think if you are comfortable with a flexible nasal intubation for these kids that maybe even as you get older, I'll give you an example. Last night I got called for an OAV kid who can't open her mouth even two millimeters because she's got such bad trismus and anesthesia couldn't intubate, but I can go do the flexible intubation.
That's, again, that's what sets you apart from an airway standpoint. That's my final step. Last but not least, I always have a trach set in the room ready to go. I don't think you can underestimate having that available. You hope you never have to use it because you hope that with different techniques and maneuvers you're going to be fine without it. That's my stepwise ladder.
Now along those things is talking with your anesthesiologist. I've had a number of kids where the minute we didn't attempt to do the DL and we didn't have a view, immediately turning the baby onto their side and mask ventilating on the side or even prone, bagging them up and then putting them back supine, because a lot of them are going to be a really hard mask. I always have a towel clip on the field and I hate grabbing the tongue, but I've certainly done it before.
These are just little tricks, have everything ready. The one thing I have, I really have emphasized with my fellows is, there are some cases where you give people lots of attempts and you're not worried and they're an easy mask and they're an easy ventilation, and so you keep trying the same thing a few times before you move on. Most of these kids, if I trust my fellows technique or my own technique and I take a look and I can't even remotely get what I need, I don't say, "Okay, well, let me try that again." It's next up the ladder because the longer you spend on these kids, the more likely they are to either have a spontaneous laryngospasm or something, and or because bleeding or something like that.
These are the kids that you don't mess around with multiple attempts. In communicating with anesthesiologists, I flat out say, "Here's my ladder approach. Here's what I'm going to try," so that they know what's going through my mind. We try to keep them spontaneously breathing because they're always going to be able to support themselves better than me, but they always have a paralytic on the field ready to go just in case there's a laryngospasm. Those are the things that, again, you've got communicate it with your anesthesiologist ahead of time.
[Dr. Gopi Shah]
I, in my mind, have a very similar ladder, like what if they're maskable, what are we using to mask to the DL. I like a number one Phillips, which I know every time I might ask for one, especially in a preemie or a infant, a newborn, they're like, "That's so big." Like you said, with the Parsons, it's the same thing. It's never too big. It gets the tongue out the way. That's something that Romaine Johnson is like always get-- I always just use the number one Phillips based on what I learned with him.
Let me ask you a question. Do you ever go out of order in the sense of do you ever just start with the flex through the nose, because you're going to have to intubate or we prefer that. Try to intubate, secure the tube with your flex, maybe, but also been able to look at the airway, and then do a DL, or do you feel like you don't have a good assessment of the airway to then pass on because you have a nasal intubation that's precessing your DL?
[Dr. Brianne Roby]
Yes. The answer to your question is yes, I have gone straight to a flexible fiber optic nasal intubation, but then I do not think doing a DL afterwards is realistic. Do you have something to guide your way? You have something holding the tongue forward. Your brain automatically knows, if I just follow this nasal tracheal tube, I'm going to get to where I'm supposed to be going. I have done it, but then I don't pass off to my NICU that, hey, this is going to be an easy intubation, because if I have a nasal tracheal tube in and then I do a DL with a Phillips laryngoscope and it's like, I'm like, "Oh, this was easy. It's a grade one view."
It's probably not an easy grade one view and my mind is automatically playing just distortion tricks on me. That's the only difference. I don't think it's wrong. I think there are plenty of people who go straight to a flexible intubation and that's their airway assessment. They're looking for laryngomalacia, whether or not they believe that can happen when you have glossoptosis, but they're looking for subglottic stenosis. Without doing true measurements, can you say-- You would notice if there was a super congenital stenosis or looking for tracheomalacia.
They're looking for all those things with a flexible scope and you can certainly do it. I think in terms of me, to me, the bigger purpose of doing like a DL bronch at the beginning is less so looking for secondary lesions, honestly, and more so that I can hand off to teams who might be taking care of this child that this airway is really bad, or this airway is actually not too bad and an experienced anesthesiologist would be able to intubate this child without any help type situation.
[Dr. Gopi Shah]
Now let's say the airway is secured and you're getting set up for your distraction. Do you monitor marge on these babies?
[Dr. Brianne Roby]
I do. Honestly, it always goes off the entire time. To me, you still have to be safe looking for the marge and it's not like other cases where you rely on it to identify it. For me, it's that constant reminder that the anatomy is really tiny and that you just need to be safe in your anatomy. To me, it's just more of that reminder not to take it for granted that the marge is there.
To do the dissection, I automatically find, and it's a little bit challenging in newborns because their submandibular glands are like the size of a pinky nail, but my goal automatically is to go straight, subplatysmal and find that submandibular gland and then go straight up to the mandible, and so then you know that you'd be keeping it safe, but I do monitor it. I think you could easily do this procedure and not monitor it. For me, it's just that reminder.
Surgical Management of Micrognathia
In terms of surgical management, both external and internal fixation options exist. Internal distractors are placed under the skin, with only a small part of the device visible, while external devices have pins that go through the mandible, leaving more hardware exposed on the infant's jaw. The advantage of external devices is their ability to distract in multiple directions, whereas internal devices typically allow distraction in only one direction. For children with Robin Sequence who generally only require anterior-posterior distraction, internal devices are usually preferred. Internal devices often leave a subtle scar, while external devices can result in multiple small scars.
[Dr. Gopi Shah]
There's internal fixation and external. Can you tell us the differences in those?
[Dr. Brianne Roby]
Yes. The internal distractors mean that the plates and the screws and the most importantly, the part of the device that's going to distract, so spread apart when you do the distraction is all under the skin. In those cases, the only thing that sticks out from the skin is where the actual distraction arm is. Most of us put it under the mastoid tip. Some people will actually have it come out anteriorly underneath in the front part of the neck, but most of us go posteriorly.
That's the only part of the device that shows. As opposed to external devices, which has the pins that go through the mandible, but the rest of the distraction arm sticks out as an external fixation device from it. They look like they've got this massive hardware on their inferior border of their mandible for the time that it's there. The benefit to the external devices, you can get it so that it distracts in multiple directions, as opposed to the internal devices, which generally only distract in one direction.
Now for straightforward Robin sequence kids, they usually only need distraction in one direction, and that's just in the anterior posterior direction. I, 95 out of 100 times will do an internal device especially for Robin sequence kids, primarily because nursing care is easier. Nurses aren't so freaked out by it. Parents aren't so nervous about it. I think in the end, the scarring that's from it is much better. The internal devices, the only scar is you have a small incision just in their neck, but you can make that look pretty subtle, and then just the pin site underneath their ear.
For the external devices, you still have to have the neck incision, but you've got these pins that go in essentially on the face near the inferior border of the mandible. When all is said and done, they've got these scars on their actual face. They're small, they're little pin sites, but you can see them. I don't like that scar as much, but primarily I don't like the external device because I just think the nurses get really nervous about caring for it, and parents get nervous about how it looks when the kids go home.
The external device I've used a couple of times for kids that, again, syndromic that maybe their mandible was so asymmetric from one side to the other that I was going to have to distract in multiple directions. That's almost always for the older kids. That's really not likely for a newborn. That would be in the Treacher Collins asymmetry or hemifacial microsomia where you're really having to distract different amounts for different directions. Those are the ones where I'm more likely to use the external devices.
After Micrognathia Surgery: The Mandibular Distraction Process
The mandibular distraction process typically begins 36 to 48 hours after surgery is complete. For newborns, the distraction involves turning the pins twice a day, usually one millimeter in the morning and one millimeter at night, although some surgeons may opt for slightly less. The goal is to achieve a slight underbite or underjet of the mandible, usually over-correcting by about two millimeters to account for future growth. This overcorrection helps ensure that by the time the child is one year old, they have a balanced jaw alignment.
During the distraction process, the infant is typically kept intubated until about four or five millimeters of distraction is achieved, usually around four days post-surgery. At this point, the airway improvement is significant enough to consider extubation, which is often done at the bedside in the NICU. Most infants undergo a total distraction of 12 to 14 millimeters, though this can vary depending on the individual case.
Regarding the removal of distractors, both internal and external devices must be removed, but the timing and process differ. For internal devices, the plates and screws are left in place for about 8 to 12 weeks during the consolidation phase, allowing the new bone to harden. The hardware is typically removed in a quick follow-up surgery, where the same incisions are reopened to extract the plates. Although this requires another trip to the OR, it’s generally a straightforward procedure with an overnight stay for observation. External devices offer the benefit of being removed without reopening the incisions. The pins are simply undone, and the device is slid out.
Once the distraction is complete and the breathing tube is removed, focus shifts to feeding, with the help of speech therapists and feeding specialists. Infants typically show improvement in feeding as the distraction progresses, and most transition well to bottle feeding once the distraction process is complete.
[Dr. Gopi Shah]
You've done the surgery. Do you start distracting or doing the rotations post-update one? How does that work?
[Dr. Brianne Roby]
Once you make the osteotomies, you want to wait a day or two. Most people wait somewhere around 36 to 48 hours, which is what I do as well. To be honest, these surgeries almost always get added on late at night for me. I won't do it the next day, but I'll do it the morning after that. That's usually 36 hours. That's when I start the distraction process.
For newborns, you're usually doing pin turns twice a day. What's interesting is babies can actually make like three millimeters of new bone per day. I don't push it that much. I do two millimeters of distraction a day, a millimeter in the morning and a millimeter at night. I know some people do a little bit less than that. They'll do like one millimeter in the morning and a half millimeter at night or 0.75 millimeters twice a day. They'll just do 1.5. I think their worry is that they won't form as nice new bone, but I've never had an issue. I think I'm in that equal number of people who do the two millimeters a day.
I do two millimeters, one morning, one at night. My goal is to get them so that they have, what I tell parents, is a little bit of an underbite or the jaw, the mandible just has a little bit of an under jet, meaning it's a little bit farther out than the upper jaw so you're over-correcting it. I usually will over-correct by about two millimeters. The reason for this is we know that in Robin sequence, just with the natural growth pattern of the maxilla and the mandible, that the maxilla is still going to catch up and probably correct itself.
If you over-correct by about two millimeters, usually a year later, they're nicely back in a Class I occlusion. If you don't over-correct, what you usually find is that a year or two later, you're like, "God, everything's good. Everything looks great, but I wish--" maybe they have just a really subtle overbite and I really wish I would have distracted them just slightly more. I tell all those parents that their children will look like Jay Leno when we're done and by a year later, they won't notice that any longer.
[Dr. Gopi Shah]
During this process, they're intubated the whole time while you're rotating?
[Dr. Brianne Roby]
No, not the whole time. I usually will keep them intubated until I've distracted around four or five millimeters. That's usually, if you start distraction at 36 hours and you do 2 millimeters a day, it's usually around 4 days after their surgery that I extubate them. I just extubate them in the NICU at the bedside, nothing special. Depending on how bad their airway is, sometimes I'll just round on the morning and I'll be like, "Just pull the tube when you're ready, put in a nasal trumpet, they'll be fine."
Sometimes I will let them know that I'm at least physically on campus. If I really think I need to be at the bedside, then they're probably not ready to extubate yet. Usually for these kids, if they were getting by ahead of time, just needed positioning and stuff, if you wait and now they're four or five millimeters ahead, that's a big change for them. That's a 50% improvement in their airway from glossoptosis. I usually think they're ready at that point.
Usually they still have another couple of days of distraction after that because I would say on average, most of these kids, I'm distracting between probably 12 and 14 millimeters. There's no set number. The last one I did it was 20. That's the farthest I've had to go, was 20. Probably on average it's 12 to 14 millimeters. Usually that's six or seven days of distraction. If you pull the tube after four days, you should have another couple of days of distraction and they usually tolerate it just fine.
[Dr. Gopi Shah]
The external distractors have to come off, but the internal ones you leave in place?
[Dr. Brianne Roby]
The external devices, they both have to come out, the hardware always has to come out. Once you're done with distraction, you have to leave the actual plates in the bone for somewhere between 8 and 12 weeks. That's called the consolidation phase where that bone is hardening up from soft little new bone callousy type bone to actual bone. I wait 10 weeks after distraction to take out the hardware, but some people do a little bit less and some people do a little bit more.
Waiting longer than three months, because they're babies, they usually start to grow a lot of bone that overgrows the plates and it becomes really hard to take the plates out. The benefit of the external device is when it's time to remove it, you actually don't have to reopen their incision because there's a way to undo the pins and you just slide it out. You undo the pins and you slide it out from the bone. That is probably, in my mind, the one benefit of doing the external device along with being able to do multidirectional, but otherwise most of the other things are downsides.
For the internal devices, I usually will end up taking them back to the OR about 10 weeks later, and it's a quick surgery. You just open up their incisions, you take out the plates and the hardware. I usually just keep them in an observation unit overnight and they go home the next day. The only reason I keep them overnight after that surgery is because they're still babies and I want to make sure they're going to feed and that we have pain control, honestly, which is Tylenol. That's why I keep them overnight at that point.
That's a quicker procedure, but it's just opening up the same incisions and taking out the plates. That's probably the one downside with the internal devices, is that you do have to go back and open up the incision. For older kids, if you were going to do distraction, maybe they had a trach, maybe they're stable enough that you're going to send them home with the parents and the distraction. For newborns, you're not going to do that.
I start to work on feeding a lot with them. As soon as the breathing tube is out, we have the speech therapists, our feeding people really start working with them to try to get them to bottle by mouth. What I generally find is the first few days while you're still distracting them, they're doing better with feeding, but they're still not 100% because every 12 hours you're adjusting how that feel of their mandible is.
Once you stop doing the distraction, most of them take to feeding quickly pretty well. The pins that are out where the distraction arm for the internal devices, those slide out. There's a little pinching device that's got this little ball socket thing that you just pinch right underneath the skin and those slide out. When they go home, they do have the hardware under their skin, but there's nothing that's showing. Strangers on the street wouldn't see any weird hardware or anything.
Mandibular Distraction Complications in Children with Micrognathia
Post-operative complications in children undergoing mandibular distraction can vary, particularly in syndromic cases. For children with Stickler syndrome, post-op complications are generally consistent with those seen in non-syndromic children, as their micrognathia is not typically associated with other mandibular deformities like hypoplastic temporomandibular joints (TMJs). Therefore, the success rate for these children is comparable to that of non-syndromic children.
However, in children with conditions like Treacher Collins syndrome, the situation can be more complex. Some of these children may not be candidates for distraction due to abnormal mandibular anatomy, such as a missing fossa for the joint. In such cases, distraction is not feasible, and these children may require a tracheostomy and later distraction when they are older and their anatomy is more developed.
For syndromic children who are suitable candidates for distraction, there is often a higher risk of postoperative complications, such as the need for a tracheostomy or a gastrostomy tube (G-tube), particularly if they have global hypotonia or other complicating factors. It’s essential to communicate clearly with parents about these risks and the potential outcomes.
Additionally, for children with severe syndromes like Nager syndrome, where mandibular development is severely deficient, a tracheostomy may be necessary from birth, with distraction being delayed until the mandible has developed enough to support the procedure. The goal in these cases is often to eventually remove the tracheostomy, but this may take time and multiple procedures.
Overall, while the success rate for mandibular distraction can be high, especially in non-syndromic or mild syndromic cases, more complex syndromic children require careful consideration, clear communication with families, and a willingness to adapt the surgical plan based on the child's unique anatomy and post-operative progress.
[Dr. Gopi Shah]
I feel like this is probably an entire different podcast, not topic, but extension. I guess just really quickly, the kids that are syndromic, are there other special considerations in terms of post-op complications or intra-op with your Sticklers, Treacher Collins or other kids, or is it pretty consistent?
[Dr. Brianne Roby]
Stickler kids, not really. Honestly, their syndromic stuff is unrelated really to the shape and development of their mandible. They're micrognathic, but they don't tend to have hypoplastic TMJs or anything like that, so not really. In terms of syndromic, I tend to tell parents of kids with Stickler, they're going to have the same success rate as a just normal non-syndromic-proband-sequence child.
Oftentimes, to be honest, we don't even have the genetics back on any of these kids when we take them to do distraction. You're gauging syndromic either if they have that classic Stickler appearance or if there's a parent that has Stickler and you're like, "Yes, okay, you have Stickler." For the Treacher Collins, first of all, some of them are just not going to be candidates for distraction because they're not going to have normal mandible anatomy.
If they don't have a fossa where their joint sits, where the condyle sits, you can't really distract them. There's nothing to hold that in place until you distract them. There's not that joint, the fossa holding it in place. There are some kids with syndromic type features that either eliminates them from being a distraction candidate as a newborn, and they might be more likely to need a trach and you need distraction when they're older and you can do more stuff with their anatomy, or if they do need it, so let's say they're a syndromic, but they are a good distraction candidate, but maybe they've got some global hypotonia or something like that.
Those are the parents that I'm very upfront with, like, "Look, we're going to do this surgery. They have a higher rate of needing a trach afterwards and they have a higher rate of still needing a G-tube. Do I think it's worth doing the distraction? Yes, because our goal is to try to avoid those. I think our numbers show that we're maybe 50% or 70% success in these situations. If it doesn't work, then the next step is going to be trach and or G-tube or both, depending on how bad it is."
Those are the ones that I don't think it's wrong to do the surgery, but I think you have to be really upfront with the parents about the success rate and let them decide do they even want you to try that, or would they rather go to like a definitive trach G-tube and deal with the smaller job down the road?
[Dr. Gopi Shah]
Also sometimes the airway is so bad that if they're not intubatable, and yes, you can put a trach in, but let's say the trach comes out in six months and there's obstruction, some sort of event. If the baby's not intubatable, for that reason itself, sometimes, like you said, the expectation should be there that they might still need the trach, but maybe we're doing that to help prevent a catastrophe despite a trach.
[Dr. Brianne Roby]
Yes, for sure. If I think about the last child that was a Nager syndrome child, we had like no mandible. He got a trach on minute zero of life, and got distracted at eight or nine months of life because by then he finally had enough mandible that we could get plates on him. The whole point when we put the trach in was like, "Look, we want to get the trach out. We know you want the trach out, we just have to wait until we can do the surgery that will help us do that."
I think you can have those discussions with the parents and say, "We've got options, just the options aren't quite there yet. A trach is going to be the best option now." As soon as we put that trach in, we'll start having the discussion of what needs to happen to get it out.
[Dr. Gopi Shah]
Similar conversations that we have with a choanal atresia or pyriform aperture stenosis sometimes depending on the comorbidities in the child. As we're wrapping up, this has been so great because you can tell there's stuff that I know about these babies, but there's definitely those little things because we're not-- it's our craniofacial plastics team here that does actual distractions that we're involved with all the airway stuff. Any other follow-up, post-op, complications ever have to go back within three months to revise? Do those things happen?
[Dr. Brianne Roby]
Occasionally. You always warn them about infection. I have them on antibiotics while I'm actually doing the pin turns and distracting them, but then I usually don't keep them on antibiotics, but they're-- It's hardware. It's hardware in the body and it's usually very close to the skin surface and at some point it was external to the skin as well. There is a risk of infection. You want to be watching for that.
I saw this once as a fellow where the baby's bone was just so soft that like at-- we distracted like eight millimeters and the plate came out. The screws just didn't have good enough purchase. In that case had to go back, took the plates out and just pretended it was a mandible fracture and put a normal non-distraction plate in that. The distraction was just done at that point. The baby did fine because they'd already been distracted like eight or nine millimeters. It wasn't ideal.
You do have to have those conversations with them. The biggest thing once they're at home is just worrying about infection and just watching for that. My hope and what I always say this is a success is then when we go back again, I think over 90% of these kids have cleft palates. You go back then either when you remove their hardware at 3 months or then when they get their palate surgery at 10 or 12 months of age and you do the DL and if they're that grade one view, you're like, "Hooray, like this is why we did this surgery," because again, from an airway, that was the whole point, was the airway.
That's the follow-up that I always love, is when I get to go tell parents that like, "Hey, remember when I told you that your kid was a really hard to get a breathing tube in, they're no longer hard. They're a normal anatomy." It's fun to be able to tell them that and to also tell the anesthesia, "Hey, take difficult airway out of their label. They're not a difficult airway."
Podcast Contributors
Dr. Brianne Roby
Dr. Brianne Roby is a pediatric otolaryngologist with Children's Minnesota and assistant professor at the University of Minnesota Medical School.
Dr. Gopi Shah
Dr. Gopi Shah is a pediatric otolaryngologist and the co-host of BackTable ENT.
Cite This Podcast
BackTable, LLC (Producer). (2021, December 14). Ep. 39 – Evaluation & Management of an Infant with a Small Jaw [Audio podcast]. Retrieved from https://www.backtable.com
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