Transcription:
Multidisciplinary Cleft and Craniofacial Program

Melanie:  There's no handbook for your child's health, but we do have a podcast featuring world-class clinical and research physicians covering everything from your child's allergies to zinc levels.

This is Kids Health Cast by Weill Cornell Medicine. I'm Melanie Cole. And today, we're discussing the multidisciplinary cleft and craniofacial program at Weill Cornell Medicine.

Joining me in this panel is Dr. Caitlin Hoffman, she's an Assistant Professor of Pediatric Neurosurgery and the Director of Craniofacial Surgery at Weill Cornell Medicine; and Dr. Vikash Modi, he's an Associate Professor and the Chief of Pediatric Otolaryngology Head and Neck Surgery at New York Presbyterian Hospital. And they are both co-directors of the craniofacial team at Weill Cornell Medicine.

Doctors, thank you so much for joining us today. And Dr. Hoffman, I'd like to start with you. If you would give us a little overview, a brief overview of craniofacial anomalies and how common they are, speak about the different types that you see and treat.

Dr Caitlin Hoffman: Absolutely. Craniofacial anomalies can be quite simple and quite complex. It's a spectrum, which therefore requires a very deep and broad team to address that spectrum, which is what's unique about our program here at Cornell, which we'll get to in a bit. But regarding the anomalies themselves, they are typically those that involve the calvarium or the skull, the connection of those bones then to the mid-face as well as involving the palate.

From my perspective, from a neurosurgical perspective, I treat predominantly all of the cranial and facial disorders, meaning those that involve the skull and how those bones relate to the mid-face or the base of that skull. These disorders can be quite simple, meaning that two bones in the skull simply fused together too early, and that's known as craniosynostosis. And it can be quite complex, in the form of what we would call a syndromic synostosis, wherein multiple bones of the skull, as well as bones of the midface all fuse together prematurely. And the consequences therefore of course are much more severe in the forms of syndromic synostosis, and require a much more multidisciplinary and prolonged care through multiple stage surgeries versus simple synostosis requiring one simple surgery, usually either by myself or with my plastic surgery partner, Dr. Imahiyerobo, as a one-time treatment.

Dr Vikash Modi: And I'd like to add the portion that I treat or my part of the team is cleft portion. And it's where the mid-face, which is the middle of the face intersects with the lower part of the space. So that involves the palate, involves the nose and involves the mid-face, also involves the jaw. And there are times where we have to interface with our neurosurgical colleagues and the cranial vault team. And there are times where we don't have to interface with them. So that's the beauty of the team. Craniofacial anomalies are so sort of varied.

And in, the cleft world, there can be issues with the nose. There can be issues with the cleft lip. There can be issues with the palate. There can be issues with the jaw. There can be issues at the tongue. But sometimes, it also relates to the calvarium as well and so we all need to work together as a team.

The incidence of cranial facial anomalies is about one in 200 live births that require specialized care. And it's important to have a multidisciplinary team that all work in concert together to treat these conditions.

Melanie: Well, thank you both for that. And we will talk about that multidisciplinary team that you both are co-directors with. But Dr. Modi, do we know why these happen and how and when are they generally diagnosed for women that are pregnant? Is this something that is seen on ultrasound? Do they not find out until baby is born? Tell us a little bit for the listeners when these are diagnosed.

Dr Vikash Modi: Well, prenatal diagnoses have come a long way just given in the last 10 years. We've gone from just routine ultrasonography to ultrasonography with full anatomical scans to 3D renderings. There was a time where people didn't know if a child had a cleft until the baby was actually born.

I'd say nowadays, we're picking up clefts earlier and earlier and earlier. And it's mainly because they're paying attention to the anatomy a lot more now. Cleft lips are oftentimes able to be diagnosed on prenatal ultrasonography at a very young gestational age. Cleft palates, because of the orientation and location where the palate is located and the vector in which the ultrasound is done, are oftentimes more difficult to pick up. At our team, we are now offering prenatal consultations. And we've been doing that for about five years now, where we will have a sit down with the family and they get to meet our entire team and talk to them about what to expect based on preliminary ultrasonography.

Dr Caitlin Hoffman: And I'll add the same to that, Dr. Modi, that it's very similar in the sense that there used to not be any known incidence prenatally in terms of syndromic synostosis or even simple synostosis. And we're seeing a far greater rate of prenatal referrals to our clinic for this purpose, but really helps guide parents' understanding expectations, what the natural history of this is going to be, but also sets those resources in place to support parents as they move through this process well before the fact, which is really essential during that time right after delivery and during delivery. It's really essential to feel that you have the right resources and support in place to ease that transition and then to move towards necessary treatment as quickly as possible.

Melanie: Well, Dr. Hoffman, if you would expand just a little bit about the right time to consider treatment, does this start right away once it's diagnosed? Is this something that takes many years over many treatments? Speak a little bit about the treatment options available and kind of how the process works for parents listening.

Dr Caitlin Hoffman: So, as you're getting the sense, the answer to this also runs a spectrum, which is fine. We'll review the whole spectrum. So for simple synostosis, meaning we're only dealing with one fused abnormal suture of the skull with no other abnormalities in the mid-face or in the palate as Dr. Modi was indicating, that is something that is evident at birth and is treated very quickly. There has been an acceleration of minimally invasive approaches endoscopically to synastosis over the last decade to the point where it is now essentially standard of care under the age of four months.

So children that are diagnosed prior to four months, we are able to correct this endoscopically through small incisions and use of helmet therapy afterwards. If a child with a single suture synostosis is diagnosed after the age of four to five months, certainly after the age of six months, then it requires a larger open procedure called a frontal orbital advancement or cranial vault remodeling depending on the suture that's involved. And while it's a larger incision to access the amount of bone that we need to remodel, it is extremely safe in the hands of a team that this is the majority of what we treat. And it's something that is very simple to treat in our hands. But from a parental standpoint, it does mean far more in terms of the recovery process and the intraoperative time.

So early diagnosis is essential and becoming much more easy as Dr. Modi mentioned based on advanced imaging modalities, and just the tools that we have to treat this now, endoscopically versus open. Building upon that, we also have advanced several of the ways in which we even remove the abnormal bone with newer advanced technology with what's called ultrasonic aspiration, so that that actually helps to decrease blood loss during surgery, et cetera. There are a lot of minimally invasive techniques that we as a team are developing together and continually pushing forward, which I think we've seen a great benefit in our patients outcomes.

The more syndromic synostosis that start to then begin to interface with each other as a team with other aspects beyond just the skull. There are also fusions of the mid-face. There are abnormalities of the palate and the jaw and the teeth. That's where then we start to work together as a team in multi-stage procedures.

So those procedures begin with remodeling usually aspects of the frontal bone and the orbits to protect the eyes, to give them enough room and to allow the brain enough room to. And then as a child matures, we then move on to working together with our oral and maxillofacial colleagues and plastic surgery colleagues and ENT colleagues to address the mid-face and the palate. And then the last would be at skeletal maturity in the teenage years to advance the jaw so that everything finally lines up for functional maturity. So you can see that gets quite complex and utilizes all aspects of our team's resources.

Dr Vikash Modi: And I'd like to add, when referring to the mid-face and the lower jaw and the nose and the lip and the palate, there are two primary concerns. There's the cosmetic concern, which is obviously the appearance on the outside. But then there's also the functional component. And as soon as the baby is born, whether they've got a cleft lip or they've got a cleft palate, we always want assess their functional status usually within the first 24 hours of life. So we're looking at breathing, we're looking at feeding. Those are the two major things. And in craniofacial anomalies, children can have problems with both. They can have problems with breathing because sometimes their jaw is under underdeveloped or their mid-face is underdeveloped. So, their airway is compromised. When your airway is compromised, feeding can also be a problem. For example, if there's a cleft palate, the baby has the inability to suction. As a result, bottle-feeding unless you've got a specialized bottle will be challenging and breastfeeding is not possible.

And then further on down the line as the child develops to when they're around 12 months of age, we have to think about the other functional aspect of speaking and talking and developing proper speech. So this is where the multidisciplinary team really comes into play because everybody has their own cog in this wheel. And we all work together for a common outcome and we all work in conjunction with each other.

Melanie: Really, it's such an interesting topic. And Dr. Modi, we've mentioned this a few times so far, this multidisciplinary approach for these children as they go through all of these treatments and for their families. Can you tell us a little bit more about the multidisciplinary cleft and craniofacial program at Weill Cornell Medicine and why this approach is so important for these patients and their families?

Dr Vikash Modi: Yeah, I'm glad you highlighted that. So not many teams in the country are accredited. We are actually accredited by the American Cleft Palate and Craniofacial Association. And you have to have certain specialists that are part of the team in order to be considered sort of certified. And in our team, we've got developmental pediatrics, we've got facial plastic surgery, we have genetics, neonatology, neurology, neuropsychology, pediatric dentistry, pediatric gastroenterology, pediatric neurosurgery, pediatric ophthalmology, oral maxillofacial surgery, orthodontics, prosthodontics, otolaryngology, pulmonary, sleep medicine, speech pathology. So we have all of these specialists sort of working in conjunction together.

So the minute a baby is born with a cleft lip and palate, the first thing we assess for is feeding and breathing. That's done by our speech and swallow specialists and our otolaryngologists. After that, if there is a very wide cleft, we'll get our prostadontics involved on day of life one where they'll insert nasoalveolar molding, so they can bring the segments of the cleft closer together so that when the facial plastic surgeon is going to repair the lip, the lip comes together tension-free. And then I work together with the speech pathologists to ensure that the cleft palate repair, not only is the hole not there, but that the speech develops properly. So you can see that's just one aspect of how just right now I mentioned six specialists will work together to treat a process.

Melanie: Thank you so much for telling us about that approach, because it really is so important for parents to hear. And I'd like to give you each a final thought. So Dr. Hoffman, tell us what life is like for these children and their families and how you support them as they go through these many different procedures and treatment modalities, and really how you approach the families and help them as they go through these.

Dr Caitlin Hoffman: I think this was one of the most important drivers in creating a clinic like this, was to better support and to provide a continuity of care that was relatively unique for the patients, but also their families. Because these families go through this together and there are often siblings, of course, that are involved in the process that becomes their life as these children move through the necessary steps of treatment. So absolutely, you know, we are focused medically on what we need to do to optimize the function of patients as Dr. Modi indicated, that's of course the central aspect of our clinical assessment and care. But it is also just as essential that the parents are part of the decision-making process at every turn. And that's why we hold this clinic together all in one space and then the patients receive a consensus opinion from us. They meet everyone face to face and we work together as a team. And the parents and the patients when they're old enough are part of that team.

And I think it's very important for parents to hear that, one of the other advantages and why we like working together in this team to provide this type of care for patients is that we do stick with parents and patients through the entirety of this process, through the spectrum, until we get patients to the point where they are at their functional best. And that is absolutely a possibility.

I think that while this can be a very overwhelming process for parents that are in the more complex aspects of this spectrum with syndromic synostosis, that there is tremendous hope for a wonderfully functional outcome and approach, especially working alongside a team that really cares about the family unit and a patient's best outcome.

Dr Vikash Modi: I'd like to add, I think one of the most important aspects of our team is that we have a clinical team coordinator, we have two nurse practitioners that help run the program. So essentially, when we have one of our team meetings, the patient is in the room, all the providers rotate and cycle to that room. Then we all meet together and we sort of go over the order in which everything is going to take place, meaning the surgeries and the therapies and the next appointment. And oftentimes that can be very overwhelming for parents, so to have a concierge, so to speak, or a clinical patient navigator, where the parents have this point person that they can talk to and call and email at any time to help them sort of do the entire process. This clinical care coordinator also puts together an entire list of all of the recommendations, the dates, the addresses, the phone numbers, the emails and also follows up with them once a week, once a month, to make sure that they're kind of on target so that they can make it to the next scheduled sort of appointment or the visit or the surgery or the treatment or the post-op care. So they have this common space that they're able to connect with. And that's what really brings the whole multidisciplinary team together. That's when we could all work together. There's a communication and there's coordination. And, with that, our nurse practitioner kind of helps pull it all together, which is just wonderful..

And for further information about cleft and craniofacial care at Weill Cornell, you can either email us at craniofacial@med.cornell.edu, or you can email us at craniosynostosis@med.cornell.edu.

Melanie: Thank you both so much. What an informative episode. Thank you again for joining us.

To learn more about pediatric neurosurgery, you can visit weillcornell.org/pediatricneurosurgery. And Weill Cornell Medicine continues to see our patients in person, as well as through video visits and you can be confident of the safety of your appointments at Weill Cornell Medicine.

That concludes today's episode of Kids Health Cast. We'd like to invite our audience to download subscribe, rate, and review Kids Health Cast on Apple podcasts, Spotify and Google podcast. For more health tips, visit weillcornell.org and search podcasts. And don't forget to check out our Back To Health. I'm Melanie Cole. Thanks so much for listening  

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