After Cony Puac delivered her daughter Evany, birthing attendants placed the newborn in her arms and cleared the room.
Born in a remote Guatemalan village surrounded by snow-capped volcanoes, even in the first moments of life, children born with facial clefts are ostracized from society. Evany was diagnosed with a severe midline cleft palate by an orthodontist in her community. An opening at the center of her face spanned from her bottom lip to the space between her eyes. On either side of the opening, her eyes were separated by 38 centimeters — 20 centimeters wider than that of an average child’s. Evany’s nostrils were pushed to the left side of her face in cluster of tissue. At the crown of her head, Evany suffered from several holes in the cranium bone beneath the skin.
Evany also lacked an upper lip, which she needed to receive nourishment early in life through breastfeeding.
Whenever we think of cleft lip, we always think of the little baby with an obvious cleft in the lip. As I had mentioned before, the lip is the most obvious, but the cleft affects others things too, including the nose. It may not be obvious, since we are so distracted by looking at the lip, but the nose is almost always asymmetric and I feel, like a lot of the cleft surgeons out there, that this is also just as important in the initial repair. But as much as we would like to hit a home run, the unpredictability of growth will most likely require a revision later in life. Rhinoplasty is a perfect example of this. Those cute kids eventually, and inevitably grow up to be teenagers, and a lot of times they want their nose done.
It isn’t all about looks though. Nasal obstruction is very common since the septum and turbinates are usually obstructing a clear nasal passage with a cleft lip. Many times my patients are more happy with being able to breathe through their nose than they are with the visual changes.
Rhinoplasty, aka nose jobs, are integral to cleft care. Traditionally we have waited until 14 to 16 years old, but I think now with cleft care being more aggressive, 10 to 12 years old is reasonable depending on the child’s maturity and wants and wishes.
Below are some examples of the subtle, and not so subtle changes possible with cleft rhinoplasty.
I often get the question, what surgery is your favorite? That is like asking, what is your most favorite book or movie. Better yet, if you were stuck on a desert island, which would be your only food. Although as adults, we kinda write off these questions as being silly, but the 1st question is a pretty legitimate one.
But just like favorite books and movies, there is no favorite, but usually a couple. And of those, there are very different reasons why they are favorites. So with that in mind, I figured I’d go through the list of cleft care surgeries and list why they are ALL my favorites.
1st surgery (cleft lip)
There is a reason why mission groups love to put up pics of repaired cleft lips. This is the most dramatic part of cleft care…this is the face of cleft care, no pun intended. And I won’t pretend to lie to you and say this isn’t one of my favorites for the same reason. Changing a child’s appearance and making things whole is obviously very rewarding. It’s the 1st surgery that will influence the course of this child’s cleft care. A “bad” lip repair can be difficult to overcome with later surgeries. Because of this, those experienced in cleft care take a dim view of those who do not routinely do these surgeries, yet tout themselves as cleft surgeons.
From a surgical point of view, this is where technical finesse merges with the “artistic” part of surgery. Each cleft is different and requires analysis to give a complete idea or plan, on how the lip is supposed to look like at the end. After this “artistic part”, you have to have the technical ability to complete the plan. As you can imagine, pulling these two elements together is never the same no matter how many cleft you do, and will continuously challenge as I feel the same way as my mentors. When you stop trying to reach for perfect, you will stop making each cleft lip repair the best you have done.
2nd surgery (cleft palate)
This is probably the most important surgery. Without a palate, a child cannot talk and be understand. And a child who cannot be understood will have a very difficult life. Since there is not much “worry” about the aesthetic part of surgery, it allows me to focus on the technical part of the surgery. There is a level of technical expertise required and for this part alone I enjoy the surgery. Add on the fact that a good repair is allowing a child to have normal speech and be able to socialize normally with their peers, is truly gratifying. My fistula rate is 5%, which is below the national average, and something I’m proud of.
3rd surgery (bone grafting)
This is another technically satisfying surgery. A lot of resources go into this surgery in terms of planning with orthodontics and dentistry. Another thing I like about this surgery is that it epitomizes, or is an example, of the philosophy of cleft care. One way to look at it, is this surgery is about saving a tooth, which is erupting into the cleft bone. In other countries where I do mission trips, the population is usually worrying more about food, shelter, and human rights. Worrying about a saving a tooth with a bone graft, and establishing a normal dental arch is a luxury they usually don’t have. Plus they don’t have an orthodontist and dentist to plan out the pre care needed for this surgery. However the way I look at it is different and I don’t think this is a “silly” or “vain” surgery. I see it as another reason to be grateful we live in a country with the resources and social stability to make this standard of care. The fact that we are dedicating these resources to making sure we achieve normalcy is one of the main points of cleft care.
4th surgery (cleft rhinoplasty)
By now, these children have grown up and developed their own personalities, their lives, and for better or worse, are usually in the midst of their peers with all the social pressures. This is where I really listen to the child and parents in what THEY want…not what I think looks good. Also, there is a large functional part of this surgery as we are able to correct their breathing out of the nose. Many patients tell me this is the best part of the surgery, not the “looks” part of it.
As for the “looks” part, I’ve found that no matter how slight or how dramatic the change, my patients tend to be quite happy with their results. This constantly remind me that its’ the patient who this surgery is for, and not for anyone else.
5th surgery (jaw surgery)
I’m a little hesitant to put this in here, since jaw surgery is NOT always indicated. The main reasons are usually to move the upper jaw forward and this tends to happen more in children with bilateral cleft lip and palates. It can be for both functional issues, as well as aesthetic. The upper jaw movement can help open up the airway and improve overall breathing. Aesthetically, it helps correct an “underbite” look. The planning required and timing can be complex and involved dentistry as well as orthodontists. We can make large changes in this surgery, however we need a lot of planning and coordination with these kinds of surgeries.
Although this is sort of a general blueprint of surgeries, by no means is this meant to be an extensive list or a “for definite” plan. This is why I’m here! to help decipher, discuss, and plan out what is needed.
I attended the American Society of Plastic Surgeons annual meeting this October and had a very interesting time discussing clefts with other plastic and reconstructive surgeons dedicated to cleft care. During this meeting, several thoughts popped into my head:
It is a small group nationally, but one that is passionate about and dedicated to cleft care.
Evolution of cleft care and art has progressed toward focusing on “perfection.”
The evolution of cleft care and techniques continue to change.
What do I mean?
It is a small group nationally, but one that is passionate about and dedicated to cleft care.
Since plastic surgery covers both cosmetic and reconstructive surgery, there is a lot of ground to cover at these national meetings. With about 4,500 participants, there is plenty of good energy and sharing of ideas. There are sessions where acknowledged national experts will give talks and panels, and the sessions are organized as being either aesthetic or reconstructive. Because there is so much information, the two sessions will run at the same time. Naturally, the session dedicated to cosmetic surgery (such as face lifts, eyelid tucks, breast augmentations, belly tucks and liposuction) is usually standing room only, whereas the reconstructive sessions tend to attract a smaller crowd. While talking about cleft care in the reconstructive session, I looked around the room and saw a lot of familiar faces, all of whom have dedicated themselves to clefts and pediatric plastic surgery. I couldn’t help but think that this is truly a small and dedicated group that easily could walk next door and focus on the more lucrative practice of aesthetic surgery. Yet we remain in the quarter-filled room to share ideas and discuss where we are taking cleft care in the future. It is this continual drive for perfecting cleft craft that keeps us coming back year after year.
Evolution of cleft care and art has progressed toward focusing on “perfection.”
It used to be that closing a cleft was what was expected and nothing else. Then we began to focus more on the aesthetics and the function. Now, we have evolved to the point of understanding and have enough experience gathered up in our specialty that the lip repair is actually the easier part of the operation. We also focus on making the nose perfect and trying to make repeat surgeries a less frequent occurrence. The goal is to make it look like there was never a cleft to begin with. And although trying to predict how a child’s lip and palate repair will grow over time is difficult, we have come to a point where we can get a pretty good idea. Besides focusing on altering our procedures to give a beautiful natural repair of the lip and nose, we also focus on minimizing future surgical procedures if possible.
The evolution of cleft care and techniques continue to change.
I keep in touch with several other cleft surgeons, as well as my mentors. All of them have at least 20 years of experience fixing cleft lips and palates. We often share pictures of our patients to discuss cases and see where things went well or discuss areas that could be done differently. These are wonderful exchanges since they allow a good flow of constructive discussion and criticism of what works and what doesn’t. It’s this continual discussion that keeps us striving for perfection in each case. It’s humbling when the older cleft surgeons still are open to change and new ideas about operations and techniques they have been doing for the past 20 years.
It is the same during national meetings since we see results from other cleft surgeons and have lively discussions about what works and what doesn’t. It probably comes as no surprise, but cleft surgeons are their own toughest critics. We are self-described perfectionists, and this is perfect for improvement! Because of this attitude of “We can always do better,” cleft techniques continue to change. I think this is great since the ones who benefit the most are the kids.
These national meetings recharge my professional batteries. Rubbing elbows with the “experts” and seeing that your own results are on par with theirs is reassuring. Dr. Ralph Millard is the modern father figure of North American cleft surgery and he labeled it Cleft Craft. This is the perfect description since although medicine prides itself on the science, cleft care it is still an art and craft. Its practice requires constant dedication, constant study, and above all, constant care of each individual patient.
– James Liau, MD
Although we’d like to only have to do one surgery on the lip and palate, realistically most kids will need a “touch up” or revision. Usually this happens between 3 and 6 years of age when children become more socialized from day care or school. This also is when kids become more self-aware and notice differences between each other.
Why do we need revisions?
There are two main goals of cleft care:
- Creation of a normal appearance.
- Establishing normal function.
Since our social interactions are so important to us, both appearance and speech shape who we are and how we relate to people. As children grow, their own tissue and scars change, and they essentially grow out of their initial repair. I like to refer to the revisions as touch ups – typically outpatient surgeries that consist of a few readjustments.
I find there usually are two times in a child’s life when we need to do touch ups. The first time is usually before kindergarten or at about preschool age. This is when kids begin to notice differences between one another, and they begin to make comments. In this age group, these often are not mean or hurtful comments, but questions stemming from curiosity. Parents of children with clefts often say it’s best to address this topic directly. Let your child know that when he or she was born, “they had a boo-boo (or whatever your equivalent term is) of their lip and nose. Doctors fixed it, and now things are good.” Multiple parents have told me this works well. Kids at this age usually just want an explanation, and once they have it, they’ll let it go without a second thought.
The other time we consider a touch-up procedure for children is during their preteen and teenage years. As I’m sure we all remember, these are the awkward years where what happens socially is what defines the whole world. Unfortunately, this is also the age where kids can be mean to one another. It is difficult for “tweenagers” to see past residual traces of a cleft lip or the airy sound of the cleft palate. Revisions at this stage are dependent on the child’s wishes, as well as what can be reasonably expected. With current techniques and experience-based knowledge, I would expect a child with a cleft to live a healthy and happy life without having any limitations because of cleft lip and/or palate.
Touch ups tend to be grouped as such:
- The lip
- The nose
The lip: As a child grows, what can happen is his or her lip grows “apart.” The sides don’t match up, and because of the imprecise nature of scarring, what originally looked great now looks off. Also, depending on how wide the cleft is, the muscle layer can thin and look like it has pulled apart. These changes can result in a look called a “whistler’s deformity.” There is notching of the vermilion (or otherwise known as white roll) of the lip, and perhaps some degree of asymmetry of the entire lip itself. It looks like one could “whistle” through this area.
There is no defined timeframe to do a lip touch up, but most families and patients prefer to do this before kindergarten and then later if needed.
The nose: The full effect of clefting can be seen as a child grows older and the nose begins to achieve adult proportions. Not only is the lip involved, but so too are the nasal septum and tip cartilages (which gives the nose tip its shape). This presents two problems. One is the ability to breathe well from one or both nostrils. The other is nose asymmetry. At this point, we begin to see how much the nose really can change the appearance of the entire face. Traditionally nose operations didn’t happen until age 16, when the child’s bone structure was more like an adult’s. However, experience shows that operating on noses earlier doesn’t pose a problem later in adulthood. Not only can we improve breathing, but we also can help improve self-image and self-esteem, too. Typically, though, we would consider doing nose work around 12 years of age. Of course, this is subjective and differs from child to child. We have to take into consideration the jaw growth, level of breathing impairment, social pressure and the maturity of the child. This is why I like to take extra time to explore all of these issues and make sure we do what is right at the right time.
Speech: As we all know, kids like to talk, and preteens talk even more! It’s at this age that our children begin to really develop their personalities and their ability to relate to others. Having poor speech becomes a barrier during this important time and can have a detrimental effect on the child. Speech is very complex because it’s affected by a number of variables, including: the ability to hear well, learned behavior, mechanical issues with the throat and palate, and the availability of resources such as speech therapy. Luckily at UK, we have a great speech pathologist, Rebecca Hancock, M.Ed., CCC-SLP, who specializes in Video Nasoendoscopy, or VNE. This procedure uses a very small camera that is guided down the nose to see the palate and throat work while the patient speaks. This helps us figure out whether it is a mechanical issue with the palate and throat, or whether more therapy is required. Rebecca is vital in providing information for a clinical decision.
If we decide that the throat and palate are not working properly, there are two procedures available, depending on what is wrong. One is called a pharyngeal flap and the other is called a sphincterplasty. These will be covered in a future blog post.
As you can see, there are many decisions to make during a child’s transition into young adulthood. These can be especially challenging times, as the child begins to develop his or her personality and self-identity. Although we know our kids are more than their appearance, it can be very hard for them to see that. I’m here to help coordinate and make sure everything is evaluated and treated accordingly. It’s my goal to address these issues, and then take care of them in the best way possible. – James Liau, MD
I will never forget the day doctors at UK told me that my baby had a cleft lip and possibly a cleft palate as well, I proceeded into the restroom to dress after the ultrasound and I dropped to my knees in that very restroom and asked GOD to just lay his hand on my baby and help me through the long road ahead and he did just that by sending a very passionate, caring and loving doctor to us and I would like to thank Dr. Liau for taking time out of his busy schedule to make my son whole again and I would recommend him to anyone. He has a special love for his work when it comes to children and thank you, thank you and thank you again Dr. Liau for everything you did for my son. ….Sherry F.
We’d love to hear from you. Send us your comments or questions.
My daughter, Emma, was born with bilateral cleft lip and palate. When we first received the news we were very worried and scared because we didn’t know what we were dealing with. No one in our families were ever born with this, so we had no idea of what to expect. Thankfully, we were sent to UK and this is where we met Dr. James Liau.
Joyce K. asks, “Does your team use the NAM device pre-surgery?”
Nasoalveolar molding (NAM) is a technique to help mold the nose and alveolar segments in a child prior to cleft lip repair. This can be helpful with wider clefts. My experience with them in my year of fellowship training have generally been positive, however it does require a fair amount of maintenance, care, and usually weekly to biweekly follow up appointments. If the child is unable to tolerate the device, or if it is difficult for the child and parent to maintain, NAM has the potential to be quite stressful.
We do not routinely offer this service here for several reasons.
- This is an orthodontic appliance and traditionally managed by an orthodontist. Unfortunately at the University of Kentucky, there is not an orthodontist on staff who does nasoalveolar molding, however, I do know of an orthodontist in Lexington who has done a fellowship in NAM and I’d be more than happy to put you in contact with her.
- If the child or parent are unable to complete the NAM protocol, then there may be unwarranted feelings of “guilt” or “not being able” to do what is necessary. There can be feelings of being a “bad parent”. THIS IS NOT TRUE! NAM can be quite difficult depending on the child and social circumstances, as well as the weekly to biweekly scheduled visits. Because of this potential dynamic, I do not tout or pressure any family to do NAM. This leads us to the next question, how effective is NAM.
- NAM works to readjust the nose and even out the alveolar ridges. But the main question is whether this continues to be effective or any different than non-NAM treated children over time; basically the question is whether this makes a difference in the long term. Only several institutions have enough long term data to support the superiority of NAM, and there is argument that their experience is difficult to duplicate.
Personally I feel nasoalveolar molding allows a very active approach to the 1st several months life of the child prior to his or her cleft lip repair. How a family adjusts to this can range from adding more stress to the parent and child, or it may be exactly what the parents want since they can actively alter their child’s cleft. Whether this makes a difference in less wide clefts I cannot answer that, although my gut feeling is that is does not.
Does it produce a “nicer” cleft lip repair? My experiences with NAM suggest that if you have a great NAM treatment, but a less than adequate repair, you will have an inadequate repair. If you have no NAM or partially completed NAM protocol, but an excellent repair, then you have a child with an excellent repair, and this is what I focus on with every one of my cleft children. Once again, I stress this is a my own humble opinion on the NAM from personal experience.
James Liau, M.D.
Division of Plastic Surgery
University of Kentucky
Having a child with a cleft lip and/or palate is stressful and can detract from the joy of bringing a child into the world. Fortunately prenatal screening can usually see cleft lips and cleft palates.
Although we cannot catch them all, depending on how the child is laying in the belly, we usually have a very good idea of whether a child will be born with a cleft lip and/or palate. When there is a chance a child has a cleft lip or palate, I like to meet with the parents before the delivery so we can discuss what the plan will be after birth.
This usually happens around 32-36 weeks of the pregnancy. My goal during this time is to let the family and parents know that we have a plan.
Also, I want to say that in this day and age, cleft care has gotten to the point where this problem can be corrected and corrected well. I fully expect the kids I treat to grow up beautiful, healthy and happy. My passion is to make sure they will not be denied anything in life because they were born with a cleft.
Cleft lip and palate alone doesn’t affect whether the birth is a natural birth or C-section birth. If the child was born here, or comes to Kentucky Children’s Hospital immediately after delivery, I will see you and your child right away and visit as long as needed.
Just let the nurses know that Dr. James Liau saw you in prenatal consultation.
Generally speaking, there is nothing to do when a child with a cleft is born except work on feeding. At Kentucky Children’s Hospital, and the Commission, we have speech therapists and nutritionists who are experienced in cleft feeding and will help with cleft feeding.
Sometimes this can be frustrating and disheartening, but with a little parental bonding and persistence, this is easily overcome. In the meantime, I want parents to enjoy their child as any normal family should!
There is close follow-up until the time of surgery to help make sure everything stays on track and to address any questions or issues which may arise. I also like to give a tentative date for the lip repair so we can plan ahead and not have any scheduling issues.
3- 6 months
3- 6 months old is when I would proceed with a cleft lip repair (cleft palate is fixed later at 10 months). Although this is a very dramatic appearance-changing surgery for the child, it is probably one of the “easier” surgeries for them.
I am able to block most of the nerves with local anesthetic and the kids do great post-surgery. They can have their bottle right after surgery, and they stay overnight as a precaution. This is so that any later effects from anesthesia have time to wear off and in case they need some pain medication in the I.V.
There will be big changes in both the nose and lip appearance, but the lip will be covered up with Steri-Strips, which help support the skin repair. All the stitches I use will dissolve with time, so there is no need to take any out.
After an overnight stay, 99 percent of my kids go home the next day, with a follow-up in the clinic the following week and then three weeks after that.
Next we start to plan the palate repair, if there is a cleft palate.
9-12 months is usually when the palate is fixed, whether or not there is cleft lip. The reason is because the palate surgery is a little harder on their feeding and airway, so I would like them to be a little bit older and more coordinated.
Also, as you will find out, the palate is not necessary for eating, but it is very necessary for speech. Since most kids begin to say words around a year of age, I like to fix the palate and let it heal before their minds want to start talking. This way, when the mind wants to talk, they have a palate to begin talking.
This surgery is somewhat harder on the child, but 95 percent of all my kids with palate repairs go home the next day. The issues tend to be more with postoperative discomfort, as well as feeding.
After palate repair surgery, the kids are taken to the Pediatric Progressive Care Unit, which is a step down from the Pediatric Intensive Care Unit, but a step above the regular pediatric floor.
Immediately post op, I want my kids to eat or drink. The limitations are as follows though.
- For drinking
They cannot make any sucking forces since that will break down the palate repair. Before the cleft palate surgery, we should work on weaning them from the bottle and get them onto a sippy cup that pours into the mouth. Also a pacifier is a no-no since that will directly push onto the palate repair, as well as create suction.
- For eating
Stage 2 or an applesauce consistency diet is fine. As long as we keep the spoon in the front area of the mouth, then I’m not too worried about hurting the palate repair.
After discharge, I will see you the following week, and then three weeks after that.
Cleft palate children may also require a set of ear tubes during surgery. This is for fluid buildup in their middle ear. This is quite common with children with cleft palate because the muscles which help open the middle ear to drain fluid don’t work as well.
Tubes are used to help drain the middle ear to prevent further infections and possible loss of hearing. We like to do the ear tubes in the same surgery with the cleft palate if possible. I will coordinate this with the ear, nose and throat (ENT) doctors.
These are general guidelines for the first year of life, and there is a separate PDF file with the same guidelines (308KB), which you can view as well.
Hopefully this helps answer the questions of when and how often a child with a cleft lip and/or palate will require surgery in the first year. Naturally, these guidelines can change depending on the child’s progress, and I will be intimately involved in the care plan, making sure everything goes as smoothly as possible.
In the next blog entry, I’d like to address the procedures which may be required as a child gets older towards school age.