Cleft Lip and Palate
Clefts of the lip and palate are one of the most commonly seen conditions affecting the face in newborn babies. Clefts occur somewhere between 1 in
500 to 1 in 2000 births, depending on the race of the parents. Children can be born with just a cleft lip, a cleft lip and palate, or just a cleft palate. Most commonly, 50% of the time, the cleft patient has components of both cleft lip and palate. The cleft lip can be incomplete, complete or bilateral.
A cleft lip is a separation in the lip that results from a disturbance in lip growth during the first trimester of development. The parts of the lip that are separated vary from child to child. Some children can simply have a notching or cleft in the lip. This notching can vary in degree and is called an incomplete cleft. Other children have a complete separation of the lip that extends all the way up into the nose. This is called a complete cleft lip. This type of cleft often distorts the nose as well. Clefts of the lip can be on one side (unilateral) or on both sides (bilateral) of the lip.
Children with a cleft lip often have a hole in the gum below the lip as well. This is called an alveolar cleft. There can also be a separation in the roof of the mouth called a cleft palate. However, because the lip and palate develop separately, not all children with cleft lip have a cleft palate and vice versa.
A cleft palate may be divided into the back of the palate (soft palate) or front of the palate (hard palate) or both. This is based on when the developmental event occurs in-utero as the different portions of the palate develop at different stages, which occur between the fourth and ninth week of gestation. The soft palate is soft because it consists of muscles, which are primarily used for speech. The hard palate has bone and its function is a barrier between the nasal cavity and the oral cavity. Therefore, with a cleft of the hard palate there is often food regurgitation into the nasal cavity as well as difficulty acquiring suction for bottle feeding.
The soft palate is generally repaired around one year of age as this is the time when an infant starts to acquire speech. The hard palate may be repaired anywhere from nine months to four years of age. Surgeons who choose to repair the hard palate later often place a prosthesis in the mouth until the time of repair for ease of feeding and to allow for non-surgical narrowing of the bony cleft of the palate. There are many opinions as to the timing of closure of the cleft palate. However, most surgeons agree that speech is the foremost concern. It is the concern for surgical scarring of the hard palate that prompts varying opinions regarding timing of closure. It is our philosophy that by delaying surgical intervention until approximately 1-4 years of age, the hard palate is allowed to migrate toward the midline over time narrowing the cleft and thus allowing for an easier surgical closure. Until the time of surgery for formal cleft palate closure, We leave a palatal prosthesis (placed around 3 months of age) in place to allow for undisturbed growth of the bony cleft palate toward the midline, without the tongue in the way. This enables easier feeding with less food regurgitation into the nasal cavity.
Children with an isolated cleft lip (no cleft palate) typically have no problem eating. They can be fed with normal nipples, although a crosscut nipple can be helpful. Feeding of a child with a cleft palate is more challenging if the cleft encroaches on the hard palate or involves the entire hard palate. This is because of the difficulty and expenditure of the child’s energy during feedings as they cannot acquire a vigorous suck. If parents try to feed their baby (born with a cleft palate) by breast feeding, or using a standard nipple and bottle, they will find that after about 30 minutes their baby will get tired of trying to get enough milk and will fall asleep. Then, within 60-90 minutes, the baby will wake up, crying and hungry again. The result of this cycle is that the parents become exhausted, and the baby does not gain weight. Therefore, right after a baby is born with a cleft palate, a feeding specialist (typically a speech pathologist or occupational therapist) is called in to help. With this in mind, there are several different approaches to bottle feeding. In essence, they involve either putting pressure on the bottle to extrude the milk and deliver to your baby or releasing the milk by learning to tongue the nipple. Either method can be effective as there are several different types of nipples and bottles to assist in feeding. The critical point is to find an effective method for your baby that allows feeds to offer enough volume in a reasonable amount of time with minimal effort on the part of your baby.
A cleft of the soft palate creates a speech problem in the child. When we speak, the soft palate uses six muscles to intermittently close off the nose from the mouth. This is important in the creation of certain sounds like s, b, p, etc. When there are problems creating these sounds, the patient is said to have velopharyngeal insufficiency or VPI. Without proper closure and repair of these muscles, normal speech can not be expected. With proper surgery, most children will have normal speech (national average is 80%); however, speech therapy is frequently required.
Children born with a cleft palate are more likely to develop fluid behind their eardrums (serous otitis), which may progress to ear infections. Often, babies will need tubes put in their ears to improve hearing (children who do not hear well develop speech delays). Cleft lip and palate teams should try to coordinate the placement of tubes, when needed, with the lip or palate repair in order to reduce the total number of operations a child will receive.
There are many causes of a cleft lip, most of which have nothing to do with the parents. Therefore parents should not blame themselves for “causing” the cleft. In about one third of cases, one of the baby’s relatives will have had a cleft; in the remaining two thirds, there is no family history. A cleft does not occur because of any one thing in particular that a woman did during her pregnancy. A number of genes have been discovered that are associated with cleft lip and palate, and a number of different drugs (alcohol, cigarettes, some seizure medications, vitamin A derivatives) have also been shown to make clefts more likely. However, it is most probable that a number of factors must come together for a cleft to occur. Children born with a cleft lip and palate need to be evaluated by experienced physicians to make sure that there are no other associated problems. Once a family has a child with a cleft lip and palate, the chances of having a second child with a cleft is about 4% (many families find it helpful to meet with a geneticist to further discuss these issues). There is some evidence suggesting that if a mother is at risk for having a child with a cleft (there is a family history for clefts) taking multiple vitamins (including folic acid) may reduce the chance of having a child with a cleft.
As ultrasound technology improves, more clefts are being diagnosed in-utero before the baby is born. Most families agree that it is better to learn if their child has a cleft lip before the birth, in order to give them time to better understand this condition. Typically, families will then meet with a cleft lip and palate team, in order to prepare for the delivery. Children with an isolated cleft palate, especially if they also have a small jaw, need to be seen by a geneticist to rule out Pierre Robin Sequence, Velocardiofacial syndrome, or other syndromes.
Cleft Lip Repair
Some surgeons may recommend taping the lip together, in an attempt to narrow the width of the cleft before surgically closing it. The timing for the lip repair is most often between 3-4 months of age. The traditional rule has been that the baby should be ten weeks old, and weigh at least ten pounds for safe administration of anesthesia.
The actual surgical technique used to correct a cleft lip is fairly standard; most surgeons perform some fashion of a “rotation advancement” repair. This repair recruits skin and tissue from the sides, in order to vertically lengthen the lip, while bringing the edges together. There are different ways of closing the skin at the end of the lip repair: regular stitches, dissolving stitches and tissue glue. Regular stitches must be taken out 3 to 5 days after being placed, in order to prevent bad scarring. Dissolving stitches have the advantage of not needing to be removed. We only use dissolvable stitches and/or tissue glue in our patients so that the child does not need to have additional procedures for suture removal.
NAM (Nasoalveolar Molding)
Nasoalveolar molding is a non-surgical method of bringing the gum and lip together by redirecting the forces of natural growth. It is non-painful and easy to use. This molding process is coordinated by our orthodontist and usually takes about 12 weeks. It also allows for correction of the flattened nose prior to surgery, and facilitates nose repair at the time of lip repair. This technique is becoming the “gold standard” for cleft lip repair and we are proud to be able to bring this technique to the families.
Essentially, our orthodontist fashions a mold of the oral cavity and uses an passive process over several weeks to narrow the cleft. There is also usually an outrigger device placed into the nose to help round out the cartilage of the nose to a more normal anatomy. Towards the end of the process there is usually taping of the lip to relax the tension for ease of closure of the lip just prior to surgery. This is a labor-intensive process for both the parents and the orthodontist. This process usually takes about 3 months with visits at least every other week to remodel the molding device. The goal is to narrow the cleft, align the cleft segments, round out the nose, and bring the lip segments closer together. This all enables the cleft lip and palate to migrate closer together for easier surgery and allow for improved scarring. Over the last ten years the results from institutions that perform NAM have been impressive, especially regarding scars and alignment of the nose. We offer this to all cleft lip and palate patients who are willing to comply with this regimen. We will specifically try to have all bilateral cleft patients engage in this process due to the exemplary results.
Cleft Palate Repair
Children with a cleft of the soft palate undergo a definitive closure between 9-15 months of age. At this time, we often perform a Z-plasty of the soft palate, which is a lengthening procedure. This type of closure takes a little longer (about 2 hours) but is both more anatomic and provides additional length, allowing for fewer post-operative speech problems than traditional straight-line soft palate repair.
If a hard palate cleft is present, then a static palatal prosthesis is placed in the first 3 months of life. The goal is to make feeds easier, preventing reflux into the nose, as well as allowing the hard palate to migrate to the midline over time. This is a slow process, but by preventing the tongue from interfering during feeds and other activities the prosthesis allows the hard palate to grow towards the midline. When the prosthesis is removed we wait about 30 days before operating on the palate to allow the surrounding tissues to strengthen enough to hold suture. Patients undergo hard palate repairs between 1-3 years of age, depending on the width and severity of the cleft deformity.
After palate surgery, your child will be watched closely for any problems with breathing, and a nursing specialist will work with you to start feedings as soon as possible. Feeding is liquid or puree only for 2-3 weeks via a sippy cup or a syringe allowing time for the stitches to dissolve. Your child may also wear elbow immobilizers during this time to prevent the placement of any unwanted objects inside the mouth. The immobilizers can be taken off when your child is being held. After about 4 weeks, the palate should be healed, and we will advance the diet to regular foods.
Most patients will stay in the hospital 1 day following surgery. Children can go home when they are able to drink well. Your child may be fed with a syringe initially, but after 1-2 days, the use of the soft specialized nipple may be reinitiated. Your child may also wear protective elbow immobilizers, which prevent your baby from scratching his or her face or reaching into the mouth and disrupting the sutures. These immobilizers are worn for the first 2-3 weeks but may be removed when you are holding your baby.
After 2 weeks of healing, you will begin lip massage 3-5 times a day. This is essential because it decreases scar and helps prevent lip “notching” which always occurs after surgery. Massage helps you get the best result for your child and our staff will instruct you in the proper method. We usually recommend vitamin E over the counter lotion to apply 3-5 times per day for a minute at a time along the length of the scar with some digital pressure. This massage will continue for up to 6 months after surgery.
Following the lip and palate repair, many children are done with their lip and palate surgery. It is important to watch dental and speech development as well as the maturation of the lip scar and the growth of the upper jaw. Occasionally, however, some additional surgeries are required to optimize the final result. As your child grows, he or she may need some “touch up” lip surgery.
Alveolar Bone Grafting
Alveolar bone grafting is usually done between 6 and 8 years of age to allow for bony continuity and adult dentition to form properly. An alveolar cleft is a breach in the gum line where there is usually teeth and bone. Because children lose their initial “baby” teeth it is not until the adult teeth begin to form that we consider bone grafting. If bone is not placed into the alveolar cleft prior to tooth eruption, then the adult tooth will never erupt. Currently alveolar bone grafting involves obtaining bone cells from the marrow of the hip and placing them into the cleft. This is a painful procedure and usually requires at least one overnight stay in the hospital. However, we are currently researching and employing other modes of obtaining bone graft that are less painful and do not require overnight stay. Definitive nasal surgery, when necessary, is usually delayed until after alveolar bone grafting so there is a stable bony platform for nasal reconstruction to rest upon.
LeFort I Advancement
Children who have a retruded maxilla or under bite may need additional jaw surgery. This occurs in approximately 20% of cleft patients. Most think that this is attributed to the scarring caused by the multiple surgical interventions. Because our practice intervenes surgically on the hard palate later in age, the need for upper jaw surgery has decreased. Those who still require surgery to align their jaws usually have this done between the ages of 5-8 years with a distraction device. Those who are older, with dental skeletal maturity in their teenage years, undergo a more traditional advancement of the upper jaw with surgical plating.
The procedure involves cutting the upper jaw and moving it forward. It is moved forward by one of two methods: either by traditional plates or screws that are placed at dental maturity (between 13-17 years of age based on gender) or through distraction. Distraction is a method of slowly moving bone and soft tissue forward through either an internal or external distractor. External distractors are cumbersome but provide a great deal of control. Internal distractors and dissolvable materials are preferable and are being used more often now. The benefits of the internal distractor are that there is less of a social stigma and less interference with daily activities. Disadvantages are that there is less control in the post-operative period, they often require a second procedure, and they are still relatively new.
Distraction involves phases which include a consolidation period from 4-6 weeks upon completion of the forward movement. This allows the new bone that has grown to harden over time and retain the forward movement. During these 4-6 weeks the child continues to have hardware as well as maintain a soft diet. External distracters are removed with very little pain in the office. Internal distracters, unless made of dissolvable material, require a secondary procedure for removal. The results are usually impressive and lasting.
VPI (Velopharyngeal Insufficiency) Procedures
VPI, essentially, is when the soft palate is not working properly and allows speech to escape through the nose rather than solely through the mouth. This is assessed clinically by the surgeon or speech pathologist who is listening for nasal air escape with speech. This can also be assessed through special types of MRI or endoscopy. When it is determined that the causes of speech problems are result of a short or inappropriately closing soft plate often surgical intervention is required. This usually entails either lengthening the palate through a Z-plasty or taking tissue from the back wall, called the pharynx, and laying the tissue over the opening. This is usually done in one of two methods: the first called a pharyngeal flap and the other a sphincteroplasty. The surgical intervention is determined by the anatomy and location of air escape. The outcomes for both of these procedures are essentially equivalent.
In light of NAM techniques and better cleft surgeries the need for rhinoplasties has been reduced. Children with bilateral clefts have a higher incidence for rhinoplasty. However, it is somewhat dependent on the individual in determining if nasal aesthetics and/or breathing need to be improved. If necessary, the surgery is put off until either social stigmata dictates or after early teens have completed the alveolar bone grafting process. The nose is degloved and the internal architecture including cartilage, nasal septum and bone can be addressed. Often times the cleft deformities require cartilage grafting to round out the lower portion of the nose. The cartilage may be obtained from either the nasal septum or from the ear cartilage through a separate well-hidden incision behind the ear.