Overview of Cleft Palate Repair
Understanding and treating a condition like a cleft palate needs a deep knowledge of how the roof of the mouth grows and develops. It also requires knowing how this part of the body helps us to talk, swallow, breathe, and hear. The way in which a baby’s upper lip and roof of the mouth wasn’t joined together when they were developing in the womb can occur in different ways and is found in approximately 1 to 25 in every 10,000 babies born. This issue occurs more often in white babies, and less often in black babies.
A cleft palate can come in many forms including a thin membrane over a split in the roof of the mouth (submucous clefting), splits in the back portion of the roof of the mouth (secondary palate clefting), splits in both the front and back portions of the roof of the mouth (primary and secondary palate clefting), and most severe splits which affect the gum and lip as well (complete clefting). The exact way in which the clefting happens along with the direction and the degree of the voice-producing part of the throat not working properly varies in different people. Some other medical problems can also occur along with a cleft palate.
About half of the babies born with a cleft palate don’t have any other health conditions (non-syndromic), but there are many other health conditions (syndromes) that can also be linked with cleft palates. Each baby is different, and each way in which cleft palate occurs can also be different. That’s why it’s crucial to fully understand the options for treating a cleft palate and the potential impacts, not only of having a cleft palate, but also of having its repair done. These understandings will help the team of healthcare professionals in providing the safest treatment to these babies, in making the outcomes as good as possible, and in lessening the complications.
Anatomy and Physiology of Cleft Palate Repair
The structures of our skull and face begin to form between the 4th and 8th week of being in the womb. If something goes wrong during this important period, the result could be a cleft, or split, in the mouth and face. There are two types of these clefts. A primary cleft palate happens when parts of the mouth’s roof, or palate, don’t come together properly. A secondary cleft palate occurs when the sides of the palate and the wall dividing the nostrils don’t fuse.
In the womb, the lining of the palate takes shape differently on each side. On the side facing the nasal cavity, it becomes a layer of column-like cells with tiny hair-like structures. On the side facing the mouth, it turns into a layered, flat cell type.
If we look at the roof of the mouth, or the palate, it’s broken down into two parts. The main dividing line is a small hole called the incisive foramen. The primary palate is in front of this hole and contains the front teeth and part of the bony ridge where teeth sit. The secondary palate is behind this hole and includes the rest of the teeth, bony ridge, and the remaining parts of the palate, both hard and soft. If a cleft occurs, it can affect any of these areas and can appear on one or both sides.
People with a cleft palate also have muscle abnormalities. One of the main muscles affecting speech, the tensor veli palatini muscle, is not attached properly. Similarly, another muscle, the levator veli palatini muscle, attaches incorrectly. In surgery, the correct placement of these muscles is crucial for improving the movement of the palate after surgery.
The palates get their blood supply from a blood vessel called the descending palatine artery. In surgery, it’s essential to avoid damaging this blood vessel as it can lead to the death of tissue in the palate.
In some patients, a hidden or ‘submucous’ cleft palate may be present. They might have abnormal speech or a weak palatal muscle, even though no visible cleft is apparent. Physical exams might find a split uvula, a thin spot in the roof of the mouth, or a noticeable dent at the back of the hard palate. Depending on the patient’s individual case and their age, management can range from speech therapy to surgery and post-operative speech therapy. It’s crucial to correctly adjust these muscles during surgery to improve pronunciation, especially of syllables like “p,” “k,” and “s.”
Why do People Need Cleft Palate Repair
If a person has a noticeable cleft in their palate, they will usually need surgery to fix it. This is mostly because this condition can lead to problems with speaking and swallowing. Doctors usually wait until a child is between 6 to 18 months old before doing the surgery. People with a hidden (“submucous”) cleft palate may also need surgery, especially if they’re having trouble speaking or swallowing. This is particularly considered if they’ve already tried speech therapy without success. If someone still has trouble with speaking or swallowing after cleft palate surgery and speech therapy, the doctor might think about doing another surgery to improve the situation.
When a Person Should Avoid Cleft Palate Repair
There’s no absolute reason that someone couldn’t have a cleft palate repair surgery. However, there are some situations where it might be less safe or need some other issues to be dealt with first. For example, someone with a serious illness or a heart condition might not be able to have the surgery because they can’t undergo general anesthesia, which is necessary for this surgery.
Also, some people with conditions that affect their airway might need those problems treated before they can have their cleft palate repaired. This includes conditions like retrognathia, where the lower jaw is set further back than the upper jaw, or Pierre Robin sequence, a condition present at birth where a baby has a smaller-than-normal lower jaw, a tongue that falls back into the throat, and difficulty breathing.
Equipment used for Cleft Palate Repair
If your doctor is planning on repairing a cleft palate, there are a number of tools that they will need to use. Here’s a list of them:
- A fine suction cannula, such as a Frazier 7 Fr, which is a tool for removing fluids or gases from body areas during surgery.
- A mouth retractor like the Dingman or Fisher. This tool is used to pull open the mouth and keep it open for the surgery, with different-sized tongue blades for different patient sizes.
- Periosteal elevators, which include tools like the Freer, Cronin, Molt #9, Warwick-James, Mitchel’s trimmer, and Barsky cleft palate rasp. These tools help to separate the periosteum (the layer of connective tissue that surrounds the bones) from the bone, creating a space to work in during surgery.
- Various types of retractors, like a single hook or a Guthrie hook, which are used to hold tissues away from the surgical area.
- Long forceps like the Gerald toothed or non-toothed forceps. Forceps are similar to tweezers and used in surgery for grabbing or holding things.
- Long scissors such as Metzenbaum long scissors for cutting tissue.
- A long needle holder like the Rider for holding suturing needles during the sewing up process.
- An electrosurgical knife, also known as electrocautery, which uses an electric current to cut tissue and minimise bleeding.
- Diathermy with a long Colorado tip. Diathermy is a technique using heat to destroy abnormal cells, and the Colorado tip allows for precision cutting and coagulation.
- Long bipolar electrocautery forceps for conducting electric current during surgery to prevent bleeding.
It’s also strongly suggested that the surgeon use surgical loupes with 2.5-4x magnification to view the surgery area better. However, some surgeons might prefer to use an operating microscope instead, which provides an even clearer and more magnified view of the surgical area.
Who is needed to perform Cleft Palate Repair?
A doctor, known as a reconstructive surgeon, who specializes in fixing cleft palates, or the split in the roof of the mouth, is a part of the medical team. A surgical assistant is also present to support the surgeon during the operation. An anesthesiologist is there to manage your pain and make you sleep during the surgery; for children, they prefer a pediatric anesthesiologist who specializes in working with kids. A nurse circulator is also there to make sure everything goes well around the surgery area. Lastly, a technician or nurse is there to help pass the medical tools and instruments to the surgeon during the operation.
Preparing for Cleft Palate Repair
During pregnancy check-ups, many parents worry about the chance of their baby having a cleft lip or cleft palate (a split or opening in the lip, upper lip area, or roof of the mouth). If a family has one child with a cleft lip and palate and no family history of it, there is a 4% chance of a second child having the same condition. This likelihood increases to about 9% if two children have a cleft lip and palate. Also, if one parent and one sibling have a cleft lip and palate, the risk goes up to around 17%. More severe clefts can also lead to a higher chance of recurrence.
Half of the patients with a cleft palate have a type known as nonsyndromic clefting, where the cleft lip or palate is not linked to any other physical or health problems. The other half are linked to genetic syndromes, which may be due to a single abnormal gene (monogenic syndromes) or multiple abnormal genes. One such monogenic syndrome is velocardiofacial syndrome (also known as Shprintzen syndrome), where there is a deletion of a part of chromosome 22. Patients with this syndrome often have features like a prominent nose, notched nostrils, and a small chin. In such cases, it is crucial for doctors to check the location of major blood vessels (like carotid arteries) using imaging tests before operating in the mouth or throat area. This is because these arteries can be displaced and accidentally injured during surgery, which can have serious consequences.
Before surgery, doctors have a detailed discussion with parents or caregivers about the procedure. This includes explaining that the surgery will be performed under general anesthesia, and discussing the various risks involved. These risks can include bleeding, infection, complications with the surgical wound, the need for follow-up surgery, and the possibility of the patient needing a breathing tube after surgery and monitoring in the intensive care unit (ICU). Although these complications are rare, it’s important to be aware of them.
Before undergoing surgery, the patient must be healthy, free from infections, and have healthy levels of hemoglobin (a protein carrying oxygen in the blood). The patient must not eat or drink anything for a short period before surgery. The surgery is carried out while the patient is under general anesthesia, lying on their back. Any factors that might prevent neck extension (like a genetic disorder called trisomy 21) need to be taken into account, as the position of the neck can make the surgery easier. A special tool called a mouth retractor is used to keep the oral cavity open during surgery. It’s important to protect and lubricate the lips and tongue during surgery to prevent unintended injuries.
Patients with a cleft palate often experience middle ear infections which can affect their hearing in the first two years of life. This can impact their speech, language, and emotional and intellectual development. Therefore, newborns with a cleft palate should receive hearing screenings soon after birth, and tests for middle ear problems before cleft palate repair surgery. If these tests reveal any dysfunction, an ear, nose, and throat (ENT) specialist may need to place tubes in the ears to equalize pressure. This can usually be done during the same surgical session as the cleft palate repair, reducing the overall anesthesia exposure for the patient.
How is Cleft Palate Repair performed
Doctors use a few different surgical techniques to fix a cleft palate. No matter which method is used, the first step is always cleaning the nose, mouth, and throat with an antiseptic solution — this prevents infection. After that, the surgeon injects a local anesthetic with medicine to help slow down bleeding and make it easier to see what they’re doing.
One technique is known as Von Langenbeck. With this method, the surgeon makes cuts along the sides of the cleft, or gap, in the soft part of the roof of the mouth. After making these cuts, they carefully separate the different tissues in the palate. Once this step is complete, they sew together the different layers of the palate. The same technique is used on the hard part of the roof of the mouth too. If needed, they can use a special tool to make more room for the tissues in the mouth to move around. All of this helps fix the cleft. They might also use a related procedure called Intravelar veloplasty to help with this.
The Bardach method, aka “Two-flap” palatoplasty, is another common surgical technique. This method uses the same steps as Von Langenbeck’s, but with some differences. The surgical team can make more cuts to create flaps made of the muscle and skin of the roof of the mouth. Then, they can move the tissues around and sew them together to fix the cleft. Sometimes they add some supporting materials to the area where the bone is exposed after surgery.
Veau-Wardill-Kilner’s method, or “V to Y pushback”, is another way to fix the cleft palate. It’s similar to the previous method, but they make the cuts in other places in the palate. Doing this allows the surgical team to move the tissues in the mouth, which can make it easier to fix the cleft. They sew everything in place after that. However, some doctors avoid this method because it might cause problems after surgery.
The Furlow Double Opposing Z-Palatoplasty is also used. This method uses a special way of making cuts — in a Z shape — to fix the cleft in the soft part of the roof of the mouth. The Z-shaped cuts let the doctor move the tissues around more easily, which helps fix the cleft. They might use this method with the other methods if there’s a cleft in the hard part of the roof of the mouth too.
Remember, these are all ways that surgeons help children who are born with a cleft palate. The details behind each method might be a bit complex, but the aim is always the same: to fix the cleft and allow the child to eat, speak, and breathe more easily.
Possible Complications of Cleft Palate Repair
After surgery for a cleft palate, some patients might have complications that occur immediately, within two weeks of the procedure.
One issue can be a swollen tongue, which can block the airway in the mouth. This generally occurs if the mouth retractor used during surgery was the wrong size or put too much pressure on the tongue. The swelling can become quite serious and block the airway. In infants, who often lie on their backs, a swollen tongue can fall back and block their throat. Swelling of the area where the surgery was done, or “palate,” could also block the airway by narrowing the spaces through which air usually passes in the back of the throat. Doctors usually deal with this issue by putting in a stitch that pulls the tongue forward to open the airway or by putting a special tube in through the nose to keep the swollen area out of the way to allow for breathing through the nose.
Sometimes, patients with other health conditions, special needs patients, or those who had issues with heart and lung stability during the operation might not be able to have their breathing tube taken out right after surgery. These patients usually need to be transferred to intensive care for monitoring. In some cases, the voice box might go into a persistent spasm after the breathing tube is removed. If this happens, doctors might need to give the patient a breathing mask or put the breathing tube back in.
Bleeding after surgery can also occur when the patient first wakes up from anesthesia or starts crying hard. This can cause blood clots to shake loose from the surgery site. Usually, putting gentle pressure on the area with a small piece of gauze for five minutes can stop the bleeding.
Another complication is when the surgical repair separates, either partially or totally. This can happen if there was too much tension across the repair site or if the site was hurt in some way after the surgery. Infections might also happen. These can show up as a hole, or “fistula,” in the repair, or as a more obvious infection with redness, heat, pus, and pain.
Long-term complications can happen two or more weeks after the surgical repair.
One such issue is a fistula, which is a hole that can make a pathway between the mouth and nose. This can be caused by the repair failing in a spot, an infection, or injury. These fistulas might allow fluids and solid foods to go from the mouth into the nose, and they usually require another surgery to fix.
In some cases, the joint where the repair was done or the flap of tissue at the back of the throat might partly separate. This is usually due to the technique used to close the flap. Both surfaces of this flap need to be carefully aligned to prevent this from happening.
Sometimes, the repaired palate might not move the way it should, which is usually due to badly managed tissues, too much scar tissue, healing problems, or neurological issues. In very rare cases, the repaired palate might even start to break down, a condition caused by injury to a major artery during the repair. This can happen right away or later on.
What Else Should I Know About Cleft Palate Repair?
The goals of repairing a cleft palate, which is a gap in the roof of the mouth, are as follows:
1. Adjusting the muscles in the roof of the mouth to make it easier for you to speak clearly.
2. Separating the mouth from the nasal cavity.
3. Fixing the hole in the roof of the mouth.
One of the methods used in cleft palate repair is called “robbing Peter to pay Paul.” This means using tissue from one area to repair another. In this case, the surgeon will remove a layer of tissue from the sides of the roof of the mouth to close the gap in the middle. This tissue will grow back in about two to three days.
Another important part of the surgery is to carefully move and sew the muscles involved in swallowing and speech (the tensor and levator veli palatini) so they’re closer to a normal position. This should help improve your ability to speak and swallow.
One of the most important things for a surgeon to remember during the procedure is to avoid stretching the area they’re repairing too much. Too much tension can lead to the repaired area opening up again and creating a small channel between the mouth and nose.
