Cleft lip and palate

Embryology

During intrauterine growth, differentiation of the face begins in the 4th week. All normal structures are in place by the end of the 10th week, meaning that cleft lip and palate develop before this time. Clefts of the lip, maxilla and palate result from an interruption in the development of the nasal cavities during the embryonic stage. This interruption may occur on one side of the nose or on both sides, and at different stages of development, which explains why there are many different types of cleft.

It is important to know that, in clefts, no part is missing; rather, some parts have not fused.

Types of clefts

Cleft lip (25%)

They may be left-sided, right-sided or bilateral, complete or incomplete.        

In some children, a small bridge remains connecting the two sides of the cleft beneath the nostril; in this case, the nostril will be better formed. Cleft lip is often accompanied by a cleft of the maxillary arch located between the lateral incisor and the canine.

Cleft palate (25%)

Unlike cleft lips, these are located centrally.

The palate consists of two parts: the hard palate and the soft palate. The hard palate, located at the front, is covered by a fibrous mucous membrane against which the tongue rests during chewing. Behind the hard palate is the soft palate, which ends at the back in the uvula. The soft palate is made up of palatal muscles that lift it during speech and swallowing. A cleft palate may also be covered by mucous membrane and may therefore be more difficult to diagnose (submucous cleft). These clefts do not cause problems with swallowing, but they do cause problems with speech, which is why it is important to operate on them.

Cleft lip and palate (50%)

These combine a cleft lip with a lateral cleft of the maxillary arch extending backwards into the midline cleft of the palate. They may be unilateral or bilateral.
 

Genetics

One in 500 children has a cleft lip or cleft lip and palate (0.2%), and one in 2,000 has a cleft palate (0.05%). In most of these children, there is no family history of clefts.
  
There are naturally occurring parental factors: the older the parents, the greater the risk. The use of medication to treat the mother’s diabetes or epilepsy may increase the incidence of this malformation.
As regards external factors, a whole range of factors acting during the first weeks of pregnancy may cause cleft lip and palate: rubella, toxoplasmosis, etc.

If one child in the family has a cleft lip or cleft lip and palate, the risk for a second child is 3–7%. If one parent has a cleft, the risk is 2–5%. If both a child and a parent have a cleft, the risk is 15%.
For reference, the risk in a population without a cleft is 0.2%.
If one child in the family has a cleft palate, the risk for a second child is 2–5%. If one parent has this type of cleft, the risk is 3–7%, and if both a parent and a child are affected, the risk is 17%.
For reference, the risk in a population without a cleft is 0.05%

Although cleft lip and/or palate is the only congenital anomaly in most children, in 15% of cases this anomaly is associated with others.
More than 300 craniofacial syndromes involving cleft lip and/or palate have been described. It is therefore essential to carry out a complete assessment of your child to determine whether any other malformations are present and thus establish an appropriate treatment plan.

• The plastic surgeon
  • They will operate on your child from the age of three months and follow them throughout their growth.
  • They will perform any subsequent corrective procedures if necessary.
• The maxillofacial surgeon and the orthodontist: The maxillofacial surgeon will make the small plates your baby will need to protect their palate before and after surgery. This small plate, called an orthosis, will facilitate the surgical procedure performed by the plastic surgeon. It may also help your baby suck. The maxillofacial surgeon will follow your baby as they grow, when their milk teeth and then permanent teeth appear, and will monitor the development of their upper jaw.
• The orthodontist will begin a treatment to straighten the teeth as needed. Orthodontic treatment may be started from the age of 5, depending on the deformation of the jaws resulting from the cleft. It will continue once the permanent teeth have appeared, as these may show delayed eruption as well as significant malpositioning.
• The otorhinolaryngologist: They will examine your baby’s nose, throat and ears and assess their hearing throughout their growth. It is not uncommon for transtympanic drains to be inserted. They will also give you advice on facilitating the drainage of nasal secretions, which is often difficult in young children.
• The geneticist: They will inform you of the risk of recurrence of a cleft lip and/or palate in your future children and in your child’s future children.
• The psychologist: They will help you and your child overcome any social, psychological or physical difficulties that may result from the malformation.
• The specialist nurse: They will guide you during your first weeks with your baby. Advice on breastfeeding at home, help with feeding…
• The speech therapist: They will advise you regarding your baby’s breathing, phonation, swallowing and sucking. They will then monitor the development of your child’s language and guide you as to the appropriate time to begin speech therapy, if necessary.
• The paediatrician: They will follow your child throughout their growth and will be an integral part of the team caring for your child.
   

Primary surgical treatment

The aim of surgical treatment is to close the cleft in order to restore function while disturbing facial growth as little as possible.

In most cases, the cleft lip and/or palate will be repaired at the age of 3 months. In the rare cases where only the lip is cleft, an earlier procedure may be considered. In the case of a wide cleft lip and palate, the procedure will be performed in two stages: repair of the soft palate at 3 months and closure of the lip and hard palate at the age of 6 months.

• The lip: the skin, muscles and mucous membrane are separated, then brought together and sutured to restore the structures and give the lip a normal shape and function. The scar will be vertical, parallel to the philtral column on the unaffected side.
• The hard palate: separation and suturing are performed only on the mucous membrane. The bones are not moved, in order not to interfere with their growth.
• The soft palate: closure involves separating the tissues and suturing the mucous membrane and muscles of the soft palate in 3 layers. No tissue graft is required.

For this first surgical procedure, your child will be hospitalised for a few days. On the day of the operation, your child must remain fasting (including milk) for 6 hours before the procedure. Immediately after the operation, your child will be allowed to drink. Slight bleeding may be observed around the palate immediately after surgery.

Secondary surgical treatments

In 15% of children with a cleft palate, unresolved speech problems will be observed at around the age of 5 years despite speech therapy. When the cleft palate is very wide, it is not always possible to achieve sufficient length of the soft palate when closing it. An endoscopic or videofluoroscopic examination enables us to confirm the diagnosis of a short palate and, in this case, a pharyngoplasty will be performed. Speech therapy may be necessary after this type of procedure. Following this surgical procedure, your child will need a puréed and liquid diet for approximately two weeks.

At around the age of 10 years, most children with a cleft of the dental arch will require an intermaxillary bone graft. To help the canine tooth erupt on the side of the cleft, bone taken from the iliac crest must be placed in the dental arch. For this surgical procedure, your child will be hospitalised for a few days. During the week following the operation, your child will need a puréed and liquid diet.

During adolescence, if the upper jaw does not develop harmoniously, it may be necessary to perform an expansion procedure called distraction osteogenesis. This operation is performed by the maxillofacial surgeon in consultation with the orthodontist.

At around 17–18 years of age, children with a cleft lip or cleft lip and palate who wish to do so may undergo corrective surgery for both aesthetic and functional purposes (septorhinoplasty, revision of the lip scar, etc.). These procedures can only be performed once growth is complete, to avoid the risk of hindering the development of the child’s face.

Orthodontic treatments

If your child has a cleft of the hard palate, orthodontic treatment will begin during the first few weeks. The orthodontist, or more often the maxillofacial surgeon, will make a small plate that will be attached to the palate using a specific adhesive powder. This plate will be cleaned and put back in place after each feed. Its role is to gently correct the malposition of the palatal bones and enable your child to create negative pressure with their tongue, thereby making sucking possible. In addition, this plate will protect the intraoral scars and will be worn until a few weeks after complete closure of the palate, in order to protect the suture from tongue movements.

In the case of a very severe cleft, elastic pressure may sometimes be applied to the central fragment of the dental arch (which will support the upper incisors) in order to gradually move it backwards into a more anatomical position, thereby facilitating closure of the palate.

Subsequently, dental alignment treatment will be undertaken as needed. When the dental arch is cleft, the teeth do not grow in the correct alignment and treatment is often necessary. This may be started as early as the age of 4 years. Good dental hygiene is therefore essential to ensure that the teeth can support the corrective appliances.

In addition, some primary and permanent teeth may be missing. A panoramic X-ray taken at the age of 5 years will provide a complete dental assessment, and a lateral cephalometric X-ray will make it possible, as early as the age of 5 years, to measure the development of the upper jaw and mandible.

Hearing treatments

The middle ear communicates with the inside of the mouth through a canal called the Eustachian tube. Among other functions, this canal drains secretions that accumulate behind the eardrum in the middle ear. This function is ensured by muscles connecting the soft palate and the Eustachian tube. When the soft palate is cleft, the palatal muscles are abnormally positioned and the middle ear cannot drain properly, resulting in an accumulation of fluid behind the eardrum, an increased risk of infections and partial hearing loss.

When the palate is closed, the ENT specialist may sometimes insert a drainage tube into the eardrum to ensure proper drainage of the middle ear and consequently improve hearing. This procedure will be decided during the preoperative ENT consultation on the basis of the ear and hearing examination. A more precise hearing test (auditory evoked potentials) is generally performed in hospital on the day before the operation.

Careful hearing monitoring is required, even after closure of the soft palate. Indeed, reduced hearing acuity, which is already detrimental to the child’s harmonious development, may also compromise speech and language therapy. 

Generally, these problems improve around the age of 10 years, when the tonsils and adenoids spontaneously regress.

However, as Eustachian tube function remains suboptimal even after surgery, annual ENT follow-up is recommended until adulthood.

Speech and language therapy

Your child’s speech will probably develop normally. However, minor speech problems may occur. 

Speech and language therapy is sometimes necessary, either to help a child whose palate is sufficiently long to make contact with the posterior wall of the pharynx during speech, or after pharyngoplasty in children whose palate is too short. 

It only exceptionally continues for more than 12 months.

In the event of a cleft lip, your child may have difficulty breastfeeding, but if this is the case, you can feed them with a bottle.

In the event of a cleft palate, a little milk may pass through the nose when your baby drinks. There is no danger. You will quickly find the right position to minimise this as much as possible. Avoid laying the child down to feed them and burp them frequently during feeding, as they swallow more air than other children. 

Use a soft teat with a wide opening: previously used teats are preferable to new ones. It is important for you to know that your child will be fed like any other child. They do not need any special diet because of their cleft palate. They will receive thicker foods and solid pieces at the same age as other children.