Cleft Lip Before and After palate is one of the most common congenital malformations in humans. A gap in those affected partially or completely traverses the upper lip, upper jaw, and palate. Cleft Lip Before and After and palate is cosmetically disruptive and can hinder breathing and food intake. However, it can be treated well at specialized centers—Cleft Lip Before and After Read more about cleft lip and palate.
Cleft lip and palate: description
The term “cleft lip and palate” encompasses a group of congenital malformations of the face. The upper lip, the upper jaw, and the hard and soft palate have a gap. This either runs through all of these facial structures or only parts of them.
The aperture is reminiscent of the Y-shaped indentation towards the nose on the upper lip of a hare in the upper lip. That is why the cleft lip and palate are colloquially referred to as a harelip. The medical term is cheilognathopalatoschisis.
Cleft lip and palate: shapes
Strictly speaking, a cleft lip and palate only exists if the aperture is consistently present in the upper lip, upper jaw, and palate. This collective term also includes gap shapes in which only one or two of these structures are affected:
Cleft lip: Cleft only on the upper lip.
Cleft lip and jaw: cleft on the upper lip and upper jaw. There is no isolated cleft jaw without a cleft lip, just as there is no cleft jaw and palate without a cleft lip.
Cleft palate: Cleft only on the palate.
A cleft palate either runs through the entire palate – i.e., the complex (anterior area of the palate) and the soft palate (posterior region) – or only affects the soft palate (soft cleft palate, cleft palate). There is no single cleft palate, however.
Cleft lip and palate: position
On the upper lip, upper jaw, and hard palate, the aperture position is not in the middle but slightly to the right or left of the symmetrical center (paramedian). For example, a cleft lip is always located in the so-called philtrum edge, i.e., at the edge of the vertical groove that runs from the middle of the upper lip to the nose (philtrum). On the upper jaw, the cleft runs in the region of the lateral incisor. There it can reach up to the floor of the nose. In the hard palate, too, there is always a gap to the side of the axis of symmetry; only in the soft palate is it in the middle.
A cleft lip and palate can also be present on both sides. The patients then have two columns, one to the right and one to the left of the center. Only in the soft palate are there no bilateral crevices.
Cleft lip and palate: severity
A cleft lip and palate can be differently pronounced in all sections. A complete cleft lip affects all tissue layers (mucous membrane, muscle, skin ) of the upper lip and extends to the nasal entrance. In the case of an incomplete (partial) cleft lip, on the other hand, the lip is not divided up to the nose. Doctors also speak of lip notch here.
A gap in the upper jaw can also vary in terms of width and extent (upwards, i.e., towards the nasal floor). It often widens in a V-shape towards the top. The adjacent teeth are often misaligned.
The aperture can be complete or incomplete in the hard and the soft palate and of different widths. In the area of the cleft palate, the oral mucosa, the bony palate plate, and the overlying mucous membrane of the nasal cavity are interrupted. The oral and nasal cavities are then not separated from each other. The oral mucosa and the overlying muscle layer are affected along the cleft palate. Only the uvula is split open in the minor form of a soft cleft palate (uvula Bifida).
A unique form of the cleft lip and palate is the submucosal cleft palate. The oral mucosa is preserved in the soft palate, but the muscles above it and sometimes the uvula is split. The air-filled nasopharynx then typically shimmers black through the mucous membrane.
Cleft lip and palate: frequency
The cleft lip and palate is a common malformation. In Central Europe, around every 500th child is born with it, with boys being affected slightly more often than girls at 60 percent. In 40 to 65 percent of all cases, there is a continuous cleft lip and palate. Almost a third are pure cleft palates. Isolated cleft lip and cleft lip and jaw together make up about 20 to 25 percent. One-sided columns appear twice as often on the left as on the right. The reason for this is unknown.
Cleft lip and palate: symptoms
A cleft lip and palate can lead to various symptoms and complications, both directly and indirectly. The symptoms also vary depending on the form and severity of the malformation. Above all, cleft formations with palatal involvement are often associated with numerous functional disorders. Some of the possible symptoms of a cleft lip and palate include:
In the case of cleft palates, the tongue lacks the palate vault as an abutment. This can cause it to fall back in the newborn and obstruct the airways. Serious breathing problems in babies with cleft lip and palate are rare.
Difficult food intake
Here, too, the lack of palatal support is the cause. Infants do not suck the nipple; they milk it by massaging it between the tongue and the roof of the mouth. Without a palatal vault, you won’t get enough milk. Cleft lip alone, on the other hand, hardly affects food intake.
Impaired language development
Because there is no closure between the oral and nasal cavities in cleft palates, the sound formation is often disturbed. The affected children nibble when speaking (rhinophonia). A cleft lip has little effect on speaking. Only complete forms that are left untreated cause speech disorders.
Lack of middle ear ventilation
There is a connection between the middle ear and the oral cavity, the Eustachian tube. When swallowing, it opens automatically for a short time and thus ventilates the middle ear or ensures pressure equalization. This mechanism is often disturbed in a cleft palate, and the Eustachian tube then no longer opens appropriately. This often leads to problems such as the accumulation of secretions and inflammation in the middle ear.
A cleft lip and palate often cause an unbalanced distribution of forces in the face. For example, in a cleft lip or palate, specific muscles are interrupted, which then start incorrectly. This creates a muscular imbalance that can lead to stunted growth.
Many children with a cleft lip and palate therefore also have malformations of the nose, such as a crooked nasal septum or too small nostrils. In pronounced cases, this hinders nasal breathing and forces those affected to breathe through the mouth. A gap can also impede jaw growth. The upper jaw then develops too short compared to the lower jaw (maxillary retrognathia).
Damage and malformations on the teeth
Both the position and the correct number of upper teeth are often affected by a cleft lip and palate. The adjacent teeth are particularly affected. The lateral incisor is often stunted or even missing. In addition, the teeth near the gap often have little enamel, which makes them more prone to tooth decay.
Dry air to breathe
If you have a cleft palate, the air you breathe in is not sufficiently humidified in your nose. Dry air, in turn, promotes tooth decay and infections in the mouth, nose, and throat.
Cleft lip and palate: causes and risk factors
Various factors favor developing a cleft lip and palate – external (exogenous) and internal (genetic) influences. It is believed that external factors cause most cleft malformations. This includes:
- High-energy radiation (such as x-rays or gamma rays)
- Certain chemicals and medications such as the anti-epileptic hydantoin
- Viral infections of the mother and child (such as rubella ) during pregnancy
- Alcohol abuse and smoking during pregnancy
- The severe physical and emotional stress of the expectant mother
How significant the influence of the individual factors is cannot be said precisely. It is only considered specific that they promote malformations in the unborn child in general and lead to a cleft lip and palate.
A certain proportion of cleft lip and palate is familial, i.e., caused by faulty genetic makeup. However, it is not a single gene that is the trigger; instead, several genes are involved (polygeny). The more relatives have the malformation and the closer the relationship, the greater the likelihood that a child will also develop a cleft lip and palate.
For example, if one of the parents already has a child with a cleft lip and palate, another child is also affected with a probability of four to six percent. If one of the parents also has a cleft lip and palate, the risk increases to 17 percent.
Combination with other malformations
A cleft lip and palate often occurs together with other malformations as part of specific syndromes. A syndrome is a clinical picture made up of various typical symptoms. About half of all cleft lips and palates are part of a more complex syndrome. A total of over 400 syndromes are known that can be associated with a cleft lip and palate.
Some of these syndromes are inherited, while others are unclear. Examples of syndromes with cleft formation are trisomy 13 and Pierre Robin syndrome. In the latter, the affected children have a U-shaped cleft palate and, in addition, a lower jaw that is too small (micrognathia) and a tongue that is often too large shifted into the throat (glossoptosis).
Cleft lip and palate: examinations and diagnosis
A cleft lip and palate are usually noticed immediately upon first examining the newborn. Only a submucosal cleft palate is not consistently recognized immediately. An ENT doctor or pediatrician may only become aware of them if the child suffers from otitis media unusually often.
Because the affected children often have other malformations, it makes sense to examine them in the first few days of life extensively. For example, the heart, eyes, and hearing are checked more closely.
You may be able to detect a cleft lip and palate on cleft lip ultrasound images before birth. Although there are three ultrasound examinations for pregnant women as part of prenatal care in Germany, an exact representation of the unborn child’s face is usually not included.
This facial examination is only included at specialized centers. An experienced doctor can detect a cleft lip and palate with a high probability. Isolated cleft palates are harder to identify. Even in specialized houses, only one in five is discovered.
Cleft lip ultrasound examination of the fetal face is usually carried out if the family is already aware of cleft malformations. If a cleft lip and palate are diagnosed, the shape of the gap can be determined more precisely with the so-called volume lip ultrasound. If the malformation is part of a hereditary syndrome, it may be possible to decide on this with specific examinations of the genetic material (karyogram, molecular genetic analysis).
Cleft Lip Repair – Bilateral Cleft Lip Repair
The therapy of a cleft lip and palate is lengthy and complicated. It usually takes place at specialized cleavage centers. Oral and maxillofacial surgeons, orthodontists, ENT specialists, and speech therapists work together to create a suitable therapy plan for each patient.
As a rule, treatment of a cleft lip and palate is started soon after diagnosis. The main steps take place in the first years of the life of the children. Cleft lip repair of adult patients is scarce in Germany.
The primary goal is to close the gap or gaps in all sections (immediate cleft lip repair). Functional restrictions and subsequent growth disorders are to be avoided. In addition, care is taken to ensure a cosmetically satisfactory result. In addition to the actual cleft lip surgery to close the gap, this usually requires some preparatory measures and possibly surgical interventions in further therapy (secondary treatment).
There is no uniform therapy standard for a cleft lip and palate. The different treatment centers sometimes proceed very differently; significantly, the timing varies. The basic procedure is usually the same.
The type of treatment depends on the shape and severity of the cleft lip and palate. The therapy goal is always the same: On the one hand, a standard external appearance should be achieved. On the other hand, a good speech, hearing, eating, drinking, and breathing function should be established, which allows the child an unimpaired development. Therefore, treatment should take place in a center that specializes in malformation. There, experts from various disciplines work hand in hand: pediatricians and oral and maxillofacial surgeons, orthodontists, ENT doctors, podiatrists as well as speech therapists (speech therapists) and psychologists.
If the cleft formation affects the soft and hard palate, treatment in many cleft centers begins in the first days of life. The doctor then takes an impression of the upper jaw and has a palatal plate made, which is inserted into the child before breastfeeding begins. It separates the nasal cavity from the mouth and makes it easier for babies to drink. In addition, the plastic plate, which has to be readjusted at regular intervals, promotes the correct position and function of the tongue and the shaping of the jaw and palate. This simplifies the subsequent operation.
Cleft lips are surgically closed at three to six months of age. As a rule of thumb, the baby should have reached a weight of five kilograms during this procedure. There are different opinions about the “best” time to have lip surgery on the palate. On the one hand, the palate should be closed at an early stage to allow unhindered language development. On the other hand, the procedure should be done as late as possible because the lip scar can disrupt the growth of the jaw. In most centers, it has become established that the children are operated on in about a year – when they start learning to speak.
To achieve the best possible treatment result, further lip surgery may be necessary into adulthood. This includes aesthetic corrections such as lengthening the lips or the bridge of the nose and interventions to improve language. Sometimes the alveolar ridge has to be closed when it is already ossified. This happens around the age of nine.
First of all, it is essential that a baby with a cleft lip and palate can eat without being disturbed. Like other children, it should be nursed to the breast if possible. This is usually not a problem with a purely cleft lip, but children with cleft palates need a special palate plate. This is made from plastic-based on a plaster cast and inserted into the baby. So he has an abutment for the tongue.
The palate plate must be regularly adjusted to the growing bone. It not only enables the child to eat but also controls jaw growth. In this way, it can help narrow the cleft jaw and palate. This, in turn, makes the operation easier later. If this is not possible with a palatal plate alone, there are also other orthodontic methods for this purpose.
ENT medical treatment
Secretions often accumulate in the middle ear, especially in connection with the cleft palate. This can lead to hearing loss, which in turn later interferes with language development. Therefore, a specialized ear, nose, and throat doctor examines whether such a collection of secretions is present and whether the hearing is impaired before the first operation.
If so, he will cut a small slit in the eardrum during the first operation so that the secretion can drain off (paracentesis). The slot grows closed again by itself. To prevent secretion from accumulating again in the middle ear after a while, the doctor can also insert a so-called ventilation tube into the slot. This allows the secretion to drain away permanently.
Operative gap closure
The different sections of a continuous bilateral cleft lip and palate are usually not closed at once but at other times. Various surgical techniques are available for the closure.
Lip: One usually starts with the closure of the cleft lip, preferably between the 4th and 6th months of life. Some cleft centers carry out the procedure from the 3rd month on.
Cleft palate: A cleft in the soft palate should also be closed as early as possible, by the first birthday at the latest – but preferably just a few months after the birth, like the cleft lip, to enable middle ear ventilation in good time.
You wait a little longer to close the hard palate to avoid possible surgical cleft lip scarring from disrupting the jaw’s growth. On the other hand, the nasal cavity must be separated from the oral cavity to learn to speak correctly. Therefore, a crack in the hard palate is closed around the second birthday, at the latest two and a half years after the birth.
Jaw: A narrow cleft jaw can be closed together with the cleft in the hard palate. However, if the aperture in the jaw is too broad, conventional surgical techniques will not ossify the gap, and a cleft osteoplasty will be necessary. To do this, the surgeon takes some bone material from the patient, for example, from the hipbone or shin, and inserts it into the gap. After a specific time, it grows together with the crack edges and thus closes the gap.
A jaw gap closure using osteoplasty is usually carried out between the ages of 7 and 11, preferably before the canine erupts. The dog can then be orthodontically moved into the gap where it now has a bony abutment.
Subsequent operations and accompanying treatment
Even if the surgical closure of a bilateral lip goes according to plan, functional and aesthetic problems often remain. Sometimes they are also the result of surgical scars.
Further interventions may be necessary to correct these defects, such as language-improving operations, nose corrections, or the closure of remaining holes in the palate. It would be best if you were done as soon as possible. For nose corrections, however, one usually waits for the end of the nose to grow. Otherwise, malformations can arise again.
In addition to the surgical interventions, the patients are regularly examined orthodontically and treated if necessary. Above all, when the milk teeth erupt and later the permanent teeth, you have to watch for misalignments.
Speech therapy support for the children is also essential. This should begin in the first year of life and, among other things, check the development of the lip, tongue, and palate muscles. In the 2nd and 3rd years of life, the speech therapist monitors the sound formation and intervenes therapeutically if necessary.
Cleft lip and palate: disease course and prognosis
Cleft lip and palate are complicated malformations that, if left untreated, lead to numerous complications. It takes a specialized treatment team and a lot of time to remedy all aesthetic and functional limitations. The primary operations for a lip and palate usually occur within the first three years of life. Still, subsequent corrective surgical interventions can sometimes be necessary into young adulthood.
Check-ups are also carried out at regular intervals in the non-operation phases. So the patients have to endure a lot. That can be mentally stressful for them. Therefore, the children and their parents can receive psychotherapeutic support as part of the treatment. There are also self-help groups and parenting initiatives that offer support.
But the lengthy procedure is worth it because there is usually a good result at the end of the therapy. The patients live symptom-free, and of the former bilateral cleft lip and palate, you can hardly see more than a minor lip scar on the upper lip.
What gap shapes are there? ( unilateral cleft lip – bilateral cleft lip )
Depending on when a facial development disorder occurred during pregnancy and how severe it was, there are other clefts (gap types). The gap can be a unilateral lip or bilateral. About 20% of the cracks are lip notches, lips, or lip and jaw. Around 30% are isolated cleft palates. More than 50% of cleft carriers have complete lip and palate (combination of lip and cleft palate).
Particularly in patients with unilateral lip and palate, asymmetrical development of the outer and inner nose can occur in the course of growth. If this results in significant nasal breathing disorders and/or significant impairment of appearance, a so-called septorhinoplasty is often performed. In addition to the aesthetic correction of the outer nose to eliminate nasal breathing disorders, the nasal septum is straightened, and too large turbinates are made smaller.