Cleft lip and palate

Cleft lip and palate: Description

The term “cleft lip and palate” includes a group of congenital malformations on the face. Here, the upper lip, the upper jaw and hard and / or soft palate have a gap. This pervades either all of these face structures or only parts of them.

In the upper lip, the cleft is reminiscent of the Y-shaped retraction to the nose on the upper lip of a hare. Therefore, the cleft lip and palate is colloquially referred to as harelip. The medical term is cheilognathopalatoschisis.

Cleft lip and palate: shapes

Strictly speaking, a cleft lip and palate is only present if the cleft is continuously present in the upper lip, upper jaw and palate. In fact, 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 of the mouth: Column on upper lip and upper jaw. There is no isolated cleft lip with no cleft lip, nor is there a cleft lip and palate without a cleft lip.
• Cleft palate: cleft only on the palate.

A cleft palate penetrates either the entire palate – ie the hard (front of the palate) and the soft palate (posterior region) – or affects only the soft palate (soft cleft palate, soft palate column). There is no single hard-cleft cleft, however.

Cleft lip and palate: position

On the upper lip, upper jaw and hard palate, the position of the cleft is not central, but slightly to the right or left of the symmetrical middle (paramedian). For example, a cleft lip is always in the area of ​​the so-called Philtrum edge, ie at the edge of the vertical groove that runs from the middle of the upper lip to the nose (Philtrum). In the upper jaw, the cleft runs in the region of the lateral incisor. It can reach there up to the nasal floor. Even in the hard palate, a column is always located laterally of the axis of symmetry, only in the soft palate is it in the middle.

A cleft lip and palate may also be present on both sides. Patients then have two columns, one to the right and one to the left of the center. Only in the soft palate there are no two-sided columns.

Cleft lip and palate: severity

A cleft lip and palate may vary in severity in all sections. A complete cleft lip affects all tissue layers (mucous membrane, muscle, skin) of the upper lip and extends to the entrance of the nose. In the case of an incomplete (partial) cleft lip, on the other hand, the lip is not severed to the nose. Doctors also speak of lip score here.

A column in the upper jaw can also vary in terms of width and extent (upwards, ie towards the floor of the nose). Often it becomes wider V-shaped upwards. The adjacent teeth often have malpositions.

In both the hard and the soft palate, the column may be complete or incomplete and of varying width. In the area of ​​the hard-palate cleft, the oral mucosa, the bony palate plate and the overlying mucous membrane of the nasal cavity are interrupted. The oral and nasal cavities are not separated from each other. Along the soft palate cleft, the oral mucosa and overlying muscle layer are affected. In the smallest form of a cleft palate, only the uvula is split (uvula bifida).

A special form of the cleft lip and palate is the submucous cleft palate. Here, the oral mucosa is preserved in the soft palate, but the overlying muscles and sometimes the suppository split. Through the mucous membrane then typically the air-filled nasopharynx shimmers through black.

Cleft lip and palate: frequency

The cleft lip and palate is a common malformation. In Central Europe, about every 500th child is born with it, with boys are affected at 60 percent more often than girls. In 40 to 65 percent of all cases there is a full-length cleft lip and palate. Just under a third are pure cleft palates. Isolated cleft lip and cleft lip together account for about 20 to 25 percent. One-sided columns appear twice as often on the left side as on the right. The reason is unknown.

Cleft lip and palate: Symptoms

A cleft lip and palate can lead directly and indirectly to various symptoms and complications. Depending on the form and severity of the malformation, the symptoms also vary. Especially clefts with involvement of the palate are often associated with numerous dysfunctions. Possible symptoms of a cleft lip and palate include:

respiratory disorders

In cleft palate the tongue lacks the palate vault as an abutment. As a result, it can fall backwards in the newborn and relocate the respiratory tract. Serious respiratory problems in babies with cleft lip and palate are rare.

Difficult food intake

Again, the missing palate abutment is the cause. Because infants do not suck at the nipple, they milk them by massaging them between the tongue and the palate. Without a palate, they do not get enough milk. Sole cleft lip, on the other hand, hardly affects food intake.

Disturbed language development

Because there is no closure between the oral cavity and the nasal cavity in cleft palate, the phonation is often disturbed. The affected children nasal when speaking (rhinophony). A cleft lip has little effect on speech. Only very broad forms that are not treated cause speech disorders.

Missing 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 aerates the middle ear or ensures pressure equalization. This mechanism is often disturbed in a cleft palate, the Eustachian tube then no longer opens properly. This often leads to problems such as secretions and inflammation in the middle ear.

stunted growth

A cleft lip and palate often causes an unbalanced distribution of forces in the face. Thus, in a cleft lip or cleft, certain muscles are interrupted, which then start wrong. This creates a muscular imbalance that can lead to growth disorders.

Many children with a cleft lip and palate therefore have malformations of the nose, such as a crooked nasal septum or too small nostrils. In severe cases, this hinders nasal breathing and forces those affected to breathe through the mouth. Also, the jaw growth may be hampered by a gap. The upper jaw then develops too short (maxillary retrognathia) compared to the lower jaw.

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. Especially the adjacent teeth are affected. The lateral incisor is often atrophied or even absent. In addition, the teeth close to the teeth often have very little tooth enamel, making them more susceptible to decay.

Dry breathing air

In cleft palate, the inhaled air in the nose is not sufficiently moistened. Dry respiratory 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 the development of cleft lip and palate – both external (exogenous) and internal (genetic) influences. It is believed that most clefts are caused by external factors. This includes:

• high-energy radiation (such as X-rays or gamma rays)
• certain chemicals and medicines like the antiepileptic Hydantoin
• Viral infections of mother and child (such as rubella) in pregnancy
• Alcohol abuse and / or smoking during pregnancy
• strong physical and / or mental stress of the expectant mother

How big the influence of the individual factors is, can not say exactly. It is only considered certain that they generally promote malformations of the unborn child and thus can lead to a cleft lip and palate.

Genetic influences

A certain amount of cleft lip and palate is familial, that is caused by faulty genes. But not a single gene is the trigger, but several genes are involved (polygenia). The more relatives have the malformation and the closer the relationship, the greater the probability for a child to also get a cleft lip and palate.

If, for example, a parent already has a child with a cleft lip and palate, another child is also affected with a probability of four to six percent. If a parent also has a harelip, the risk increases to 17 percent.

Combination with other malformations

A cleft lip and palate often occurs together with other malformations in certain syndromes. A syndrome is a disease that is composed of various typical symptoms. About half of all cleft lip and palate is part of a more complex syndrome. In total, more than 400 syndromes are known, which can be associated with a cleft lip and palate.

Some of these syndromes are inherited, 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 too small lower jaw (micrognathia) and a displaced into the pharynx, often too large tongue (Glossoptosis).

Cleft lip and palate: investigations and diagnosis

A cleft lip and palate is usually noticeable immediately upon the first examination of the newborn. Only a submucosal cleft palate is not always recognized immediately. An ENT or pediatrician may only become aware of them if the child suffers from middle ear infections unusually often.

Because the affected children often have other malformations, it makes sense to examine them extensively in the first few days of life. For example, the heart, the eyes and the hearing are checked more closely.

Prenatal diagnosis

You may recognize a cleft lip and palate even before birth on ultrasound images. Although there are three ultrasound examinations for pregnant women in Germany in the context of antenatal care, but a precise representation of the face of the unborn child is usually not included.

Only at specialized centers this facial examination is included. An experienced doctor can detect a cleft lip and palate with high probability. Isolated cleft palates are harder to identify. Even at specialized houses only every fifth of them is discovered.

An ultrasound examination of the fetal face is usually carried out when there are already known gap malformations in the family. If a cleft lip and palate is diagnosed, one can determine the shape of the cleft with the so-called volume sonography. If the malformation is part of a hereditary syndrome, this may be determined by certain genetic examinations (karyogram, molecular genetic examination).

Cleft lip and palate: treatment

The treatment of a cleft lip and palate is tedious and complicated. It is usually done at specialized cleavage centers. Oral and maxillofacial surgeons, orthodontists, otolaryngologists and speech therapists work together to create a suitable treatment plan for every patient.

As a rule, the treatment of a cleft lip and palate is approached soon after the diagnosis. The essential steps take place during the first years of childhood of the children. Treatment on adult patients is rare in Germany.

The primary goal is to close the gap or the gaps in all sections (primary treatment). Functional restrictions and subsequent growth disturbances are to be avoided. In addition, attention is paid to a cosmetically satisfactory result. In addition to the actual operations for crevice closure usually requires some preparatory measures and any surgical intervention in the further course of therapy (secondary treatment).

There is no standard of treatment for a cleft lip and palate. The different treatment centers are sometimes very different, especially the timing vary. The basic procedure is usually the same.

Orthodontic treatment

First, it is important that a baby with cleft lip and palate can eat undisturbed. Like other children, it should be nursed as possible on the chest. Normally this is not a problem with a pure cleft lip, but children with cleft palates need a special palate plate for this purpose. This is made to fit a plaster cast from plastic and used the infant. So he has an abutment for the tongue.

The palate plate must be regularly adjusted to the growing bone. It not only allows the child to feed, but also controls the growth of the jaw. This can help to narrow the jaws and palate. This in turn facilitates the operation later. Should this not be possible with a palate plate alone, there are other orthodontic methods for this purpose.

ENT-medical treatment

Especially in connection with soft cleft crevices secretions accumulate in the middle ear. This can lead to deafness, which in turn disturbs speech development later on. Therefore, a specialized otorhinolaryngologist examines before the first operation, if such a secretion collection is present and if the hearing is impaired.

If so, in the course of the first operation, he will cut a small slit in the eardrum so that the secretion can drain away (paracentesis). The slot grows again by itself. In order to prevent secretions from accumulating again in the middle ear after some time, the doctor can also insert a so-called tympanic tube into the slot. This allows the secretion to drain off permanently.

Operational crevice closure

The various sections of a full-length cleft lip and palate are usually not closed at once, but at different times. For the closure various surgical techniques are available.

Cleft lip: It usually begins with the closure of the cleft lip, preferably between the 4th and 6th month of life. Some cleavage centers perform the procedure as early as the 3rd month.

Cleft palate: A cleft in the soft palate should also be closed as early as possible, at the latest until the first birthday – but best like the cleft lip a few months after birth, to allow a middle ear ventilation in time.

With the closure of the hard palate, one waits a little longer to avoid possible surgical scars disturbing the jaw growth. On the other hand, it is important that the nasal cavity is separated from the oral cavity so that the child can correctly learn to speak. A gap in the hard palate is therefore closed around the 2nd birthday, at the latest two and a half years after birth.

Cleft: A narrow pine cleft can possibly be closed together with the cleft in the hard palate. If the cleft in the jaw but too wide, takes place with the conventional surgical procedures no ossification in the gap statt.Dann a Spaltosteoplastik necessary. For this purpose, the surgeon removes some bone material from the patient, for example from the hip bone or tibia, and inserts it into the gap. After a while, it fades with the gap edges and thus closes the column.

A jaw splint closure by means of osteoplasty is usually performed between the ages of 7 and 11, preferably before the canine has broken through. Because the canine can then be moved orthodontically into the gap, where he now has a bony abutment.

Subsequent operations and accompanying treatment

Even though the operative closure of a cleft lip and palate is on schedule, there are often functional and aesthetic problems. Sometimes they are also the result of surgical scars.

To correct these defects, further procedures may be needed, such as voice enhancement surgery, rhinoplasty, or closure of residual cavities in the palate. They should be done as soon as possible. For nose corrections, however, one usually waits for the end of the nasal growth, otherwise malformations may occur again.

Accompanying the surgical procedures, the patients are regularly examined orthodontically and treated as needed. Especially at the eruption of the deciduous teeth and later the permanent teeth you have to pay attention to malpositions here.

In addition, a speech therapy care of children is important. This should start in the first year of life and check, among other things, the development of the lip, tongue and palate muscles. In the second and third year of life, the speech therapist monitors the sound and, if necessary, intervenes therapeutically.

Cleft lip and palate: disease course and prognosis

Cleft lip and palate are complicated malformations, which, if left untreated, lead to numerous complications. It takes a specialized treatment team and plenty of time to fix any aesthetic and functional limitations. Although the most important operations for a cleft lip and palate are usually within the first three years of life, subsequent, corrective surgical interventions may sometimes be necessary until young adulthood.

Even in the non-surgical phases, check-ups take place at regular intervals. So the patients have to go through a lot. This can be stressful for the soul. Therefore, the children and their parents can be accompanied by psychotherapeutic treatment. There are also self-help groups and parent initiatives that provide support.

But the long procedure is worth it, because at the end of the therapy is usually a good result. The patients live without complaint and from the former Cleft lip and palate You can hardly see more than a small scar on the upper lip.