Cleft lip and palate

Synonyms

Medical: Cheilo-Gnatho-Palatoschisis, English: cheilognathouranoschisis

introduction

In Europe, there is statistically 1 child out of 500 newborns with a cleft lip and palate (Cheilo-Gnatho-Palatoschisis) born. This is a relatively high number, which is why the gap formation is one of the most common congenital facial deformities.

They arise during the transition period from the second to the third month of pregnancy. However, not at the same time, but first the cleft lip and jaw develop and later the cleft palate.

While both halves of the face develop separately and usually unite at a specific time, the cleft lip and palate does not.
The formation of a gap can take on different dimensions. E.g. only the lip has a gap that extends to the nostril, or there is an extension through the row of teeth of the upper jaw to the complete gap formation including the hard and soft palate. Sometimes just a split uvula is the only indication of a cleft lip and palate.

Cleft lip and palate are sometimes detected on ultrasound, but no later than at birth. The crevices can be complete or incomplete, unilateral or bilateral. The lips, jaw, and roof of the mouth can be affected together or individually. The left side is affected more often than the right.

In the past, cleft formations were also known as "harelip" or "wolf's throat". Since these are not medical expressions and can be perceived as offensive by those affected, one should stick with cleft lip and palate.

causes

The cause of the cleft lip and palate is a malformation in the 4th-10th Embryonic week. Between the 5th and 7th Cleft lip and jaw appear from week 8, cleft palate from week 8. The tissues do not grow together during this time due to external and internal influences.

One then often speaks of one multifactorial history, since mostly several factors act simultaneously and with one another.

In about 70% of cases, the cleft lip and palate occurs in isolation, i.e. alone, as the only defect. In the remaining 30%, the newborn has another malformation. Often times, a column is associated with different syndromes, such as the Trisomy 21, also called Down syndrome. If the cause of the disease lies in genetics and heredity, one speaks of internal factors. If gaps have already appeared in the family, the risk of further gaps forming with more offspring is slightly higher. Consultation with the responsible doctor often helps here.

The external factors are influences that have an external effect on the pregnant woman. If the mother smokes during pregnancy, it can harm the baby in a number of ways. It does not matter whether you smoke actively or passively. The toxic substances contained in the cigarettes act like poison on the fetus's body. The same applies to the mother's alcohol consumption during this time.

Read more on the topic: Alcohol during pregnancy

Ionizing radiation, i.e. the production of X-ray images, can also be the origin for the formation of a gap. The X-rays can damage the child's DNA, which can cause tissues to develop improperly. To prevent this from happening, it is always very important to inform the doctor treating you that you are pregnant! Taking medication during pregnancy can also have serious consequences. Especially Corticosteroidssuch as cortisol, which has an anti-inflammatory effect, are suspected of causing such a disease.

In the event of stress and psychological stress, the body also releases its own cortisol, which is also not beneficial for the fetus. Other factors include being too old or too young, as well as improper nutrition for the mother, bleeding in the uterus or fever are dangerous for the unborn child.

Symptoms

In the case of cleft lip and palate, one does not speak directly of the symptoms that occur in the patient. Rather, it is different effects or functional disorders that arise from the disease. These disorders mainly affect the nose, ears and the organ of speech.

Difficulty breathing often occurs because the nostrils may be flattened or the nasal septum is curved. It is more difficult to eat because the sucking does not work properly. However, drinking plates can be made for the youngest ones a few days after birth to avoid this problem.

Many children also find it difficult to speak, rubbing sounds, plosives and vowels are pronounced differently. One last important point is the problem of middle ear ventilation. A cleft palate can change it, which can lead to constant otitis media or impaired hearing. However, this can be prevented by inserting a tube. If the upper jaw is affected by the gap, misaligned teeth are possible. Sometimes some teeth are not even created due to the gap. At an advanced age, operations are often necessary to connect the facial parts that have not grown together. The psyche often suffers from this.

Problems with breastfeeding

Since a cleft lip and palate is to be equated with a connection between the mouth and nose, problems with food intake and absorbency arise. It is important to know that feeding newborns with crevices always takes up to 30 minutes longer than children without crevices, regardless of whether they are fed with a bottle or breastfed.

If sucking is not possible with the help of a custom-made drinking plate that separates the two rooms and thus in many cases facilitates nutrition, other aids must be used.

The attempt is made to feed the child with the help of a syringe while it is lying against the chest. The milk can be pumped out beforehand with a pump. Another variant is to put a finger in the baby's mouth and simultaneously pour in milk with a syringe. This variant is known as finger feeding. There are also elongated bottles for feeding when breastfeeding does not work. These so-called Habermann vacuum cleaner have a longer mouthpiece, which should simplify the act of swallowing. “SpecialNeeds suckers”, which have been specially developed for children who are unable to exercise the sucking reflex due to diseases and genetic defects, are also helpful. With a return valve, no air can enter the bottle and a slit valve can then regulate how much milk enters the child's mouth. For example, an isolated cleft lip can simply be covered with a finger so that the baby can suckle the breast. Of course, there are also many operations to be performed in the first few weeks and months so that all malfunctions can be eliminated as quickly as possible.

In most cases, however, these aids are not necessary and the drinking plate is sufficient for practicing the correct suction.

If you have any questions, it is advisable to consult the treating physicians, orthodontists and midwives.

therapy

Help the child as early as possible to improve food intake. A drinking plate can be individually made a few days after the birth. It is inserted into the gap and thus separates the nose from the mouth or throat. This spatial separation enables the child to swallow better and it gets used to the feeling of normal anatomical conditions. The drinking plate has recesses in places to allow the tissue to grow further.

The cleft lip and palate should be closed by surgical interventions by the age of 3 at the latest. As a first measure, the gap on the palate can be closed with a palate plate.

However, several operations are necessary to fully restore normal anatomical conditions. These eliminate not only functional but also aesthetic defects.

A quick normalization of the aesthetics and complete restoration of function speak in favor of an early operation.

The minimization of the surgical risk and the growth inhibition caused by the surgery speak in favor of a late appointment.

Typically, the baby is said to be ready for surgery when they are 10 pounds and 10 weeks old. After approximately 6 months, the lip, jaw and nasal entrance surgery is the first thing to do. Hard and soft palates are closed at less than a year. Further operations are carried out at an advanced age.

Above all, language difficulties can be eliminated through operational measures. Anomalies in the position of the teeth must be eliminated by orthodontic measures. The susceptibility to tooth decay is increased in these children; they therefore require careful oral hygiene.

If speech disorders are present, speech therapy exercises are indicated.

Soother for a cleft lip and palate

In the case of a cleft lip and palate, the first operation takes place quite early. A pacifier must not be used immediately after the operation, as there is a risk that the suturing could burst the seam. Otherwise, pacifiers are allowed, but it is not easy to find the right pacifier. The simple ones often simply fall out of the mouth through the crack. Cherry-shaped pacifiers with a longer stem are recommended, but in general you should simply check which pacifier suits the individual situation of the child and which the child accepts.

Cleft lip and palate on both sides

The bilateral cleft lip and palate is the most severe form of the defect, in which patients have two isolated gaps to the right and left of the center of the lip, which meet again in the soft palate. This variant of the gap formation was formerly known as "wolf's mouth".

In several operations by the maxillofacial surgery combined with an ear, nose and throat specialist, orthodontics and speech therapy, the gaps are closed and an attempt is made to restore the defect. The two primary operations are lip closure and palate closure. In the course of growth, cleft osteoplasty, rhinoplasty and orthodontic treatments may also occur.

Therefore, each case is individually different and planned differently depending on whether a column is complete, incomplete, or isolated. The more pronounced the shape of the crevice, the longer the therapy path. In the case of the bilateral cleft lip and palate, the greatest amount of therapy is necessary.

Diagnosis

Ultrasound as prenatal diagnosis

The first signs of a cleft lip and palate can already be seen on ultrasound. Modern ultrasonic devices in particular have a very high level of accuracy. An experienced practitioner can recognize a crack as early as the 14th week of pregnancy, provided that the child lies conveniently during the ultrasound examination. The expectant parents are also immediately informed of the suspicion so that they can be informed about treatment options as quickly as possible.

The gynecologist and obstetrics do this before the birth. Sometimes the crevice is not recognized until after the birth. This mainly happens with very little pronounced or internal crevices.

A detailed diagnostic examination has to be paid for privately by the parents and costs between € 200 and € 400. A referral from the gynecologist for the reimbursement of costs by the health insurance can only take place in special cases - for example, if there is already a case of cleft lip and palate in the family. In families with multiple occurrences in the family tree, there is an increased risk that the unborn child will also have this defect. With prenatal ultrasound diagnostics, however, it is only possible to see the cleft lip, but for the cleft jaw and palate the inside of the mouth of the fetus would have to be inspected, which is not possible before birth.

If the crevice is discovered early during pregnancy, then other organ systems should also be accurately scanned. The risk of another malformation is around 30%.

Inheritance

The basic risk with which a child develops a cleft lip and palate is around 0.2%, i.e. 1 child in 500 children is born with it. This malformation occurs more frequently in families in which a cleft lip and palate has already occurred. If one parent is among the victims, the risk for the first child is around 3%, for the second it is even 15-17%. If both parents are affected, one can expect in about 35% of the cases that the children will also become ill.

If the child has the disease for the first time in the family, the risk rises to approx. 4-6% that another baby will be born with this disease. If two babies are born like this, the risk increases even further. According to several studies, crevices occur more frequently among the natives of Australia or America, Europe is in the middle. The cleft lip and palate is the least common in Africa. How exactly the inheritance proceeds has not yet been clearly clarified at the present time.

frequency

The frequency of occurrence of one type of cleft lip and / or cleft palate is 1: 500. However, with the exception of van der Woude syndrome, it is not an isolated genetic defect. It is an interplay of multifactorial environmental influences, but further research is being carried out, as it is still partly not understood why this malposition occurs.

The Cleft lip occurs during development in the 5th-8th Embryonic week on that Cleft palate and jaw during the 7th-9th Embryonic week. Therefore, the risk of a cleft lip and palate increases through the external use of medication and drugs in the first trimester (1st to 3rd month of pregnancy).

This primarily includes smoking and dioxins, which are still considered carcinogenic and are hidden in many foods.In addition, benzodiazepines, commonly known as sedatives or sleeping pills, are considered to be a possible factor in cleft lip and palate. These include Valium, Diazepam, or Oxazepamthat people like to fall back on to find peace. Mental illnesses in the expectant mother and chronic starvation such as eating disorders are another factor. According to the latest research, rubella disease in the mother's first trimester is also the cause of a cleft lip and palate.

Summary

The cleft lip and palate is a defect that occurs during the embryonic facial development. The gap formation can reach different dimensions. There are functional and aesthetic complaints. Therapy consists in closing the crevices through several surgical interventions that lead to the restoration of normal function and aesthetics.

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