Cleft lip in children. Cleft lip: causes, types of pathology, ways to eliminate the disease What kind of disease is a cleft lip

With the advent of a child in the family there is great joy, but sometimes children are born with certain defects.

In some groups of children, some types of deformities of the mouth area and the face as a whole can be detected, some of which are the cleft palate and cleft lip. A feature of these defects can be called the fact that the formation process takes place even in the first few months of pregnancy, when the child is in the mother's womb.

Major defects in children at birth

The formation of such defects is facilitated by incorrectly connected parts of the body, which is caused by a lack of tissue in some areas.

Such children need the help of doctors from the very first day of birth. Fortunately, today's medicine has a considerable number of methods that allow such children to return to normal life.

The disadvantages include the fact that the process takes a lot of time and requires a huge number of operations. But if you follow the recommendations of specialists and follow all the prescribed procedures, in most cases the treatment of the disease will not be so difficult and will give good results!



Modern science has not yet been able to fully understand all the processes occurring in the womb. Even if all tests and examinations show the normal development of the fetus, features that require medical attention may be found after birth. One of them is the cleft of the upper lip, which occurs even during the formation of the nasal processes. This is one of those congenital pathologies that are easily eliminated. If a child develops a cleft lip or its combination with a wolf palate, plastic surgery is required.

What is cleft lip

Cheiloschisis is a pathology of fetal development that occurs around the second or third month of gestation. Often it is caused by a violation of the formation of the bones of the skull, abnormal development of the fetus during the period when the maxillofacial elements are formed. A cleft forms in a child's upper lip and often deepens into the nasal cavity. The defect sometimes appears on one side, it is bilateral or located in the middle of the lip. Often, a deformed lip is combined with a splitting of the hard palate, known as cleft palate disease.

The defect almost does not carry functional disorders, but needs to be surgically eliminated. The statistics of the occurrence of deformation is different for different countries. The minimum level is considered when such a pathology occurs in 1 case per 2500 children, the maximum is 1 per 500. Boys often have birth defects of this kind. In order to eliminate the cleft lip at the age of 3 months to six months, an operation is performed. In the future, visits to the dentist and speech therapist may be required, which are completed by the age of six. Later, surgery is done to remove scars.

Is it hereditary

In about a quarter of cases, "hare lip" occurs due to a genetic disorder that is passed on to children. The presence of a deformed lip in one or both parents significantly increases the risk of the same defect in a child. During pregnancy, compliance with a certain regimen is shown, and its violation can also lead to abnormalities in the development of the fetus. There can be many reasons. It is not yet possible to predict what leads to the appearance of a defect in a child.

What does a cleft lip look like in humans?

The defect is manifested by external ugliness: it is a gap in the lip located on one or two sides. Common is a unilateral cleft, located to the left of the median line of the lips. If there is a bilateral cleft, the presence of other maxillofacial defects is also common. The child has an incomplete cleft. The presence of a deep defect often results in the exposure of the upper jaw, creating a visual resemblance to a hare.

In some cases, the defect affects the alveolar process of the upper jaw. In the presence of a wolf's mouth, which can be considered a kind of "hare's lip", the splitting of the sky is different. It may be a small hole. A wide cleft palate with deformation of both hard and soft tissues is possible. The cleft palate in humans is caused by a gene mutation.

Causes

"Hare lip" and cleft palate can occur as a result of genetic abnormalities such as Van der Wud's syndrome or Stickler's syndrome. There are a number of factors that increase the likelihood of a child having a cleft lip. Risk factors include:

  • late pregnancy. Childbirth after 40 years can lead to the formation of a defect in the fetus.
  • Alcohol and smoking.
  • Viral diseases of the mother in early pregnancy.
  • Genetic disorders.
  • Bad ecology.
  • Chronic or other diseases suffered during pregnancy.
  • Heredity. The presence in the family of people born with an unfused lip can be the cause of the appearance of pathology.

Classification

In most cases, the cleft is located on the upper lip on the left, less often on the right side of the midline. Sometimes the defect occurs on both sides. In mild cases, the cleft affects only the outer soft tissues. In severe forms of the defect, the bones of the palate and the upper jaw can be deformed. In some cases, nose deformity occurs. "Hare lip" can be:

  • one-sided and two-sided;
  • isolated;
  • complete;
  • partial;
  • with a defect on one lip;
  • light form;
  • heavy form.

What is dangerous disease

The presence of this anatomical defect does not have a direct impact on the health of the child, but it causes discomfort. Children with a cleft lip can become the object of ridicule of other children. Deformation makes it difficult to speak, eat, the child catches a cold more easily, there are otitis media. The defect is recommended to be corrected in infancy at the earliest opportunity and as early as possible. In adulthood, it will be much more difficult to restore anatomical usefulness.

Diagnostics

In some cases, the presence of a defect in a child can be seen on ultrasound starting from the 14th week of gestation. It should be borne in mind that the accuracy of such a diagnosis will never be absolute. After the birth of the baby, parents can immediately notice the presence of a defect. To identify other anomalies, the child's eyesight, hearing are checked, and a general assessment of the condition and development of the body is made.

Surgical correction

Currently, surgical intervention completely eliminates nonunion. Some people born with such a defect accidentally find out already in adulthood that they once had such a problem. A cleft lip repair is highly recommended for all children with this diagnosis, unless there are individual contraindications. If the newborn has other pathologies or jaundice, the operation may be refused.

Doctors need to make sure that the child does not have other pathologies, that the gastrointestinal tract, cardiovascular, endocrine and nervous systems are functioning normally, and that there is no weight loss. The sooner the correction is made to the baby, the less the result of the operation will be subsequently noticeable. Given the peculiarities of the physiology of newborns, the operation is postponed until the age of three to six months. It is done under general anesthesia. The result will be the elimination of the cleft, the restoration of tissue integrity, the normal development of the maxillofacial part.

By the age of three, the correction should be completed. At this age, the process of speech formation begins, and it is important that all sounds are pronounced correctly by the child. All muscles involved in speech, including facial muscles, should not encounter any obstacles in their work. In some cases, the help of a speech therapist may be needed. In the presence of a cleft of the alveolar process, the operation is performed during the period of mixed dentition, that is, at about 8-11 years.

Cheiloplasty

Correction of congenital clefts often requires several stages of reconstructive cheiloplasty. During the operation, the tissues are cut and reconnected, tampons are placed in the nasal passages, and then tubes are placed in order to prevent the sutures from opening, which are removed after 10 days. The operation takes several hours. There are several ways to cut:

  • Linear. It leaves behind an almost imperceptible postoperative scar, it is used only for minor defects.
  • Triangular flap method. With this method of incision, it is possible to significantly lengthen the lip and make it symmetrical, but a scar remains.
  • Quad flap method used to repair deep crevices.

Rhinocheiloplasty

The upper lip and nasal septum are corrected. Surgery is often part of a more comprehensive surgical correction program. During the primary correction, the nasal cartilages are exposed, the cleft of the upper lip is eliminated. Subsequent operations correct the cleft palate and other defects. The first days after the operation, the child will not be able to eat normally, so he is fed from a spoon or with a probe. The duration of the operation is several hours.

Rhinocheilognatoplasty

This operation is sometimes performed together with cheiloplasty and is used if the cleft lip is adjacent to the cleft palate. During the treatment of the patient, the mucous membrane is exfoliated from the edges of the gap, due to which the nasal opening near the alveolar process is restored. The operation involves the nasal cavity, often affecting the nasal cartilage. To eliminate the jaw defect, periosteum material is used, which is taken from the lower leg. In the future, the child will need to visit an orthodontist.

Photo of cleft lip

Video

Cleft lip or cheiloschisis is a congenital defect in a child, which is a cleft lip, which is formed by non-fused tissues of the nasal cavity and upper jaw. Cleft lip is a rare disease, 0.04% of babies are born with a defect. The disease does not affect the mental and physical state of the child, but it causes aesthetic and physical discomfort to children: it is difficult for children to talk and eat, they cannot smile, the formation of the dentoalveolar system is disturbed, and special dental care products are needed. A cleft lip is primarily a cosmetic defect that can and should be corrected with plastic surgery.

Main symptoms

The defect is formed at the 8th week of pregnancy, when the maxillofacial organs are laid in the child.

A cleft lip can be seen immediately after the birth of a child during an external examination. The presence of a defect is indicated by a characteristic deformation of the upper or lower lip with a one- or two-sided cleft.

In infants, the processes of sucking and swallowing are significantly difficult; with deep clefts, a nasal probe may be required to feed the child. In the future, the bite is disturbed and the teeth are deformed, which makes it difficult to chew food.

The development of the dentition is disturbed in a child, some teeth are missing or, on the contrary, there are additional ones, teeth grow at the wrong angle, caries develops, the process of sound formation is disturbed, rhinolalia (speech function disorder) manifests itself. Speech in adult children with a congenital defect is fuzzy, nasal, with problematic pronunciation of consonants.

Causes of the disease

The following factors lead to the appearance of a cleft lip in newborns:

  • Viral infectious diseases carried by mothers in the first trimester of pregnancy, for example, toxoplasmosis, herpes, rubella, cytomegalovirus.
  • Complications during pregnancy, chronic diseases of the mother, taking medications with side effects, STDs.
  • The impact of an unfavorable environment on a woman's body during pregnancy (for example, chemical or radiation exposure).
  • Genetic factor: the defect can manifest itself as a result of a mutation taking place in the genes.
  • Heredity: the disease often develops in children in whose families several members were born with this pathology (for example, both parents, one of the parents, the eldest child).
  • Maternal smoking during or before pregnancy, drug use, alcohol use.
  • Late birth (after 40 years).

The main types of cleft lip

There are the following types of disease:

  • Unilateral cleft lip and bilateral. With a one-sided form, the upper lip is cut deep, usually on the left side. With bilateral - the defect looks like a deep furrow, tearing the lip on the right and left from the midline to the very nose (sometimes deeper).
  • Isolated cleft lip and disease associated with other maxillary anatomical defects (eg, cleft palate, cleft palate, nasal deformity).
  • Complete and partial cleft lip. The full form is a deep crack (chip) that rises from the lip to the nose area on one or both sides. Partial appears as a small pit or deep cleft on one part of the upper lip.
  • Defect only on the upper lip (most common), cleft lip on the lower lip, on both lips.
  • The disease is in a mild form (cleavage affects only the soft tissues of the lips), in a severe form (the defect is associated with the bone of the upper jaw or with the palatine bone).

How to prevent cleft lip in a newborn

To prevent the development of the disease in a child, the mother must observe the following rules:

  • Before planning a pregnancy, make sure that the period of validity of her vaccination has not expired, undergo a full comprehensive examination of the body, pass the necessary tests.
  • During and before pregnancy, eat a balanced diet, take vitamin and mineral complexes, eat fresh vegetables and fruits.
  • Avoid hypothermia before and during pregnancy.
  • Minimize the risk of contracting infectious diseases during pregnancy, such as deep frying or boiling meat before eating, as eating undercooked or undercooked meat can lead to toxoplasmosis.
  • Do not take medications without first consulting a doctor, it is especially dangerous to take antibiotics on your own. Taking even prescription drugs during pregnancy increases the risk of developing cleft lip in the newborn.
  • Avoid exposure to adverse environmental factors.
  • Do not smoke, drink alcohol or use drugs during and before pregnancy. Not only active, but also passive smoking (staying in smoky rooms) increases the percentage of births of children with a defect.
  • Avoid medical examinations during pregnancy where there is a risk of irradiation of the fetus.
  • Do not communicate with people who have infectious diseases (for example, chickenpox, rubella).

Diagnosis of the disease

Diagnosis can be established by conducting intrauterine ultrasound (ultrasound) at 16-20 weeks of gestation. This diagnosis is not always reliable.


To correct the defect, mandatory surgical intervention is necessary. The number of operations, terms and volumes of their implementation are determined by the doctor at the consultation.

The removal of the cleft lip is carried out using the following types of reconstructive plastic surgery:

  • Cheiloplasty.
  • Rhinocheiloplasty.
  • Rhinocheilognatoplasty.

Cheiloplasty

The operation includes the restoration of the aesthetic, anatomical and functional usefulness of the split lip. Cheiloplasty is desirable to carry out at the age of 3-6 months of the child.

If there is a defect in a severe form, the operation can be performed in the first days or the first month of the child's life, subject to the following conditions:

  • The child must have sufficient weight gain.
  • There should be no diseases such as anemia, pathology of the intestines, endocrine, cardiovascular or nervous systems.

In the video below you can see the effect of this operation (Cheiloplasty)

Cheiloplasty has a positive effect on the development of the lips and nose of the child in the future. But it should be remembered that newborns have an increased tendency to blood loss and some physiological functions are not yet perfect.

Rhinocheiloplasty

Primary plasty is performed if it is necessary to correct the abnormal location of the muscles of the oral region, cleft lip, and nasal cartilage. Rhinocheiloplasty is a more complex surgical intervention than cheiloplasty; it is indicated for severe forms of cleft lip, facial skeleton defects, and nose deformities.

Rhinocheilognatoplasty

The operation helps to restore the anatomical usefulness of the lip, eliminate cartilaginous deformity of the nose, defects in the alveolar process and underdevelopment of the upper jaw on the side of splitting, and contributes to the formation of a full-fledged muscular frame in the oral region.

Contraindications for all types of plastic surgery to eliminate the cleft lip are birth injuries, physiological jaundice, malformations of vital organs (for example, the heart, kidneys), acquired diseases of an infectious and viral nature.

Preoperative period

During the period of preoperative exposure, the use of special dental splints, cast dental inlays, and medical adhesive plaster is indicated.

Postoperative period

After the operation, a gauze tampon is installed in the nasal passage to protect the suture area from mucus and food, and to prevent narrowing of the nasal opening. After removing the gauze swab, a plastic tube can be installed in the nasal passage for 3 months to prevent its narrowing and deformation of the wings of the nose. The sutures are removed on the 7-10th day.

After plastic surgery, to enhance the effect, the child must wear a special headdress that passes through the upper lip and is attached to the head and cheeks. Thanks to this device, the child's lips are protected from stretching, the seams remain intact. The headgear promotes healing of the lips after surgery.

The movements of the hands of a small child for 3 weeks must be limited with the help of a splint, other special means so that the child does not damage the seams.

After plastic surgery, parents must also observe the following rules:

  • Take all necessary measures to prevent infection from entering the wound.
  • Regularly show the child to the surgeon, consult with a pediatric dentist and orthodontist.

How many surgeries are required to correct a cleft lip

The number of operations depends on the degree of damage to the lips, the type of disease, the presence of defects in the nose.

If only a cleft lip without a cleft palate is found in a child, then it is necessary to carry out:

  • One operation if the gap is small and one-sided.
  • Two operations if the gap is one-sided but wide or if the gap is two-sided (each part of the lip is restored separately during the operation).

A child with a cleft lip and nose defects needs at least 2 surgeries.

If the cleft lip is combined with a cleft palate, then 2 or more operations will be required for correction.

Effect

Plastic surgery in 90% of cases helps children born with cleft lip achieve good functional and aesthetic results, adapt them to a full social life.

In 70-80% of cases, additional interventions may be required to correct residual deformities of the nose and lips at an older age (correction of irregularities on the smile line, removal of a postoperative scar). The timing of additional surgeries is usually delayed until adolescence (before the onset of puberty).

There is a risk of developing cicatricial deformity of the lips on the part of the mucous membrane after the operation, to eliminate which it is necessary to perform plastic surgery of the vestibule of the oral cavity.

Photos before and after the operation

A cleft lip is a congenital defect in the development of the bones of the facial skull, when a gap is noted in the upper lip of a child through which the oral cavity can be clearly seen; The cleft may also lead into the nasal cavity. Such a defect can be observed on one, two sides or above the place where the middle of the lip should be. Often, a cleft lip, or, as they say, a cleft lip, is combined with a non-occlusion of the palate - "cleft palate", but in every fifth case it is combined with more severe malformations.

A cleft lip can occur due to a large number of reasons. In some cases, this is a gene defect that can be inherited, but often these are damaging factors that affected the fetus during the formation of its face. Pathology is not life-threatening, but leads to malnutrition, speech, and hearing. This is the cause of frequent ear infections, abnormal growth and dental caries.

The disease in different regions occurs with different frequencies: at best, it is 1 baby out of 2500, but sometimes 2 out of 1000 children. Boys are more often affected. Treatment - only surgery, which is performed at the age of 3-6 months of the child. After the operation to eliminate the cleft lip, rehabilitation measures are carried out: classes with a speech therapist, dentist, pediatrician. They should completely end by the age of 6, so as not to complicate the child's getting used to society.

Why a cleft lip occurs, what dangers it threatens, and what conditions are needed for the operation, read on.

Where does cleft lip come from

You will get a better understanding of how facial defects are formed if you consider how a multicellular embryo is formed from 1 maternal and 1 paternal cell, and how it acquires a face.

So, after the fusion of the egg and the male cell, one cell is formed, which begins to divide into 2, then, 4 and further cells, and they are all the same. At the beginning of cell division, the cells become smaller, but after a while, the volume of the embryo also begins to grow. Over time, as the cell mass grows, the next significant event occurs: the cells from which the body of a developing person is built become different. They form 3 layers:

  • internal - endoderm, from the cells of which the intestines, liver, lungs, pancreas are subsequently formed;
  • outer - ectoderm, giving rise to skin, nails, hair, nervous system and sensory organs;
  • the middle one is the mesoderm, from which muscles, bones, blood vessels, the heart, genitals and kidneys develop.

At the 2nd week of life, an impression is formed between the developing brain and the area from which the heart will be formed - the primary mouth. It connects to a cavity within the endoderm called the primary gut. This is how the alimentary canal is formed.

At 4 weeks, longitudinal impressions appear on one and the other side of the primary mouth - gill arches. They appear in 4 pieces on each side, and they deepen so that structures that look like bumps are obtained. The anterior ends of the I and II gill arches bifurcate, forming several processes resembling petals. From the first gill arch, which gives rise to the whole face, there are 5 of them:

  • 1 nasolabial process;
  • 2 maxillary;
  • 2 mandibular.

Between the nasofrontal and maxillary processes there is a gap where the eye sockets will subsequently be. In the gap between the maxillary and mandibular processes, a mouth will form, and when they are connected, cheeks will form in the lateral sections. From 1 gill arch, the anterior third of the auricle will also be formed.

The frontal and maxillary “petals” are the first to move towards each other, then the cheeks, upper and lower jaws are formed: skin, mucous, salivary glands, tooth enamel are formed from the ectoderm; from the mesoderm - the bones and muscles of the face, the inner parts of the teeth. If the fusion of the processes does not occur, which can happen in any one or more places, the face is modified. The severity of this change varies from a small cleft in the upper lip to a completely disfigured face.

Starting from the 4th and until the end of the 8th week of intrauterine development, these processes should connect, but their complete fusion occurs up to and including the 11th week. It turns out that in the period from the second to the 11th week, the fetus is extremely sensitive to damaging factors that can affect it through the mother. And the sooner the harmful circumstance acts, the more severe the vice will be. But the damaging factor leads to the formation of a defect only if it acts until the end of the formation of the face. The period from 3 to 6 weeks is considered the most dangerous for a person, and just at this time, the expectant mother usually does not know about her pregnancy, continuing to lead a normal life with smoking, drinking alcohol or habitual drugs.

Causes of cleft lip

The reasons due to which a cleft lip occurs are diverse, as a result of which they are divided into groups:

  1. Genetic background. It is believed that the main gene that causes the development of this disease is called TBX-22. Many other genes can also do this: MMP3, BMP4, VAX1, pTCH1 and others. If one parent has cleft lip, there is only a 7% chance that they will pass the condition on to their child.
  2. Harmful chemical effects on the mother's body in the first trimester (they account for 22.8% of cases). It:
    • smoking;
    • alcohol;
    • addiction;
    • taking medications: anticonvulsants, lowering blood pressure, hormonal drugs, drugs containing sodium, salicylates, insulin;
    • exposure to pesticides, lead, mercury vapor, insecticides, organophosphates, harmful environmental factors, epoxides, arsenic, benzenes;
    • ingestion of additives: tetrazine, sodium cyclomate, aromatic hydrocarbons.
  3. Harmful biological effects on the body of a developing fetus in the first 11 weeks of its formation (this factor accounts for 5% of the causes):
    • viral diseases: SARS (especially if the temperature rises), herpes, rubella, cytomegalovirus infection, mumps, chicken pox;
    • diseases caused by certain protozoan microorganisms, such as toxolasmosis or malaria;
    • infections that a woman "acquired" sexually: chlamydia, gonorrhea, syphilis.
  4. Physical effects on a pregnant woman (2% of causes):
    • injuries (especially a blow to the lower third of the abdomen),
    • freezing,
    • radiation exposure,
    • vibration in the workplace where the pregnant woman works,
    • overheating in production (hot shop, laundry), in a bath, sauna,
    • tumors of the uterus (mainly fibroids), which prevent the fetus from developing normally,
    • multiple pregnancy,
    • adhesions or "strings" that are formed from the fruit shells.
  5. Stress factors, due to which the level of adrenaline in the blood rises, and this has a teratogenic (disfiguring) effect on the fetus: quarrels, fear, overwork.
  6. If this pregnancy occurred after 40 years, especially if before that the woman did not lead the most “righteous” lifestyle.
  7. Inadequate or unbalanced nutrition of a pregnant woman, when the diet lacks proteins, folic acid, zinc, manganese, copper, or, conversely, a lot of vitamin A.
  8. Maternal obesity.
  9. Anemia (low hemoglobin) in the mother for which she received no treatment.
  10. Diseases of the cardiovascular system in the mother.
  11. Severe toxicosis.
  12. Threatened miscarriage or uterine bleeding during pregnancy.
  13. Diabetes.

Oral and maxillofacial surgeons have conducted numerous studies that have proven that cleft lip is only 10-15% hereditary. The remaining 80-85% is due to the fact that the impact on the fetus occurred from several sides at once, while, perhaps, he was genetically predisposed to the development of this particular facial defect.

This defect does not always occur in isolation: in some cases, the cleft lip is only one of the multiple defects that form in a child in the prenatal period. It:

  • Van der Wood syndrome, which is caused by a specific change in the IRF4 gene. This is the most common disease in which cleft lip and palate occur. It is characterized by the appearance of cracks in the lip, palate, together with the development of pits on one or both lips;
  • Lois-Dietz syndrome, in which the child not only has a cleft lip, but also damage to the palate, and a bifurcated tongue, and a large distance between the eyes, and an aortic aneurysm;
  • Stickler's syndrome, in which a cleft of both lips and palate develops, as well as myopia and joint pain;
  • Hardikar syndrome, in which there is a combination of cleft palate, cleft lip with impaired intestinal patency, hydronephrosis of the kidney.

What is dangerous disease

If a child was born only with a cleft lip, and the hard palate remained intact, this leads to the following disorders:

  • under the age of one year, when the child eats only liquid food, it is difficult for him to suck and swallow; food can enter the nasal cavity, and this requires special tricks when feeding, sometimes - even placing a probe - a tube that will lead from the nose to the stomach;
  • if it is not possible to perform the operation when the teeth begin to form, this may lead to the absence of the necessary teeth or the appearance of their additional units;
  • teeth begin to grow at the wrong angles - the bite is broken. From this, both the chewing of food suffers and, accordingly, its digestion worsens, and caries often occurs;
  • the process of sound formation is disrupted: a sound wave, entering the nasal cavity, makes the voice nasal, and speech fuzzy, with problems in pronouncing consonant sounds;
  • hearing problems;
  • often there are otitis media;
  • cleft lip, even mildly pronounced, is a significant cosmetic defect, which will make it difficult for a child to adapt in a children's society

That is why the cleft lip, even if it is not very pronounced, must be operated on. And this needs to be done up to a year in order to have time to go through the necessary rehabilitation measures before the formation of speech and before the start of the child's socialization.

Disease classification

A cleft lip can be:

View Subspecies What does
Unilateral cleft lip Complete The cleft goes from the lip to the nose
incomplete The cleft only affects the lip
Hidden Only the muscles are split, and the mucous membrane and skin above them are not changed
Bilateral cleft lip Complete The defect goes from lip to nose
incomplete The defect is located only within the mucosa of the lip
symmetrical The defect is the same on both sides
Asymmetrical Larger on one side, smaller on the other

The cleft lip can be on one upper lip, one lower lip, or both at once.

The classification is used when choosing the method of operational correction.

Symptoms

Signs of this disease are visible immediately after the birth of the child. It:

  • a defect in the upper (more often) or lower lip;
  • it can look like a small gap in the red part of the lip, or it can be manifested by a significant divergence of the tissues of the lip from the lip to the nostrils, or even in general - with entry into the nasal cavity;
  • it can be on one or two sides (in the second case, the lip consists of three fragments);
  • through this gap in the lip, the mucous membrane of the upper jaw is often visible.

Diagnostics

The diagnosis of "cleft lip" in children is made after birth - on the basis of only one external examination. At the same time, an ENT doctor is examined to find out if there is a defect in the nasal cavity, on the hard or soft palate.

A cleft lip is usually seen on an ultrasound performed at 14-16 weeks or later, but the first routine ultrasound is usually performed earlier - from 12 to 14 weeks. In addition, no, even the best sonologist, who examines the fetus using ultrasound, can guarantee that the child has formed a cleft lip. This diagnosis before childbirth can only be made by a council of doctors, and usually this is when this malformation prompts a woman to decide on an abortion. According to the legislation of the Russian Federation, abortion of a fetus that is older than 12 weeks is possible only if, in addition to the cleft lip, there are other malformations that threaten the life of the fetus.

Anatomy of the affected area

Let us briefly consider which tissues suffer from cleft lip. This will make it clearer the amount of work that the surgeon has to perform.

The lip is a complex musculoskeletal formation. The outer layer consists of three parts:

  • skin that contains mucous and sweat glands;
  • the skin passes into the intermediate part, which already has a slightly different structure, and is rich in blood vessels (therefore it has a red color);
  • the intermediate part passes into the mucous membrane, which is in direct contact with the teeth.

Beneath the mucocutaneous outer layer is a loose layer of connective tissue, and below that is the orbicularis oculi muscle and several other muscles. Under the lip are the gums - the mucous membrane that covers the bone of the upper and lower jaw.

The lower jaw is solid bone. It consists of a body, on which there are cells for teeth, and processes that connect to the skull. The upper jaw is more complicated: it not only has cells for teeth, this bone continues higher and forms the entrance to the nasal cavity, as well as the maxillary sinus.

Treatment

Cleft lip before and after surgery

Only surgery will help eliminate the cleft lip. It is recommended to perform it up to a year, and better - from 3 to 6 months after birth. If this is not possible, it can be performed later - the main thing is that all treatment of a small patient, including further postoperative rehabilitation measures (classes with a speech therapist, wearing orthodontic appliances) should be completed by the age of 6.

The surgeons Musgrave and Willelmessen, who improved surgical interventions for the correction of the cleft lip back in 1969, introduced the "rule of 10", in which the operation is possible:

  1. the child must be at least 10 weeks old;
  2. the weight of the baby must not be less than 10 pounds (4.5 kg);
  3. hemoglobin in his blood should not be less than 10 g / dl (that is, at least 100 g / l).

In our country, cleft lip surgery is usually performed at 6 months or later. Here are added such conditions as: a sufficient increase in the weight of the child, the absence of pathology of the intestines, nervous and cardiovascular systems.

Preoperative period

Before surgery, the child needs to be fed. How to feed a newborn with such a defect depends on the type and degree of the defect.

So, if the cleft is incomplete and one-sided, it will be possible to breastfeed (optimally) and from a bottle, while keeping the child not in a lying position, but half-sitting or upright (“soldier”).

If the defects are deep, but the hard palate is not affected, you will have to abandon breastfeeding in favor of special nipples (they are produced, for example, by NUK and Avent), which are put on a bottle into which formula or breast milk is poured. This nipple should be pushed as far as possible into the child's mouth, to the root of his tongue. If the baby cannot suckle because of the small hole in this nipple, it can be widened with a thick needle that has been decontaminated with fire.

If the cleft lip is combined with a cleft palate, then you can only feed through a probe. It is installed in the nose in a children's hospital or maternity hospital, after which the mother must learn how to use it correctly.

It is recommended, at least in the preoperative period, to feed the child with breast milk as much as possible, if he does not have enzyme deficiency or other contraindications. Only women's milk contains the necessary substances for the development of immunity, digestion and other processes occurring in the body of a child.

Operation

To correct a cleft lip, one of three types of surgery can be performed.

Cheiloplasty

This intervention is performed when the cleft is only in the lip. One of three methods for performing this operation can be used. Its choice depends on the shape of the cleft lip:

  1. Triangular flap method. In this case, a triangle is created from the tissues of the damaged lip, which is installed in such a way as to lengthen the tissues of the lip and make them symmetrical. As a result of this intervention, a transverse scar is formed between the mouth and nose.
  2. Method with the formation of a quadrangular flap. It is used when the cleft is very pronounced.
  3. Linear method. It is suitable for the correction of small lip defects, as it does not allow to compensate for significant tissue deficiencies.

If the lip is split on both sides, then cheiloplasty is performed either as the first stage of the operation, after which the defect of the nose is corrected in the second stage, or rhinocheiloplasty is used immediately.

Here is how the cleft lip looked before and after cheiloplasty:

Rhinocheiloplasty

This intervention involves the correction of a complete cleft lip, when the cartilage of the nose and the muscles of the oral cavity are affected. In this case, the cartilages of the nose are released from the skin and subcutaneous tissue, they are placed in the correct position and fixed. After that, the tissues of the upper lip are sewn together. After a while, if the defect was significant, a second operation may be necessary.

The operation ends with the installation of a tampon in the nasal passage. This cotton-gauze device will prevent food from entering the nose, as well as the narrowing of the nasal passages. For 2-3 days, the tampon is removed, and a polyvinyl chloride (PVC) tube is installed instead, the purpose of which is to prevent narrowing of the nose and deformation of its wings.

Rhinocheilognatoplasty

This intervention is used to recreate the correct position of the lip, nasal cartilage and hard palate. The intervention is complex, traumatic. After it, tampons, various PVC and plastic fixatives can stand in the mouth for some time.

Postoperative rehabilitation

It is carried out in three stages:

  1. In the hospital - immediately after the operation. Anesthesia, prevention of suppuration of injured tissues, feeding and correction of the water-salt balance are carried out here. A special device is put on the child's face, which will prevent the seams from coming apart when the lips move. A splint can be worn on the baby's arms for 3 weeks, which will prevent combing and damage to the seams.
  2. With the help of clinicians. This stage begins immediately after discharge, when the mother should come with the child to the local therapist at the place of residence, and he will write which specialists and how often he will need to go, which physiotherapy procedures to attend and which pills to take.
    At the outpatient stage are carried out:
    • Work with a speech therapist - if the consequences of surgical intervention for speech cannot be eliminated up to 3 years. Then the speech therapist should be involved in the formation of the baby’s speech, and these classes should be completed before the age of 6, when the child will have to join the school team.
    • Orthodontic treatment for bite correction. It consists in wearing various braces, caps or plates. The method depends on the specific situation and the financial capabilities of the family.
    • Treatment by an audiologist if the child has hearing problems.
  3. At home, when classes with a child are carried out with less intensity, an ENT, an orthodontist and a dentist-therapist, a speech therapist are periodically visited.

Until the complete elimination of violations of speech formation, bite, from the side of the respiratory and digestive systems, the child is diagnosed with disability.

If the postoperative scar turned out to be ugly, it can be corrected with a laser beam. Additional corrective interventions may also be performed subsequently to correct smile irregularities.

cheiloschisis or cleft lip- This is a congenital anomaly of the facial region, in which the upper lip is divided into two parts. Cleft lip in this case can be limited only to the upper lip, but can also affect the upper palate, combined with other malformations.

Statistical data

The cleft lip is one of the most common congenital anomalies. With this defect, one child out of 1000 newborns is born, which is approximately 0.04 percent of the total population of the planet. Most often, boys are born with a split lip. In most cases, the cleft is located on the left side of the upper lip. In the United States of America, the incidence of children with cleft lip varies by state. In New York, 0.78 children per 1000 newborns are born with such a defect, in Alabama - 1.94, in New Mexico - 2.5.

There is a certain relationship between race and the frequency of this pathology. Compared to fair-skinned Asians, cleft lip is twice as common. The Negroid race is characterized by the formation of a defect in 50 percent of newborns.

According to the World Health Organization ( WHO) today there is a tendency to increase the number of children born with this anomaly. This fact is associated with environmental degradation and the emergence of a large number of factors affecting the occurrence of congenital anomalies. Thus, in the Republic of Belarus, where every 5th inhabitant suffered from the Chernobyl accident, there is an annual increase in the number of children with cleft lip by 0.25 times per 1000 newborns. Attempts to explain the reasons for the birth of people with a split lip were made in the days of ancient civilizations. Anomalies were assigned religious significance. In ancient Egypt, it was believed that people born with this defect were punished by the gods. Representatives of other cultures associated vice with a sign of the introduction of representatives of evil forces into a person. In Russia, children born with such a lip belonged to special people endowed with supernatural powers. It was believed that they could transform into animals.

The first to attempt the surgical treatment of a cleft lip were the ancient Egyptians. During the excavations, mummies were discovered, on the remains of whose faces there were signs of an overgrown cleft lip. The defect was sewn together by Egyptian healers with the help of thin animal veins.
The Chinese were the first to describe the procedure for correcting this anomaly. The principle of the method was based on cutting out an even gap and its subsequent stitching in parts. In the middle of the 17th century, special plates began to be used for facial reconstruction.
Ayurveda explains the reasons for the formation of a defect in an interesting way ( an ancient science of healthy living that originated in ancient India). According to Ayurveda, cleft lip belongs to the group of diseases Janma-vala-pravritta ( diseases acquired in the mother's womb). Factors in the development of such pathologies is the wrong behavior of a woman during pregnancy. It was believed that a woman could give birth to a child with a split lip if she was sexually active during pregnancy, committed sinful deeds, and often experienced anger and irritability.

Cleft lip is not a sentence, and its consequences are successfully corrected by modern surgery. Many people who were born with such a defect achieved success and prosperity in their lives. One of the famous people who had this pathology was, for example, Glenn Turner, who is today considered the king of network marketing. From 1962 to 1967, Glenn Turner, with a starting capital of $5,000, earned $300 million. This man is dedicated to a series of books under the general title "Glenn Turner - Cleft Lip", written by the Soviet journalist Melor Georgievich Sturua.

Among modern celebrities, Joaquin Phoenix has a scar, indicating a surgery to correct a cleft lip. There is also evidence that such stars as Mikhail Boyarsky, Andrei Makarevich, Andrei Mironov were born with a split lip.

Lip Anatomy

Lips are musculoskeletal formations located on the anterior surfaces of the upper and lower jaws, around the entrance to the oral cavity. The upper and lower lips are distinguished, which together form the oral fissure.

The lips are formed by several layers of different tissues.

The main tissue layers that form the lip are:

  • skin layer;
  • loose connective tissue layer;
  • muscle layer;
  • slime layer.
Almost the entire skin layer of the lips is formed by stratified squamous keratinized epithelium. The term keratinizing means that the process of keratinization is inherent in it. Only at the outer edge of the lip there is a non-keratinizing epithelium, due to which the skin is thinner. Subcutaneous vessels are visible through it, giving the lip a pinkish color.

The loose connective tissue layer is moderately pronounced. It contains a large number of sebaceous glands, vascular plexuses and nerve fibers.

The muscular layer of the lip is represented mainly by the circular muscle of the mouth. Part of its muscle fibers is located circularly, forming a rounded sphincter. When these fibers contract, the lips close and press against the teeth. Another part of the fibers runs radially from the edge of the lips to the bones of the skull. As a result of their contraction, the lips move forward, and the oral fissure opens. The muscular layer of the lips also includes a number of facial muscles.

Mimic muscles located in the thickness of the lips are:

  • muscle that raises the upper lip;
  • muscle that lifts the upper lip and wing of the nose;
  • muscle that raises the corner of the mouth;
  • zygomatic minor and major muscles;
  • buccal muscle;
  • muscle that lowers the upper lip;
  • muscle that lowers the corner of the mouth;
  • subcutaneous muscle of the neck.
As a result of contraction of facial muscles, the lips change their position, expressing various human feelings and emotions.

The mucous layer lining the entire inner surface of the lip passes into the skin layer on the outer surface. The zone of transition from one layer to another is called the border of the lip. It has a bright red color due to highly translucent blood vessels. When the mucous layer passes into the gums along the midline, a transverse mucous fold is formed, called the frenulum. Many excretory ducts of the salivary glands of the upper lip come to the surface of the mucous layer.

The structure and anatomy of the upper jaw

The upper jaw is a massive pair of bones involved in the formation of the eye sockets, nose and oral cavity. The anterior surface of the upper jaw is covered by the upper lip.

According to the anatomical structure in the upper jaw, a body and four bone processes are distinguished. The body of the upper jaw is a hollow bone with a large airy sinus. This sinus is called the maxillary or maxillary sinus. It has a connection with the nasal cavity through a wide opening.

The bony processes of the upper jaw are:

  • the frontal process, which fuses with the frontal bone and is involved in the formation of the nasal cavity;
  • palatine process, which is involved in the formation of the hard palate ( bony plate that separates the oral cavity from the nasal cavity);
  • the alveolar process, which is provided with dental cells for the attachment of eight teeth;
  • zygomatic process, which fuses with the zygomatic bone.

Intrauterine development of the face

Prenatal development of the face is a complex process of formation and fusion of bones and tissues, which starts at the end of the first month of embryonic development.
At the fourth week, five tubercles begin to stand out in the embryo ( processes) limiting the oral cavity.

The embryonic tubercles involved in the intrauterine development of the face are:

  • frontal tubercle;
  • paired maxillary tubercle;
  • paired mandibular tubercle.
Embryonic tubercles gradually grow and grow together.

The maxillary and mandibular tubercles grow laterally ( to the sides) and are connected on the sides. Thus, the lateral part of the upper jaw and lips, as well as the cheeks, are formed. Then there is a gradual convergence of the mandibular processes and their fusion, which gives rise to the development of the lower lip and lower jaw.

The maxillary tubercles, unlike the mandibular ones, do not reach the midline. The resulting gap fills the nasal process of the frontal tubercle, which grows from top to bottom. It is wedged between the maxillary tubercles, forming the outer part of the nose, the middle part of the upper jaw and the middle of the upper lip.
Thus, the processes of the maxillary tubercles and the nasal process of the frontal tubercle are involved in the formation of the upper jaw and upper lip.

As a result of the growth and convergence of the embryonic tubercles, crevices are formed between their processes.

Embryonic clefts are:

  • median cleft, which is formed at the point of convergence of the maxillary or mandibular tubercles;
  • transverse cleft, which is formed by the maxillary and mandibular tubercles;
  • oblique and lateral cleft lip, formed at the site of convergence of the nasal process of the frontal tubercle and the processes of the maxillary tubercles.
By the beginning of the eighth week of intrauterine development, the fusion of facial clefts ends with the formation of the main lines of the face.
When for some reason there is no complete fusion of the processes of the embryonic tubercles, the clefts persist as congenital anomalies. So, with non-union of the lateral cleft, a cleft lip is formed, and with the preservation of the transverse cleft, macrostomia is observed ( pathologically large mouth).

Reasons for the formation of a defect

Cleft lip defect refers to congenital developmental anomalies, the exact causes of which in most cases remain unclear. Experts note that the formation of a cleft lip can be due to both one factor and a combination of several reasons.

The reasons for the formation of a defect are:

  • endogenous factors;
  • adverse environmental conditions;
  • the influence of radiation;
  • fetal intoxication with chemicals;
  • lack of vitamins;
  • wrong way of life of the mother;
  • taking medications;
  • infectious diseases of a pregnant woman;
  • other external factors.

Endogenous factors

Endogenous factors include internal causes of the development of an anomaly.

Endogenous causes of cleft lip formation are:

  • heredity;
  • the age of the parents;
  • biological inferiority of germ cells.

Heredity
This pathology is often formed in children whose parents or other family members had a similar defect. According to statistics, if one of the parents was born with a cleft lip, the probability of having a child with the same pathology reaches 4 percent. If both parents had a cleft lip, the risk of developing a defect is 9 percent.

Hereditary pathologies arise as a result of the influence of internal and external factors, as a result of which various mutations occur at the genetic level. According to a discovery made in 1991, cleft lip develops due to a mutation in the TBX-22 gene.

Factors that can provoke an anomaly of this gene are called mutagens. By nature of origin, mutagens can be physical, chemical or biological. The most significant physical mutagen is ionizing radiation. Chemical mutagens are chemicals that cause changes primarily in the structure of DNA ( a molecule that provides storage and transmission of genetic information). Biological mutagens include various microorganisms that enter the body and cause mutations.

Age of parents
Experts define parental age, which exceeds 40 years, as one of the reasons for the formation of a cleft lip in a child. The age of the mother is of the greatest importance.

Biological inferiority of germ cells
The inferiority of the germ cell is its inability to form a cell with a complete set of chromosomes, which is called a zygote and is formed as a result of the fusion of a male sperm and a female egg. Both male and female defective germ cells can cause the formation of a split lip.

The reasons for the inferiority of germ cells are:

  • "overripe" ( an increase in the period from ovulation to the fusion of the sperm with the egg);
  • addiction to alcohol;
  • unfavorable environmental conditions.

Unfavorable environmental conditions

In some cases, genetically healthy embryos, while in the womb, acquire this pathology under the influence of environmental factors.

Negative environmental factors include:

  • unfavorable ecological situation;
  • electromagnetic radiation;
  • radiation.
Unfavorable environmental situation
The group of increased risk of having a child with a cleft lip includes women living or working in areas of environmental pollution.

Sources of pollution are:

  • thermal power plants;
  • metallurgical enterprises;
  • chemical production;
  • oil companies;
  • agricultural organizations.
In the course of the activities of these institutions, various chemical compounds are released into the atmosphere and soil ( sulfur oxides, ammonia, hydrogen sulfide, etc.). These substances, entering the body of a woman, cause various developmental disorders of the fetus, including the cleft lip.

One source of pollution that has grown in importance in recent years is motor vehicles. Car exhaust gases contain a large amount of toxic compounds that have a negative impact on the development of the fetus.

Electromagnetic radiation
An expectant mother can be exposed to electromagnetic radiation both at work and at home.

Sources of electromagnetic radiation are:

  • personal computer, laptop, tablet;
  • electronic books;
  • Cell phones;
  • devices for copying documents;
  • scanners and printers;
  • devices for destruction of documents;
  • microwaves;
  • refrigerators;
  • TVs.

Radiation

Ionizing radiation is one of the key adverse environmental factors that provoke the development of cleft lip. Entering the female body, radioactive substances can linger in it for a long time. The degree of danger to the embryo is determined by such factors as the time of entry of the radionuclide ( radioactive substance), the duration of exposure and the ability of the substance to penetrate the placental barrier. Radiation sources can be natural or artificial.

Natural radionuclides are divided into terrestrial and space. A pregnant woman can be exposed to strong cosmic radiation during an airplane flight. Terrestrial radionuclides are located in the earth's crust, of which radon is the most significant. It is possible to prevent the penetration of this substance into the body using a special radiometer device.

Artificial sources of radiation are used in energy production, the creation of nuclear weapons, and the manufacture of some consumer goods. Staying close to these radiation factors, the expectant mother puts herself at risk of giving birth to a child with a cleft lip.
A large number of radiation sources are used in modern medicine.

Medical sources of radiation include:

  • x-ray machines;
  • radiotherapy devices;
  • equipment operating on the basis of radioisotopes.

Fetal chemical poisoning

The penetration of certain inorganic chemical compounds into the female body can cause the birth of a child with a cleft lip. Substances that can cause birth defects are called teratogenic poisons. Teratogenic poisons are part of some cosmetics, household chemicals, drugs used in agriculture. One of the most dangerous and widespread elements with a teratogenic effect is lead. This substance can enter the body through the skin, respiratory tract, along with food. Mercury, arsenic, and cadmium can also provoke the formation of a cleft lip.

Other teratogenic poisons are:

  • agricultural poisons ( pesticides, fungicides, herbicides);
  • mineral fertilizers ( nitrates, nitrogen);
  • nutritional supplements ( cyclamic acid, amaranth dye);
  • cosmetic ingredients ( retinoids, accutane, sodium lauryl sulfate);
  • household chemicals ( chlorine, ammonia, phosphates, xylene).

Vitamin deficiency

An insufficient amount of vitamins in the body of a pregnant woman can cause the birth of a child with a split lip. The most dangerous is folic acid deficiency. This substance is necessary for the normal formation and development of the fetus. Folic acid takes an active part in such processes as cell division, tissue growth, doubling of nucleic acids. Also, in the process of bearing a fetus, a woman should receive vitamins such as A, E, C with food or in the form of supplements. The need for such a vitamin as B6 increases by 30 percent. Women who follow a vegetarian diet may give birth to a child with a split lip due to a lack of vitamin B12. Expectant mothers living in the northern regions need to supplement their diet with vitamin D3.

Wrong way of life

According to many experts, the likelihood of having a child with a cleft lip increases if a woman drinks alcohol during pregnancy. The level of negative influence of alcohol is determined by its quantity. When using up to 30 milliliters of ethanol per day ( no more than 1 glass of dry wine) there is no negative effect on the fetus. If a pregnant woman drinks alcohol every day, which contains from 30 to 60 milliliters of ethyl alcohol, the chance of giving birth to a child with this defect is 12 percent.
Women who use tobacco products and drugs during pregnancy are at risk of having a child with a split lip.

Infectious diseases

Infectious processes in the body of a pregnant woman increase the likelihood of the formation of a cleft lip in the fetus. Infections of both viral and bacterial nature have a harmful effect. The effect of the virus can spread directly to the fetus, causing it to become infected. Also, viral infections can have a negative effect indirectly, causing hyperthermia in the mother ( high temperature). Infections caused by bacteria also cause fetal overheating, which can cause cleft lip.

Diseases that can cause this anomaly are:

  • cytomegaly;
  • coxsackie virus;
  • smallpox.

Medications

Some drugs are teratogenic. The level of negative effect on the fetus depends on the degree of penetration of the drug through the placental barrier.

High risk funds are:

  • psychotropic drugs ( lithium);
  • antiepileptic drugs ( valproic acid, phenytoin);
  • cytotoxic drugs ( methotrexate);
  • antibiotics ( dactinomycin, exifin);
  • antidepressants ( sertraline, fluoxetine).
Medications with a significant degree of risk include anticonvulsants, antipsychotics, antidiabetic drugs, and anti-inflammatory drugs.

External factors

Physical factors such as uterine tumors, attempts to terminate the current pregnancy, and previous abortions can cause the development of a cleft lip in a child. Falls of a pregnant woman from a height, unsuccessful landings, blows to the lower abdomen can also affect the formation of a cleft lip in the fetus.
One of the external circumstances that can cause this congenital developmental anomaly is thermal exposure. Overheating of a woman in the sun, high temperature due to illness, visiting a steam room - all this increases the risk of having a child with a cleft lip.

Hypoxia can cause congenital cleft lip ( oxygen starvation) fetus. Due to the insufficient amount of oxygen in the fetus, the metabolism is disturbed, which causes various pathologies in the formation of tissues. Hypoxia can be caused by diseases of the cardiovascular system, blood diseases, severe toxicosis. In some cases, a lack of oxygen provokes a predisposition to miscarriage, pathological processes in the uterus.

What does a cleft lip look like?

A cleft lip defect looks like a unilateral or bilateral cleft lip. Also, the defect can affect the lower lip, which is extremely rare. Most often there is a unilateral cleft, which is localized on the left side of the midline. Bilateral cleft lip is much less common and, as a rule, is combined with other malformations of the maxillofacial apparatus.

Unilateral cleft lip

Most often located on the left, but may be located on the right. The defect looks like a cleft, the length of which can vary. This may be a shallow defect that will not reach the wings of the nose. At the same time, the upper lip looks as if it were slightly dissected. In this case, the upper jaw with teeth and the nasal cavity are not visible. However, as a rule, the cleft extends from the edge of the upper lip to the wings of the nose, exposing the front jaw ( thereby giving the child a resemblance to a hare). Through this defect, both the nasal cavity and the intermaxillary process with teeth are visible.

Unilateral cleft lip can be hidden or open. An open defect is characterized by the absence of all layers of the upper lip. The cleft in this case is through, and through it the nasal cavity and the intermaxillary process are visible. With a hidden cleft of the upper lip, some of the tissues remain intact. In this case, the bone base undergoes splitting ( maxillary process) and the muscles of the lips, and the skin of the lips and their mucous membrane remains intact. Visually, such a defect is not immediately recognized, since the skin and mucous cover the cleft lip.

Bilateral cleft lip

This type of anomaly can be symmetrical or asymmetric. In the first case, clefts are localized on both sides of the midline of the upper lip. They can also be complete and reach the wings of the nose) and incomplete ( look like shallow furrows). Complete bilateral splitting of the upper jaw is characterized by a deep cleavage ( a crack that goes from the wings of the nose to the soft palate). Parts of the upper lip in this case are completely separated. With an asymmetric version of the cleft lip, on the one hand, the cleft may be complete, and on the other, incomplete.

In both cases, with a bilateral cleft lip, the intermaxillary process of the upper jaw protrudes slightly forward. This type of cleft lip is almost always accompanied by a cleft in the upper palate. Thus, the anomaly affects not only the upper lip, but also other structures of the maxillofacial apparatus.

Other manifestations of cleft lip

Cheiloschisis is not only a cosmetic defect, but also deep respiratory and speech disorders.

The main manifestations of cheiloschisis are:

  • sucking and swallowing disorders;
  • violations of the dentition;
  • chewing disorders;
  • speech disorders;
  • other anomalies in the development of the maxillofacial apparatus.
Sucking and swallowing disorders
They are most pronounced with deep, through defects, which are characterized by direct communication between the oral and nasal cavities. Due to the lack of tightness between these two cavities, the necessary pressure is not created in the oral cavity, which would provide the child with a sucking reflex. If the defect also affects the muscles of the soft palate, then the swallowing process is also disturbed. In this case, a newborn with a cleft lip is transferred to artificial feeding through a tube. If this is a unilateral and shallow defect of the upper lip, then the sucking and swallowing reflex is preserved.

Dentition disorders
Due to the split of the upper jaw with a cleft lip, the process of tooth growth is disrupted. Dentition disorders can be characterized by missing teeth, an incorrect growth angle, or the presence of additional teeth. The teeth of children born with a cleft lip are prone to caries and decay quickly. Sometimes, even after defect plasty, such children have malocclusion, which further requires the intervention of an orthodontist.

Chewing disorders
Violations of the chewing processes are noted at a later age. They develop in several cases - if the plastic of the defect was not made, and also if the wrong bite was formed. Most often, a violation of the chewing process occurs due to improper bite and deformation of the teeth. Incorrect chewing also contributes to the weakness of the muscles of the pharynx and palate, which is observed with a bilateral cleft lip in conjunction with a cleft palate.

Speech disorders
Due to the violation of the integrity of the upper jaw in children, the process of formation of sounds is disrupted. This manifests itself in the development of rhinolalia. With this defect in sound pronunciation, speech acquires a pronounced nasal tone, sounds become fuzzy.

Other developmental anomalies of the maxillofacial apparatus
Most often, the cleft lip is combined with such an anomaly of development as the cleft palate. In this case, the gap cuts not only the lip, but also the upper palate. Disorders of sound pronunciation, breathing and nutrition in this case are expressed as much as possible. The defect affects not only the bone structures, but also the muscle aponeurosis ( tendon plates). Weakness and dysfunction of the muscular apparatus of the oral cavity lead to serious problems in the nutrition of children. The greatest danger is a violation of swallowing. Also, in children with multiple anomalies of the maxillofacial apparatus, respiratory disorders are noted. Shallow breathing leads to the development of oxygen deficiency, since less oxygen enters the body. All this leads to physical underdevelopment of children. It should be immediately noted that such an unfavorable outcome is observed in cases where surgical correction of the defect is not timely resorted to.

Similarly, a cleft lip can be combined with anomalies in the development of the nose, face, and malformations of internal organs. Cleft lip is also found in the pattern of Patau syndrome. This is a chromosomal disorder characterized by the presence of an extra thirteenth chromosome. With this syndrome, multiple anomalies in the development of internal organs are noted, for example, defects in the interatrial septa and blood vessels. In children with Patau's syndrome, in addition to malformations of the internal organs, there are also multiple external anomalies. For example, narrowing of the palpebral fissure, deformation of the auricles, as well as non-closure of the upper lip ( cleft lip) and upper sky ( cleft palate).

Surgical repair of the defect

At what age is it better to have surgery?

The most favorable time for surgical treatment of cleft lip is determined by the surgeon. Factors such as the nature of the anomaly are taken into account ( location and severity of the defect), the weight of the child and other features of his development. The optimal time, in the absence of contraindications, is the period from the 2nd to the 12th birthday and the interval between 6 and 8 months. Contraindications for the operation may be the unsatisfactory weight of the patient, the presence of diseases of the cardiovascular system or breathing problems, and other congenital malformations. A number of experts believe that operations performed at the age of 6 to 8 months are more appropriate. Surgical intervention in the first weeks after birth contributes to better development of the upper lip and nose. But patients at this age react heavily to blood loss that occurs during surgery. In addition, in such children, the upper lip is small, which makes surgical intervention difficult. Upon reaching 6-8 months, the child's condition allows all operations to be carried out in full, while the risk of complications is significantly reduced. The rate of development of bone tissue in the middle region of the face is stabilizing, which is favorable conditions for the operation.

If the defect is expressed as a bilateral cleft, the operation is not possible in the first weeks of birth and is postponed until the child reaches six months. If necessary, repeated operations are carried out after a few months.
If the damage is deep, soft tissue correction is performed at an early age. Correction of bone and cartilage structures is prescribed for a period of 4-6 years. The final surgical correction of the jaw and nose is best done after 16 years of age, when the growth of the facial bones stops.

Defect repair technique

In medical practice, there are a large number of methods for correcting a cleft lip. You can eliminate the defect using only one technique or a combination of several techniques. Regardless of the type of plasty used, the goal of surgery is to restore the anatomical integrity of the lip and eliminate associated deformities. Surgical treatment should provide favorable conditions for the growth and development of all structures of the middle part of the face throughout the patient's childhood.

Preparing for the operation
Any type of operation is preceded by a number of preparatory procedures. The surgeon explains to the parents the principle of the chosen technique, the type of anesthesia used, the possible risks and complications. Before the operation, the patient is assigned several examinations and tests in order to identify possible contraindications. Within 2 weeks before plastic surgery, patients should not take drugs that contain acetylsalicylic acid and various anticoagulants. In some cases, surgical treatment involves some additional manipulations. These can be special dental splints or molded dental linings.

The number of operations and types of plastics in the treatment of cleft lip
The optimal method of plastic surgery of the split lip and the number of required operations are determined by the surgeon. The doctor takes note of the nature of the defect and the general condition of the patient.

Factors that a plastic surgeon takes into account are:

  • type of cleft - can be complete or partial;
  • type of damage to the lip - meaning one-sided or two-sided cleft;
  • the presence of concomitant defects on the face - the presence of a cleft palate or nose defects requires complex surgical intervention;
  • the age of the child, his weight, features of physical development;
  • the possibility of deterioration after surgery.
If the child has a small unilateral cleft, the defect is eliminated during one operation. With a wide unilateral gap, in most cases two operations are required, which are carried out with a pause of several months. With a bilateral defect, each part of the lip is restored during a separate operation. If the cleft lip is accompanied by a defect in the nose, the choice of approach depends on the opinion of the surgeon. Some doctors prefer to carry out simultaneous correction of the lip and nose, believing that this will reduce the difficulties in mastering speech skills and adapting the child. Other experts suggest performing separate lip and nose plastic surgeries, prescribing rhinoplasty at the time when the child is 5-6 years old. In their opinion, operations at this age will help to avoid disproportion of the nose. If the cleft lip is associated with a cleft palate, two or more surgeries may be necessary. Additional surgeries are performed in some cases to correct the smile line or remove a postoperative scar on the lip. Such activities are most often postponed until adolescence.

Types of plastic surgery for cleft lip are:

  • cheiloplasty- performed with a cleft lip;
  • rhinocheiloplasty- is prescribed when not only the correction of the lips is necessary, but also the correction of the muscles of the oral cavity and the cartilaginous tissue of the nose;
  • rhinocheilognatoplasty- is used for severe pathologies of the facial skeleton with a violation of the structure of the alveolar process ( bone to which teeth are attached).
Cheiloplasty
During this plastic surgery, deformities in the area of ​​the lips and nose are eliminated and the anatomical and functional usefulness of the lip is restored. Depending on the severity of the defect, the correction can be performed in one operation or several successive stages. In the course of surgical treatment, the doctor performs reposition ( restoring the correct position) tissues and their connection. All the methods that are used by modern surgeons to eliminate the cleft lip can be divided into three categories. The key difference is the shape of the incision on the lip.

The incision methods are

  • Linear way. The positive side of this method is an inconspicuous postoperative scar. The disadvantage of such operations is insufficient elongation of the lip, so they are not performed in the presence of large clefts. Linear cuts include the methods of Evdokimov, Limberg, Millard.
  • Triangular flap method. This group includes techniques developed by Tennyson and Obukhova. Their principle is to correct the defect using triangular flaps. This method allows you to obtain the necessary elongation of tissues and form a symmetrical shape of the lips. The negative side of the method is the formation of a transverse scar on the crease between the mouth and nose.
  • Quad flap method. This category includes the methods proposed by Hagedorn and Le Masurier. They consist in the correction of the defect with the help of a quadrangular flap. These methods are used in the plastic of strongly pronounced crevices.
In the case of bilateral splitting of the lip, cheiloplasty is performed in two stages. Sometimes the cleft is corrected on both sides first, after which the defect in the nose area is corrected ( rhinoplasty). In other situations, one-sided editing of the cleft is carried out together with nose correction ( rhinocheiloplasty). Then, during the second stage, the gap is corrected on the other side.
After cheiloplasty, postoperative scars remain on the patient's face. If the operation was carried out professionally and there were no complications after it, the scars look like thin thread-like strips that are almost invisible.
Residual deformities in the area of ​​the lip or nose remain after the first cheiloplasty in 70-80 percent of those operated on. As you grow older, postoperative defects may become more pronounced. In such cases, reconstructive cheiloplasty is performed to correct cosmetic defects.

Rhinocheiloplasty
This type of plastic involves the simultaneous correction of the upper lip and nasal septum. Such operations can be carried out both independently and as part of a complex surgical treatment. Distinguish between primary and secondary rhinocheiloplasty. The goal of primary rhinocheiloplasty is to eliminate the incorrect position of the nasal cartilage and restore the anatomical integrity of the lip.

Secondary rhinocheiloplasty is performed in cases where, after the first operation, various deformities develop immediately or over time.

Indications for secondary rhinocheiloplasty are:

  • shortening of the columella ( part of the septum in front of the nose);
  • flattening of the tip of the nose;
  • deformation of the wings of the nose.
In secondary rhinocheiloplasty, incisions are made along the edges of the existing postoperative scar. After that, the cartilages of the nose are released and their correct position is restored. Next, the tissues of the upper lip are sutured and sutures are applied.

Rhinocheilognatoplasty
This type of plastic is a complex operation, during which a number of problems are solved.

The goals of rhinocheilognatoplasty are:

  • elimination of deformation of the anterior jaw;
  • improving the shape of the upper lip;
  • nose deformity correction.

This operation can be performed in conjunction with cheiloplasty or after it. Rhinocheilognatoplasty is recommended for patients in cases where a cleft lip is combined with a cleft palate. During the surgical intervention, mucosal flaps are exfoliated on both sides of the gap, which are used to correct the nasal opening in the zone of splitting of the alveolar process. To restore the integrity of the jaw, a transplant of the periosteum removed from the anterior surface of the leg is used. The wound is sutured by moving fragments cut from the upper lip.
After rhinocheilognatoplasty, orthodontic treatment is indicated after 3 months.

Under what kind of anesthesia is the operation performed?

Cleft lip correction surgery can be performed under local or general anesthesia.

Local anesthesia
Local anesthesia is used in cases where patients with incomplete and shallow clefts are operated on. Anesthesia is carried out by the method of infiltration anesthesia ( frosts) by injecting a solution of novocaine or trimecaine.

General anesthesia
With local anesthesia, the child most often behaves restlessly, which makes the operation difficult. Therefore, with bilateral clefts and other complex types of defect, the operation is performed under general anesthesia. Tissue freezing with novocaine can also be used with this type of anesthesia, especially if newborns are operated on. Infiltration increases the volume of tissues, which facilitates their dissection.

The stages of general anesthesia are:

  • premedication;
  • induction ( induction into anesthesia);
  • introduction of the main anesthetic;
  • intubation ( lung ventilation);
  • exit from anesthesia.
Premedication is carried out in order to prepare the patient for surgery, reduce anxiety, increase the effect of the anesthetic and reduce the secretion of the salivary glands. This procedure is carried out using a combination of drugs, one of which is most often atropine.
Introduction to anesthesia is carried out using the inhalation method. Through a special mask, the child breathes a gas that consists of oxygen and an anesthetic. With an older patient, induction can be done intravenously. After the child falls asleep, a catheter is inserted into the vein ( with intravenous induction, it is administered immediately) through which the anesthetic is administered. The choice of anesthetic is carried out by the anesthesiologist in accordance with the age of the child.

Intubation is carried out using a tube that is inserted into the airways and connected to a special device. Intubation ensures that the patient breathes normally during the operation.
During the operation, the anesthesiologist controls the supply of the drug to ensure the state of anesthesia. With the help of medical equipment, the doctor monitors the well-being of the child, checking blood pressure, respiration and cardiac activity.

At the end of the operation, the anesthesiologist stops the drug supply and ensures that the patient regains spontaneous breathing. After that, the tube is removed from the respiratory tract.
The child stays in the intensive care unit for 2-3 hours after the operation, where he is observed by a doctor.

The duration of rehabilitation after surgery

The duration of rehabilitation depends on the characteristics of the child, the nature of the operation performed and how the child's body reacted to anesthesia. In the process of patient recovery after surgery, there are several stages.

The stages of rehabilitation are:

  • stationary;
  • polyclinic;
  • restorative.
inpatient rehabilitation
The purpose of this stage of rehabilitation is to provide conditions for the proper healing of the postoperative wound and the prevention of complications. If surgery was performed under local anesthesia, you can start feeding after a few hours. After general anesthesia, the time of the first feeding is determined by the doctor.
To avoid maceration of the skin ( swelling), the sutures on the lip are not bandaged. Daily it is necessary to treat the seams with an antiseptic. Drug therapy during inpatient rehabilitation is based on a complex of drugs and has several goals.

The objectives of drug therapy are:

  • anesthesia;
  • detoxification;
  • prevention of bacterial infections;
  • correction of violations of water-salt metabolism;
  • stimulation of tissue regeneration;
  • support for immune functions.
In order to protect the sutures from food, and the nasal cavity from narrowing, a gauze tampon is inserted into the patient's nose. The sutures are removed after 7-10 days, after which a special tube is inserted into the opening of the nose, which is left for 3 months. This helps to prevent deformation of the nasal cavity and wings of the nose. To prevent the divergence of postoperative sutures, the patient should avoid trauma to the face.
To enhance the effect of the operation, in some cases, the child is assigned to wear a special headgear. The device is a support bandage that passes through the upper lip, being fixed in the cheek area. Such a dressing helps prevent the lip from stretching and maintain the integrity of the postoperative sutures. The duration of use of the device is determined by the doctor. To prevent the child from damaging the seams with his hands, hand movements are limited with a splint or other device.

Polyclinic rehabilitation
This stage of rehabilitation begins from the moment the child is discharged from the hospital. The date of discharge is determined by the doctor, who assesses the general condition of the patient. Polyclinic rehabilitation includes a systematic visit to a medical institution and the implementation of activities aimed at eliminating residual effects after surgery.

Restorative rehabilitation
The task of this stage is to restore all body functions and return the patient to a normal lifestyle. If the operation was performed late, the child may need the help of such doctors as an ENT ( otolaryngologist), orthodontist, speech therapist, dentist. Observation by these specialists is necessary in order to prevent malocclusion, defects in the formation of the dentition, speech problems.

This period lasts at least a year. Only after 12 months, the doctor can make a conclusion about how successful the operation was and whether all functions were fully restored. In the presence of aesthetic or functional problems, the following stages of surgical treatment are planned.

Complications
One of the complications of the surgical treatment of the cleft lip is the divergence of the edges of the wound. This can happen due to mistakes made during the intervention, the development of the inflammatory process in the wound, the injury received by the patient after the operation. As a postoperative complication, shallow scars in the vestibule of the mouth are considered. Over time, exerting pressure on the alveolar process, scars cause deformation of the upper jaw. In addition, such a defect does not allow subsequent treatment by an orthodontist.

Other complications after surgery are:

  • facial deformity;
  • narrowing of the nasal opening;
  • deformation of the wings of the nose;
  • speech disorder.
Cleft lip entails disability. This does not mean at all that children born with this anomaly are physically handicapped. With timely correction of the defect, complications do not occur. At the same time, if a child with such an anomaly was born in the family, the pediatrician is obliged to send him for examination for disability. The basis is disorders of the digestive and respiratory systems or speech formation. Disability is established until the violations are eliminated at the age of 3 to 7 years.

To receive disability compensation, the child must pass an examination. To do this, the parent must apply to the social security authorities. The child is removed from the disability register only after the end of the rehabilitation period. Rehabilitation methods should be focused not only on the plasticity of the defect, but also on the correction of concomitant disorders. These are, first of all, respiratory and digestive disorders. Also, in order to deprive a child of a disability group, he must have no speech defects. If the cleft lip caused severe, irreparable violations, then the group remains for life.

Plastic results

After plastic surgery of the cleft lip, a barely noticeable scar remains in the region of the nasolabial triangle. This scar can be easily corrected with a laser in the future. The position and length of the postoperative scar depends on the method of the operation used. It is worth noting that well-perfused facial tissues heal very quickly. The earlier the treatment is undertaken, the less visible the scar becomes. This is due to the fact that in childhood, cartilage and bone tissue is not yet formed. As a result of this plastic defect is easier and with fewer complications.



How to feed a newborn with a cleft lip?

How newborns with cleft lip should be fed depends on the type of defect. So, if there is only a small unilateral cleft lip without other associated anomalies, then breastfeeding is possible. However, in this case, it will be somewhat different from conventional breastfeeding. Firstly, the child must not be placed horizontally, but slightly upright or half-sitting. Secondly, feeding should be carried out in small portions.

Deep, penetrating defects of the upper lip require the use of special nipples, which differ in shape from the usual ones. This is due to the fact that such volumetric clefts are accompanied by weakness and dysfunction of the muscles of the maxillofacial apparatus. As a result, the child has difficulty sucking. The most common are teats from NUK and Avent. Such nipples are put on a bottle ( the same or another company), where breast milk is previously expressed. It is recommended to move the nipple as far as possible to the root of the tongue. If the sucking process is difficult, then the hole in the nipple is recommended to be made larger. This can be done by the mother herself with the help of ordinary scissors.

If the cleft of the upper lip also affects the palate, then special nozzles are used. These nozzles look like inserts that are placed in the child's mouth, thus closing the defect. With massive through crevices, when the defect is too voluminous, and the sucking and swallowing reflex is impaired, they switch to feeding through a tube.

It is very important to continue breastfeeding and not switch to artificial formulas, unless, of course, the child has concomitant metabolic pathologies ( e.g. lactase deficiency). It is necessary to do this, because children born with a cleft lip are prone to frequent colds. Mother's milk contains all the necessary substances to strengthen the immune system.

Why are babies born with cleft lip?

There are several opinions about why children are born with a cleft lip. Today, heredity is considered the most studied prerequisite for this pathology.

Heredity
Numerous studies among close relatives have shown that the risk of developing a cleft lip is highest in those children in whose family anomalies of the maxillofacial apparatus have already been encountered. This is due to the phenomenon of a genetic mutation in the TBX-22 gene. As a result of this mutation, from 8 to 12 weeks of intrauterine development, the alveolar processes do not coalesce.
According to the same studies, the likelihood of a child developing a cleft lip reaches 4-5 percent if one of the parents suffered from a similar pathology. The percentage of risk doubles if both parents had a cleft lip.

Environmental factors
At the same time, some children with a cleft lip do not have relatives with a similar anomaly. This suggests the involvement of external factors in the development of the cleft lip. Today, it is a generally accepted fact that the bad habits of the mother play a decisive role in the development of this anomaly. It has been proven that women who smoke have a 6 to 7 times higher risk of having a child with a cleft lip than non-smokers. If a woman abused alcohol during pregnancy, then the risk to the child will be more than 10 percent.

External factors such as infections during pregnancy or the use of medications can increase the likelihood of having a baby with a cleft lip. Herpes, measles, Coxsackie and cytomegalovirus viruses have the greatest teratogenic effect on the fetus. If a pregnant woman had one of these infections during the first trimester of pregnancy, the risk of developing cleft lip in the fetus ( even if the mother does not smoke or abuse alcohol) increases several times.

Another factor that increases the risk of developing cleft lip is medication. Antidepressants have the highest risk ( fluoxetine), anticonvulsants ( phenytoin), cytostatic drugs ( methotrexate). Even if the mother took the listed medications before pregnancy, they can still have a negative effect on the fetus. This is due to the long-term excretion of drugs from the body, as well as their teratogenic effect on the cells of the body.

It should be noted that the maximum risk of developing a cleft lip is observed with the simultaneous impact of several causes.

Is cleft lip hereditary?

According to modern theories about the causes of the development of cleft lip, this defect is inherited. However, the type of inheritance of this disease is currently unknown. It is possible that this is an autosomal dominant type of inheritance, in which the anomaly is passed from generation to generation. It is known that the risk of inheriting a cleft lip increases if both parents suffered from defects in the maxillofacial apparatus.

If a couple has already had a child with a similar pathology, then the risk of its development for a subsequent child is from 8 to 10 percent. If the parents suffered from this anomaly, then the probability increases to 50 percent. However, there is no 100% risk of inheriting cleft lip. Scientists have found that the disease appears due to the complex interaction of genetic predisposition and environmental prerequisites. Therefore, the risk of having a child with this anomaly, despite the hereditary predisposition, can be reduced to zero, if all the factors that provoke the formation of a defect are taken into account. It is necessary to undergo examinations for the presence of chronic infections, take the necessary trace elements ( e.g. folic acid) even during pregnancy planning, as well as to exclude smoking and drinking alcohol during the period of expectation of the child.

What does a cleft lip look like after surgery?

The operation, which is performed with a cleft lip, restores a tissue defect, regardless of the type of plastic surgery used. After its implementation, the anatomical integrity of the lip is restored, and the accompanying deformities are eliminated.

During the operation, the surgeon restores the correct position of the tissues and connects them. After that, an inconspicuous postoperative scar remains in the area of ​​​​the nasolabial triangle. The location of the scar depends on the type of operation performed. So, if plastic surgery was performed in a linear way, then a barely noticeable scar remains in the lip area. If the triangular flap method was applied, then a transverse scar is located on the crease between the mouth and nose. The number of scars corresponds to the number of clefts. If there was a bilateral cleft, then a scar remains on both sides of the midline.

With deep defects, when the dissection of the lip reaches the wings of the nose, a nose plastic surgery is also performed. In this case, flaps of mucous tissue are exfoliated on both sides of the gap, which go to the plastic of the nasal opening in the zone of splitting of the alveolar process. The extent of the scar after that depends on the professionalism of the surgeon and the quality of the rehabilitation period. As a rule, if there are no complications, the scars look like thin, thread-like strips that are almost invisible.

It should be noted that today, thanks to modern technologies, it is possible to eliminate scars ( or make them less visible) of any size.

Is the cleft lip visible on ultrasound?

A cleft lip can be diagnosed by ultrasound as early as the 16th week of pregnancy. Some experts argue that cheiloschisis is visible on ultrasound much earlier, namely from 14 weeks. However, most often this anomaly is detected in the period from 4 to 5 months of intrauterine development.

It is important to note that the first scheduled ultrasound examination during pregnancy is carried out somewhat earlier ( from 12 to 14 weeks). Therefore, it is impossible to see the formed defect during this period. However, even subsequently, ultrasound diagnostics is not 100% correct. This is explained by a large percentage of errors, since the images on the screen are interpreted by different specialists in different ways. Proof of this is the fact that only 5 to 10 percent of anomalies are detected during fetal development. Basically, parents learn about this defect after childbirth.

When is cleft lip surgery necessary?

The time for surgical treatment of a cleft lip is determined by the attending physician. It is believed that the optimal time for the operation is the first year of a child's life, namely the period between 6 and 8 months. Of course, the degree and extent of the defect, the nature of the anomaly are taken into account ( location), the weight of the child and other features of its development, as well as the presence of concomitant complications.

Contraindications for surgery in the first year of life are:

  • prematurity of the child and its low weight;
  • diseases of the cardiovascular system;
  • breathing problems;
  • associated birth defects.
A number of experts believe that an operation performed at the age of 6 to 8 months is more appropriate, as it contributes to a better development of the upper lip and nose. At the same time, babies at this age react heavily to blood loss that occurs during surgery. This is especially difficult for premature babies who already have congenital anemia ( anemia among the people). In addition, in children of the first year of life, the upper lip is small in size, which is an obstacle to surgical intervention. After 4 - 5 months, the child's condition allows for surgery ( or multiple operations) in full, while significantly reducing the risk of complications. The rate of development of bone tissue in the middle region of the face stabilizes, which is a favorable condition for the operation.

It happens that the cleft lip requires a multi-stage surgical intervention. This happens when it is combined with the cleft palate and other facial defects. In this case, the timing of the surgical intervention is extended. Given this, you need to know that the correction of the defect is best completed by the age of three, that is, before the formation of speech.

If the defect affects the bone and cartilage structures of the face, then the operation is prescribed for a period of 4-6 years. The final surgical correction of the jaw and nose is best done after 16 years of age, when the growth of the facial bones stops.