Hare lip. Congenital malformations of the face

Congenital malformations of the MFR are a stoppage of development (underdevelopment) or a deviation from the normal formation of certain anatomical formations, organs or systems. Depending on this, pathology can be of varying severity - from difficult-to-detect anomalies, sometimes interpreted as variations of the karyotype, to severe malformations that are incompatible with life.
The formation of the facial part of the embryo ends mainly by the 10-12th week. prenatal development, therefore, the formation of pathological changes is possible only in this period. Numerous factors of genetic and teratogenic origin lead to the formation of malformations.

The overall frequency of morphological malformations in children under 1 year of age is approximately 27.2 per 1000 population. About 60% of them are detected in the first 7 days of life already in obstetric institutions. One of the leading places among the malformations is occupied by orofacial clefts. They are included in the "big five" deformities, occupying the 2nd place in terms of frequency. Cleft lips account for 86.9% of all congenital malformations of the face. Almost every 5th typical cleft is a component of a severe syndrome.

Some authors believe that the number of newborns with these anomalies is increasing and in the next decade the frequency of such cases will be 2 times higher than 100 years ago. In other works, the prognosis is not so gloomy, but everywhere the trend towards an increase in their occurrence is emphasized. Every year, for every 100 thousand of the population, the number of newborns with cleft lip and palate increases by 1.38 (Gutsan A.I., 1984). As a result, there is a constant increase in the number couples in which at least one spouse is a carrier of the anomaly.

Among newborns with cleft lip, boys always predominate (0.79 boys and 0.59 girls per 1000 newborns). In men, as a rule, more severe forms of pathology are found. In most cases, cleft lip is not an isolated defect in a child. Detection of additional phenotypic or morphological changes indicates the presence of the syndrome. If in 1970 there were 15 syndromes, the phenotypic picture of which included clefts, then in 1972 72 syndromes were described, and in 1976 - 117 syndromes with an orofacial cleft. More than 150 have been described so far.

ETIOLOGY AND PATHOGENESIS.

With cleft lip, there are sharp changes in the bone skeleton of the face, as well as an incorrect location of the premaxillary bone and the teeth located in it. Sometimes the number of rudiments is reduced or they are absent (anodentia). Deformation of the dental arch and palatine plates can be combined with underdevelopment upper jaw- micrognathia.

The narrowing of the upper jaw is often congenital and as the child grows, its degree increases. congenital deformity of the upper jaw with a cleft palate can be combined with a deformity of the lower jaw.

On examples of cleft lip of various etiologies, the general principles characteristic of any monogenic, multifactorial and chromosomal hereditary diseases can be traced. In the autosomal dominant type, the disease can occur both when a mutant gene is transmitted from a parent with a cleft lip and palate, or when a sporadic mutation occurs in the germ cell of one of the parents. However, in both cases, the risk for the offspring of a child with a cleft will be 50%.

In the past, when cleft lip caused the death of children in the first years of life, almost all newborns in the population with autosomal dominant syndromes appeared as a result of new mutations. At present, due to the significant improvement surgical technique and holding whole system rehabilitation measures the number of operated persons with autosomal dominant syndromes, who marry and pass on the mutant gene to their children, is increasing. Autosomal dominant mutations are characterized by an increase in the average age of parents, especially fathers. The degree of increase in the age of fathers is approximately the same in various autosomal dominant syndromes with cleft lip and palate and is 32.7 + 7.4 years, which is 5 years higher than the average age of fathers in the control group. The consanguinity of the parents, determined by the coefficient of inbreeding or by "marriage distance" (the distance from the husband's birthplace to the wife's birthplace), does not matter in autosomal complementary syndromes.

In autosomal recessive cleft lip syndromes, a child with a defect is born from two healthy parents who are heterozygous carriers of the abnormal gene. The risk for another child in this family is 25%, as for the first child, while the risk for proband children with cleft is minimal. Naturally, the age of the parents and the number of proband pregnancy in such syndromes does not matter. At the same time, the "marriage distance" is significantly reduced. In some cases, the parents of a sick child are blood relatives. The frequency of new recessive mutations is negligible, almost always the parents of a child with this syndrome are heterozygous.

The most rare monogenic forms of cleft lip are sex-linked syndromes. X-linked mutations are more common, in which a woman is an unaffected carrier of the mutant gene. In this case, in the pedigree, the corresponding defects are found in males. With X-linked dominant inheritance, the syndrome is detected in heterozygous women, and the lesion of hemizygous men is so pronounced that, as a rule, it is incompatible with extrauterine existence.

Cleft lip and palate can occur as one of the components of multiple malformations in chromosomal abnormalities. Common features all syndromes of chromosomal etiology are prenatal hypoplasia, symmetry of lesions and oligophrenia. Such children with cleft lip and palate are clinically the most severe. Cleft lip and palate are not specific to any one chromosomal syndrome. They occur with anomalies of 50% of chromosomes (1; 3; 4; 5; 7; 10; 11; 13; 14; 18; 21 and X), both with deletions and translocations. This does not mean that any child with Down syndrome, for example, has cleft lip and palate, but the incidence of cleft lip and palate in Down syndrome is 10 times higher than in the general population.

Multifactorially inherited cleft lip is characterized by features common to all multifactorial diseases. For the occurrence of such forms, it is necessary to have a genetic susceptibility (predisposition) and the impact of any adverse factors environment conducive to the implementation of exposure to malformation. By themselves, adverse environmental conditions, regardless of a certain genetic background, are not capable of causing the appearance of such syndromes. characteristic feature Such inheritance is the difference in the "susceptibility threshold" for men and women (the formation of a defect occurs only when the "gene concentration" exceeds a certain value - the "threshold"). The combined effect of genes that can cause a cleft (like any other defect) in members of the same sex, for example, in men, is insufficient to cause it in females. In this regard, the frequency of affected girls and boys with cleft lip and palate of a multifactorial nature is different, while with monogenic forms (with the exception of X-linked, which, as a rule, are extremely few), this indicator is the same for men and women.

Finally, a group of cleft lip and palate syndromes is described, the occurrence of which is associated with specific environmental factors. These syndromes can be divided into two groups:

1) syndromes resulting from teratogenic effects (for example, thalidomide or fetal alcohol);

2) syndromes that occur as a result of non-specific effects various factors realized through a common pathological mechanism (for example, through " vascular factor leading to hepoxia and necrosis). Currently, 6 specific teratogenic syndromes with cleft lip and palate have been described:

· fetal-alcoholic;

· thalidomide;

· aminopterin;

· hydantoin;

· amneotic ligament syndrome;

· trimethadione.

Nonspecific syndromes are characterized by the influence of the same factors that are "risk factors" for the implementation of the hereditary assumption in multifactorial cleft lip. These include:

· increase in body temperature of a pregnant woman;

· vitamin deficiency;

· micronutrient deficiency (copper);

· reception medicines with mutagenic activity, as well as steroid hormones, androgens, estrogen, insulin, adrenaline;

· infectious diseases of the mother;

· diabetes;

· gynecological diseases.

Extremely importance has a description of the phenotype of the sick child.

Some monogenically inherited cleft lip and palate syndromes.

CLASSIFICATION.

When diagnosing clefts upper lip at the clinic of the Department of Dentistry childhood Moscow Medical Dental Institute use the following clinical and anatomical classification:

1. Congenital hidden cleft of the upper lip (unilateral or bilateral).

2. Congenital incomplete cleft lip: a) without deformation of the skin and cartilage of the nose (unilateral or bilateral); b) with deformation of the skin and cartilage of the nose (unilateral or bilateral).

3. Congenital complete cleft of the upper lip (unilateral or bilateral).

Other classification congenital pathologies CHLO:

1. Unilateral cleft lip.

2.Bilateral cleft lip

ü symmetrical

ü asymmetrical

· combined (cleft palate + lips)

· individual

· isolated

CLINICAL PICTURE.

Depending on the degree anatomical changes There are three forms of clefts of the upper lip: hidden, incomplete and complete. With a hidden cleft of the upper lip, a splitting of the muscle layer is observed with preservation of continuity skin and mucous membranes. With an incomplete cleft of the lip tissue, only in its lower sections do not grow together, and at the base of the nose there is a correctly developed area or a thin skin bridge connecting both sections of the lip to each other. With a complete cleft, all tissues do not grow together along the entire length of the lip from the red border to the bottom of the nasal cavity. Regardless of the severity of the cleft, the upper lip (middle part) is always shortened. The tissues are pulled up to the top of the cleft, the correct anatomical ratio of the lip sections is broken, the red border is stretched along the edges of the cleft.
With complete clefts of the upper lip in all cases, there is irregular shape wing of the nose, located on the side of the cleft. The wing is flattened, stretched, the tip of the nose
asymmetrical; the cartilaginous part of the nasal septum is curved. A similar deformation of the nose can also occur in some forms of incomplete cleft lip, which is explained by the anatomical and functional inferiority of the tissue layer of the upper lip.
With clefts of the upper lip from the first days of life, the child's sucking function is impaired due to leakage of the oral cavity. With hidden and incomplete clefts of the upper lip, the child can take the mother's breast, pressing the breast tissues against the normally developed alveolar process of the upper jaw and palate, compensating for the inferiority of the muscles lips active inclusion of the tongue in the act of sucking.In other forms of clefts, the child's nutrition can only be artificial.The most severe disorders of the sucking function are observed in children with simultaneous cleft lip and palate.

DIAGNOSTICS.

Diagnosis is carried out using fetoscopy and fetoamniography. Fetoscopy is performed under ultrasound control at the 16th-22nd week of pregnancy using a selfoscope. This technique allows you to see the face of the fetus and, if there is a cleft, invite the family to interrupt abnormal pregnancy. In addition to fetoscopy, fetoamniography is used. The study is performed at 20-36 weeks of gestation. Under ultrasound control, a transabdominal amniocentesis is performed and a solution of a radiopaque substance (myodil or verografin) is injected into the vessels of the placenta. In progress x-ray examination in the presence of a cleft, there is no closure of the end sections of the contrasted vessels of the fetal face. Both methods are invasive and are used only if there is high risk the birth of a child with a cleft lip and palate in combination with such anomalies as oligophrenia, etc.

SURGICAL TREATMENT OF CLISCHES OF THE UPPER LIP.

Treatment of children with cleft lip should be comprehensive and include both surgery, and orthodontic, speech therapy by a speech therapist, etc. There are certain age indications to cheiloplasty:

· Early plastic surgery of the upper lip is performed in maternity hospitals or specialized surgical departments for newborns on the 2nd-4th day or after the 11th-14th day of the child's life. Contraindications to early lip plasty in a child are concomitant congenital malformations, trauma during childbirth, asphyxia, postpartum inflammatory process in the mother. results early operations worse than after a lip plasty performed at a later age. Currently, the age of 4-6 months is considered optimal for lip plastic surgery.

· Newborns are operated on only for special indications.

Upper lip plasty for unilateral clefts.

To restore correct anatomically shaped and full-fledged function of the lips, it is necessary: ​​1) to eliminate the cleft; 2) lengthen the upper lip; 3) correct the shape of the nose.
The methods of lip plastic surgery currently used by dental surgeons can be conditionally divided into three groups depending on the shape of the incisions on the skin of the lip. The first group includes the so-called linear methods: Evdokimov, Limberg, Millard. These methods differ in the way the nasal vestibule is formed with complete cleft lip. positive side linear method is the aesthetics of the scar line, coinciding with the border of the filtrum. However, these methods do not allow to obtain a sufficient elongation of the lips, which is necessary for wide complete clefts.
After scarring, one half of the "Cupid's bow" is pulled up, breaking the symmetry of the red border line. In addition, a few months after the plasty, there is an ingrowth along the scar of the mucous membrane of the red border in the form of a triangle.
The second group includes those proposed by Tennyson (1952) and L.V.
Obukhova (1955) methods, which are based on movement on the skin in lower third lips of triangular skin flaps with different angles.
They make it possible to obtain the necessary elongation of the tissues of the lip, which depends on the size of the triangular flap, borrowed from a small part of the lip; allow you to match the tissues of the lips and get a symmetrical shape of the "bow
Cupid." The anatomy of the methods allows you to clearly plan the operation.
Their disadvantage can be considered the need to cross the filtrum line in the transverse direction. Such direction postoperative scar reduces the aesthetic result of the operation. It is recommended to use these methods for incomplete clefts of the upper lip in the absence of nose deformity.
With complete cleft lip and incomplete, accompanied by deformation of the skin and cartilage of the nose, a good anatomical and functional effect is achieved by combining one of the described methods of the second group with the method
Limberg. This combination of two methods with some additional techniques is used in the clinic of the Department of Pediatric Dentistry
Moscow Medical Dental Institute (Fig. 2.10), which allows you to get a good cosmetic and functional result in a child of any age (Fig. 2.11).
The third group includes the methods of Hagedorn (1884) and Le Mesurier (1962), in which lip lengthening is achieved by moving a quadrangular flap cut out on a small fragment of the lip. However, the quadrangular flap is inactive and inconvenient for repair of incomplete unilateral clefts, when a large lengthening of the lip is not required.

Plastic surgery of the upper lip with bilateral clefts that are not combined with a cleft of the alveolar process and palate.

This operation is performed using most of the methods described above, used for each party separately. Simultaneous plasty of bilateral cleft lip in children with cleft alveolar process and palate does not provide a high functional and aesthetic result. This is hampered by the complex anatomical relationships of the jaw bones and the deficiency of soft tissues. The upper lip turns out to be of an irregular anatomical shape, inactive, soldered with scars to the surface of the premaxillary bone.
Subsequently, due to the absence of the vestibule of the mouth, orthodontic treatment of such children is difficult.
At the Department of Pediatric Dentistry, MMSI, a two-stage method of upper lip plasty has been developed, which is based on elements of several methods. Incisions on the skin of the lips are made according to the Limberg-Tennyson method, the vestibule of the mouth is formed according to the method proposed by a group of American surgeons. With complete clefts on the lateral fragment of the lips, triangular flaps are cut out according to the described method of Limberg and Obukhova. At the first stage of the operation, the cleft is closed on one side only. The other side of the cleft is closed after 2-2 "/a month. Applying this technique plastics of the upper lip, it is possible to achieve high aesthetic and functional results. A well-formed oral vestibule allows for early orthodontic treatment.

The timing and scope of surgical intervention for clefts is determined by the dental surgeon in accordance with the recommendations of all other specialists. Cheiloplasty is performed in the maternity hospital in the first 2-3 days of life or 15-16 days after birth, and in a hospital - at the age of 3-4 months. With a bilateral cleft lip, surgery is performed in two stages with a break of 3-4 months. From the age of 3, the child actively learns from the orthodontist and speech therapist.

COMPLICATIONS AFTER CHEILOPLASTY.

Complications after plastic surgery of the upper lip. After surgery, there may be a divergence of the edges of the wound. The reason for this may be the tension of the edges of the wound due to poor preparation of tissues, insufficiently thorough layer-by-layer suturing of tissues, the development of postoperative, inflammatory process in a wound, trauma. When the wound edges diverge in newborns, it is not recommended to apply secondary sutures, as this worsens the result of the subsequent corrective operation.

The final effect of the operation is determined by the long-term results.
A shallow cicatricial vestibule of the mouth should be considered as postoperative complication. Lip scars put excessive pressure on alveolar ridge, causing flattening over the years anterior section alveolar arch of the upper jaw. Severe deformities of the upper jaw are caused by cicatricial changes in the tissues of the lips in children with complete clefts of the upper lip, alveolar process and palate. A poorly formed, shallow vestibule of the mouth does not allow for orthodontic treatment and requires additional surgical interventions.

POSTOPERATIVE CARE OF THE CHILD.

The line of seams on the lip is left without bandages to avoid maceration of the skin. Feeding the child begins 2-3 hours after anesthesia or 1-2 hours if the operation was performed under local anesthesia. Before removing the stitches, it is better to feed with a spoon, after removing the stitches, the baby can be applied to the mother's breast or fed with a nipple.
The nipple should be large, made of soft rubber, with a small hole. Babies with cleft palate should be kept in vertical position to avoid aspiration of liquid food.
For a warning inflammatory phenomena antibiotics are prescribed intramuscularly. Every day, the toilet of the wound should be carried out in the form of lubricating the suture line with alcohol. The sutures are removed on the 6-8th day after the operation. The earlier the stitches are removed, the more cosmetic the scar is.

Thus, congenital clefts- this is the result of non-union of the germinal tubercles that form the face of the embryo on early stages embryonic development. exact reason occurrence this disease is currently unknown. The impact of unfavorable factors on the most early stages pregnancy (in the first trimester) leads to the formation of clefts, i. is a multifactorial disease. They can occur as an isolated malformation and be one of the symptoms of congenital syndromes.

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Modern science has not yet been able to fully understand all the processes occurring in the womb. Even if all tests and examinations show normal development fetus, already after birth, features that require medical attention. 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 splitting hard palate known as cleft palate disease.

Almost no defect functional disorders but needs to be surgically removed. 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, the “hare lip” occurs due to the fault 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 is shown certain regime, and its violation can also lead to fetal abnormalities. 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 early dates 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 from the left, less often - from right side from 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.

Physicians should make sure that the child has no other pathologies, that the gastrointestinal tract, cardiovascular, endocrine and nervous systems are functioning normally, and that there is no weight loss. How rather baby correction is made, 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 made 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 is corrected and nasal septum. 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.

A congenital cleft lip is also called a cleft lip. In connection with varying degrees non-fusion of the protrusions of the head end of the embryo of the form of cleft lip involved in the formation of the face and lips are very diverse. regular shape cleft lip - a vertical lateral cleft of the upper lip, which is formed due to non-fusion or incomplete fusion of the fronto-nasal protrusion with the maxillary one. Other forms of cleft lips are very rare.

congenital cleft lip it can be unilateral or bilateral, extends to part of the lip or to its entire height, is limited to the lip or goes far beyond its limits. When the gap spreads to the nasal opening, the latter expands and deforms; when spreading deep into the upper jaw, the alveolar process is split, often the hard, and sometimes the soft palate.

If the bilateral cleft lip continues to the palate, then the intermaxillary bone, isolated from the sides, sometimes strongly moves forward, dragging the nasal septum and the middle part of the lip along with it.

Cleft lip, unilateral or bilateral, uncomplicated by cleft palate, disfigures the face but does little to interfere with suckling and therefore does not affect general condition child. This cannot be said about forms complicated by cleft palate.

The operation of congenital cleft lip is best performed at 2-6 months of age. operational methods Much has been proposed to restore the lips.

A. M. Orlovsky describes his method in the following way: “From the lateral edges of the labial fissure, flaps of the mucous membrane are cut out, penetrating through the entire thickness of the labial border. The incisions are made at the border between the skin and the mucous membrane and reach until it passes into the mucous membrane of both halves of the lip, which has horizontal direction. The flaps are turned down, then the skin parts of the lip are sutured, starting from the top.

After the last suture is applied, one of the incisions is continued along the visible part of the mucous membrane of one of the halves of the lip, and a flap of the opposite side is sewn into it. The remaining flap is inserted into the incision, which is already extended from the side of the back surface of the other half of the lip. Thus, both halves of the lips, as it were, change in flaps.

With this method, doctors are completely insured against the divergence of the edges of the wound, without losing a single piece of tissue, with the exception of an arcuate flap in the upper corner of the gap, and, finally, very simply, without any tricky cuts, we reach the goal. In addition, the proposed method allows us to equalize both halves of the lip in the event that one of them is thicker. To do this, you only need to cut out a larger flap on the thicker half, and a smaller one on the thinner one.

The cosmetic results of a correctly performed operation are excellent.

According to Mirov's method, the median edge of the gap of the lip is cut off entirely, while the lateral edge is only half. The uncut lower half of the lateral edge serves to form a small flap with a lower base. Further, the incision is continued for a short distance along the horizontal edge of the lip, going along the border of the skin and mucous membrane. The flap is then folded down and sutured to the bloodied median edge of the lip. With bilateral splitting of the lips, the same method is used on both sides.

To correct the often concomitant deformity of the nasal wing, the latter is mobilized by separating it from the bone. The lip after that also becomes more mobile and moves more easily towards the middle.

Very rarely there is a bifurcation of the lower lip along the midline, transverse and oblique cleft lips, as well as an oblique cleft face, originating from the corner of the cleft lip and heading obliquely to the eye. The last deformity is called a coloboma of the face.

A congenital cleft palate and upper jaw, also known as the cleft palate, is formed due to the failure of the clefts existing in the early period of the embryo's life in the region of the future face, which, when normal course development at the end of the second month of uterine life are closed without a trace. In half of the cases of congenital cleft palate is accompanied by cleft lip.

Cleft palate causes sucking disorders, since the presence of a gap that communicates the oral cavity with the nasal cavity excludes the possibility of creating negative pressure in the oral cavity necessary for sucking liquid food. Milk entering the mouth flows into the nose and flows out, as a result of which the baby's nutrition deteriorates. Phonation is disturbed, speech becomes nasal, slurred. Nasal breathing disorder causes frequent illnesses respiratory tract.

The most favorable period for the operation (see the course of dentistry) is the age of 4 to 5 years. The painful situation of patients with cleft palate before surgery is greatly facilitated by the use of a prosthesis - an obturator.

Cleft lip is the most common congenital malformation of the face of a child. It occurs in one baby out of 1000 newborns. Often referred to as a "cleft lip", a facial defect is formed in the first months of fetal life and is determined by ultrasound as early as the 16th week of pregnancy. It manifests itself as a dissection of the upper lip in depth from a small gap to a deep groove on one or both sides. With timely treatment to the doctor, the anomaly can be successfully corrected. Get qualified medical care maxillofacial surgeon offered by our Clinic.

Reasons for the formation of pathology

One can lead to the formation of a cleft harmful factor affecting the body of the expectant mother, or several reasons at once. The most common ones are:

• internal factors in the development of the pathology of the "cleft lip": hereditary, exceeding 40 years of age of parents, violations in the formation of germ cells;
• external influences: unfavorable ecology, radiation, chemical intoxication with alcohol, smoking, drugs, medicines;
• abnormal course of pregnancy, lack of vitamins;
• infectious diseases: rubella, herpes, etc.

Various processes during a woman's pregnancy are capable of leading to the birth of a child with a "cleft lip", which increase the likelihood of the formation of fetal malformations. This leads to overheating of the fetus heat body due to illness, sun exposure or bathing. Increasing the degree of permeability of the placenta, through which they enter, having a negative effect on the fetus, medical preparations. The cause of a congenital cleft lip is often uterine tumors, previous abortions, a predisposition to miscarriage, severe toxicosis.

Manifestations of a cleft lip defect

A defect in the form of a cleft is usually localized on the upper lip on one, less often on both sides, sometimes accompanied by a cleft palate and splitting of the alveolar process of the jaw. Apart from external manifestations the defect is characterized by symptoms of a disturbed dentition, the processes of sucking, swallowing and sound pronunciation. Bilateral anomaly is less common, often in combination with other pathologies. maxillofacial apparatus. Often, a cleft lip is accompanied by a "cleft palate" defect, in which the gap extends to the upper palate.

According to the degree of severity of the defect and the place of localization " cleft lip» newborns have:

• unilateral:
  complete(with splitting to the nostril);
  incomplete(with partial preservation of tissues);
  hidden(with splitting of muscles while maintaining the mucous membrane and skin);
• bilateral:
  symmetrical(equally full or partial on both sides);
  asymmetric(with complete splitting on one side and incomplete or hidden on the other).

The importance of timely treatment

A baby diagnosed with cleft lip should be shown to a specialist as soon as possible in order to find out the degree of complexity of the case, outline a treatment plan and increase the likelihood of successful resolution of the problem. Competent diagnosis of the disease reveals not only a noticeable external cosmetic defect but also a number of other violations. It is necessary to prevent their development and negative effect on the health of the child until the defect is corrected by means of surgery, which is possible not earlier than 4-6 months of age.

Among dangerous consequences not delivered on time medical care children with congenital complete or incomplete cleft lip:

• eating disorders, when the child is able to swallow only liquid food or receive it through a tube directly into the stomach;
• improper formation of teeth, their lack, growth at an angle;
• problems with voice and speech (nasal, fuzzy pronunciation of consonants);
• hearing impairment;
• tendency to otitis, colds.
• psychological difficulties of adaptation to children's team due to differences in appearance.

Methods for correcting the "cleft lip"

In the absence of contraindications, this defect, called medical term"cheiloschisis", corrected plastic surgery. Cheiloplasty involves the restoration of anatomically correct form mouth, orbicularis continuity, and nose correction. The degree of complexity of the case determines the volume of preventive, surgical measures, the duration of treatment and rehabilitation.

Patients with double cleft lip are given a pressure elastic bandage immediately after birth to prevent excessive protrusion of the maxilla. IN difficult cases treatment is carried out in several stages:

• primary operation - in 4-6 months;
• repeated (in case of protrusion of the intermaxillary bone by 10 mm or more) - after 2.5-3 months;
• elongation of the skin of the septum, convergence of the wings of the nose at the base - at 4-6 years;
• final correction of the nose - at the age of 16-18 (after the growth of the bones of the face is completed).

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Premaxillary agenesis is a severe defect, which is based on gross violations of the development of the brain of the arynencephalic group (arynencephalic anomaly). Outwardly, it manifests itself as a cleft lip and palate, a flattened nose, hypotelorism and a Mongoloid incision of the palpebral fissures. Disturbances in the structure of the face are associated with hypoplasia and aplasia of the ethmoid bone, bone and cartilage parts of the nose, as well as the palatine process of the jaw.
Anomaly of the median cleft of the face (str.: frontonasal dysplasia, nasal cleft, double nose, dirinia, split nose, "dog's nose") - a complete or skin-covered longitudinal defect of the back of the nose, sometimes passing to the alveolar process and forehead (Fig. 25). The defect is accompanied by hypertelorism, wide root nose and in some cases anterior cerebral hernia. There are 3 degrees of median cleft:

where Fig. 25. Anomalies in the development of the face (Kupriyanov V.V., Stovichek G.V., 1988): a bifurcated nose; b - underdeveloped lower jaw, dystopia of the auricle; c - non-union of the rudiments of the lower jaw; g - button-shaped nose without nostrils; e - tubular nose under a single underdeveloped eye; e - cyclopia, tubular nose
I - hidden cleft (tip of the nose is bifurcated), II - open cleft of the tip and back of the nose, III - total cleft of the soft tissues and bone and cartilage parts of the nose with deformity of the orbits. Quite often at such forms there are no wings of a nose. Sometimes there is a complete doubling of the nose. In some cases, there are brachycephaly, microphthalmia, epicanthus, eyelid colobomas, congenital cataract, psauricular skin outgrowths, low-lying auricles, sometimes conductive deafness, clinodactyly, campto-

dactylia, cryptorchidism, lipomas and dermoids. There are combinations of frontonasal dysplasia with hydrocephalus, arinencephaly and microgyria, agenesis of the corpus callosum, craniosynostosis. In 20% of cases there is mental retardation medium severity. Population frequency severe forms- from 1:80000 to 1:100000.
Anomalies in the shape of the nose (Fig. 26):
a) a wide back of the nose with a sunken bridge of the nose;
b) protruding bridge;
c) upturned nose with turned-out nostrils;
d) fleshy tip of the nose;
e) hooked nose;
e) button-shaped nose;
g) proboscis nose.

a B C

Aprosomia is the absence of a face as a result of a halt in the development of facial anlages. Only individual nodes are noted on the surface of the face.
Arinia - complete absence external nose.
Acephaly is the congenital complete absence of the head. May be associated with absence upper limbs(acephalobrachia), stomach (acephalogastria), heart (acephalocardia), lower extremities (acephalopodia), spinal column(acephalorachia), chest(acephalothoration).
deviated septum of the nose - frequent vice, develops with a lag in the growth of the arch and bottom of the oral cavity.

A facial cyst is a tumor-like formation of congenital origin, found in places of bone sutures on the face. Its origin is associated with the growth in the depths of the tissues of the ectoderm, laced into embryonic period. There are dermoid and epidermal cysts. The most typical localization is the bridge of the nose, the border of the bone and cartilaginous parts of the nose, the outer edge of the orbit.
Dermoid nasal cyst - located on the back of the nose, formed as a result of non-closure of embryonic fissures. It is predominantly localized under the skin at the junction of the nasal bones with cartilage.
Coloboma of the ala of the nose is a transverse, shallow unilateral or bilateral fissure of the free edge of the ala of the nose. It often accompanies complex facial malformations.
"Bird's face" - a face with a sloping and receding chin with underdevelopment of the lower jaw and ankylosis of the temporomandibular joint. It is observed in the Franceschetti-Tsvalen syndrome (Fig. 27).
"Fish face" - a face with a sharply narrowed mouth opening. It is observed in Franceschetti-Tsvalen syndrome.
("bird's face") (Kupriyanov V.V., Meloskhiz - cleft cheek with an increase in the size of the mouth.
StovichekG. V., 1988)
Microforms of cleft lip and palate - in addition to the pronounced forms of clefts mentioned above, there are also small signs, called microforms. These include hidden or obvious cleft of the tongue only, diastema, hidden and initial cleft of the red border of the lips, deformity of the wing of the nose without the presence of a cleft lip.
Additional nose (syn.: proboscis, proboscis) - in mild cases, it is an outgrowth in the form of a tube located at the root of the nose. In severe cases, instead of a nose, there is a tubular leathery formation with one blindly ending hole.
The absence of the nasal septum - it can be complete or partial. Occurs rarely.
The absence of half of the nose is congenital - aplasia of the wing and lateral surface of the nose within the cartilaginous part, usually accompanied by atresia of the bony opening leading into the nasal cavity from the same side. The preserved half of the nose is hypoplastic.
Congenital perforation of the nasal septum - a hole in the bony or cartilaginous part of the nasal septum.
The section of the eyes is anti-Mongoloid - the outer corners of the palpebral fissures are lowered. It occurs as part of many syndromes of malformations.
Cleft lip soft tissues lip extending to the side of the filtrum. It can be unilateral or bilateral, complete or partial, subcutaneous or submucosal.
Cleft of the upper lip and palate through (syn.: cheilognatopathoschis) - a gap of the lip, alveolar process and palate. It can be one- or two-sided. With through clefts, there is a wide communication between the cavities of the nose and mouth (Fig. 28). It can be combined with polydactyly and anomalies of the genitourinary apparatus (Grauhan's syndrome).

Rice. 28. Clefts of the upper lip (Kupriyanov V.V., Stovichek G.V., 1988): a - unilateral partial cleft of the upper lip; b - unilateral complete cleft of the upper lip; c - bilateral complete cleft lip
Cleft upper lip median (syn.: cleft upper lip prepalatine) - a gap in the soft tissues of the upper lip, located along the midline. Accompanied by frenulum and diastema; can be combined with a cleft of the alveolar process and a double frenulum. The anomaly is very rare (Fig. 29, 31).

Rice. 29. Defects in the development of the face along the lines of fusion of its parts (PattenB. M., 1959):
a - median cleft of the upper lip; b - median cleft of the lower jaw; c - bilateral cleft lip and microcephaly; d - bilateral cleft lip, mid-nasal components located at the tip of the nose; e - open orbito-nasal fissure and complete absence of the medial part of the upper lip and jaw; e - open orbito-nasal fissure in combination with nonunion of the upper lip

Rice. 31. Non-union of the upper lip in combination with a defect in the palate ("cleft lip" and "cleft palate")
(Kupriyanov V.V., Stovichek G.V., 1988): a - on the one hand; b - on both sides; 1 - median nasal process;
2 - maxillary process; 3 - nasal septum; 4 - palatine protrusion
Oblique facial cleft (syn.: paranasal cleft, lateral cleft, oblique coloboma) is a rare, usually unilateral malformation. There are nasopharyngeal and oropharyngeal forms. Both forms in some cases extend to the forehead and temporal region may be complete or incomplete. Oro-orbital clefts occur 2 times more often than nasopharynx and are often combined with other defects: cleft lip and palate, cerebral hernia, hydrocephalus, microphthalmos, deformity of the fingers and toes (Fig. 30, 32).

Rice. 30. Defects in the development of the face (Kupriyanov V.V., Stovichek G.V., 1988):
a - unilateral complete cleft of the upper lip; b - bilateral complete cleft of the upper lip; c - unilateral partial cleft of the upper lip; d - nonunion of the lip extends to the base of the nose; d - open orbital-nasal fissure; e - open orbito-nasal fissure in combination with nonunion of the upper lip

Cleft lip and mandible median - a very rare defect. There are partial and complete forms. At full forms the alveolar process and the body of the lower jaw are connected by a connective tissue bridge. Both halves of the jaw are moderately mobile relative to each other. The tongue end section can be fused with lower jaw. There are cases of simultaneous median cleft of the upper, lower lip and lower jaw.
Dermoid nasal fistula - located on the back of the nose, formed as a result of non-closure of embryonic fissures.
Synophrysis - fused eyebrows.
Telekant - displacement of the inner corners of the eye ^
Rice. 32. Bilateral orbito-oral fissure of the fissures laterally with normally located- (Kupriyanov V.V., Stovichek G.V., 1988)
orbits.
Tricephaly - the presence of three facial surfaces on one head with a common torso.
Cebocephaly - underdevelopment of the external nose up to its absence, combined with a reduced distance between the eyes, as a result of which the patient's face resembles the face of a monkey. The volume of the skull is usually reduced. The fusion of both hemispheres of the brain, the presence of one common ventricle is characteristic. Olfactory nerves, corpus callosum and septum pellucidum are not developed.