Cleft lip. Congenital malformations of the face

Congenital malformations of the maxillofacial area are an arrest of development (underdevelopment) or deviation from the normal formation of certain anatomical formations, organs or systems. Depending on this, the pathology can be of varying degrees of severity - from difficult to detect anomalies, sometimes interpreted as variations in 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 intrauterine development Therefore, the formation of pathological changes is possible only in this period. Numerous genetic and teratogenic factors lead to the formation of developmental defects.

The overall incidence 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 maternity institutions. One of the leading places among developmental defects is occupied by orofacial clefts. They are among the “big five” deformities, ranking 2nd in frequency. Cleft lips account for 86.9% of all congenital facial malformations. 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 tendency towards an increase in their occurrence is emphasized. Every year, for every 100 thousand population, the number of newborns with cleft lip and palate increases by 1.38 (Gutsan A.I., 1984). In this regard, there is a constant increase in the number married couples, in which at least one spouse is a carrier of the anomaly.

Among newborns with cleft lips, boys always predominate (0.79 boys and 0.59 girls per 1000 newborns). In men, as a rule, more severe forms of pathology occur. 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 orofacial clefts. Currently, more than 150 of them have been described.

ETIOLOGY AND PATHOGENESIS.

With cleft lips, sharp changes in the skeletal structure of the face are observed, as well as irregular placement 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 palatal plates can be combined with underdevelopment upper jaw- micrognathia.

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

Using examples of cleft lips of various etiologies, 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 lips caused the death of children in the first years of life, almost all newborns in the population with autosomal dominant syndromes were the result of new mutations. Currently, due to significant improvement surgical technique and conducting the whole system rehabilitation activities The number of operated individuals 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 for 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 the “marriage distance” (the distance from the place of birth of the husband to the place of birth of the wife), does not matter in autosomal accessory syndromes.

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

The most rare monogenic forms of cleft lip are sex-linked syndromes. More common are X-linked mutations, in which the woman is an unaffected carrier of the mutant gene. In this case, the corresponding defects in the pedigree are found in men. With X-linked dominant inheritance, the syndrome is detected in heterozygous women, and the lesion in 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 with chromosomal abnormalities. Common signs All syndromes of chromosomal etiology are prenatal hypoplasia, symmetry of lesions and mental retardation. 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 abnormalities 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 every child, for example with Down syndrome, has a cleft lip and palate, but the incidence of clefts in Down syndrome is 10 times higher than that in the general population.

Multifactorially inherited cleft lips are characterized by symptoms common to all multifactorial diseases. For the occurrence of such forms, it is necessary to have a genetic susceptibility (predisposition) and exposure to any unfavorable factors environment conducive to the realization of susceptibility to developmental defects. Unfavorable environmental conditions themselves, regardless of a specific genetic background, are not capable of causing the appearance of such syndromes. A characteristic feature Such inheritance is the difference in the “threshold of susceptibility” for men and women (the formation of a defect occurs only when the “concentration of genes” exceeds a certain value - the “threshold”). The cumulative effect of genes that can cause a cleft (like any other defect) in representatives of one sex, for example, in men, is not sufficient to cause it in women. 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 forms, which, as a rule, are extremely rare), this indicator is the same in 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 arise as a result of nonspecific effects various factors, realized through a general pathological mechanism (for example, through " vascular factor", leading to hepoxia and necrosis). Currently, 6 specific teratogenic syndromes with cleft lip and palate are 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:

· increased body temperature of a pregnant woman;

· vitamin deficiency;

· deficiency of microelements (copper);

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

· maternal infectious diseases;

· diabetes;

· gynecological diseases.

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

Some monogenically inherited cleft lip and palate syndromes.

CLASSIFICATION.

When diagnosing clefts upper lip in the clinic of the Department of Dentistry childhood The Moscow Medical Dental Institute uses 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-cartilaginous part of the nose (unilateral or bilateral); b) with deformation of the skin-cartilaginous part 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)

· separate

· 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, there is a splitting of the muscle layer with preservation of continuity skin and mucous membrane. In case of incomplete cleft tissue, the lips do not fuse together only in its lower parts, and at the base of the nose there is a properly developed area or a thin skin bridge connecting both parts of the lip to each other. With a complete cleft, all the tissues along the entire length of the lip from the red border to the bottom of the nasal cavity do not grow together. 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 relationship of the parts of the lip is disrupted, 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 the 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 parts of the lip.
With clefts of the upper lip, from the first days of life, the child's sucking function is impaired due to leaks in the oral cavity. With hidden and incomplete clefts of the upper lip, the child can take the mother's breast, pressing the breast tissue against the normally developed alveolar process of the upper jaw and palate, compensating for the inferiority of the muscles lips by 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 sucking function are observed in children with simultaneous clefts of the lip and palate.

DIAGNOSTICS.

Diagnosis is carried out using fetoscopy and fetoamniography. Fetoscopy is performed under ultrasound control at 16-22 weeks of pregnancy using a selfoscope. This technique allows you to see the face of the fetus and, if there is a cleft, suggest the family to abort pathological pregnancy. In addition to fetoscopy, fetoamniography is used. The study is carried out at 20-36 weeks of pregnancy. Under ultrasound control, transabdominal amniocentesis is performed and a solution of an x-ray contrast agent (myodil or verografin) is injected into the placental vessels. 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 mental retardation, etc.

SURGICAL TREATMENT OF CLEFTES OF THE UPPER LIP.

Treatment of children with clefts of the upper lip should be comprehensive and include: surgery, and orthodontic, speech production 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 2-4th day or after the 11-14th day of the child’s life. Contraindications to early lip surgery in a child are concomitant congenital malformations, trauma during childbirth, asphyxia, and postpartum inflammatory process in the mother. results early operations worse than after lip surgery 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.

Plastic surgery of the upper lip for unilateral clefts.

To restore the correct anatomical shape and full function of the lip it is necessary: ​​1) to eliminate the cleft; 2) lengthen the upper lip; 3) correct the shape of the nose.
The lip plastic methods used by dental surgeons today can be 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 they form the nasal vestibule for complete cleft lips. On the positive side linear method is the aesthetics of the scar line coinciding with the border of the philtrum. However, these methods do not allow obtaining sufficient lip lengthening, 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 plastic surgery, an ingrowth of the mucous membrane of the red border in the form of a triangle is observed along the scar.
The second group combines those proposed by Tennyson (1952) and L.V.
Obukhova (1955) methods 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 lengthening of the lip tissue, which depends on the size of the triangular flap borrowed from a small part of the lip; allow you to compare lip tissues and obtain a symmetrical bow shape
Cupid." The anatomical nature of the methods makes it possible to clearly plan the operation.
Their disadvantage can be considered the need to cross the filtrum line in the transverse direction. This 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 nasal deformity.
For complete clefts of the lip and incomplete ones, accompanied by deformation of the cutaneous-cartilaginous part 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 plastic surgery of incomplete unilateral clefts, when large lip lengthening is not required.

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

This operation is performed using most of the methods described above, used for each side separately. Simultaneous repair of bilateral cleft lip in children with cleft of the alveolar ridge and palate does not allow obtaining 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 is of irregular anatomical shape, inactive, welded by scars to the surface of the premaxillary bone.
Subsequently, due to the absence of the vestibule of the mouth, orthodontic treatment of such children becomes difficult.
At the Department of Pediatric Dentistry, MMSI, a two-stage method of upper lip plastic surgery has been developed, which is based on elements of several methods. Incisions on the skin of the lip 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. For complete clefts on the lateral fragment of the lip, 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 only one side. The other side of the cleft is closed after 2-2"/a month. Using this technique plastic surgery of the upper lip, you can achieve high aesthetic and functional results. A well-formed oral vestibule allows for early orthodontic treatment.

The timing and extent of surgical intervention for clefts is determined by the dental surgeon in accordance with the recommendations of all other specialists. Cheiloplasty is performed in a maternity hospital in the first 2-3 days of life or on the 15-16th day after birth, and in a hospital setting - at the age of 3-4 months. For bilateral cleft lip, surgery is performed in two stages with an interval of 3-4 months. From the age of 3, the child actively studies with an orthodontist and speech therapist.

COMPLICATIONS AFTER CHEILOPLASTY.

Complications after upper lip plastic surgery. After surgery, divergence of the wound edges may occur. The reason for this may be tension of the wound edges due to poor tissue preparation, 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 subsequent corrective surgery.

The final effect of the operation is determined by long-term results.
A shallow scarred vestibule of the mouth should be considered as postoperative complication. Lip scars put excess pressure on the alveolar ridge, causing flattening over the years anterior section alveolar arch of the maxilla. Severe deformities of the upper jaw are caused by cicatricial changes in the lip tissue 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 A CHILD.

The suture line on the lip is left without bandages to avoid maceration of the skin. They begin to feed the child 2-3 hours after anesthesia or 1-2 hours if the operation was performed under local anesthesia. Before the stitches are removed, it is better to feed with a spoon; after the stitches are removed, the baby can be placed on the mother's breast or fed with a pacifier.
The nipple should be large, made of soft rubber, with a small hole. Children with cleft palates should be kept in vertical position to avoid aspiration of liquid food.
For warning inflammatory phenomena Antibiotics are prescribed intramuscularly. The wound should be cleaned daily by smearing the suture line with alcohol. Sutures are removed 6-8 days after surgery. The sooner the stitches are removed, the more cosmetic the scar becomes.

Thus, Congenital clefts- this is the result of non-fusion of the embryonic tubercles that form the face of the embryo on early stages embryonic development. Exact reason emergence of this disease currently unknown. The impact of adverse factors on the most early stages pregnancy (in the first trimester) leads to the formation of clefts, i.e. is a multifactorial disease. They can occur as an isolated developmental defect and be one of the symptoms of congenital syndromes.

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Modern science has still not been able to fully understand all the processes occurring in the womb. Even if all tests and examinations show normal development fetus, after birth, features may appear that require medical attention. One of them is cleft lip, which occurs 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 cleft palate, plastic surgery is required.

What is a cleft lip

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

There is almost no defect functional disorders, but requires surgical removal. Statistics on the occurrence of deformation vary from country to country. The minimum level is considered to be when such a pathology occurs in 1 case in 2500 children, the maximum is 1 in 500. The presence of birth defects of this kind in boys is often noted. To eliminate cleft lip, surgery is performed at the age of 3 months to six months. Further visits to the dentist and speech therapist may be required, which are completed by age six. Later, surgery is performed to remove the scars.

Is it inherited?

In about a quarter of cases, the "hare's lip" is caused by genetic disorder which is passed on to children. The presence of one or both parents with a deformed lip significantly increases the risk of the same defect occurring in the child. During pregnancy, compliance is indicated a certain regime, and its disruption can also lead to fetal developmental 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 a person?

The defect manifests itself as an external deformity: it is a gap in the lip located on one or both sides. A common cleft is a unilateral cleft located to the left of the midline of the lips. If there is a bilateral cleft, the presence of other maxillofacial defects is often found. A child may have an incomplete cleft. The presence of a deep defect often results in 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 cleft palate, which can be considered a type of “hare lip,” cleft palate varies. It may be a small hole. A wide cleft palate with deformation of both hard and soft tissues is possible. Cleft palate in humans is caused by a gene mutation.

Causes

Hare's lip and cleft palate can occur as a result of genetic abnormalities such as Van der Woude syndrome or Stickler syndrome. There are a number of factors that increase the likelihood of a child developing 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 stages pregnancy.
• Genetic disorders.
• Bad ecology.
• Chronic or other diseases suffered during pregnancy.
• Heredity. The presence of people in the family who were born with a non-fused lip may be the cause of the pathology.

Classification

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

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

How dangerous is the disease?

The presence of this anatomical defect does not have a direct impact on the child’s health, but it does cause discomfort. Children with a cleft lip may become the object of ridicule from other children. The deformity makes it difficult to speak and eat, the child catches colds more easily, and there are otitis media. It is recommended to eliminate the defect in infancy at the first opportunity and as early as possible. In adulthood, returning to anatomical fullness will be much more difficult.

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 baby is born, parents can immediately notice the presence of a defect. To identify other anomalies, the child’s vision and 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 this defect accidentally discover in adulthood that they once had this problem. Correction of cleft lip is strongly recommended for all children with this diagnosis, unless there are individual contraindications. If the newborn has other pathologies or jaundice, surgery may be refused.

Doctors must make sure that the child does not have other pathologies, that his gastrointestinal tract, cardiovascular, endocrine and nervous systems are functioning normally, and that he is not experiencing weight loss. How rather the baby If a correction is made, the less noticeable the result of the operation will be subsequently. Taking into account 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, restoration of tissue integrity, and 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 the child pronounces all sounds correctly. 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. If there is an alveolar cleft, the operation is performed during the period of mixed dentition, that is, at approximately 8-11 years.

Cheiloplasty

To correct congenital clefts, several stages of reconstructive cheiloplasty are often required. During the operation, the tissues are cut and reconnected, tampons are placed in the nasal passages, and then tubes are placed to prevent the sutures from coming apart, which are removed after 10 days. The operation takes several hours. There are several cutting methods:

• Linear. It leaves behind an almost invisible post-operative scar and 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 eliminate deep crevices.

Rhinocheiloplasty

The upper lip and nasal septum. The operation is often part of a more comprehensive program of surgical correction. During the primary correction, the nasal cartilage is exposed, and the cleft of the upper lip is eliminated. Subsequent operations correct the cleft of the hard 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 using a tube. The duration of the operation is several hours.

Congenital cleft lip is also called cleft lip. Due to different degrees non-fusion of the protrusions of the head end of the embryo involved in the formation of the face and lips, the shapes of the cleft lip are very diverse. Regular form cleft lip - a vertical lateral cleft of the upper lip, formed as a result of non-fusion or incomplete fusion of the frontonasal protrusion with the maxillary one. Other forms of cleft lips are very rare.

Congenital cleft lip It can be one-sided or two-sided, extends to part of the lip or its entire height, is limited to the lip or extends far beyond its limits. When the gap spreads to the nasal opening, the latter expands and becomes deformed; when spreading deeper into the upper jaw, the alveolar process, often the hard and sometimes the soft palate, splits.

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

A cleft lip, unilateral or bilateral, not complicated by a cleft palate, disfigures the face, but almost does not interfere with sucking and therefore does not affect general condition child. This cannot be said about forms complicated by a cleft palate.

Surgery for congenital cleft lip is best performed at 2-6 months of age. Operative methods A lot has been proposed for lip restoration.

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 connected with sutures, starting from the top.

After applying the last suture, 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, extended from the back surface of the other half of the lip. Thus, both halves of the lip seem to be replaced by flaps.

With this method, doctors are completely protected from 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 incisions, we achieve 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 half.”

The cosmetic results of a properly performed surgery are excellent.

According to Mirov’s method, the middle edge of the lip slit is cut off entirely, while the side edge is cut off only to half. The uncut lower half of the side edge serves to form a small flap with a lower base. Next, 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 bloody midline of the lip. For bilateral cleft lips, the same method is used on both sides.

To correct the often accompanying deformity of the nasal wing, the latter is mobilized by separating it from the bone. After this, the lip also becomes more mobile and moves more easily to the middle.

Very rarely, there is a bifurcation of the lower lip in the midline, transverse and oblique cleft lips, as well as an oblique cleft of the face, originating from the corner of the cleft lip and running obliquely towards the eye. The latter deformity is called facial coloboma.

Congenital cleft palate and upper jaw, also known as cleft palate, is formed as a result of non-closure of the gaps existing in the early period of the embryo’s life in the area of ​​the future face, which, when normal course developments at the end of the second month of uterine life close without a trace. In half of the cases, congenital cleft palate is accompanied by a cleft lip.

Cleft palates cause sucking disorders, since the presence of a gap connecting the oral cavity with the nasal cavity eliminates the possibility of creating in the oral cavity the negative pressure necessary for sucking liquid food. Milk entering the mouth flows into the nose and flows out, as a result of which the child’s nutrition deteriorates. Phonation is disrupted, speech becomes nasal and slurred. Nasal breathing disorder causes frequent illnesses respiratory tract.

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

Cleft lip is the most common congenital malformation of a child's face. Occurs in one baby out of 1000 births. Often called a “cleft lip”, the facial defect is formed in the first months of the fetus’s life and is already detected by ultrasound at the 16th week of pregnancy. It manifests itself as a dissection of the upper lip deep from a small fissure to a deep groove on one or both sides. If you consult a doctor in a timely manner, the anomaly can be successfully corrected. Get qualified medical care maxillofacial surgeon Our Clinic offers.

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 cleft lip pathology: hereditary, parental age exceeding 40 years, disorders in the formation of germ cells;
• external influences: unfavorable environment, 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 can lead to the birth of a child with a “cleft lip,” increasing the likelihood of developing fetal malformations. This leads to overheating of the fetus heat body due to illness, exposure to the sun or visiting a bathhouse. Increasing the degree of permeability of the placenta, through which they enter, having a negative effect on the fetus, medical supplies. The cause of congenital cleft of the upper lip is often uterine tumors, previous abortions, predisposition to miscarriages, and severe toxicosis.

Manifestations of a cleft lip defect

The defect in the form of a cleft is usually localized on the upper lip on one, less often two sides, sometimes accompanied by a cleft palate and cleft of the alveolar process of the jaw. Besides external manifestations The defect is characterized by symptoms of impaired dentition, sucking, swallowing and sound pronunciation processes. 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 severity of the defect and location “ cleft lip"Newborn babies have:

• one-sided:
  full(with cleavage up to the nostril);
  incomplete(with partial preservation of tissue);
  hidden(with muscle splitting while preserving the mucous membrane and skin);
• double-sided:
  symmetrical(equally full or partial on both sides);
  asymmetrical(with complete cleavage on one side and incomplete or hidden on the other).

The importance of timely treatment

A child diagnosed with a cleft lip should be shown to a specialist as early as possible in order to find out the degree of complexity of the case, outline a treatment plan and increase the likelihood of successfully eliminating the problem. Competent diagnosis of the disease reveals not only externally noticeable cosmetic defect, but also a number of other violations. It is necessary to prevent their development and negative effect on the child’s health until the defect is corrected through surgery, which is possible no earlier than 4-6 months of age.

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

• feeding 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, lack of teeth, growth at an angle;
• problems with voice and speech (nasality, unclear pronunciation of consonants);
• hearing impairment;
• tendency to otitis media, colds.
• psychological difficulties of adaptation to children's team due to differences in appearance.

Methods for correcting cleft lip

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

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

• primary surgery - at 4-6 months;
• repeated (in case of protrusion of the premaxillary bone by 10 mm or more) - after 2.5-3 months;
• lengthening of the skin of the septum, bringing the wings of the nose closer together at the base - at 4-6 years;
• final correction of the nose - at 16-18 years of age (after the growth of the facial bones has completed).

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Premaxplary agenesis is a severe defect, which is based on gross disturbances in the development of the brain of the arinencephalic group (arinencephalic anomaly). Externally it manifests itself as a cleft lip and palate, a splayed nose, hypotelorism and a Mongoloid shape of the palpebral fissures. Disorders of the facial structure are associated with hypoplasia and aplasia of the ethmoid bone, bone and cartilaginous parts of the nose, as well as the palatine process of the jaw.
Anomaly of the median cleft face (sil.: frontonasal dysplasia, nasal cleft, double nose, dirynia, cleft nose, “Dog nose”) is a complete or skin-covered longitudinal defect of the nasal dorsum, sometimes extending to the alveolar process and forehead (Fig. 25). The defect is accompanied by hypertelorism, broad root nose and in some cases anterior cerebral hernia. There are 3 degrees of median cleft:

where Fig. 25. Anomalies of facial development (Kupriyanov V.V., Stovichek G.V., 1988): a bifurcated nose; b - underdeveloped lower jaw, dystopia of the auricle; c - non-fusion of the rudiments of the lower jaw; d - button-shaped nose without nostrils; d - tubular nose under the only underdeveloped eye; e - cyclopia, tubular nose
I - hidden cleft (the tip of the nose is forked), II - open cleft of the tip and dorsum of the nose, III - total cleft of the soft tissues and osteochondral parts of the nose with deformation of the orbits. Often with such forms there are no wings of the nose. Sometimes there is a complete doubling of the nose. In some cases, brachycephaly, microphthalmia, epicanthus, eyelid colobomas, congenital cataracts, presauricular skin outgrowths, low-lying ears, sometimes - conductive deafness, clinodactyly, campto-

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

a B C

Aprosomy is the absence of a face as a result of a stop in the development of facial anlages. Only individual nodes are noted on the surface of the face.
Arinia - complete absence external nose.
Acephaly is a congenital complete absence of the head. May be combined with absence upper limbs(acephalobrachia), stomach (acephalogastria), heart (acephalocardia), lower extremities (acephalopodia), spinal column(acephalorachia), chest(acephalotoration).
Deviated nasal septum - common vice, develops with retarded growth of the vault and floor of the mouth.

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 ectoderm tissues, laced into embryonic period. There are dermoid and epidermal cysts. The most typical location 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 clefts. It is predominantly localized under the skin at the junction of the nasal bones and cartilage.
Coloboma of the nasal wing is a transverse, shallow one- or two-sided fissure in the free edge of the nasal wing. More often accompanies complex facial defects.
“Bird's face” - a face with a sloping and receding chin with underdevelopment of the lower jaw and Ankyloses of the temporomandibular joint. Observed in Franceschetti-Zwahlen syndrome (Fig. 27).
“Fish face” is a face with a sharply narrowed mouth opening. Observed in Franceschetti-Zwahlen syndrome.
(“bird face”) (Kupriyanov V.V., Meloschisis - cleft cheek with an increase in the size of the mouth.
StovichekG. V., 1988)
Microforms of clefts of the upper 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 clefts of the red border of the lips, deformation of the wing of the nose without the presence of a cleft lip.
Accessory nose (syn.: proboscis, proboscis) - in mild cases it is a tube-shaped outgrowth 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 a nasal septum can be complete or partial. Rarely seen.
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 bone opening leading into the nasal cavity on the same side. The remaining half of the nose is hypoplastic.
Congenital perforation of the nasal septum is a hole in the bone or cartilaginous part of the nasal septum.
The eye shape is anti-Mongoloid - the outer corners of the palpebral fissures are drooping. Occurs as part of many developmental defect syndromes.
Cleft lip (syn.: cleft lip, cheiloschisis, “cleft lip”) - a gap in soft tissues lips, passing laterally from the philtrum. It can be unilateral or bilateral, complete or partial, subcutaneous or submucosal.
Cleft of the upper lip and palate through (syn.: cheilognatopachatoschis) - a gap in the lip, alveolar process and palate. Can be one- or two-sided. With through clefts, there is a wide connection between the cavities of the nose and mouth (Fig. 28). It can be combined with polydactyly and abnormalities of the genitourinary system (Grauhan 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
Median cleft of the upper lip (syn.: prepalatal cleft of the upper lip) is a gap in the soft tissues of the upper lip, located in the midline. Accompanied by a frenulum and diastema; can be combined with an alveolar cleft 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 orbital-nasal fissure and complete absence of the medial part of the upper lip and jaw; e - open orbitonasal fissure in combination with nonunion of the upper lip

Rice. 31. Non-union of the upper lip in combination with a defect of 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 - palatal ledge
Oblique facial cleft (syn.: paranasal cleft, lateral cleft, oblique coloboma) is a rare, usually unilateral, developmental defect. There are naso-ocular and oro-ocular forms. Both forms in some cases extend to the forehead and temporal region, can be complete or incomplete. Oroorbital clefts are twice as common as nasoorbital clefts and are often combined with other defects: cleft lip and palate, cerebral hernias, hydrocephalus, microphthalmia, deformation of the fingers and toes (Fig. 30, 32).

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

Median cleft of the lower lip and lower jaw is 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's end section can be fused with lower jaw. There are known cases of simultaneous median clefts 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 clefts.
Sinophriz - fused eyebrows.
Telecanth - displacement of the inner corners of the eyes ^
Rice. 32. Bilateral orbital-oral fissure of fissures laterally with a normally located - (Kupriyanov V. V., Stovichek G. V., 1988)
orbits.
Tricephaly is the presence of three facial surfaces on one head with a common body.
Cebocephaly is 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. Characterized by the fusion of both hemispheres of the brain, the presence of one common ventricle. The olfactory nerves, corpus callosum and septum pellucidum are not developed.