Cleft lip - Pediatric maxillofacial surgery. Anomalies in the development of the palate

A congenital cleft lip is also called a cleft lip. In connection with different degrees of non-fusion of the protrusions of the head end of the embryo involved in the formation of the face and lips, the forms of cleft lip are very diverse. The usual form of cleft lip is 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 and bilateral, not complicated by cleft palate, disfigures the face, but almost does not interfere with sucking and therefore does not affect the general condition of the 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. Many surgical methods have been proposed to restore the lips.

A. M. Orlovsky describes his method as follows: “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 before it passes into the mucous membrane of both halves of the lip, which has a 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 of the palate and upper jaw, also known as the cleft palate, is formed due to the non-closure of the gaps in the early period of the embryo's life in the area of ​​​​the future face, which, during normal development, close without a trace at the end of the second month of uterine life. 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 respiratory diseases.

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.

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 of intrauterine 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). In this regard, there is a constant increase in the number of married 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. The 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 of the 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 a significant improvement in surgical techniques and a whole system of rehabilitation measures, 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 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 of 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 emergence of such forms, it is necessary to have a genetic susceptibility (predisposition) and the impact of any unfavorable environmental factors that contribute to the implementation of susceptibility into a developmental defect. By themselves, adverse environmental conditions, regardless of a certain genetic background, are not capable of causing the appearance of such syndromes. A characteristic feature of 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 arise as a result of non-specific effects of various factors that are realized through a common pathological mechanism (for example, through the "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);

· taking drugs with mutagenic activity, as well as steroid hormones, androgens, estrogens, insulin, adrenaline;

· infectious diseases of the mother;

· diabetes;

· gynecological diseases.

The description of the phenotype of a sick child is extremely important.

Some monogenically inherited cleft lip and palate syndromes.

CLASSIFICATION.

When diagnosing cleft lip in the clinic of the Department of Pediatric Dentistry of the Moscow Medical Dental Institute, the following clinical and anatomical classification is used:

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).

Another classification of congenital pathologies of the maxillofacial area:

1. Unilateral cleft lip.

2.Bilateral cleft lip

ü symmetrical

ü asymmetrical

· combined (cleft palate + lips)

· individual

· isolated

CLINICAL PICTURE.

Depending on the degree of anatomical changes, there are three forms of cleft lip: hidden, incomplete and complete. With a hidden cleft of the upper lip, a splitting of the muscle layer is observed with the preservation of the continuity of the 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, an irregular shape of the wing of the nose, located on the side of the cleft, is observed. 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, offer the family to terminate the pathological 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 the process of 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 a high risk of having 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 surgical treatment and orthodontic treatment, speech therapy by a speech therapist, etc. There are certain age indications for cheiloplasty:

· Early plastic surgery of the upper lip is carried out 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. The results of early operations are worse than those after 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 the correct anatomical shape and full 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. The positive side of the linear method is the aesthetics of the scar line, which coincides 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 the movement of triangular skin flaps with different angles on the skin in the lower third of the lip.
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. This direction of the 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. Using this technique of upper lip plasty, high aesthetic and functional results can be achieved. 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 tissue preparation, insufficiently thorough layer-by-layer suturing of tissues, the development of a postoperative, inflammatory process in the wound, and 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 a postoperative complication. Lip scars exert excessive pressure on the alveolar process, causing flattening of the anterior alveolar arch of the upper jaw over the years. 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. Children with cleft palate should be kept upright during feeding to avoid aspiration of liquid food.
To prevent inflammation, 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.

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

Congenital cleft lip and alveolar process ( pathology of the primary palate).

Anatomical disorders in patients with pathology of the primary palate depend on the degree of its non-closure. This can be manifested by non-closure of the muscular layer of the lip (hidden cleft), all parts of the primary palate: skin, muscles, mucous membranes of the vestibule of the mouth, alveolar process.

There are various variants of lesions: on one, two sides and their combinations. In addition to the cleft itself, constant anatomical features are shortening of the upper lip, deformities of the nose and alveolar process. The severity of these symptoms varies.

Clefts of the primary palate are often combined with other developmental disorders and pathological conditions (non-closure of the secondary palate, underdevelopment of the lower jaw, heart defects, chondrodystrophy, syndactyly, mental retardation, cerebral hernia, various deformities of the skull bones, etc.).

Congenital cleft palate ( pathology of the secondary palate). Isolated clefts of the secondary palate are manifested in the clinic by various types of clefts (from partial hidden to complete).

With a slight degree of non-closure, only the muscular layer of the soft palate remains split, while maintaining the integrity of the mucous membranes of the oral cavity and nasopharynx. With a pronounced degree of non-closure, all tissues of the secondary palate are split: mucous membranes, muscles and bone.

The length of non-closure is different, as well as the side on which the pathology is formed. In this regard, hidden clefts of various lengths are distinguished (there is no muscle layer or bone tissue and muscle layer) and clefts of various sections of the palate on one side or bilateral.

Congenital cleft lip, alveolar process and palate ( pathology of the primary and secondary palate).

Non-closure of the primary and secondary palate, the so-called through clefts of the upper lip and palate, are the most severe form of this pathology.

Deformations of the dentition are always noted with a combination of cleft lip and palate. These deformations can have different degrees of severity. Most often, narrowing of the upper jaw, microgenia, malocclusion and position of individual teeth or groups of teeth are noted.

Significant anatomical changes in several parts of the face and oral cavity determine the severity of the clinical picture. Functional disorders inherent in clefts of only the lips or only the palate reach a high degree. The respiratory function is sharply disturbed with the development of significant secondary changes in the nasal cavity, nasopharynx, and lungs.

Transverse cleft face. Synonym - macrostomy. It happens one- and two-sided. The degree of severity of the pathology is different - from a slight increase in the oral fissure to an ugly large, as if stretched mouth. The cleft is often combined with other anomalies and malformations.

Oblique cleft face. A severe form of pathology, which is often combined with other manifestations of developmental disorders: cleft palate, aplasia of the eyelids, anomalies of the auricle, skull deformities, hypertelorism, hairy nevus, etc. Oblique cleft face can be one- or two-sided.

A hidden cleft is a retracted furrow on the skin of the face, running obliquely from the corner of the mouth to the outer or inner corner of the eye. The lower eyelid is underdeveloped and drooping.

Full crevices gape. The oral fissure, as it were, continues obliquely upwards. Functional disorders depend on the degree of damage and associated pathological changes. Facial expressions, speech, breathing suffer, food intake is difficult.

Etiology, clinical manifestations, principles of treatment of facial malformations.

CONGENITAL DEFECTS OF DEVELOPMENT OF THE PERSON.

Congenital malformations of the face include cleft lip and palate, transverse and oblique (coloboma) clefts of the face, congenital defects of the nasal septum and individual parts of the external nose, macrostomia, atresia of the oral fissure and nasal passages, etc. .

Treatment of all facial malformations is surgical.

Defects in the development of the face:

a-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.

Anomalies in the development of the face:

a - forked nose;

b - underdeveloped lower jaw (micrognathia), 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.

Cyclopia

Cyclopia is a malformation in which the eyeballs are completely or partially fused and placed in one eye socket, which is located along the midline of the face. "Cyclops" die in the first days of life.

Anomaly of language development

Aglossia

Lack of language. It is observed extremely rarely in severe hypoplasia of the face and jaws.

Ankyloglossia (short frenulum of the tongue)

Attachment of the frenulum in the region of the tip of the tongue or its shortening, leading to a restriction of the mobility of the tongue. The extreme degree of such an anomaly is the increment of the language.

Glossoptosis

The combination of congenital underdevelopment and retraction of the tongue.

Macroglossia

Excessive enlargement of the tongue with pronounced folding of the mucous membrane. Often combined with macrogenia, occurs relatively often.

microglossia

Small size of the tongue. It has not been described as an isolated anomaly. One-sided microglossia is one of the signs of combined defects of the I and II gill arches, with a median cleft of the lower jaw, Robin's anomaly.

Double tongue

Splitting of the tongue in the midline.

Additional language

The presence of an additional muco-muscular protrusion at the root of the tongue. In appearance and mobility it resembles a tongue, only much smaller. An extremely rare defect.

Anomaly in the development of the auricle

Congenital malformations of the auricle are determined immediately after birth due to cosmetic defects

excessive size (macrotia)

small size of the ear (microtia)

distance from the head (protrusion of the auricles).

These defects can be corrected surgically. When the ears are protruding, an oval-shaped skin flap is cut out from the region of the behind-the-ear fold; when suturing the wound, the auricle is attracted to the surface of the head. Macrotia and phlegm can be eliminated with a number of plastic surgeries.

Anomaly of the development of the nose

Congenital anomalies of the external nose due to impaired embryonic development are relatively rare. This is the complete absence or insufficient development of the nose, excessive growth of its parts, abnormal location and development of the entire nose or its departments.

In practice, such deformities of the nose as:

double nose

cleft nose

the formation of the nose in the form of one or two trunks

absence of one or both halves of the external nose

fistulas or cysts of the nose

concha malformations

choanal atresia (holes through which the nose connects to the pharynx).

Deformities of the nose are often combined with malformations of other organs and parts of the body, for example, non-closure of the hard or soft palate, upper lip, underdevelopment of the brain, limbs, etc.

Treatment of deformities of the nose is only surgical. The indications for surgery depend on the nature of the anomaly, the degree of the defect, and the presence of other malformations.

Less severe and more accessible for surgical treatment are nasal fistulas and dermoid cysts that occur due to impaired embryonic development of epithelial rudiments. Usually the cyst is located under the back of the nose at the junction of the nasal bones with the cartilage. The cyst may open with a fistula from which hair protrudes. The pressure of the cyst causes atrophy of the adjacent areas of the bones, which causes deformation of the external nose.

Operations are performed in early childhood, which allows to achieve the best cosmetic results and prevents abnormal development of the bones of the facial skeleton due to deformity.

Congenital atresia of the choanae occurs due to the fact that in the embryonic period the mesenchymal tissue that covers the lumens of the choanae in the form of a membrane does not completely or partially resolve. In the future, this membrane most often ossifies (bone choanal atresia is formed) or turns into connective tissue.

Bilateral choanal atresia can cause asphyxia and death of the newborn, because he does not have a reflex to open his mouth for breathing.

With partial choanal atresia, pathological phenomena also occur in the form of abnormal development of the facial skeleton, in particular, the upper front teeth grow incorrectly, a high palate is formed, and if the atresia is unilateral, then a high palate is formed only on one side, the nasal septum deviates towards atresia.

Diagnosis of congenital choanal atresia is carried out using sounding, infusion into the nose through a catheter of water (with complete atresia, it pours back).

Early surgical intervention in the first year of life is necessary. In cases where the infection of the choanae is life-threatening, it is necessary to operate on the newborn.

Anomalies in the development of the lips

A significant place among the malformations of the face is occupied by congenital cleft lips, due to both hereditary factors and intrauterine development disorders. The formation of this defect may be associated with impaired fusion of the mandibular processes (median cleft of the lower lip), maxillary and median nasal processes (the so-called cleft lip). The size of the clefts ranges from a slight notch in the area of ​​the red border to its complete communication with the opening of the nose. When tissue splitting is limited to the muscle layer, a hidden cleft occurs in the form of a retraction of the skin or mucous membrane. In general, cleft lip and palate are polygenic multifactorial diseases. They can occur as an isolated malformation or be one of the symptoms of congenital syndromes (Robin's syndrome, etc.).

Cleft lip (cleft lip, cleft lip)

Fissure in the soft tissues of the lip, passing to the side of the filtrum. It can be unilateral or bilateral, complete or partial, subcutaneous or submucosal. Congenital incomplete cleft lip can be both with deformation of the skin and cartilage of the nose, and without this defect.

With a hidden cleft of the upper lip, a splitting of the muscle layer is observed while maintaining the continuity of the skin and mucous membrane. In the case of incomplete cleft tissue, the lips do not grow together only in its lower sections, 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 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 ratio of the lip sections is broken, and the red border is stretched along the edges of the cleft.

Complete clefts of the upper lip in all cases are characterized by an irregular shape of the wing of the nose, located on the side of the cleft. The wing is flattened, stretched, the tip of the nose is asymmetrical. In addition, the cartilaginous part of the nasal septum is curved. A similar deformity 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.

Perforated cleft lip and palate (cheilognatopalatoschis)

Fissure 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. It can be combined with polydactyly and anomalies of the genitourinary apparatus.

Median cleft lip (cleft cleft lip)

A gap in the soft tissues of the upper lip, located along the midline. This anomaly is accompanied by a diastema, can be combined with a cleft of the alveolar process and a double frenulum.

Median cleft lip and mandible

This is a very rare malformation. There are partial and complete forms. In the latter case, 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 the lower jaw. There are cases of simultaneous median cleft of the upper, lower lip and lower jaw.

Microforms of cleft lip and palate

They represent a hidden or obvious cleft of the tongue only, a diastema, a hidden and initial cleft of the red border of the lips, a deformity of the wing of the nose without the presence of a cleft lip.

Acheilia

Absence of one or both lips.

brachycheilia

Congenital shortening of the middle part of the upper lip, in which it does not overlap the upper teeth.

Double lip (lip doubling)

A fold of mucous membrane, located parallel to the red border of the upper lip and resembling an additional lip. It occurs predominantly in men.

Macrocheilia

Excessively enlarged lips due to the growth of connective tissue in their thickness.

microcheilia

Very small lips.

Fistula of the lower lip

Usually a paired lesion that is located on the red border of the lip on either side of the midline. It is a duct of accessory mucous glands. Occurs very rarely.

Syncheilia

Fusion of the upper and lower lips.

frenulum of the upper lip

Low attachment of the frenulum of the upper lip, reaching the base of the interdental papilla of the central incisors. A short frenulum of the tongue is a mucosal fold that limits the movement of the tongue due to the short attachment of the anterior third of the tongue to the mucous membrane of the floor of the mouth. It attaches directly to the papilla between the central incisors and affects the mobility of the upper lip (difficulty turning the upper lip outward). Often combined with a central diastema. Occurs very often.

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

Statistical data

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

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

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

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

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

Lip Anatomy

The lips are formed by several layers of different tissues.

The main tissue layers that form the lip are:

• skin layer;
• loose connective tissue layer;
• muscle layer;
• slime layer.

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

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

Mimic muscles located in the thickness of the lips are:

• muscle that raises the upper lip;
• muscle that lifts the upper lip and wing of the nose;
• muscle that raises the corner of the mouth;
• zygomatic minor and major muscles;
• buccal muscle;
• muscle that lowers the upper lip;
• muscle that lowers the corner of the mouth;
• subcutaneous muscle of the neck.

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

The structure and anatomy of the upper jaw

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

The bony processes of the upper jaw are:

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

Intrauterine development of the face

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

• frontal tubercle;
• paired maxillary tubercle;
• paired mandibular tubercle.

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

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

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

Embryonic clefts are:

• median cleft, which is formed at the point of convergence of the maxillary or mandibular tubercles;
• transverse cleft, which is formed by the maxillary and mandibular tubercles;
• oblique and lateral cleft lip, formed at the site of convergence of the nasal process of the frontal tubercle and the processes of the maxillary tubercles.

Reasons for the formation of a defect

The reasons for the formation of a defect are:

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

Endogenous factors

Endogenous causes of cleft lip formation are:

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

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

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

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

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

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

The reasons for the inferiority of germ cells are:

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

Unfavorable environmental conditions

Negative environmental factors include:

• unfavorable ecological situation;
• electromagnetic radiation;
• radiation.

Sources of pollution are:

• thermal power plants;
• metallurgical enterprises;
• chemical production;
• oil companies;
• agricultural organizations.

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

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

Sources of electromagnetic radiation are:

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

Radiation

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

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

Medical sources of radiation include:

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

Fetal chemical poisoning

Other teratogenic poisons are:

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

Vitamin deficiency

Wrong way of life

Infectious diseases

Diseases that can cause this anomaly are:

• cytomegaly;
• coxsackie virus;
• smallpox.

Medications

High risk funds are:

• psychotropic drugs ( lithium);
• antiepileptic drugs ( valproic acid, phenytoin);
• cytotoxic drugs ( methotrexate);
• antibiotics ( dactinomycin, exifin);
• antidepressants ( sertraline, fluoxetine).

External factors

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

What does a cleft lip look like?

Unilateral cleft lip

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

Bilateral cleft lip

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

Other manifestations of cleft lip

The main manifestations of cheiloschisis are:

• sucking and swallowing disorders;
• violations of the dentition;
• chewing disorders;
• speech disorders;
• other anomalies in the development of the maxillofacial apparatus.

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

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

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

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

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

Surgical repair of the defect

At what age is it better to have surgery?

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

Defect repair technique

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

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

Factors that a plastic surgeon takes into account are:

• type of cleft - can be complete or partial;
• type of damage to the lip - meaning one-sided or two-sided cleft;
• the presence of concomitant defects on the face - the presence of a cleft palate or nose defects requires complex surgical intervention;
• the age of the child, his weight, features of physical development;
• the possibility of deterioration after surgery.

Types of plastic surgery for cleft lip are:

• cheiloplasty- performed with a cleft lip;
• rhinocheiloplasty- is prescribed when not only the correction of the lips is necessary, but also the correction of the muscles of the oral cavity and the cartilaginous tissue of the nose;
• rhinocheilognatoplasty- is used for severe pathologies of the facial skeleton with a violation of the structure of the alveolar process ( bone to which teeth are attached).

The incision methods are

• Linear way. The positive side of this method is an inconspicuous postoperative scar. The disadvantage of such operations is insufficient elongation of the lip, so they are not performed in the presence of large clefts. Linear cuts include the methods of Evdokimov, Limberg, Millard.
• Triangular flap method. This group includes techniques developed by Tennyson and Obukhova. Their principle is to correct the defect using triangular flaps. This method allows you to obtain the necessary elongation of tissues and form a symmetrical shape of the lips. The negative side of the method is the formation of a transverse scar on the crease between the mouth and nose.
• Quad flap method. This category includes the methods proposed by Hagedorn and Le Masurier. They consist in the correction of the defect with the help of a quadrangular flap. These methods are used in the plastic of strongly pronounced crevices.

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

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

Indications for secondary rhinocheiloplasty are:

• shortening of the columella ( part of the septum in front of the nose);
• flattening of the tip of the nose;
• deformation of the wings of the nose.

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

The goals of rhinocheilognatoplasty are:

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

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

Under what kind of anesthesia is the operation performed?

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

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

The stages of general anesthesia are:

• premedication;
• induction ( induction into anesthesia);
• introduction of the main anesthetic;
• intubation ( lung ventilation);
• exit from anesthesia.

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

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

The duration of rehabilitation after surgery

The stages of rehabilitation are:

• stationary;
• polyclinic;
• restorative.

The objectives of drug therapy are:

• anesthesia;
• detoxification;
• prevention of bacterial infections;
• correction of violations of water-salt metabolism;
• stimulation of tissue regeneration;
• support for immune functions.

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

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

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

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

Other complications after surgery are:

• facial deformity;
• narrowing of the nasal opening;
• deformation of the wings of the nose;
• speech disorder.

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

Plastic results

How to feed a newborn with a cleft lip?

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

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

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

Why are babies born with cleft lip?

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

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

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

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

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

Is cleft lip hereditary?

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

What does a cleft lip look like after surgery?

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

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

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

Is the cleft lip visible on ultrasound?

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

When is cleft lip surgery necessary?

Contraindications for surgery in the first year of life are:

• prematurity of the child and its low weight;
• diseases of the cardiovascular system;
• breathing problems;
• associated birth defects.

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

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