Cleft lip. Causes, symptoms, plastic surgery and rehabilitation. Cleft lip is not a death sentence: plastic surgery can help

A cleft lip is not something terrible and irreparable. If parents find out that their baby will be born with such a pathology, do not panic and despair, because now there are modern and safe methods surgical intervention, allowing you to get rid of the disease almost without a trace.

Cleft lip is a congenital pathology that occurs due to unfused tissues of the nasal cavity and upper jaw in the intrauterine state. The pathology is a cleft in the upper lip, dividing it into two parts.

The medical name for the disease is cheiloschisis. According to medical statistics, per 1,000 newborn children, there is 1 baby with a cleft lip.

Besides negative influence on appearance, the defect creates functional problems in eating and the formation and development of the child’s speech. But this defect does not have a pronounced negative effect on the body as a whole and on the overall development of the baby.

There are 2 types of cleft lip:

  1. Unilateral or bilateral cleft lip.
  2. Isolated or continuous cleft lip.

The cause of the pathology lies in a genetic mutation associated with a change in the TBX22 gene. The mutation occurs between 8 and 12 weeks of pregnancy. It can occur for various reasons:

Based on the strength of influence of the above reasons on the formation of a defect, the most significant can be identified: chemical factors - approximately 22%, mental - 9, biological - 5, physical - 2 percent.

In the first trimester of pregnancy, the internal and external organs of the fetus are formed, so this is a very responsible and dangerous time. The mother definitely needs to isolate her body from the effects of teratogenic factors (reasons that may cause disruption of the development of the fetus and its organs).

A family that already has one child with this disease is recommended to consult a geneticist.

In most cases, newborns have a cleft on the upper lip and very rarely you can find a defect on the lower lip.

There are several types:

1. One-sided splitting:

  • Incomplete - part of the lip tissue remains untouched in the upper part.
  • Complete is complete splitting upper lip.
  • Hidden - only the muscles of the lip are split, and the skin and mucous membrane are intact.

2. Bilateral splitting:

  • Symmetrical - incomplete or complete cleft on both sides.
  • Asymmetrical - incomplete or hidden on one side, complete on the other (and other options).

A split on one side is a depression on the upper lip. In this case, the middle nasal and right maxillary processes do not have fusion. The defect can have different configurations, for example, in one case it affects only the soft tissues of the lip, and in another it can also affect the bones of the upper jaw.

Nowadays, the existence of such a pathology in the fetus is easily determined using ultrasound in the 3rd trimester of pregnancy.

If a cleft lip is discovered, then this is not a reason to terminate the pregnancy, because the development of the fetus is not disrupted, and the child will not suffer from delays after birth mental development(unless, of course, cheiloschisis is not part of congenital pathological syndromes). Children born with a cleft lip do not differ in any way in mental and mental development from other kids.

After the birth of the baby, the doctor must fully examine the child, after which he can accurately diagnose the disease and establish the fact that cleft lip is in no way connected with mental illnesses, and that it is an independent disease.

Modern medicine can help patients with such genetic disease. By using plastic surgery You can completely and without problems eliminate cleft lip in children. There are 3 types of plastic surgery to eliminate cleft lip:

  • Cheiloplasty.
  • Rhinocheilognatoplasty.
  • Rhinocheiloplasty.

The operation is selected taking into account the nature and configuration of the defect and is performed under general anesthesia.

Surgical intervention can only be performed on those children who were born in due date, and which have no contraindications: serious pathologies vital important organs, serious illnesses(cardiovascular, respiratory tract etc.), birth injuries etc.

The operation can be performed as early as the 1st month of life. But as a rule, the operation is performed on babies aged three to six months. However, with deep lesions, treatment can begin as early as the 1st week of the child’s life.

Surgeries can completely eliminate the disease in almost ninety percent of cases. The full result can be assessed after a year has passed from the time of the operation.

Cheiloplasty is an operation to eliminate cleft lip. Before cheiloplasty, it is mandatory to conduct a complete medical examination of the baby.

During plastic surgery, the split tissues are connected and restored. correct position bones. After the operation is completed, a gauze swab is inserted into the nasal cavity for some time to protect the sutures. After removing the tampon, a tube is inserted into the nose for three months.

Sutures are removed approximately ten days after surgery. Very often, after the main operation, additional cosmetic and other procedures are subsequently carried out to correct the remaining consequences of the cleft lip.

Rhinocheilognatoplasty is a complex operation used when it is necessary to eliminate pathology of the alveolar process and for severe anomalies of the facial part of the skull. It helps to form the normal position of the mouth muscles. The operation improves the shape of the upper lip, reduces the nasal cartilage defect, and eliminates the likelihood of developing dental anomalies.

Rhinocheiloplasty is a more complex operation that eliminates not only the cleft lip, but also corrects the muscles of the mouth and nasal cartilage. Reconstructive plastic surgery is performed at any age. Depending on the severity of the defect, procedures may be carried out to correct residual defects in the child.

Many patients require subsequent surgeries to correct nasal deformities. At the age of 4 to 6 years, it is worth having surgery to correct the wings of the nose and lengthen the skin part of the nasal septum. And the final operation to correct the nose is best performed at 16–18 years of age, since at this age children experience a slowdown in the growth of the facial skeleton due to age-related changes. You will also need Plastic surgery for removing a scar on the lip.

Children, after removal of a cleft lip, must be periodically observed by an otolaryngologist, because they are prone to colds and otitis media. It is necessary to be constantly monitored by a speech therapist and dentist, and also to visit an audiologist and phoniatrist due to speech, sound perception and hearing disorders.

Famous people, including actors and musicians, also include people who were born with cheiloschisis and have had surgery to correct it. For example, the famous Hollywood actor Joaquin Phoenix. A scar on his upper lip is clearly visible on his face. The harelip is attributed to the famous Russian musician Andrei Makarevich and TV presenter Masha Malinovskaya.

The risk of having a child with a cleft lip can be minimized if you carefully monitor your health during pregnancy and be attentive to factors that can trigger the formation of a defect in the fetus. It is best to plan your pregnancy and undergo all examinations before conception to identify all diseases, folic acid and vitamin deficiencies. Be sure to lead a healthy lifestyle, completely give up alcoholic beverages and smoking. And if the mother’s job is in a dangerous workplace, it is better to refuse it.

Photos of children after surgery.

> > > Cleft lip

Congenital cleft lip is also called cleft lip. Due to the different degrees of nonfusion of the protrusions of the head end of the embryo involved in the formation of the face and lips, the forms of cleft lips 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 deep into the upper jaw it splits alveolar ridge, often hard and sometimes soft palate.

If a bilateral cleft lip continues into the palate, then the premaxillary bone, separated from the sides, sometimes moves forward strongly, dragging 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 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 until 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 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 side back surface 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.

Split is very rare lower lip along 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 what is necessary for suction liquid food negative pressure. 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.

Most favorable period To perform the operation (see course in dentistry) the age is from 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.

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 of intrauterine development, hence the formation pathological changes 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 of 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. 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 orofacial clefts. Currently, more than 150 of them have been described.

ETIOLOGY AND PATHOGENESIS.

With cleft lips, sudden changes are observed bone skeleton the face, as well as the incorrect position 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 of the upper jaw - micrognathia.

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

Examples of cleft lips of various etiologies traceable general principles, characteristic of any monogenic, multifactorial and chromosomal hereditary diseases. 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 improvements in surgical techniques and the whole system Rehabilitation measures increase the number of operated persons with autosomal dominant syndromes who marry and pass on the mutant gene to their children. 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 component of multiple malformations due to 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 for 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 environmental factors that contribute to the development of susceptibility into a developmental defect. 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 of various factors realized through a general 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:

· 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 of the 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 of anatomical changes, three forms of clefts of the upper lip are distinguished: 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 disability 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-related indications for 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 associated birth defects development, trauma during childbirth, asphyxia, postpartum inflammatory process in the mother. The results of early operations are worse than after lip plastic surgery performed at a later stage. late 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 to: 1) 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, 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 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 of the postoperative scar reduces the aesthetic result of the operation. Recommended use by the indicated methods with 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 complex anatomical relationships jaw bones and soft tissue deficiency. 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 of upper lip plastic surgery, high aesthetic and functional results can be achieved. A well-formed vestibule of the mouth allows for early orthodontic treatment.

Timing and scope surgical intervention for clefts are determined by a dental surgeon according to the recommendations of all other specialists. Cheiloplasty is performed in 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. If the wound edges diverge in newborns, it is not recommended to apply secondary seams, since 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 place excess pressure on the alveolar process, causing flattening of the anterior alveolar arch of the maxilla over the years. 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. The child begins to feed 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 pacifier must be large sizes, made of soft rubber, with a small hole. Children with cleft palates should be kept in vertical position to avoid aspiration of liquid food.
To prevent inflammation, 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. The exact cause of this disease currently unknown. Exposure to unfavorable factors in 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 developmental defect and be one of the symptoms of congenital syndromes.

Congenital clefts of the upper 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-fusion. This can manifest itself as 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.

Meet various options lesions: on one, two sides and their combinations. In addition to the cleft itself, constant anatomical signs are shortening of the upper lip, deformation of the nose and alveolar process. The severity of these signs varies.

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

Congenital cleft palate ( pathology of the secondary palate). Isolated cleft palate appears in the clinic different types clefts (from partial hidden to complete).

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

The extent of the non-union varies, as does the side on which the pathology forms. In this regard, hidden clefts of various lengths are distinguished (there is no muscle layer or bone and muscle layer) and clefts of various parts of the palate on one side or bilateral.

Congenital clefts of the upper 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 dental system are always observed with a combination of cleft lip and palate. These deformations may have varying degrees expressiveness. Most often, narrowing of the upper jaw, microgenia, malocclusion and position of individual teeth or groups of teeth are observed.

Significant anatomical changes in several parts of the face and oral cavity cause severity clinical picture. Functional disorders, inherent in clefts of only the lip or only the palate, reach high degree. Respiratory function is sharply impaired with the development of significant secondary changes in the nasal cavity, nasopharynx, and lungs.

Transverse facial cleft. Synonym: macrostoma. It can be one- or two-sided. The severity of the pathology varies - from a slight increase in the mouth gap to an ugly large, as if stretched mouth. The cleft is often combined with other anomalies and malformations.

Oblique facial cleft. A severe form of pathology, which is often combined with other manifestations of developmental disorders: cleft palate, aplasia of the eyelids, abnormalities of the auricle, skull deformities, hypertelorism, hairy nevus, etc. Oblique facial cleft can be unilateral or bilateral.

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

Complete clefts gape. The oral fissure continues obliquely upward. Functional disorders depend on the degree of damage and associated pathological changes. Facial expressions, speech, breathing suffer, and eating is difficult.

The majority are represented by clefts, which are formed as a result of disruption of the fusion of embryonic structures and arrest of their development. In this regard, clefts are localized in certain places.

Cleft lip(nonunion, cheiloschisis, “cleft lip) - a gap in soft tissues lips, passing laterally from the philtrum. It can be one- or two-sided, complete, partial, subcutaneous or submucosal, accompanied, as a rule, by a peculiar deformation of the tip and wing of the nose.

The median (prenebia) cleft of the upper lip is a gap in the soft tissue 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, can be isolated or accompanied by more severe defects, for example, maxillofacial densostosis.

Cleft palate(palatoschisis, “cleft palate”) can be complete (gap in the soft and hard palate), partial (only in the soft or only in the hard palate), median, one- and two-sided, through or divided.

Through cleft of the upper lip and palate (cheilognatopalatoschiz) - a cleft of the lip, alveolar process and palate. It can also be one- or two-sided. With through clefts, there is a wide connection between the cavities of the nose and mouth, which greatly complicates sucking, swallowing and subsequent speech.

Cleft lip- until the end of the 7th week, sky - until the 8th week. With an average population incidence of clefts of 1 case per 1000 births, pronounced regional differences are known. Thus, in Japan the frequency of these defects is 2.1 cases per 1000, in Nigeria - 0.4 cases per 1000 births. Cleft lips are more common than cleft palates; The exception is the median cleft lip. The risk of recurrence of an isolated cleft lip (or in combination with a cleft palate) for siblings in sporadic cases is 3.2-4.9%. The RNSC is approximately the same for the children of the affected subject. The risk of recurrence of isolated cleft palate in sporadic cases is 2%; for children in whom one of the parents had a cleft, -7%.

Surgical treatment. The duration of the operation depends on the location and type of defect. Surgical intervention on the lip can be performed in the first 2 days after birth. The most common period is 6 months.

Cleft fuck up to 6 months they are corrected with an obturator; after this period, plastic surgery is performed for periods from 3 to 12 years. The choice of time and methods of surgery, as well as the outcome of treatment, in addition to the severity of the defect, to a certain extent depend on the presence of concomitant malformations and the preservation of the growth potential of the tissues surrounding the cleft. In addition to surgery, such children require systematic pediatric, orthodontic, and speech therapy treatment.

Microforms 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.

Median cleft lip and lower jaw. A very rare defect. There are partial and complete forms. In 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 can be fused with the lower jaw by its coiculum. There are known cases of simultaneous median clefts of the upper, lower lip and lower jaw. TTP - up to the 5th week.

Treatment prompt, the period is determined individually.

Double lip(double) - a fold of the mucous membrane located parallel to the red border of the upper lip and resembling an additional lip. It occurs quite often, mainly in men. Treatment is surgical. Oblique facial cleft (paraiasal, lateral cleft, oblique coloboma). A rare, usually unilateral malformation. There are naso-ocular and oro-ocular forms. Both forms, in some cases, extend to the forehead and temporal region, and can be full or non-full. Orophthalmic clefts are twice as common as nasophthalmic clefts and are often combined with other clefts: cleft lip and palate, cerebral hernias, hydrocephalus, hysterelism, microphthalmia, deformation of the fingers and toes. The edges of the defects are sometimes fused with the amnion. TTP - before the 5th week of intrauterine development. With full forms the prognosis is unfavorable. Such children often die in the perinatal period.

Surgical treatment. Optimal age for surgical intervention is determined individually in each specific case.

Midfacial cleft anomaly (frontonasal dysplasia, nasal cleft, double nose) is a complete or skin-covered longitudinal defect of the nasal dorsum, sometimes extending into the alveolar process and forehead. The defect is accompanied by hypertelornosm, a wide root of the nose and, in some cases, an anterior cerebral hernia. Less common are epicanthus, microphthalmia, and a wedge-shaped hairline in the forehead. There are three degrees of median cleft: I - hidden cleft: the tip of the nose is bifurcated, II - open cleft of the tip and dorsum of the nose, Ш - total cleft of the soft tissues and bones of the cartilaginous 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. There are known cases of a combination of fronton azal dysplasia with hydrocephalus, arineicephaly and microgyria.

The vast majority of these defects are sporadic cases; seed forms are known. The population frequency for severe forms is one case per 80,000 -100,000 births. The basis of the median facial cleft is a stop in the development of the ventral sections of the 1st branchial arch, in particular the nasal capsule. TTP - before the beginning of the 6th week. Median facial clefts must be differentiated from Goldenhar and NMS syndromes, as well as nasal glandoma. Goldenhar syndrome is accompanied by epibudbar dermoid. In NMS syndrome, there are mnorotia and kidney defects and no cerebral hernias are observed. Nasal anomaly is not accompanied by hypertelorism, the tip and wings of the nose are not changed. Life prognosis for frontonasal dysplasia III degree adverse; Defects of degrees I and II are subject to surgical correction.

Premaxillary agenesis- a severe defect, which is based on gross disturbances in the development of the brain of the aryencephalic group (aryencephalic 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 ethmoid bone, bone and cartilaginous parts of the jaw, as well as the palatine process of the jaw. Population frequency of 1 case is 25,000-30,000 births. TTP - up to the 5th week. Most children die during the final period. There are known familial cases with recessive type inheritance.

Macrostomia is an excessively enlarged rotovan fissure. Caused by nonunion of the tissues of the upper and lower parts cheeks and edges of lips between each other. It can be one- or two-sided, and is a sign of anomalies of the 1st and 2nd gill arches. Population frequency is 1 case in 80,000 births.

Treatment operational.

Microstomia(small mouth) - excessively reduced oral opening. It is rarely observed as an independent defect. Known family forms with a dominant type of inheritance. Usually combined with severe defects of the derivatives of the 1st branchial arch or included as a component of Frimer-Shelton syndrome. Fistulas of the lower lip.

Fistulas of congenital origin- usually paired and located on the red border of the lips on either side of the midline. They are ducts of accessory mucous glands. They are very rare. Such fistulas are considered to be a hereditary trait, transmitted by a dominant type. May be a component of popliteal pterygium syndrome.

Treatment is surgical.

Upper lip frenulum- low attachment of the frenulum of the upper lip, reaching the base of the interdental papilla of the central incisors. In such cases, the frenulum turns out to be wider, sometimes represented by a cord that limits the mobility of the lip. Often combined with central diastema. It occurs very often.

Treatment surgical at the age of 3 years and older.

Double mouth- an extremely rare defect, manifested by an additional oral fissure, opening into an additional oral cavity smaller in size than the main one oral cavity. Both cavities do not communicate.

Treatment operational.

Extra nose, or proboscis (proboscis), in mild cases it is a tube-shaped outgrowth located at the corium of the nose. It increases as the child grows and has no connection with the cranial cavity. IN severe cases instead of a nose there is a tubular leathery formation with one blindly ending hole. It is lined with mucous membrane. Proboscis usually accompanies severe CNS defects - cebocephaly, ethmocephaly and cyclopia. In cases of cebocephaly, the base of the proboscis is localized at the level of the root of the nose; with ethmocephaly, it is located above the level of the palpebral fissures (which in such cases are close together). In the case of iclopia, the base of the proboscis is located above the medianly located single palpebral fissure. In the latter case, a double proboscis is occasionally found. Population frequency is 1 case per 37,000 births. TTP - before the 4th week of intrauterine development. Proboscis is sometimes seen in newborns with chromosomal diseases. The laterally located proboscis is accompanied by aplasia of the corresponding half of the nose, and sometimes by microphthalmos and ketotic degeneration of the optic nerve.



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