Cleft lip and palate. What is a cleft lip (cleft lip) and cleft palate (cleft palate)

Cheiloschisis or cleft lip is a congenital anomaly of the facial region in which the upper lip is divided into two parts. In this case, a cleft lip may be limited only to the upper lip, but it can also affect the upper palate, combined with other developmental defects.

Statistical data

Cleft lip is one of the most common congenital anomalies. One child in 1,000 births is born with this defect, which is approximately 0.04 percent of the total world population. Most often, boys are born with a split lip. In most cases, the cleft is located on the left side of the upper lip. In the United States, the incidence of children being born with cleft lip varies by state. In New York, 0.78 children per 1000 births are born with this defect, in Alabama - 1.94, in New Mexico - 2.5.

There is a certain relationship between race and the incidence of this pathology. Compared to the light-skinned population, Asians cleft lip occurs twice as often. 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 deterioration and the emergence of a large number of factors influencing the occurrence of congenital anomalies. Thus, in the Republic of Belarus, where every 5th resident 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 back 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 evil forces invading a person. In Rus', children born with such a lip were considered special people endowed with supernatural powers. It was believed that they could transform into animals.

The first to attempt surgical treatment of cleft lip were the ancient Egyptians. During excavations, mummies were discovered whose facial remains showed signs of an overgrown cleft lip. The defect was stitched together by Egyptian healers using 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 an even slit and then sewing it together in parts. In the mid-17th century, special plates began to be used for facial reconstruction.
Ayurveda interestingly explains the reasons for the formation of the defect ( ancient science of healthy living, originating 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 are the woman’s incorrect behavior during pregnancy. It was believed that a woman could give birth to a child with a cleft lip if she was sexually active during pregnancy, committed sinful acts, and often experienced anger and irritability.

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

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

Anatomy of the lip

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

Lips are formed by several layers of different tissues.

The main tissue layers that form the lip are:

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

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

The muscle layer of the lip is represented mainly by the orbicularis oris muscle. Some of its muscle fibers are arranged 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 muscle layer of the lips also includes a number of facial muscles.

The facial muscles located in the thickness of the lips are:

  • muscle that lifts the upper lip;
  • muscle that lifts the upper lip and ala nasal;
  • levator anguli oris muscle;
  • zygomatic minor and major muscles;
  • buccal muscle;
  • muscle that depresses the upper lip;
  • depressor anguli oris muscle;
  • subcutaneous muscle of the neck.
As a result of contraction of the facial muscles, the lips change their position, expressing different human feelings and emotions.

The mucous layer lining the entire inner surface of the lip passes into the dermal layer on the outer surface. The transition zone from one layer to another is called the lip border. 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 called a frenulum is formed. Many excretory ducts of the salivary glands of the upper lip emerge onto the surface of the mucous layer.

Structure and anatomy of the upper jaw

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

According to the anatomical structure, the upper jaw is divided into a body and four bone processes. The body of the upper jaw is a hollow bone with a large air cavity. 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 participates in the formation of the nasal cavity;
  • palatine process, which participates in the formation of the hard palate ( bony plate separating the oral cavity from the nasal cavity);
  • the alveolar process, which is equipped with dental cells for attaching eight teeth;
  • the zygomatic process, which fuses with the zygomatic bone.

Intrauterine facial development

Intrauterine facial development is difficult process formation and fusion of bones and tissues, which starts at the end of the first month embryonic development.
In the fourth week, the embryo begins to develop five tubercles ( processes), limiting the oral cavity.

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

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

The maxillary and mandibular tubercles grow laterally ( to the sides) and connect on the sides. Thus, the lateral part of the upper jaw and lips, as well as the cheeks, are formed. Next, 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 cusps, unlike the mandibular ones, do not reach the midline. The resulting gap is filled by the nasal process of the frontal tubercle, which grows from top to bottom. It wedges between the maxillary tuberosities, 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 tuberosities and the nasal process of the frontal tuberosity participate in the formation of the upper jaw and upper lip.

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

Embryonic clefts are:

  • median cleft, which is formed at the convergence of the maxillary or mandibular tubercles;
  • transverse cleft, which is formed by the maxillary and mandibular tubercles;
  • oblique and lateral cleft lips, formed at the convergence of the nasal process of the frontal tubercle and the processes of the maxillary tubercles.
By the beginning of the eighth week of intrauterine development, the fusion of facial clefts ends with the formation of the main lines of the face.
When, for any reason, complete fusion of the processes of the embryonic tubercles does not occur, the clefts persist in the form of congenital anomalies. Thus, if the lateral cleft does not heal, a cleft lip is formed, and if the transverse cleft persists, macrostomia is observed ( pathologically large mouth).

Reasons for the formation of a defect

The cleft lip defect is a congenital developmental anomaly, the exact causes of which in most cases remain unclear. Experts note that the formation of a cleft on the lip can be caused by either one factor or a combination of several reasons.

The reasons for the formation of the defect are:

  • endogenous factors;
  • unfavourable conditions environment;
  • influence of radiation;
  • fetal intoxication with chemicals;
  • lack of vitamins;
  • poor mother's lifestyle;
  • taking medications;
  • infectious diseases of a pregnant woman;
  • other external factors.

Endogenous factors

Endogenous factors include internal reasons development of anomaly.

Endogenous causes of cleft lip formation are:

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

Heredity
This pathology often develops 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 the defect is 9 percent.

Hereditary pathologies arise as a result of exposure to internal and external factors, resulting in various mutations occurring 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 abnormality 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 include chemicals that cause changes, mainly in the structure of DNA ( molecule that provides storage and transmission of genetic information). Biological mutagens include various microorganisms that penetrate the body and cause mutations.

Parents' age
Experts identify 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 greatest importance.

Biological inferiority of germ cells
Inferiority of a germ cell is its inability to form a cell with a full 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 reproductive cells can cause the formation of a cleft lip.

The reasons for the inferiority of germ cells are:

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

Unfavorable environmental conditions

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

TO negative factors environment include:

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

Sources of pollution are:

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

One source of pollution whose importance has grown in Lately, is motor transport. Car exhaust gases contain a large number of toxic compounds that have Negative influence on fetal development.

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;
  • e-books;
  • Cell phones;
  • machines for copying documents;
  • scanners and printers;
  • devices for destroying documents;
  • microwaves;
  • refrigerators;
  • TVs.

Radiation

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

Natural radionuclides are divided into terrestrial and cosmic ones. A pregnant woman can be exposed to strong cosmic radiation during an airplane flight. Earth's radionuclides are located in the earth's crust, of which radon is the most significant. You can 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 certain consumer goods. Staying near these radiation factors, the expectant mother exposes herself to the 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;
  • radiation therapy devices;
  • equipment operating on the basis of radioisotopes.

Intoxication of the fetus with chemicals

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

Other teratogenic poisons are:

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

Lack of vitamins

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

Wrong lifestyle

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

Infectious diseases

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

Diseases that can cause this anomaly are:

  • cytomegaly;
  • Coxsackie virus;
  • smallpox

Medications

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

Means with high risk are:

  • psychotropic drugs ( lithium);
  • antiepileptic drugs ( valproic acid, phenytoin);
  • cytostatic drugs ( methotrexate);
  • antibiotics ( dactinomycin, exifin);
  • antidepressants ( sertraline, fluoxetine).
TO medical drugs Anticonvulsants and antipsychotics, antidiabetic medications, and anti-inflammatory medications carry a significant risk.

External factors

Physical factors such as uterine tumors, attempts to terminate the current pregnancy, and previous abortions can cause the development of a cleft lip in a child. A pregnant woman falls from a height, fails to land, gets hit bottom part abdomen can also affect the formation of a cleft lip in the fetus.
One of the external circumstances that can cause this congenital malformation is heat exposure. Overheating of a woman in the sun, high temperature due to illness, visiting a steam room - all this increases the risk of having a child with a cleft lip.

Hypoxia can cause congenital labial cleft ( oxygen starvation) fruit. Due to the insufficient amount of oxygen in the fetus, the metabolism is disrupted, which causes various pathologies during tissue formation. Diseases can cause hypoxia of cardio-vascular system, blood diseases, severe toxicosis. In some cases, lack of oxygen provokes a predisposition to miscarriage, pathological processes in the uterus.

What does a cleft lip look like?

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

Unilateral cleft lip

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

A unilateral cleft of the upper 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 premaxillary process are visible. With a hidden cleft of the upper lip, some of the tissue remains intact. In this case, it undergoes splitting bone base (maxillary process) and the muscles of the lip, and the skin of the lips and their mucous membrane remain intact. Visually, such a defect is not immediately recognized, since the skin and mucous membrane cover the cleft in the lip.

Bilateral cleft lip

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

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

Other manifestations of cleft lip

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

The main manifestations of cheiloschisis are:

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

Dental disorders
Due to the splitting of the upper jaw with a cleft lip, the process of tooth growth is disrupted. Dental irregularities can be characterized by missing teeth, irregular growth angles, or the presence of additional teeth. The teeth of children born with a cleft lip are susceptible to caries and quickly decay. Sometimes, even after plastic surgery of the defect, such children have a malocclusion, which subsequently requires the intervention of an orthodontist.

Chewing disorders
Disturbances in chewing processes are observed in more late age. They develop in several cases - if the defect has not been plasticized, and also if an incorrect bite has been formed. Most often, disruption of the chewing process occurs due to malocclusion and tooth deformation. Improper chewing also contributes to weakness of the muscles of the pharynx and palate, which is observed with bilateral cleft upper lip together with a cleft. upper sky.

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

Other developmental anomalies of the maxillofacial apparatus
Most often, a cleft lip is combined with a developmental anomaly such as a 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 to the maximum. The defect affects not only bone structures, but also muscle aponeurosis ( tendon plates). Weakness and dysfunction of the muscular system of the oral cavity lead to serious problems in children's nutrition. The greatest danger is impaired swallowing. Also, children with multiple anomalies of the maxillofacial apparatus experience breathing disorders. Shallow breathing leads to the development of oxygen deficiency, as less oxygen enters the body. All this leads to physical underdevelopment of children. It should immediately be noted that such an unfavorable outcome is observed in cases where surgical correction of the defect is not performed in a timely manner.

Similarly, cleft lip can be associated with nasal, facial and developmental anomalies internal organs. Cleft lip is also found in the structure of Patau syndrome. This is a chromosomal disease characterized by the presence of an additional thirteenth chromosome. With this syndrome, multiple anomalies in the development of internal organs are noted, for example, defects interatrial septa and blood vessels. In addition to internal organ defects, children with Patau syndrome also have multiple external anomalies. For example, narrowing of the palpebral fissure, deformation of the ears, as well as non-fusion of the upper lip ( cleft lip) and upper sky ( cleft palate).

Surgical correction of the defect

At what age is it better to have surgery?

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

If the defect is expressed as a bilateral cleft, surgery is not possible in the first weeks of birth and is postponed until the child reaches six months. If repeated operations are necessary, they are performed after several months.
If the damage is deep, early age soft tissue correction is performed. Correction of bone and cartilage structures is prescribed for a period of 4–6 years. Final surgical correction of the jaw and nose is best performed after 16 years of age, when the growth of facial bones stops.

Defect plastic surgery technique

In medical practice, there are a large number of methods for correcting cleft lip. A defect can be eliminated using only one technique or a combination of several techniques. Regardless of the type of plastic surgery used, the purpose surgical intervention is to restore the anatomical integrity of the lip and eliminate associated deformities. Surgical treatment should provide favorable conditions for the growth and development of all structures of the midface throughout the patient's childhood.

Preparing for surgery
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, possible risks and complications. Before the operation, the patient is prescribed several examinations and tests in order to identify possible contraindications. For 2 weeks before plastic surgery, patients should not take medications that contain acetylsalicylic acid and various anticoagulants. In some cases, surgical treatment involves some additional manipulations. These can be special dental splints or cast dental onlays.

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

Factors that the plastic surgeon takes into account are:

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

Types of plastic surgery for cleft lip are:

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

The methods of making the incision are

  • Linear method. On the positive side this method is inconspicuous postoperative scar. The disadvantage of such operations is the insufficient lengthening of the lip, so they are not performed in the presence of large clefts. Linear cuts include the methods of Evdokimov, Limberg, and 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 lengthening of the tissues and form a symmetrical lip shape. The negative side of the method is the formation of a transverse scar in the fold between the mouth and nose.
  • Quad flap method. This category includes the methods proposed by Hagedorn and Le Masurier. They consist of correcting the defect using a quadrangular flap. These methods are used for plastic surgery of severe clefts.
In case of bilateral cleft lip, cheiloplasty is performed in two stages. Sometimes the cleft is corrected on both sides first, followed by correction of the defect in the nasal area ( rhinoplasty). In other situations, unilateral correction of the cleft is performed together with nose correction ( rhinocheiloplasty). Then, during the second stage, the gap is corrected on the other side.
After cheiloplasty, postoperative scars remain on the patient’s face. If the operation was performed professionally and there were no complications after it, the scars appear in the form of thin thread-like strips that are almost invisible.
Residual deformities in the lip or nose area remain after the first cheiloplasty in 70–80 percent of those operated on. As you get older, postoperative defects may become more pronounced. In such cases, reconstructive cheiloplasty is performed to correct cosmetic defects.

Rhinocheiloplasty
This type of plastic surgery involves simultaneous correction of the upper lip and nasal septum. Such operations can be performed either independently or as part of a complex surgical treatment. There are primary and secondary rhinocheiloplasty. The goal of primary rhinocheiloplasty is to correct the malposition of the nasal cartilage tissue and restore the anatomical integrity of the lip.

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

Indications for secondary rhinocheiloplasty are:

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

Rhinocheilognatoplasty
This type plastic surgery 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;
  • correction of nasal defects.

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

Under what anesthesia is the operation performed?

Surgery to correct cleft lip can be performed with local or general anesthesia.

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

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

The stages of general anesthesia are:

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

Intubation is carried out using a tube that is inserted into the airway and connected to a special device. Intubation ensures that the patient can breathe normally during surgery.
During the operation, the anesthesiologist controls the supply of the drug to ensure the state of anesthesia. Using medical equipment, the doctor monitors the child’s well-being, checking blood pressure, breathing and cardiac activity.

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

Duration of rehabilitation after surgery

The duration of rehabilitation depends on the characteristics of the child, the nature of the operation performed and how the child’s body responded to anesthesia. There are several stages in the process of patient recovery after surgery.

The stages of rehabilitation are:

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

The objectives of drug therapy are:

  • anesthesia;
  • detoxification;
  • prevention of bacterial infections;
  • correction of water-salt metabolism disorders;
  • stimulation of tissue regeneration;
  • support of immune functions.
In order to protect the sutures from food and the nasal cavity from narrowing, a gauze tampon is inserted into the patient's nose. The sutures are removed after 7–10 days, after which a special tube is inserted into the opening of the nose and left for 3 months. This helps prevent deformation of the nasal cavity and wings of the nose. To prevent postoperative sutures from coming apart, the patient should avoid facial injuries.
To enhance the effect of the operation, in some cases the child is prescribed to wear a special headdress. The device is a supporting bandage that passes through the upper lip, securing it in the cheek area. Such a garment helps prevent the lip from stretching and maintains the integrity of postoperative sutures. The duration of use of the device is determined by the doctor. To prevent the child from damaging the stitches with his hands, hand movements are limited using a splint or other device.

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

Restorative rehabilitation
The goal 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 doctors such as ENT ( otolaryngologist), orthodontist, speech therapist, dentist. Observation by these specialists is necessary in order to prevent malocclusion, defects in the formation of the dentition, and problems with speech.

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

Complications
One of the complications of surgical treatment of cleft lip is the dehiscence of the wound edges. This can happen due to mistakes made in the process of intervention, development inflammatory process in the wound, trauma received by the patient after surgery. Shallow scars in the area of ​​the vestibule of the mouth are considered as a postoperative complication. Over time, putting pressure on the alveolar process, the 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 deformation;
  • narrowing of the nasal opening;
  • deformation of the wings of the nose;
  • speech disorder.
A cleft lip entails disability. This does not mean that children born with this anomaly are physically handicapped. With timely correction of the defect, complications do not develop. At the same time, if a child is born into a family with such an anomaly, the pediatrician is obliged to refer him for a disability examination. The basis is disorders of the digestive and respiratory systems or speech formation. Disability is established until the violations are eliminated between the ages of 3 and 7 years.

To receive disability compensation, a child must undergo an examination. To do this, the parent must contact the social security authorities. A child is removed from the disability register only after the end of the rehabilitation period. Rehabilitation methods should be focused not only on plastic surgery 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 results in severe, irreparable damage, then the group remains for life.

Plastic results

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



How to feed a newborn with a cleft lip?

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

Deep, through defects of the upper lip require the use of special nipples, which differ in shape from regular ones. This is due to the fact that such large clefts are accompanied by weakness and dysfunction of the muscles of the maxillofacial apparatus. As a result, the baby has difficulty sucking. The most common are nipples from NUK and Avent. These nipples are put on the bottle ( the same or another company), where breast milk is first expressed. It is recommended to move the pacifier as far as possible to the root of the tongue. If the sucking process is difficult, it is recommended to make the hole in the nipple larger. This can be done by the mother herself using ordinary scissors.

If the cleft of the upper lip also affects the palate, then special attachments are used. These attachments look like inserts that are placed in the child’s mouth, thus closing the defect. In case of massive through clefts, when the defect is too large and the sucking and swallowing reflex is impaired, they switch to tube feeding.

It is very important to maintain breastfeeding and not switch to artificial formula, unless, of course, the child has concomitant metabolic pathologies ( for example, lactase deficiency). This is necessary because children born with a cleft lip are susceptible to frequent colds. Breast milk contains all the necessary substances to strengthen the immune system.

Why are children born with cleft lip?

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

Heredity
Numerous studies among close relatives have shown that the risk of developing a cleft lip is highest in those children whose family already has anomalies of the maxillofacial apparatus. 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, fusion of the alveolar processes does not occur.
According to the same studies, the probability of a child developing a cleft lip reaches 4–5 percent if one of the parents suffered from a similar pathology. The risk doubles if both parents have a cleft lip.

Environmental factors
At the same time, some children with cleft lip do not have relatives with a similar anomaly. This indicates the participation of external factors in the development of cleft lip. Today it is a generally accepted fact that bad habits mothers play a crucial role in the development of this anomaly. It has been proven that smoking women The risk of having a child with a cleft lip is 6–7 times higher than that of non-smokers. If a woman abused alcohol during pregnancy, 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 child with a cleft lip. The greatest teratogenic effects on the fetus are herpes viruses, measles, Coxsackie and cytomegalovirus. If a pregnant woman experiences one of these infections in the first trimester of pregnancy, the fetus is at risk of developing a cleft lip ( even if the mother does not smoke or abuse alcohol) increases several times.

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

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

Is cleft lip inherited?

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

If a couple has already given birth to 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 a cleft lip. Scientists have found that the disease appears as a result of a complex interaction of genetic predisposition and environmental conditions. Therefore, the risk of having a child with this anomaly, despite hereditary predisposition, can be reduced to zero if we take into account all the factors that provoke the formation of a defect. It is necessary to undergo examinations for the presence of chronic infections, take the necessary microelements ( for example folic acid) even while planning pregnancy, and also avoid smoking and drinking alcohol while expecting a child.

What does a cleft lip look like after surgery?

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

During the operation, the surgeon restores the correct position of the tissues and connects them. After this, a barely noticeable postoperative scar remains in the area of ​​the nasolabial triangle. The location of the scar depends on the type of surgery 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 used, then a transverse scar is located on the fold 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.

In case of deep defects, when the dissection of the lip reaches the wings of the nose, nose plastic surgery is also performed. In this case, flaps of mucous tissue are peeled off on both sides of the gap, which are used for plastic surgery of the nasal opening in the area of ​​the splitting of the alveolar process. The extent of the scar after this 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, scars can be eliminated ( or make them less noticeable) any volumes.

Is a cleft lip visible on an ultrasound?

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

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

When is surgery necessary for cleft lip?

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

Contraindications for surgery in the first year of life are:

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

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

If the defect affects the bone and cartilaginous structures of the face, then surgery is prescribed for a period of 4–6 years. Final surgical correction of the jaw and nose is best performed after 16 years of age, when the growth of facial bones stops.

Correction of a cleft lip involves surgical interventions that are performed for a congenital defect - cleft lip, a combination of nasal, labial and maxillary deformities. The goal of surgery in this case is to restore the lip and eliminate birth defects persons, creating normal conditions for adequate formation and further development middle facial zone.

Clinical picture

The cleft of the upper lip can be located on one side or mirrored (on both sides). According to experts, the most common unilateral variant is on the left side. In complex cases, a complication involving the lower lip is possible (a fairly rare occurrence).

The one-sided version may be short in length, but in some cases it can reach the nose area (in this case, the maxillary tissues become noticeable, hence the name).

Visually, the lip appears to be cut or cut (similar to injury). The dissection can be hidden or open (visible oral cavity, lip muscles and other soft tissues).

Problems and possible consequences

Many people bring their children for cleft lip treatment primarily because it makes the child look repulsive. There are also more serious problems:

  1. Violation of sucking abilities
  2. Impaired speech, swallowing and chewing functions

In addition, dental problems and developmental defects of the maxillofacial apparatus, for example, dental malocclusion, may soon arise.

Types of interventions

Experts list the following types of cleft lip surgery:

  • Rhinocheiloplasty. Involves the process of restoring the physiology of the upper lip and nose. The palatal cleft will remain in this case, but such a correction of the problem is best option. The surgeon seeks to rehabilitate the functioning of the muscles of the upper lip, correct the red border, form the correct dimensions of the oral vestibule and return the normal position of the nasal wings, improve symmetry and shape the bottom of the nasal passages. In many cases, techniques are used to minimize the visibility of scars. The need for secondary interventions is determined by the correct selection of the treatment method, the level of initial deformation of cartilaginous and tissue structures, as well as compliance with postoperative recommendations. The unilateral version can be adjusted from 3 months of life, the bilateral version - from six months. Immediately after the operation and for several more days, feeding is carried out using a spoon or a special nasogastric tube (depending on age and condition)
  • Rhinocheilognatoplasty. The intervention helps eliminate anatomical abnormalities of the upper lip, nose and alveolar process, and correct through defects. One of the main indications is a bilateral variant of the pathology. It is better to prescribe surgery in childhood (before the bite is fully formed)
  • Veloplasty. This is the name for correction of soft palate tissues. It is performed to restore the function of swallowing and speaking, and correct breathing. According to experts, a child is able to learn to consume food, avoiding it getting into the nose from the mouth, but above speech apparatus it will require difficult work. If there are serious speech changes, it is not possible to correct them yourself. It is important to take into account and control the child in the first years of life: how he speaks, sings, reads. Veloplasty can be performed on children older than 8 months. The intervention is tolerated without special problems, after about a couple of days the baby can already feed on his own
  • Palatoplasty. For babies with a cleft, the operation can be carried out in several stages (the intervals are determined by a specialist). If the congenital problem also affects the hard palate, it is a serious indication for palatoplasty. Upon completion of the restoration of the anatomical structures of the soft palate, an independent narrowing of the gap in the hard palatine tissues occurs. After a few years, it narrows as much as possible, which allows you to restore integrity without traumatic actions. Such a step-by-step correction allows short time create conditions for proper development speech functions and prevent disturbances in the maxillary growth areas. When restored in one stage, there is a risk of maxillary underdevelopment

You can learn more about the treatment and rehabilitation of children with cleft lip in the video of a member of the Russian Union of Pediatricians, O.V. Ginther, candidate medical sciences, maxillofacial surgeon:

Rehabilitation period

For several days after cleft lip repair, you will need to wear a gauze pad prescribed by your doctor. After the tampon is removed, a plastic tube is placed in the patient’s nose for about 3 months to prevent narrowing of the passage and deformation of the nasal wing.

The sutures are removed after a week. In the future, the scar will be practically invisible to others; it can additionally be eliminated using cosmetology procedures.

Forecast

In approximately 70% of cases, patients will require additional surgeries in the future to correct secondary nasal and labial deformities. Aesthetic and functional results are concluded no earlier than 12 months after surgery.

Contraindications

Treatment for cleft lip is not suitable for everyone, as it has the following contraindications:

  1. Infectious diseases
  2. Respiratory system diseases
  3. Impaired blood clotting
  4. Heart and circulatory system defects

Famous people with the defect

People with a cleft lip do not have mental or mental retardation; the problem covers only the physiological side of the issue. Many celebrities suffer from a similar anomaly, but this has not stopped them from becoming famous and in demand among millions of people.

Denis Dorokhov

Denis Dorokhov, a member of the Kamyzyak Territory Team of KVN, suffers from a cleft lip. He became famous in KVN, but previously he hid the problem with the help of an artificial mustache. Now he can be seen in a variety of roles in the humorous show “Once Upon a Time in Russia.” But the young man no longer hides the problem, and recently even began helping Russian children with this anomaly.

Target setting. Learn to diagnose congenital clefts of the upper lip and palate and create a treatment plan. Learn to organize treatment for children with such disorders depending on the territorial location of the medical examination center for children with congenital pathology of the face and jaw (city, district, region, region, republic).

Clinical picture and diagnosis of congenital clefts of the upper lip and palate

Clinical manifestations of cleft lip and palate depend on the anatomical shape and extent of the cleft.
Diagnosis of cleft lip and palate is carried out according to the clinical and anatomical classification given in the textbooks of A. A. Kolesov “Dentistry” childhood"(M.: Medicine, 1978, 1985).
Cleft lip and palate often occur in combination and rarely occur in isolation (Fig. 166).

Anatomical and functional disorders in cleft lip and alveolar process: 1) cleft of the upper lip; 2) shortening of the height of the lip due to a vicious arrangement of muscles; 3) deformation of the skin-cartilaginous part of the nose, deformation and underdevelopment of the upper jaw in the area of ​​the alveolar process and the edges of the pyriform opening.
Symptoms of functional impairment in this group of children are variable. Violation of the act of sucking can occur only with a cleft lip and alveolar process due to air entering the oral cavity through the cleft of the alveolar process. During the formation of speech, the pronunciation of labial, labial-lingual and some hissing sounds of Russian speech is disrupted.
Anatomical and functional disorders in children with cleft palate: 1) cleft palate; 2) shortening of the soft palate; 3) wide middle part of the pharynx; 4) underdevelopment of the size of the upper jaw for this group of patients is an unstable symptom and is rare.
Functional disorders (disorder of sucking and swallowing movements) appear from the first days of life. The baby does not take the mother's breast, but when artificial feeding chokes easily and may aspirate liquid food.
A wide connection between the nasal cavity and mouth with cleft palate leads to the free entry of air into the upper respiratory tract and the formation of a mixed nasal type of breathing. Children with a cleft palate get used to breathing shallowly, taking shallow inhales and weak exhales. Shallow breathing is compensated by an increase in the number of breaths per minute, and with the age of the child leads to a decrease vital capacity lungs. Inadequacy of external respiration predisposes to the development of colds inflammatory diseases upper respiratory tract and lungs. Sick children incorrectly articulate the palatal, palatoglossal and sibilant sounds of our speech.
Congenital deficiency of the tensor muscles curtain of palate, and the pterygopharyngeal part of the upper constrictor of the pharynx contributes to dysfunction of the auditory tube with the occurrence of foci of chronic inflammation and obstruction in it, which ultimately often leads to the development of acute and chronic otitis media and hearing loss.

Rice. 166. The most common combinations of clefts of the upper lip and palate are a - cleft of the upper lip and alveolar process (cleft of the primary palate); b - cleft palate (cleft of the secondary palate); c - clefts of the upper lip, alveolar process and palate (clefts of the primary and secondary palate).

Constant mechanical irritation of the nasal mucosa with food leads to the development of foci of chronic inflammation in this area (hypertrophic rhinitis, chronic sinusitis); many children develop chronic tonsillitis.
Anatomical and functional disorders in children with cleft lip, alveral process, hard and soft palate. Children with these disorders are the largest group among patients with congenital clefts of the upper lip and palate (about 60%), they are carriers of the most severe anatomical and functional pathology;
Main anatomical disorders: 1) cleft lip; 2) reduction in lip height; 3) deformation of the cutaneous cartilaginous part of the nose; 4) deformation of the alveolar process of the upper jaw and underdevelopment of the bone in the area of ​​the pyriform opening; 5) cleft of the soft or soft and hard palate; 6) shortening of the soft palate; 7) wide middle part of the pharynx.
Functional disorders in this group of children consist of functional disorders inherent in children with cleft lip and palate (see above).

Treatment of congenital clefts of the upper lip and palate

Treatment of congenital clefts of the upper lip and palate is carried out in medical examination centers for children with congenital pathology maxillofacial area. Such centers are organized throughout the territory of our country and, according to administrative subordination, are divided into city, regional, interregional, regional and republican centers.
The objectives of medical examination of children with congenital clefts of the upper lip and palate are: 1) identification of children with cleft lips and palate in the first days after birth. For this purpose, maternity hospitals fill out form No. 30 for each child and send it to the medical examination center;
2) drawing up an individual treatment plan for the child depending on the anatomical shape of the cleft, the general condition of the child and the absence or presence of concomitant congenital malformations; 3) implementation of all types of treatment for the child (orthodontic, surgical, somatic, staging correct speech, sanitation of the oral cavity, prevention of the development and treatment of foci of chronic inflammation in the nasal cavity and nasopharynx, etc.); 4) rehabilitation of children after plastic surgery of the upper lip or palate; 5) medical and genetic counseling of a sick child, his parents and relatives in order to resolve the issue of character congenital pathology(hereditary, sporadic) and the degree of risk of re-birth of a child with a congenital defect.
The listed tasks are solved by a group of specialists of various specialties: dentists (surgeon, orthodontist, therapist), speech therapist, pediatrician, otolaryngologist, psychoneurologist, geneticist, methodologist therapeutic exercises.
The child is prepared for the operation in advance. Lip and palate plastic surgeries are planned, therefore they are performed on a child in the absence of local and general somatic contraindications.
Surgical treatment of cleft lip. The operation is performed under general anesthesia. The optimal age of a child for lip surgery is 4 - 6 months.

The objectives of the operation are: a) to close the cleft lip by correct anatomical comparison of tissues; b) increase the height of the upper lip; c) correct the shape of the skin-cartilage section of the nose according to indications.
Lip surgery is performed using local tissue.
Widely used surgical techniques differ in the way they lengthen the lip. There are three main groups of methods: 1) “linear” methods (Fig. 167, a, b, c); 2) techniques with moving opposing triangular musculocutaneous flaps (Fig. 168); 3) techniques with moving a quadrangular flap (Fig. 169, a, b).
The clinic of the Department of Pediatric Dentistry of the MMSI uses a combination of the Limberg and Tennyson methods in its work.
Surgical treatment of cleft palate. The operation is performed under general anesthesia. Optimal for the operation is preschool age 6-7 years.
The objectives of the operation: 1) to close the cleft along the entire length of the palate; 2) lengthen the soft palate; 3) narrow the middle part of the pharynx.

Rice. 167. “Linear” techniques for upper plastic surgerylipsa - according to Evdokimov; b - according to Limberg; c - according to Millard

For cleft palate, the radical Limberg operation is widely used (Fig. 170, a-c).
For complete clefts of the alveolar process and palate, the Limberg technique is combined with the method of closing the anterior part of the cleft (Fig. 171, a - d).
For bilateral complete clefts of the alveolar ridge and palate, various techniques, allowing you to solve all three problems of palate plastic surgery in one or two stages.

Rice. 168. Stages of upper lip plastic surgery using opposing triangular flaps according to the technique adopted in the clinic of the Department of Pediatric Dentistry of the N. A. Semashko Moscow Medical Institute (diagram).

Rice. 169
According to Gagedorn (a); according to Le Mesurier (b).

Rice. 170. Stages of palate plastic surgery using the Limberg method (according to V. F. Rudko). a - before surgery; b - during the operation; c - after surgery.

Orthodontic treatment of children with cleft palate. Children with complete clefts of the upper lip, alveolar ridge and palate require mandatory planned orthodontic treatment. For children with isolated cleft palates, orthodontic treatment is carried out according to individual indications when signs of retarded growth of the upper jaw appear (Fig. 172).
The objectives of orthodontic treatment are: 1) elimination of congenital deformation of the upper jaw and normalization of the size and shape of the upper dental alveolar arch in the sagittal, transversal and vertical direction; 2) ensuring the formation of an orthognathic bite during the growth of the facial skeleton and after plastic surgery of the lip and palate; 3) prevention of the development of secondary deformations of the lower dentoalveolar arch and bite.
Orthodontic treatment of children with bilateral complete clefts of the upper lip, alveolar ridge and palate should begin in the first month of life. The main goal of treatment at an early age is to correct the growth of the vomer, on which the incisive bone is fixed. Orthodontic devices strive to delay the growth of the vomer in the sagittal and vertical directions and place the incisive bone within the upper dentoalveolar arch.
Orthodontic treatment of children with unilateral complete clefts of the upper lip, alveolar process and palate can begin much later, from 3 to 3 years of age, after plastic surgery of the upper lip. At this age, the fixation of orthodontic appliances becomes easier, since by this period the bite of baby teeth is formed.


Rice. 171. Stages of cleft closure in the anterior palate with complete clefts of the alveolar process and palate (diagram).
a, b - according to Zausaev; c, d - according to the method adopted at the Department of Pediatric Dentistry of the MMSI named after. N. A. Semashko.

Treatment is carried out with functional and mechanical devices. Elastic bandages that apply pressure from front to back and bottom to top (Fig. 173, a) and palatal plates (Fig. 173, b) help to retard the growth of the vomer with the incisive bone. The palatal plates are formed in such a way as to limit the growth of the vomer with the incisive bone forward and downward and at the same time ensure the free growth and development of the lateral sections of the alveolar process. At a later age, before and after palatoplasty, palatal plates with a median or sectoral cut are used, the mechanical action of which is aimed at creating the shape and size of the upper dentoalveolar arch.
Orthodontic treatment of most children with complete unilateral and bilateral clefts of the upper lip, alveolar process and palate should stop only after the end of growth of the facial skeleton (for boys - 18-20 years old, for girls - 16-18 years old).
Delivering the correct speech. A speech pathologist is involved in the formation of speech articulation in children with cleft lips and palates. At different ages of children, the tasks of a speech therapist are different.

Rice. 172. Types of deformation of the alveolar arch in clefts of the alveolar process and palate (diagram).
Above - with bilateral clefts; below - with one-sided clefts.

A speech therapist should begin working with a child and his parents in infancy. At this age, the main task of the teacher is to train external breathing and practice oral exhalation. This is achieved through gymnastics and games, during which the child learns to breathe deeply (playing a locomotive, playing the pipe, etc.). It is useful to combine the work of a speech therapist with the work of a methodologist in therapeutic exercises.
At the age of 4-4.5 years, during the period of formation of conscious speech, a speech therapist conducts classes with the child and practices the articulation of individual speech sounds. Before surgery, only the nasal tone of speech remains (open rhinolalia). After palate plastic surgery, the speech therapist consolidates the child’s acquired skills and eliminates the nasal sound of speech.
With the systematic work of a speech therapist, the child clearly and correctly pronounces speech sounds 2-4 months after the operation.

Control questions
1. What is the classification of congenital clefts of the upper lip?
2. What is the classification of congenital cleft palate?
3. List the anatomical and functional disorders associated with congenital cleft lip.
4. Tell us about the anatomical and functional disorders of congenital cleft palate.
5 List the age-related indications for upper lip surgery.
6. List the age-related indications for palate plastic surgery.
7. Name the indications for orthodontic treatment for cleft palate.
8. Name the indications for logotherapy for cleft palate.
9. Tell us about medical and genetic counseling for children with congenital clefts of the upper lip and palate and their parents as a means of preventing congenital pathology.
10. Tell us about the medical examination of children with congenital clefts of the upper lip and palate. Describe the territorial principle of organization medical care. Which specialists provide assistance? What are the indications for surgical and orthodontic treatment?

Cleft lip and palate are the most common congenital malformations of the head and neck. In addition to changes in the appearance and growth of the face, these developmental defects lead to disorders of chewing, swallowing and speech.

Examination and treatment of patients with cleft lip, palate and nasal deformities is challenging. To provide comprehensive care to these patients, an interdisciplinary team approach is required. Successful treatment requires special effort, aimed at conducting examinations and eliminating dental, psychological, speech, otiatric and aesthetic deficiencies of patients with cleft fusion.

Normal development of the lip and palate occurs during the embryonic period (the first 12 weeks of fetal development). The middle part of the face is formed anterior to the forebrain due to the differentiation of the wide frontonasal protrusion located in the midline. The primary palate forms around the 4th or 6th week and forms the primary separation between the oral and nasal cavities. The primary palate, or median palatal process, is formed by the fusion of the paired median nasal protrusions, the fusion of which in turn gives rise to the central part of the upper lip, the central maxillary alveolar arch and the corresponding lateral and central incisors, and the hard palate in front of the incisive foramen (Fig. 1) . The development of the primary palate differs embryologically from the normal formation of the secondary palate (posterior to the incisive foramen).

Rice. 1. Diagram of the development of the lip and palate at 4 and 5"/2 weeks of pregnancy. The primary palate is formed at approximately 4-6 weeks by the fusion of paired median nasal protrusions.

The paired median nasal processes fuse in the sixth week of embryonic development and ultimately form the incisive bone, philtrum of the upper lip, columella and tip of the nose. The lateral elements of the upper lip (lateral to the column of the philtrum) originate from the paired maxillary processes. The cheek, maxilla, cheekbone and secondary palate (see below) are also formed from the maxillary processes. Thus, the upper lip is formed from both the median nasal and maxillary processes.

The secondary palate begins to develop around the eighth week of gestation, after development of the primary palate has completed (Figure 2). The formation of the secondary palate occurs through the growth and movement downward and medially of the palatine folds (medial projection of the maxillary processes). As the palatal folds move downward (like a drawbridge), the developing nasal cavities expand laterally and downward.

Rice. 2. Schematic representation of an embryo at the 7th and 10th week of intrauterine development. During this time, the secondary palate is formed, the fusion of parts of which occurs in the anteroposterior direction and begins from the incisive foramen.

The paired palatal folds are initially separated by the developing tongue. The growth of the lower jaw, accompanied by the movement of the tongue forward, makes it possible for the palatal folds to move downward and assume a more horizontal position. If the development of the fetus and the movement of the lower jaw occur with deviations from the norm, the paired palatal folds cannot move downward and medially. The lack of contact between these folds leads to the appearance of a cleft in the palate. This malformation is described in detail in the works of the French pediatrician P. Robin (micrognathia, relative macroglossia and U-shaped cleft palate) (Fig. 3A, B).

Rice. 3. Child with Robin's syndrome (micrognathia, relative macroglossia and U-shaped cleft palate).

The normal sequence of normal palate formation begins when the palatal folds and nasal septum touch each other and continue in an anteroposterior direction. Closure of the palate first occurs near the incisive foramen at approximately 8 weeks of gestation and is usually completed by the formation of the uvula by 12 weeks of gestation. The degree of nonfusion of the secondary palate is associated with many factors, including the moment at which the fusion process is interrupted during fetal development. Thus, anomalies in the development of the palate can only be a bifurcated uvula, a submucosal cleft of the soft palate, or a complete cleft of the secondary palate.

Jonathan M. Sykes

Diagnosis and treatment of cleft lip and palate


Cleft lip and palate(CRGN) in the structure of congenital deformities occupy the second place in frequency and are among the most severe malformations of the face and jaws, leading to significant anatomical (cosmetic) and functional disorders. According to WHO, the frequency of births of children with CGN in the world is 0.6 - 1.6 cases per 1000 newborns. There is a tendency for this indicator to increase, one of the reasons for which is the sharp deterioration of the environment. Anatomical and functional disorders present in children with this pathology lead not only to a delay in the physical development of these patients and to frequent concomitant diseases, but also often lead to changes in the mental state of the child, caused by isolation and the development of an inferiority complex. In addition, most sick children with congenital CGN have concomitant congenital malformations of other organs and systems (heart, lungs, kidneys, etc.), which also must be taken into account when drawing up a treatment plan.

Etiological factors, leading to the appearance of malformations of the face and jaws, are divided into exogenous and endogenous.

Exogenous causes:

    physical factors:
    – mechanical (previous abortions, abnormal position of the fetus, uterine tumors, multiple pregnancies, maternal injuries in the first months of pregnancy, etc.);
    – thermal (hyperthermia);
    – ionizing radiation (external and internal);

    chemical factors:
    – hypoxia (anemia, toxicosis in pregnant women, uterine bleeding, chronic alcoholism, etc.);
    – inadequate and unbalanced nutrition;
    – hormonal imbalances (diabetes mellitus, thyroid disease in a pregnant woman, phenylketonuria);
    – teratogenic poisons (gasoline, formaldehyde, heavy metal salts, nitrogen oxide, mercury vapor, alcohol, etc.);
    medicinal substances(chemotherapy drugs, adrenal hormones, insulin, vitamin A, salicylates, diazepam, etc.);

    biological factors:
    – viruses (measles rubella, measles, cytomegalovirus, herpes simplex, mumps, chickenpox);
    – bacteria and their toxins;
    – protozoa;

    mental factors (causing hyperadrenalineemia).

Endogenous causes:
    pathological heredity (dominant or recessive);
    biological inferiority of germ cells (improper lifestyle; bad habits: alcohol, smoking, drugs);
    influence of parents' age and gender.

Pathogenesis. Under the influence of one or more of the listed etiological factors, the fusion of the edges of the “physiological gap” is delayed, which leads to congenital nonunion of the upper lip and palate. There is evidence that the primary palate forms around 6 to 7 weeks of gestation and contributes to the initial separation between the oral and nasal cavities. The primary palate is a triangular, horseshoe-shaped piece of tissue that separates the nasal passages from the oral cavity. It is located in the area of ​​the alveolar process, which includes the four upper incisors. Subsequently, the primary palate gives rise to the anterior (premaxillary) part of the final palate, as well as the middle part of the upper lip. As a result of the rapid growth of the maxillary and medial nasal processes, which come closer and fuse with each other, the anlage of the upper jaw and upper lip is formed. It must be borne in mind that the middle part of the upper jaw, bearing the incisors and the middle part of the upper lip (area philtrum), arises due to the fusion of the medial nasal processes. Therefore, in the embryonic period of development, a cleft of the upper lip often accompanies a cleft of the primary palate. These are the so-called median clefts of the upper lip and upper jaw. But the most common is the formation of lateral clefts of the upper lip, as a result of non-fusion of the maxillary process with the medial nasal process. Around 8 to 9 weeks of pregnancy, after development of the primary palate has finished, the secondary palate begins to develop. It is formed from the palatine processes, which are formations on the inner surfaces of the maxillary processes. When the tongue is lowered, the edges of the palatine processes rise, move and grow together with each other and the nasal septum. By the end of the 12th week of pregnancy, fragments of the soft palate fuse together. Thus, the pathogenesis of cleft of the hard and soft palate is associated with underdevelopment, and, consequently, nonunion of the palatine processes.

Anatomical and functional disorders with congenital CGN are diverse and depend on the type of cleft lip and (or) palate, i.e. depending on severity birth defect.

For all clefts of the upper lip There are anatomical disorders common to all types, expressed to a greater or lesser extent:

    splitting of the tissues of the upper lip;
    shortening of the middle fragment of the upper lip;
    deformation of the skin-cartilaginous part of the nose.
With a hidden cleft of the upper lip, there is a pronounced underdevelopment of the muscle layer in the absence of a violation of the integrity of the skin and mucous membrane of the upper lip. On the side of the philtrum there is a vertical scarred strip of skin in the form of a groove, under which the orbicularis oris muscle is absent. In a calm state, the defect is little noticeable and appears only during smiling, crying, etc., when muscle ridges appear on both sides of the groove due to contraction of the orbicularis oris muscle. The shortening of the upper lip with a hidden cleft is insignificant (1 - 2 mm), and the deformation of the skin-cartilaginous part of the nose is hardly noticeable.

With an incomplete cleft of the upper lip, tissue nonunion is present 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. There is almost always a deformity of the nose: the wing of the nose on the side of the cleft is stretched, flattened, its base is displaced outward and downward, the tip of the nose is shifted towards the cleft, the nasal septum is curved due to its arching in the healthy direction. 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 lip muscles by actively including the tongue in the act of sucking.

With complete clefts, all the tissues of the upper lip from the red border to the lower nasal passage do not grow together. In all cases, there is deformation of the skin-cartilaginous and bone parts of the nose. With a bilateral cleft of the upper lip, the nasal septum is shortened, the prolabium protrudes anteriorly in the form of a proboscis, the tip of the nose is flattened, often bifurcated, the wings of the nose are stretched and flattened on both sides, the nostrils are wide.

For congenital cleft palates There are also anatomical disorders common to all types of clefts, expressed to varying degrees depending on the severity of the defect:

    splitting of the tissues of the palate;
    shortening of the soft palate;
    expansion of the middle part of the pharynx.
With a hidden cleft of the soft palate, only the muscles of the soft palate are split along the midline while maintaining the integrity of the bone structures and mucous membrane of the oral and nasal cavity. With a hidden cleft of the hard and soft palate, a retracted groove is identified in the midline, which increases when pronouncing the sound “a” due to contraction of the cleft muscles of the soft palate. Typically, the mucous membrane in this area has a bluish tint as a result of the translucency of two layers of the nasal and oral mucosa that are welded together. Upon palpation, non-union of the palatine processes of the upper jaw along the midline is determined. There is almost always a bifurcation of the tip of the uvula on the palate. The soft palate is somewhat shortened. The speech of such children is nasal and is often accompanied by compensatory contractions of the facial muscles.

With an incomplete cleft of the soft palate, its anterior border does not reach the posterior edge of the hard palate. With a complete cleft of the soft palate, its non-union reaches the posterior edge of the hard palate and often continues further in the form of a hidden cleft of the hard palate. With complete and incomplete clefts, the soft palate is also shortened. The children's speech is slurred and nasal, but the growth and size of the upper jaw in these children is not impaired.

With a complete cleft of the soft palate and an incomplete cleft of the hard palate, the anterior border of the cleft does not reach the incisive foramen. If the cleft of the soft and hard palate is complete, then the anterior border of the cleft reaches the incisive foramen. In this case, the base of the vomer lies freely, without connecting to the palatine plates. With complete clefts, congenital underdevelopment of the upper jaw with malocclusion is possible. The speech of such children is even more nasal. As a rule, a child cannot suckle at the breast, and the air stream entering the nasal cavity seems to fall into the oral cavity. These disorders are caused by the inability to create a vacuum in the child’s mouth. With complete clefts of the soft, hard palate and alveolar process, which can be unilateral or bilateral, the described signs are even more pronounced. In addition, with non-union of the lip, all this is accompanied by a sharp disfigurement of the child. After teething, these children also experience all possible anomalies in the teeth in the cleft area and malocclusion.

Treatment children with congenital clefts of the upper lip and palate. Timely and correct implementation of the first stage of surgical treatment determines the success of rehabilitation of patients with congenital clefts of the upper lip and palate. Particular attention in recent years has been given to the full restoration of not only anatomical structures, but also functions with minimal traumatic impact of surgical manipulations on the subsequent growth of the facial skeleton.

The main and most effective methods of plastic surgery of the upper lip for clefts are rightfully considered to be patchwork methods of cheiloplasty, which have been reasonably undergoing a number of recent years changes and improvements. In Russia, widespread methods of primary cheiloplasty are used, described by Tennison-Obukhova and Millard. Used for palate plastic surgery traditional ways at the age of 2.5 to 7 years in order to prevent the damaging effects of surgery on the growth of the upper jaw. However, in most cases, these methods do not relieve patients from problems associated with deformation of the upper jaw, the presence of occlusion anomalies and defects in the dentition, do not allow full restoration of speech and significantly complicate social adaptation child in society.

Modern ideas about the processes of development, formation and growth of the facial skull and surrounding tissues, knowledge of the anatomy and physiology of the premaxillary-maxillary complex in normal conditions and in congenital clefts of the upper lip and palate made it possible to develop and implement (A.S. Artyushkevich, D.A. Grichanyuk , Belarusian Medical Academy of Postgraduate Education, Department of Maxillofacial Surgery) to the clinic functional and gentle methods of their correction: the most optimal may be early cheilo-uranoplasty, the essence of which is to perform simultaneous interventions on the soft palate (veloplasty) and the upper lip (cheiloplasty) at the age of 3 - 6 months with the use of mucoperiosteal flaps to close the alveolar process defect using bioactive platelet gel. This should lead to activation of natural growth factors, acceleration of reparative processes and obtaining the required bone volume in the area of ​​the bone defect. It is important to note that the ability of the upper jaw to grow after taking a mucoperiosteal flap is not impaired, since the periosteum quickly regenerates from the edges of the wound, and obtaining a bioactive platelet gel does not require donor material(except the patient's blood). In this case, the operation time is extended by only 10-15 minutes. This technique reduces the treatment period for patients with this pathology and eliminates the traumatic stage - bone grafting; the number of complications decreases; the disability of children with this pathology is reduced, which ultimately makes it possible to achieve full medical and social rehabilitation of patients, create conditions for social protection and adaptation of the child in the family and team.



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