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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.
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.
Lips are formed by several layers of different tissues.
The main tissue layers that form the lip are:
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:
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.
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 embryonic tubercles involved in the intrauterine development of the face are:
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:
The reasons for the formation of the defect are:
Endogenous causes of cleft lip formation are:
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:
TO negative factors environment include:
Sources of pollution are:
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:
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:
Other teratogenic poisons are:
Diseases that can cause this anomaly are:
Means with high risk are:
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.
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.
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.
The main manifestations of cheiloschisis are:
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).
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.
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:
Types of plastic surgery for cleft lip are:
The methods of making the incision are
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:
Rhinocheilognatoplasty
This type plastic surgery is a complex operation during which a number of problems are solved.
The goals of rhinocheilognatoplasty are:
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.
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:
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.
The stages of rehabilitation are:
The objectives of drug therapy are:
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:
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.
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.
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.
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.
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.
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.
Contraindications for surgery in the first year of life are:
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.
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).
Many people bring their children for cleft lip treatment primarily because it makes the child look repulsive. There are also more serious problems:
In addition, dental problems and developmental defects of the maxillofacial apparatus, for example, dental malocclusion, may soon arise.
Experts list the following types of cleft lip surgery:
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:
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.
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.
Treatment for cleft lip is not suitable for everyone, as it has the following contraindications:
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, 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 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 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
: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).
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:
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:
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.