Congenital nonunion of the upper lip: causes, symptoms, diagnosis, treatment

The anatomical structure and size of the lips in children and adults vary considerably; However, they have certain harmonic limits, the deviation from which we associate with the notion of an ugly or even ugly form of the lips.

The normally developed upper lip has the following anatomical components:

1. filter (filtrum);
2. two columns (columellae);
3. red border;
4. middle tubercle or proboscis;
5. line (or arc) Cupid - the so-called line dividing the red border and the skin of the upper lip.

When treating a child with a congenital defect of the lips, the surgeon must recreate all of its listed elements.

[1], [2], [3], [4], [5]

Symptoms of congenital nonsense of the upper lip

Symptoms of lip nonunions depend on their type and number. The most severe disfigurement, the difficulty of the act of suckling the mother's breast, the violation of breathing, and subsequently the pronunciation of sounds are observed with bilateral, especially complete, non-incisions of the upper lip.

Sometimes non-affection, starting from the corner of the mouth, passes into a congenital crooked defect of the cheek, causing a picture of one- or two-sided macrostomy. Non-convergence of the lip and cheeks can extend to the lower eyelid, the infraorbital margin of the upper jaw, the superciliary arbor and the entire frontal bone.

In congenital nonunions of the upper lip and palate, in 76.3% of cases, there are various deformations of the dento-jaw system, the elimination of which is an integral part of the complex treatment of patients. The most frequent form of dentoalveolar deformities in congenital non-growth of the lip and palate is narrowing of the maxilla (60.7%).

In the opinion of AN Gubskaya, the anomalous arrangement of the teeth near the area of non-affection, the abnormal forms of the teeth and their roots, the adentia, the supercomplex of the teeth must be attributed to congenital or primary deformations.

Deformations that gradually develop as a result of the interaction of the defect with the external environment, the effects of various biomechanical factors after the birth of the child, should be considered secondary. They can develop before and after the operation.

Before the operation, the following defects develop:

1. displacement of individual anterior teeth or large fragment of alveolar process with teeth in the vestibular direction;
2. narrowing of the upper jaw.

They increase with the growth of the child, the activation of his speech, the enhanced development of the language (macroglossia), and so on.

After cheiloplasty may occur:

1. displacement of individual teeth or their group in the direction of the palate, turning them around the transverse and longitudinal axes;
2. flattening of the anterior part of the alveolar process of the upper jaw. Along with these anatomical disorders, in patients with congenital non-abscesses of the upper lip and palate, there are functional changes on the part of the chewing apparatus, which are manifested by a decrease in the strength of the muscles of the lips, chewing efficiency and atypical reflex chewing movements of the lower jaw.

Classification of congenital nonsense of the upper lip

In accordance with clinical-anatomical features, congenital defects of the upper lip are divided into several groups:

1. In the transversal plane, non-injuries of the upper lip are divided into lateral ones - one-sided (about 82%), bilateral (about 17%) - and middle, dividing the lip into two symmetrical parts (about 1%).
2. In the vertical plane, they are subdivided into partial (when non-spread has spread only to the red border, or simultaneously with the red border there is a non-degeneration of the lower part of the skin of the lip) and full - within the entire height of the lip, as a result of which the nasal wing usually develops because of non-growth the bases of the nostrils.

IM Got and OM Masna (1995) have established that the dimensions of non-fractures (the wing of the nose

• upper lip - alveolar process
• palate) on the right are much larger than the left.

The sizes of nasal apertures on the party of a non-affection and to the healthy party essentially differ: accordingly up to 14 and 8 mm. With two-sided incisions, the size of the defects on each side is smaller than in the case of one-sided ones. The same applies to the size of the nose defects. All these facts are very useful both for substantiating the choice of delayed surgical intervention, and for preoperative treatment (orthopedic, orthodontic, logopedic).

3. In terms of the depth of tissue non-infestation,
   • obvious - non-delays of all layers of the lip (red rim, mucous membrane, skin and muscle layer);
   • latent - nerazhcheniya only the muscular layer of the lip, while the skin layer is somewhat thinned;
   • combined - nerazhcheniya lips, extending to the gum or palate, cheek (coloboma of the face), eyelids and superciliary arches.

With bilateral nonunions of the upper lip, their length can vary in height and depth (for example, on one side, complete incision of lip fragments extending to the alveolar process and palate, and on the other - only a hidden non-growth of the muscular layer within the red border and slightly above the line Cupid). Two-sided complete non-growth of the lip in a number of cases is accompanied by a more or less pronounced protrusion of the intermaxillary bone. As a result, the middle fragment of the lip sometimes protrudes sharply forward (in the form of a "trunk") and is soldered to the tip of the nose, extremely disfiguring the newborns. This is explained by the fact that in the embryonic period and after birth (up to 6-7 years of age) the cartilage of the septum of the nose occupies a leading position in the system of developing cartilages, therefore it is laid down and differentiated earlier than other cartilages. In the first phase of the postnatal period, the entire septum of the nose consists of cartilage.

Nonunions of the lip and palate can be combined with abnormalities of the brain area of the skull, the auricles, the tongue (macroglossia), the thorax, the spinal column, various internal organs and limbs. For example, described the syndrome of Gangart (Hanhart) - non-growth of the upper lip and palate, combined with a one- or two-sided defect of kidney development; Grauchan's syndrome is a combination of lip and palate non-development (dysphalangeia, polydactyly, six fingers), bladder, genitals, kidneys.

In addition, along with the defects of the lips or palate, children may have somatic and chronic infectious-allergic (hypotrophy, exudative diathesis, rickets, pneumonia, anemia, tubenchication, rheumatism, etc.), orthopedic (scoliosis, flatfoot, etc.), surgical (umbilical hernia, cryptorchidism, dropsy of the testicle), otorhinolaryngological (hearing loss), neuropsychiatric (neuroses, mental retardation, oligophrenia, epilepsy, deaf mute) diseases.

The most frequent malformations of internal organs in these children are the following: tetralogy of Fallot, open arterial (botallus) duct, fibroelastosis, stenosis of the pulmonary trunk, cryptorchidism, stenosis of the ureter, stenosis of the trachea, pyloric stenosis, additional anus, etc. All these circumstances explain the high up to 20%) the level of mortality of children with congenital defects of the lip and palate. Such children should be carefully and comprehensively surveyed. This is all the more necessary because in children the anomalies of the lip and palate lead to chronic respiratory insufficiency of the second degree, which causes an increase in the work of the respiratory system; the energy costs for this are covered by accelerating the metabolic processes and increasing the body's absorption of oxygen in 1 minute.

With insufficiently effective use of the respiratory surface of the lungs, the necessary rate of gas transport in the body is ensured not by accelerating blood flow and subsequent development of heart failure, but by producing erythrocytes with more hemoglobin content than usual and, consequently, greater ability to bind oxygen and carbon dioxide. The author believes that a slightly reduced level of erythrocytes in such patients (in all age periods) should primarily be associated with the features of respiratory function, rather than with elementary disorders, as was previously thought. The analysis of electrocardiograms of 122 children with non-afferent lips and palate showed the presence of significant changes in the heart: conduction, automatism, excitability, etc. Yu. A. Yusubov and E. S. Mehteev (1991) in 8 out of 56 noted prematurity; in all children aged 2.5-3 months, the propensity to respiratory diseases is increased against the background of a decrease in all indices of cellular immunity, which prompted authors to conduct a treatment before and after the operation with the help of immunomodulator-levamisole (2.5 mg per 1 kg of weight child at night for a week). And M. Pasechnik (1998), with the aim of correcting local tissue and general immunity and improving the results of plaque plastic surgery, recommends that the patient be sanitized before the operation, ingestion of sodium nucleate (at a dose of 0.01 g per 1 kg of the patient's mass 3 times a day after meals ) for 4-5 days, irradiation of the palate with helium-neon laser (at a dosage of 0.2 J / cm ² ) daily for 4-5 days, hydromassage of the palate 3-6 min (at P = 0.5 atm) for 4-5 days .

[6], [7], [8], [9]

Emergency dental orthopedic care and the duration of the operation

When determining the period of operative intervention, it is necessary to take into account the general condition of the child, the degree of expression of non-division of the lips, the state of tissues in the non-affection area, the degree to which the child has physiological functions, primarily breathing, sucking.

Important is the mental state of parents, especially the mother.

In addition, when choosing the duration of the operation and its methodology, it is necessary to take into account the possibility of post-operative scarring of the lip and the associated limitation of the rate of development of the maxilla. On the other hand, the surgeon should not forget that prolonged refusal of the operation can lead to the appearance of secondary deformations in the soft tissues of the face and jaws.

Early, as well as early operations in the conditions of the maternity hospital, i.e. In the first hours and days, are permissible for strictly limited (mainly, social) indications (only with partial one- and two-sided defects), only in full-term children in the absence of heavy congenital disorders from the central nervous system and circulatory organs, i.e., with the general satisfactory state of the newborn. In addition, the surgeon should observe the child in the next 5-8 years (at least) after the operation, providing or recommending the necessary complex therapy (orthodontic, orthopedic, speech therapy, surgical, etc.).

The experience of our clinic shows that the operations performed in the maternity hospital are technically perfectly correct and a very experienced surgeon give usually good results. However, on the basis of many years of surgical experience, we share the point of view of those authors who consider the age of 6-7 months to be optimal for carrying out cheiloplasty on the background of a noticeable increase in the child's body weight and positive blood counts (at least 120 g / l hemoglobin, 3.5x109 / L erythrocytes ), absence of concomitant diseases of bronchial tubes, lungs and other internal organs and systems and not earlier than a month after acute illnesses or preventive vaccinations. Two weeks before the operation, it is recommended to prescribe a complex of vitamins (C, B1, B2, P, PP) in therapeutic doses and desensitizing drugs.

If the operation is impossible in the conditions of the maternity hospital, it is necessary to calm the mother with all the measures (first of all showing the pictures of sick children before and after operations on the same occasion) , explaining to her that an effective operation will be performed a little later, and take care of the normalization of lactation in her as the need to feed the baby mainly mother's milk is dictated by three circumstances:

1. a very high percentage of deaths (about 30%) of children with congenital defects of the lip and palate;
2. occurrence of frequent bronchopulmonary complications due to technically incorrect feeding of the child, resulting in aspiration of food;
3. the fact that the best nutritional properties are mother's milk, and the transition to artificial nutrition threatens the child with hypotrophy, hypovitaminosis and other nutritional disorders.

Artificial feeding, often uncontrolled, chaotic, has a very negative effect on the child's physical and neuropsychological development. Therefore, it is necessary to achieve normalization of the psychoemotional status of the mother (especially the first one who gave birth), convincing her of the undeniable promise of surgical treatment to give the child cosmetic and social acceptability, and to teach her to breast-feed.

A newborn with a defect in the lip and palate should be provided with specialized help (on an emergency basis) by a team consisting of a maxillofacial surgeon, an orthopedic dentist and orthodontist, a nurse, and a dental technician. Even before the first feeding of the child, the team must produce an anterior plate, dissociating the nasal cavity and the oral cavity. If it is made, and the baby is full and the birth has passed safely, then with persistent attachment to the chest, he can learn how to suck.

Children with a one-sided, partial or complete, but isolated non-mutilation of the lip (i.e., not combined with a defect of the gums and palate) are recommended to apply to the breast in such a way that the uninflated nostril is pressed against the mammary gland. You can also breastfeed in a semi-sitting position; while the milk will drain on the tongue in the throat and not get into the nose.

With one- or two-sided, partial and complete, isolated non-division of the lips, the child adapts himself to suckling without much difficulty. In the case of a combination of a lip defect with a palate defect, the child usually "clogs" the defect with the tongue and thereby creates the necessary vacuum.

According to some authors, one should not operate a child until all the possibilities of feeding him with breast milk, even if expressed, are exhausted. If this is not possible, then with a complete non-incision of the lip, combined with non-incision of the alveolar process and palate, the use of various kinds of obturators and horns is recommended, guided, for example, by the methodical recommendations of the Ministry of Health of Ukraine "Peculiarities of feeding children of the first year of life with congenital non-abscesses of the upper lip and palate "Or the recommendations of T. V. Sharova and E. Yu. Simanovskaya (1991), who developed a technique for manufacturing preformed orthopedic devices for any kind of non-non-affection of the lips, gums, and palate.

If one of the known obturators can not be made and with its help to ensure the natural feeding of the baby, it is necessary to switch to feeding the expressed mother's or cow's milk with the help of any obturator horn, pipette, teaspoon or other devices. The best way is to make the horn-obturator VI Titarev, which is a rubber finger from a glove, connected to a rubber tube 25-30 cm long and fixed with a ribbon or a rubber ring at the neck of a graduated bottle with a pacifier. The nipple is injected into the mouth in such a way that the rubber finger is under the slit in the gum and palate. When the baby begins to suck, the mother blows air through the tube and immediately clamps her end (Mohr's clamp, a hemostatic clamp, etc.). The balloon-finger, filled with air, obturates the gap. According to the author, this device is effective when feeding children as with non-merger of the lip and palate, and with isolated non-separation of the palate only. It is also important that when feeding with this obturator, nasal passages are almost not contaminated with food, complications from the middle ear are prevented and food does not enter the respiratory tract, which is of great importance for the prevention of bronchopneumonia. The adaptation of VI Titarev is also convenient because it can be made not only by a doctor, but also by the mother herself.

With complete non-loss of the lips after each feeding, the child should be given tea or water to wash away the remains of milk and mucus, which sometimes linger in the nasal passages. It is also good to instill 3-4 drops of a furacilin solution 3-4 times a day to disinfect the nasal cavity and prevent rhinitis, eustachiitis, otitis and other complications.

[10]

Surgical treatment of congenital nonunions of the upper lip

There are over 60 methods of cheyloplasty and its modifications. Many of them have not been used for a long time, and in some ways the opinions of surgeons differ. Therefore, we will focus only on those methods and their modifications, which are used most often.