Congenital nonunions 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, deviation from which we associate with the idea of an unattractive or even ugly shape of the lips.

A normally developed upper lip has the following anatomical components:

1. filter (filtrum);
2. two columns (columellae);
3. red border;
4. median tubercle or proboscis;
5. Cupid's line (or arc) - this is the name of the line that separates the red border and the skin of the upper lip.

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

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Symptoms of congenital cleft lip

Symptoms of cleft lip depend on their type and number. The most severe disfigurement, difficulty in sucking the mother's breast, breathing problems, and subsequently pronunciation of sounds are observed with bilateral, especially complete, cleft lip of the upper lip.

Sometimes the nonunion, starting from the corner of the mouth, develops into a congenital cleft defect of the cheek, causing a picture of unilateral or bilateral macrostomia. Nonunion of the lip and cheek can extend to the lower eyelid, the infraorbital margin of the upper jaw, the superciliary arch and the entire frontal bone.

In 76.3% of cases of congenital non-union of the upper lip and palate, various deformations of the dental and jaw system are encountered, the elimination of which is an integral part of the complex treatment of patients. The most common type of dental and jaw deformation in congenital non-union of the lip and palate is narrowing of the upper jaw (60.7%).

According to A. N. Gubskaya, congenital or primary deformations include abnormal placement of teeth near the area of non-union, abnormal shapes of teeth and their roots, adentia, and supernumerary teeth.

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

The following defects develop before surgery:

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

They intensify as the child grows, his speech becomes more active, language develops more rapidly (macroglossia), etc.

After cheiloplasty the following may occur:

1. displacement of individual teeth or a group of them in the direction of the palate, their rotation 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, patients with congenital non-unions of the upper lip and palate experience functional changes in the masticatory apparatus, which are manifested by a decrease in the strength of the lip muscles, chewing efficiency, and atypical reflex chewing movements of the lower jaw.

Classification of congenital clefts of the upper lip

According to clinical and anatomical features, congenital defects of the upper lip are divided into several groups:

1. In the transverse plane, non-unions of the upper lip are divided into lateral - unilateral (accounting for about 82%), bilateral (about 17%) - and median, dividing the lip into two symmetrical parts (about 1%).
2. In the vertical plane, they are divided into partial (when the non-union has spread only to the red border, or simultaneously with the red border there is a non-union of the lower part of the skin of the lip) and complete - within the entire height of the lip, as a result of which the wing of the nose is usually turned out due to non-union of the base of the nostril.

I. M. Got and O. M. Masna (1995) found that the sizes of non-unions (wing of the nose

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

The sizes of the nasal openings on the side of non-union and the healthy side differ significantly: up to 14 and 8 mm, respectively. In case of bilateral non-union, the sizes of the defects on each side are smaller than in case of unilateral ones. The same applies to the sizes of the nasal defects. All these facts are very useful both for substantiating the choice of delayed surgical intervention and for preoperative treatment (orthopedic, orthodontic, speech therapy).

3. Depending on the depth of tissue non-union, a distinction is made between:
   • obvious - non-fusion of all layers of the lip (red border, mucous membrane, skin and muscle layer);
   • hidden - non-union of only the muscular layer of the lip, while the skin layer is somewhat thinner;
   • combined - non-fusion of the lip, extending to the gum or palate, cheek (coloboma of the face), eyelids and superciliary arches.

In bilateral non-unions of the upper lip, their length in height and depth may vary (for example, on one side - complete non-union of the lip fragments, extending to the alveolar process and palate, and on the other - only hidden non-union of the muscular layer within the red border and slightly above the Cupid's line). Bilateral complete non-union of the lip in some 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 fused with the tip of the nose, extremely disfiguring 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 nasal septum 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 nasal septum consists of cartilage.

Non-fusion of the lip and palate may be combined with anomalies of the cranial part of the skull, auricles, tongue (macroglossia), chest, spine, various internal organs and limbs. For example, Hanhart syndrome has been described - non-fusion of the upper lip and palate, combined with unilateral or bilateral developmental defect of the kidneys; Grauchan syndrome - a combination of non-fusion of the lip and palate with underdevelopment of the hand (dysphalangy, polydactyly, six-fingered), bladder, genitals, kidneys.

In addition, along with defects of the lip or palate, children may experience somatic and chronic infectious-allergic (hypotrophy, exudative diathesis, rickets, pneumonia, anemia, tuberculosis intoxication, rheumatism, etc.), orthopedic (scoliosis, flat feet, etc.), surgical (umbilical hernia, cryptorchidism, hydrocele), otolaryngological (hearing loss), neuropsychiatric (neuroses, mental retardation, oligophrenia, epilepsy, deaf-muteness) diseases.

The most common malformations of internal organs in such children are the following: tetralogy of Fallot, patent arterial (Botallo's) duct, fibroelastosis, pulmonary trunk orifice stenosis, cryptorchidism, ureteral stenosis, tracheal stenosis, pyloric stenosis, accessory anus, etc. All these circumstances explain the high (up to 20%) mortality rate of children with congenital defects of the lip and palate. Such children need to be examined thoroughly and comprehensively. This is all the more necessary because in children, lip and palate anomalies lead to chronic respiratory failure of the second degree, which causes increased work of the respiratory organs; energy costs for this are covered by accelerating metabolic processes and increasing oxygen absorption by the body per 1 minute.

With insufficiently effective use of the respiratory surface of the lungs, the required rate of gas transport in the body is ensured not by accelerating the blood flow with the subsequent development of heart failure, but by producing erythrocytes with a higher than normal hemoglobin content, and therefore a greater ability to bind oxygen and carbon dioxide. The author believes that a slightly reduced level of erythrocytes in such patients (at all ages) should be primarily associated with the peculiarities of the respiratory function, and not with elementary disorders, as was previously thought. An analysis of electrocardiograms of 122 children with cleft lip and palate showed that they also had significant changes in the heart: conduction disorders, automatism, excitability, etc. Yu. A. Yusubov and E. S. Mekhteyev (1991) found prematurity in 8 out of 56; All children aged 2.5-3 months have an increased tendency to respiratory diseases against the background of decreased all indices of cellular immunity, which prompted the authors to conduct a course of treatment with the immunomodulator levamisole (2.5 mg per 1 kg of the child's weight at night for a week) before and after surgery in order to stimulate cellular immunity. A. M. Pasechnik (1998), in order to correct local tissue and general immunity and improve the results of palate plastic surgery, recommends pre-operative sanitation of the patient's oral cavity, oral administration of sodium nucleinate (at a dose of 0.01 g per 1 kg of the patient's weight 3 times a day after meals) for 4-5 days, irradiation of the palate with a helium-neon laser (at a dosage of 0.2 J/cm ² ) daily for 4-5 days, and hydromassage of the palate for 3-6 min (at P = 0.5 atm) for 4-5 days.

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Emergency dental and orthopedic care and the duration of the operation

When determining the timing of surgical intervention, it is necessary to take into account the general condition of the child, the degree of severity of the non-union of the lip, the condition of the tissues in the area of non-union, the degree of impairment of the child's physiological functions, primarily breathing and sucking.

The mental state of the parents, especially the mother, is of no small importance.

In addition, when choosing the time of the operation and its method, it is necessary to take into account the possibility of postoperative scarring of the lip and the associated limitation of the rate of development of the upper jaw. On the other hand, the surgeon should not forget that a long-term refusal of the operation can lead to the appearance of secondary deformations in the soft tissues of the face and jaws.

Early and very early operations in a maternity hospital, i.e. in the first hours and days, are allowed for strictly limited (mainly social) indications (only for partial unilateral and bilateral defects), only in full-term infants in the absence of severe congenital disorders of the central nervous system and circulatory organs, i.e. in the general satisfactory condition of the newborn. In addition, the surgeon must monitor the child for at least 5-8 years after the operation, providing or recommending the necessary complex therapy (orthodontic, orthopedic, speech therapy, surgical, etc.).

Our clinic's experience shows that operations performed in a maternity hospital technically absolutely correctly and by a very experienced surgeon usually give good results. However, based on many years of surgical experience, we share the point of view of those authors who consider the most optimal age for cheiloplasty to be 6-7 months, against 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), the absence of concomitant diseases of the bronchi, lungs and other internal organs and systems and not earlier than a month after acute diseases 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 a maternity hospital, it is necessary to use all possible means (primarily showing photographs of sick children before and after operations for the same reason) to calm the mother, explain to her that an effective operation will be performed a little later, and take care to normalize her lactation, since the need to feed the child mainly with mother's milk is dictated by three circumstances:

1. a very high mortality rate (about 30%) of children with congenital defects of the lip and palate;
2. the occurrence of frequent bronchopulmonary complications due to technically incorrect feeding of the child, leading to aspiration of food;
3. the fact that mother's milk has the best nutritional properties, and the transition to artificial feeding threatens the child with malnutrition, hypovitaminosis and other alimentary disorders.

Artificial feeding, often uncontrolled and chaotic, has a very negative effect on the physical and neuropsychic development of the child. Therefore, it is necessary to achieve the normalization of the psycho-emotional status of the mother (especially the first time), convincing her of the undoubted prospects of surgical treatment to give the child cosmetic beauty and social acceptability, and teach her to breastfeed the child.

A newborn with a defect of the lip and palate should be provided with specialized care (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 should make a preformed plate separating the nasal cavity and the oral cavity. If it is made, and the child is full-term and the birth was successful, then with persistent attachment to the breast, he can learn to suck.

Children with unilateral, partial or complete, but isolated cleft lip (i.e. not combined with a defect of the gum and palate) are recommended to be put to the breast in such a way that the cleft nostril is pressed against the mammary gland. You can also breastfeed the child in a semi-sitting position; in this case, the milk will flow down the tongue into the throat and not get into the nose.

In case of unilateral or bilateral, partial or complete, isolated non-union of the lip, the child adapts to sucking without much difficulty. In cases of a combination of a lip defect and a palate defect, the child usually "plugs" the defect with the tongue and thus creates the necessary vacuum.

According to some authors, a child should not be operated on until all possibilities of feeding him with mother's milk, even expressed, have been exhausted. If this fails, then in case of complete non-union of the lip, combined with non-union of the alveolar process and palate, it is recommended to use various types of obturators and horns, guided, for example, by the methodological recommendations of the Ministry of Health of Ukraine "Features of feeding children of the first year of life with congenital non-union of the upper lip and palate" or the recommendations of T. V. Sharova and E. Yu. Simanovskaya (1991), who developed a method for manufacturing preformed orthopedic devices for any type of non-union of the lip, gums, palate.

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

In case of complete non-union of the lip, after each feeding the child should be given tea or water to wash away the remnants of milk and mucus, which sometimes linger in the nasal passages. It is also good to instill 3-4 drops of furacilin solution into the nose 3-4 times a day to disinfect the nasal cavity and prevent rhinitis, eustachitis, otitis and other complications.

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Surgical treatment of congenital cleft lip

There are over 60 methods of cheiloplasty and its modifications. Many of them have not been used for a long time, and surgeons have different opinions regarding some methods. Therefore, we will focus only on those methods and their modifications that are used most often.