A neonatologist will tell you about the most important reasons for the development of a cleft lip

Cleft lip - symptoms and treatment

A multidisciplinary team is involved in the treatment of patients with clefts, since even a well-performed operation does not guarantee the absence of other complaints. Team members: pediatrician, dentist, orthodontist, plastic or maxillofacial surgeon, nutritionist, speech therapist, ENT, ophthalmologist and neuropsychiatrist. The ENT doctor works to prevent concomitant pathology of the middle ear, which often develops with clefts, and, if present, treats it. Orthodontists make orthodontic structures (obturators), thanks to which the child can eat normally. Obturators are prosthetic devices that close a cleft and can be used for children of all age groups.

Dentists Hubener D.V. and Liu J.R. classified means for orthopedic treatment of the cleft into preoperative and postoperative, active and passive, extraoral and intraoral [14]. There was a long debate about how these methods affect the growth of facial bones and whether they worsen the prognosis, until naso-alveolar plates were invented, which were made first of acrylic, then of rubber. The plates closed the cleft, and the child could suck normally; in addition, the pressure of the plate helped support the wings of the deformed nose, improved the ratio of the lengths of the columella, the cleft parts of the lip, and gently moved the triangular bone [11].

Many types of surgical treatment have been described for the treatment of cleft lip and palate, although the possibility of performing such operations has arisen relatively recently.

Since early times, people have tried to find a way to get rid of this defect. They tried to connect the crevice with sharp sticks, then they used metal needles that were wrapped with thread. Due to the lack of means for pain relief, the operations were primitive and ineffective. Since the 16th century, they have tried to use special caps for external impact on the cleft, trying to cause the edges to stick together. The protruded premaxillary bone in the case of bilateral clefts was simply cut off, which most often led to worsening deformation of the midface.

Surgery to correct a cleft lip is called cheiloplasty . With the beginning of surgical corrections, cheiloplasty methods began to change and improve. The first operations were perceived as a miracle; they created a linear incision. Over time, they began to make incisions of increasingly complex configurations in order to correct associated deformities and prevent the appearance of new ones. Now, in order to correct the complex deformity that occurs in the midface area and create conditions for the normal development of the facial part of the skull, it is necessary that the operation fulfill the following conditions:

• Was safe for young children.
• I corrected the maximum in each specific case in 1 stage.
• Formed the correct line of the red border and Cupid's bow.
• Correct formation of the filtrum is desirable.
• Provided sufficient depth of the vestibule of the oral cavity.
• It eliminated improper attachment of the fibers of the orbicularis oris muscle and created a continuous layer of muscle, which will help in the correct formation of the bones of the facial skull.
• Lengthened the short medial part of the cleft lip.
• Corrected the shape and position of the nasal cartilage.

In 1938 A.A. Limberg described and in 1952 expanded the method of unilateral cheiloplasty. This method became the leading method in the USSR for a long time, because in most cases it made it possible to correct the concomitant deformation of the nasal wing on the cleft side.

In 1952, Tennyson C.W. in the USA and in 1955 Obukhova L.M. in the USSR they described a cheiloplasty method of a more complex configuration. The method is still used today; in addition, cheilorhinoplasties have been developed on its basis to reduce the likelihood of developing associated deformities. The techniques are interesting primarily for their historical significance: for the first time, complex-shaped incisions began to be made and the lip was lengthened. However, lengthening the lip on the cleft side was also a limiting factor because it itself created a recognizable deformity. Techniques are now being used to solve this problem.

The most used technique for suturing unilateral clefts in the world now is the Millard technique and its modifications. In our country this is a modification of I.A. Kozina.

Millard cheiloplasty involves simultaneous restoration of the continuity of the upper lip and correction of the nasal cartilage. On the medial fragment of the upper lip, point 1 is marked - the center of the cupid's bow. On the healthy half of Cupid's bow, look for limiting point 2 (apex of the arc), located approximately 2-4 mm from the midline. This distance is laid on a white roller on the shortened side, obtaining point 3. The length of the lateral fragment of the lip on the healthy side is measured between the limiting point 2 and the commissure of the mouth (point 6). The resulting distance (approximately 20 mm) is laid on a white roller on the damaged side of the commissure (point 7) and a limiting point 8 is obtained. Point 8 will be the top of the cupid's bow on this side; it should connect to point 3 on the medial segment. Measure the distance from point 2 to the base of the nasal wing of the healthy side (point 4), the same distance is set aside from point 8 to the base of the nasal wing of the affected side (point 10). On the shortened side, from the middle of the base of the columella (point 5) to point 3, an arcuate rotational incision is made, symmetrical to the arc of the philtrum on the healthy side. If necessary, extend the incision to point x (reverse incision towards the healthy philtrum). On the lateral (side) fragment, according to the length of the section x-5-3, point 9 is found medially from the base of the flattened wing of the nose. Modifications are mainly used to expand the intervention to the nose, with secondary cheiloplasty, hidden and incomplete clefts.

The advantages of this method of primary cheiloplasty:

• maximum tissue preservation;
• moving all elements of the upper lip to the correct position;
• formation of the bottom of the vestibule of the nasal cavity and the deep vestibule of the mouth.

The method simultaneously corrects the position of the deformed wing of the nose, and some modifications are supplemented with one-stage rhinoplasty. In addition, the arcuate scar is hardly noticeable and can be used for corrective cheiloplasty in adulthood.

Closure of a bilateral cleft can be one-stage or two-stage. This depends on the volume of the cleft, the experience and confidence of the surgeon in his techniques. Each option has its pros and cons, but it is believed that with the help of one-step closure of the cleft, it is more likely to fully create a sphincter of the orbicularis oris muscle.

Sometimes it is practiced to connect the edges of the cleft. This is not a full-fledged cheiloplasty, but only the transfer of a complete cleft into an incomplete one in the expectation that the pressure of the connected part of the lip will have an orthopedic effect on the edges of the cleft, which will lead to their convergence. In most Western countries, the Millard method of cheiloplasty remains the leading one [12].

With a two-stage closure, one side of the cleft is first closed using any method, and after some time the other side is closed.

The debate about at what age to perform surgery still continues to this day. There are those who urge treatment to be carried out as early as possible. Even operations on the fetus are offered, due to the lack of scarring in the prenatal period. Many insist on surgery in the first days and even hours of life. On the one hand, there are advantages to this (parents are not afraid of the child’s appearance, there are no intra-family conflicts, there are no problems with nutrition), but there are also disadvantages (imperfect thermoregulation of newborns, high sensitivity to blood loss, very small anatomical basis). In the 60s of the last century, a large-scale analysis of the results of operations performed in the maternity hospital in the first days of life was carried out. The result showed in almost all children much worse results than in patients who were operated on as adults [1]. N.M. Mikhelson, in one of the oldest Russian manuals on plastic surgery, advises performing the operation at the age of 5-6 months, when the child is sufficiently strong and developed. He believed that cosmetic success was easier to achieve during this period. It is also described that such operations should be performed under local anesthesia, due to the danger of anesthesia [10]. Now there are safe drugs for anesthesia, so it is difficult to imagine performing an operation on young children under local anesthesia. Most surgeons believe that primary cheiloplasty is indicated at the age of 6-12 months.

The extent of the operation depends on the degree of the cleft. Many modern authors believe that in case of a complete cleft, primary cheiloplasty (when only the cleft is sutured) should be used in exceptional cases, since it does not correct the condition of the nose. Therefore, now the primary rhinocheiloplasty technique should be used, when, together with suturing the cleft, the position of the alar cartilages, septum, and vomer is corrected. The quality of the primary operation should be maximum, since repeated corrections are more difficult to perform in conditions of soft tissue deficiency and extensive scarring [4].

Complications during surgery

When performing surgery, the danger is aspiration (inhalation by the patient) of blood and mucus, which can cause asphyxia. To prevent this, you should prevent bleeding and carefully suck out the contents of the oral cavity (blood, saliva, mucus). Possible complications also include collapse, shock, laryngospasm and other conditions.

Mortality on the operating table and shortly after surgery is usually associated with improper selection of patients. You cannot operate on malnourished and premature children, those who, even before surgery, suffer from inflammatory conditions of the respiratory system, hearing, and decompensated abnormalities in the development of the brain, heart, and blood vessels. Also, surgery is prohibited in the presence of thymic-lymphatic status (enlarged thymus). The possibility of surgery should be decided together with the pediatrician.

Complications after surgery

In the coming days after surgery, stenosis (narrowing) of the larynx, bronchitis, pneumonia, urinary retention and other somatic complications may occur, the prevention and treatment of which should be carried out with the participation of a pediatrician. The most common local early complication is suture dehiscence, which is usually associated with technical errors in the operation:

• excision of a significant amount of tissue in the area of ​​​​the edges of the cleft;
• insufficient tissue preparation;
• incorrect layer-by-layer suturing of the wound;
• tissue tension in the wound area, inflammation and swelling in the operation area.

Sometimes the sutures come apart due to injury (a child’s fall, an accidental blow to a hard object), infection, or the child’s illness with ARVI, rhinitis and other inflammatory pathologies. If the sutures diverge, the issue of applying secondary sutures is decided according to the patient’s condition.

When examining a patient who has undergone surgery, the height of the upper lip, the degree of its deformation, the depth of the vestibule of the mouth and its tightness (the presence of oronasal anastomoses and palate defects) are checked. You should pay attention to lip mobility, phonation, and changes in the structure of the dental system. The integrity of the orbicularis oris muscle can be tested by having the patient stretch his lips into a smile and purse his lips into a tube [1].

Severe psychological trauma is caused by scar changes after an unsuccessful first operation. One of the most unpleasant long-term complications of cheilorhinoplasty is deformation of the upper lip, nose and upper jaw, which can occur for various reasons [9].

After surgery, the patient may have a lack of soft tissue of the upper lip, its lengthening or shortening, stiffness due to extensive scarring, or an aesthetic defect. Alveolar cleft leads to the formation of crowding of teeth, the growth of dystopic (improperly positioned) and supernumerary (extra) teeth, which can support inflammation or, due to their location, form an oronasal fistula (an opening between the oral cavity and the nasal cavity). In general, such a fistula can also be due to excessive tension, cutting sutures, a defect in suturing, etc. Through it, the discharge of liquid part of food into the nasal cavity can persist, which in turn helps to maintain chronic rhinitis, sinusitis, etc.

Possible insufficiency of the muscle ring of the orbicularis oris muscle, which develops with defects in muscle alignment or insufficient separation of muscle fibers from the pyriform opening. Deformation of the vestibule of the mouth in the form of its flattening complicates further orthodontic treatment, which is so necessary for patients, and aggravates the retrusion of the midface (backward displacement). Deformations of the external nose and septum lead to a significant deterioration in the aesthetic result and disruption of nasal breathing, and breathing through the mouth in such patients aggravates the deformation of the facial skeleton.

“Why did this happen to me?!”

Dmitry Yuryevich Komelyagin
- Dmitry Yuryevich, there is a myth that facial clefts are a problem for children from disadvantaged families.

- No! We have many families with prosperous, beautiful parents whose children are faced with such a problem. The risk factor, rather, will be the fact that the parent himself was a carrier of this pathology in childhood. We have such families. Let's say we once operated on a girl with a cleft lip, she got married and gave birth to a child with exactly the same pathology, they came to us again. We had families in which the second and even third child was born with a similar pathology. And we successfully operated on all of them.

– How many years have you been operating?

– The department was founded 26 years ago. We have been operating for 26 years now. Facial clefts are a multifactorial problem, and it is still difficult to identify specific causes. It is known that the cause may be intrauterine fetal hypoxia. What caused this hypoxia? Anything. Stress? Maybe. Mother's infection? Taking medications? Yes, it's possible. But no one can tell you what exactly.

Today, the most substantiated theory is considered to be that the cause of facial clefts is a combination of hereditary factors and adverse environmental influences.

Mutation of several genes entails increased sensitivity to bacteria in the environment. In this case, the cleft may be caused by factors such as medications, x-rays, infections, and even stress and noise. As a result of their exposure, the fetus experiences a lack of oxygen during the few hours it takes for the face to form, and then the fused tissues suffer.

Classification of the disease

A cleft lip can be:

A cleft lip can be on one upper lip, one lower lip, or both.

The classification is used when choosing a method of surgical correction.

Causes of illnesses

Doctors believe that the reasons why cleft palate and cleft lip appear are:

• smoking by the expectant mother before and during pregnancy (scientists consider smoking to be the main cause of maxillofacial anomalies in the fetus);
• taking even small doses of alcohol in the first months of carrying a child;
• addiction;
• taking certain medications can cause many congenital anomalies in children;
• bad ecology;
• harmful working conditions;
• folic acid deficiency in the first months of pregnancy;
• long-term toxicosis;
• excess weight;
• the age of the woman (after 35 the number of mutations increases sharply);
• hereditary factor;
• mechanical injuries to the abdominal cavity of a pregnant woman;
• severe nervous shock in the 1st trimester.

Diagnostics

It is possible to recognize signs of a cleft lip in the prenatal period using ultrasound examination as early as approximately 14 weeks of pregnancy. But in order to definitively confirm the diagnosis, you will need to organize a medical consultation.

This is necessary, since this anomaly is an indication for termination of pregnancy. Undoubtedly, in this case, the decision is made only by the pregnant woman herself, but at the legislative level, in the presence of a congenital pathology, abortions for medical reasons at this stage are allowed (in other cases, for a period of more than 12 weeks, artificial termination of pregnancy is already prohibited).

It is not difficult to make a diagnosis after birth; this requires only a visual examination of the newborn baby by a doctor.

Such a child will require a mandatory consultation with an otolaryngologist (ENT). This is necessary to determine if there are any other abnormalities (for example, abnormalities in the structure of the nasal cavity or cleft palate).

How dangerous is the disease?

If a child is born with only a cleft lip, but the hard palate remains intact, this leads to the following disorders:

• under the age of one year, when the child eats only liquid food, it is difficult for him to suck and swallow; food can enter the nasal cavity, and this requires special tricks when feeding, sometimes even placing a probe - a tube that will lead from the nose to the stomach;
• If it is not possible to perform surgery when teeth begin to form, this may lead to the absence of necessary teeth or the appearance of additional teeth;
• teeth begin to grow at incorrect angles - the bite is disturbed. This affects both the chewing of food and, accordingly, its digestion worsens, and caries often occurs;
• the process of sound formation is disrupted: the sound wave, entering the nasal cavity, makes the voice nasal and speech unclear, with problems in pronouncing consonants;
• hearing problems arise;
• often there are otitis media;
• a cleft lip, even a mild one, is a significant cosmetic defect, which will make it difficult for a child to adapt to children’s society

That is why a cleft lip, even if it is not very pronounced, must be operated on. And this needs to be done before one year in order to have time to undergo the necessary rehabilitation measures before the development of speech and before the child’s socialization begins.