Cleft palate (cleft palate) in children and dental health


Causes of development of cleft palate in the fetus

The list of factors that can provoke this pathology is quite wide. Among them are both related to the expectant mother and external:

  • genetic predisposition;
  • toxicosis;
  • exposure to bacteria and viruses;
  • chronic diseases of the mother (gynecological, respiratory organs);
  • infectious diseases of the mother (herpes, sore throat, influenza, otitis and bronchitis);
  • smoking and alcohol abuse during pregnancy;
  • taking medications (antibiotics, salicylates, sulfonamides);
  • work in hazardous production;
  • stress;
  • sudden changes in atmospheric pressure;
  • radiation, including solar;
  • environmental violations [2, 3].

Environmental influence is last on the list, but not least. As a result of rapid technogenic development, the state of the environment is steadily deteriorating. Largely because of this, the percentage of newborns with cleft palates has tripled over the past 100 years [4].

Causes of facial malformations

Cleft lip and palate belong to a group of multifactorial malformations that occur against the background of hereditary predisposition, as a result of gene mutations, and also under the influence of environmental factors in the first 12 weeks of intrauterine development. Thus, the causes of pathology can be:

  • Intrauterine infections.
  • Physical effects (radiation).
  • Chemicals (aniline dyes, etc.).
  • Smoking, alcohol, drugs.
  • The age of the parents is over 35-40 years.
  • The likelihood of a child having a cleft lip and palate increases if another child or parent had the pathology.

Treatment of children with congenital cleft lips and palates, with dentoalveolar deformities and anomalies is one of the most difficult tasks of modern maxillofacial surgery and surgical dentistry. Surgical treatment of such pathologies allows you to restore speech functions, restore the aesthetic appearance of the nose and lips and ensure the normal development of the facial skeleton. During the treatment of facial malformations, a maxillofacial (plastic) surgeon, an orthodontist, a speech therapist, an otolaryngologist, a pediatrician, a neuropsychiatrist, and a geneticist are involved.

The maxillofacial (plastic) surgeon determines the timing, volume and sequence of surgical interventions. In a number of children, the question of the order and extent of surgical intervention is decided by the surgeon together with the orthodontist.

The orthodontist controls the development of the upper jaw, ensuring harmony of size and relationship of the dental arches. Preoperative orthodontic treatment with expansion plates with screws, vestibular arches and protraction springs creates favorable conditions for palate reconstruction.

A speech therapist deals with correct speech development in patients with cleft palate. Starting from the age of two, a specialist carries out advisory work and uses various techniques to regulate the child’s external breathing, practicing gradual and prolonged exhalation.

An otorhinolaryngologist systematically observes children with cleft palates, since the connection between the oral cavity and the nasal cavity, disruption of the structure and function of the velopharyngeal ring leads to the development of chronic diseases of the ENT organs in this group of patients 10 times more often than in children with a normal palate.

A pediatrician is a specialist who, from the moment of birth, must systematically monitor his general physical development. The doctor also participates in the joint preparation of the patient for surgery and monitors the postoperative care of the child.

A neuropsychiatrist should examine children with cleft lip and palate to determine concomitant malformations of the central nervous system and, if any, monitor the child’s mental development. Poorly developed speech in children with congenital cleft palate impedes normal mental development, interferes with communication with others, and can cause a feeling of inferiority and difficult experiences about this. Such patients with speech and appearance disorders require psychotherapy for social rehabilitation in society, in addition to surgical treatment.

A geneticist provides advisory assistance in diagnosing forms of hereditary diseases, a sign of which is the presence of a congenital cleft lip and palate, determines the type of hereditary transmission and determines the degree of risk of having sick children, giving appropriate recommendations.

Treatment of children with congenital cleft lip and palate should be carried out within the framework of a comprehensive program with the involvement of all necessary specialists, whose joint efforts make it possible to achieve good aesthetic and functional results, complete social rehabilitation of patients.

Surgical treatment of cleft lip and palate is carried out by Sergey Nikolaevich Bessonov, Doctor of Medical Sciences, maxillofacial surgeon and Levan Avtandilovich Eremeyshvili, maxillofacial surgeon.

You can make an appointment with a specialist by phone: 8 on weekdays from 9.00 to 19.00 Moscow time Or through the form on the website Sign up for a free consultation

Classification of defects

Based on their shape and anatomical features, congenital clefts are divided into several types and subtypes. First of all, they focus on localization:

  • clefts that affect only the soft palate;
  • clefts affecting both the hard and soft palates;
  • clefts of the soft, hard palate and alveolar process;
  • clefts of the alveolar process and the anterior part of the hard palate.

Then the degree of expression is assessed.

  • Hidden: located within the muscle tissue of the soft palate and the bone tissue of the hard palate, without extending to the mucous membrane.
  • Incomplete: the palate is partially cut.
  • Full: completely cut through the palate.

They also describe whether the defect appears on one or both sides of the face. As a result, the classification takes the following form:

  • clefts of the soft palate can be hidden, incomplete or complete;
  • the same varieties in clefts of the soft and hard palate;
  • clefts of the soft, hard palate and alveolar process are always complete and can be one- or two-sided;
  • clefts of the alveolar process and the anterior part of the hard palate can be complete or incomplete, in both cases - unilateral or bilateral [4].

Surgical treatment of cleft palate

Maxillofacial surgeons perform the first simple operation in infancy - usually before 2 years. We are talking about eliminating a defect in the soft palate, or veloplasty. Thanks to it, blood supply to tissues and development of the muscles of the soft palate improves. Moreover, even the bone plates of the hard palate also begin to grow after surgery [2].

However, veloplasty is sufficient only for cleft soft palate - the mildest form of pathology. If the cleft palate also affects the hard palate, the main stage of treatment is a much more complex operation - the so-called uranoplasty. Such interventions have a history of almost 200 years, and during this time several effective techniques have been developed. Essentially, they all boil down to three main tasks:

  1. close the cleft palate;
  2. lengthen the soft palate;
  3. narrow the middle part of the pharynx.

The main differences lie in the surgical protocol: it can be radical, when all corrections are performed in one intervention, or gentle, in which the surgeon’s actions are divided into two stages [4].

Gentle uranoplasty was developed primarily in order to perform operations as early as possible. Therefore, it is faster to return the child to normal life and begin rehabilitation. However, there is still no consensus among specialists about the optimal period for surgical intervention. Some doctors believe that it is best to eliminate the cleft at 2–4 years of age. Others, on the contrary, argue that it is not advisable to operate before 4 years of age [4].

Regardless of the operation protocol, preparation for it plays the most important role. Uranoplasty is a complex intervention (including when performed in childhood), and to minimize risks, all actions are carried out with special care:

  • restorative treatment;
  • sanitation of the oral cavity and pharynx;
  • examination of all organs and systems of the body;
  • swab from the throat and nose for pathogenic microflora;
  • testing the protective palatal plate and, if necessary, correcting deficiencies;
  • preoperative speech therapy training, which greatly facilitates the work of a speech therapist after uranoplasty [4].

The maxillofacial surgeon is the main coordinator in the rehabilitation of the child. Then ENT doctors, orthopedists, dentists , audiologists, and neonatologists (in the early stages) are involved, who guide the child according to the specifics of the corresponding pathologies.
Then we get team play and an integrated approach to rehabilitation. Grichanyuk D. A., Ph.D., Associate Professor of the Department of Maxillofacial Surgery of BelMAPO, member of the American and European Association of Craniomaxillofacial Surgeons [1]

Cleft lip and palate as predictors of EEC syndrome: clinical observation

Summary. Cleft lip and palate are one of the most common congenital facial pathologies and rank third in the structure of all congenital malformations. The isolated form of this pathology occurs in 7.6-41.4% of cases, as part of symptom complexes of cleft lip and/or palate - in 21.1-61.2% of cases. Cleft lip and palate may be predictors of ectodermal dysplasia, ectrodactyly, cleft lip and palate syndrome (EEC syndrome). It is a rare autosomal dominant disorder with high penetrance. During the newborn period, diagnosis can be difficult, since a number of signs (lack of hair and teeth) are the age norm. Significant assistance in the early diagnosis of EEC syndrome can be provided by identifying cleft lip and palate in combination with pathology of the distal extremities, especially at the stage of prenatal diagnosis. Most often, this defect can be identified at 16-30 weeks. Despite this, the diagnosis of EEC syndrome is quite rare; the condition is more often described as multiple malformations. The article presents clinical cases of diagnosing EEC syndrome in two children from the same family and describes the phenotypic characteristics of the patients. It is emphasized that multiple malformations of cleft lip and palate in combination with pathology of the distal extremities can become predictors of EEC syndrome. The great importance of early diagnosis of EEC syndrome in drawing up a correct treatment plan based on the pathogenesis of the disease is pointed out. Treatment of individual clinical manifestations included in the EEC syndrome (cleft lip and palate, dermatitis, adentia, xerostomia, lacrimal atresia), without combining them into a single nosological form, can be lengthy, expensive and unsuccessful. Detection of pathology in parents who are carriers of EEC syndrome makes it possible to predict the birth of children with this syndrome. The method of choice is the use of assisted reproductive technologies, for example, the use of donor eggs or sperm.

Congenital cleft lip and palate (CCLP) is one of the most common congenital facial pathologies and ranks third in the structure of all congenital malformations [1]. The isolated form of this pathology occurs in 7.6-41.4% of cases, as part of symptom complexes of cleft lip and/or palate - in 21.1-61.2% of cases [2]. Cleft lip and palate represent a clinically and genetically complex group of diseases, including syndromic, monogenic, chromosomal, multifactorial and teratogenic forms [3]. The W. McKusick catalog of human Mendelian traits (OMIM) contains more than 700 syndromes and conditions that are accompanied by CGN and have an established or presumed Mendelian pattern of inheritance. Rare congenital anomalies accompanied by CGN include EEC syndrome. EEC syndrome (ectrodactyly, ectodermal dysplasia and clefting syndrome) is a genetic syndrome usually manifested by a triad of symptoms: ectodermal dysplasia, ectrodactyly of the limbs, cleft lip and palate. The disease has an autosomal dominant type of inheritance and is characterized by high penetrance, reaching 93-98% [4]. Information about variants of EEC syndrome is varied. Previously, three genetic variants were identified - EEC1, EEC2 and EEC3, the latter occurring in the vast majority of patients. The EEC1 and EEC2 syndrome genes are mapped to the long arm of chromosome 7 (7q11.2-q21.3) and chromosome 19, respectively. The EEC3 syndrome gene is localized on the long arm of chromosome 3 (3q27) [5]. According to other authors, the previously described localization of the gene encoding EEC2 is considered erroneous and there are only 2 forms: EEC1 and EEC3 [5]. Clinically, all genetic forms of EEC syndrome do not differ from each other. Ultrasound diagnostics plays a huge role in identifying EEC syndrome in the fetus. Prenatal diagnosis of congenital malformations is currently quite accurate, but the detection of EEC syndrome has some peculiarities. On the one hand, facial clefts and ectrodactyly are clearly visualized by ultrasound. On the other hand, the rarity of this pathology in a doctor’s practice does not allow one to confidently establish this diagnosis; “multiple congenital malformations” are more often described. Generally, the timing of diagnosis is 16-30 weeks of pregnancy [6]. The use of new ultrasound technologies in 3D/4D mode provides great assistance in diagnosing facial clefts and associated defects [7]. Making a clinical diagnosis of EEC syndrome in newborns can also be challenging. Manifestations of ectodermal dysplasia, such as lack of hair and teeth, are the age norm in newborns. Congenital pathology of the hand does not always manifest itself in the form of a severe defect (split hands, absence of fingers); cases of syndactyly, polydactyly, and deformation of fingers have been described. In the practice of maxillofacial surgeons and dentists, manifestations of EEC syndrome occur in the form of VRGN in 80% of cases. There is also oligodentia, adentia as a consequence of a violation of the morphogenesis of dental follicle tissues [8, 9]. Patients with hereditary EEC syndrome are extremely rare; according to Doctor of Medical Sciences, Professor A. A. Mamedov, over more than 50 years of work, 15 children with CGN as part of the hereditary EEC syndrome have been identified. We present clinical examples of two patients with hereditary EEC syndrome who are being treated at the Department of Pediatric Dentistry and Orthodontics of Sechenov University.

The purpose of this work was to improve the quality of treatment of patients with CGN through early detection of EEC syndrome. Below are clinical examples of the treatment of 2 patients with hereditary EEC syndrome.

Clinical example No. 1

Patient T., 25 days of life, was hospitalized as planned in the neonatal department of the Children's City Clinical Hospital No. 9 named after. N. G. Speransky, Moscow. Upon admission, he was examined by a neonatologist, an oral and maxillofacial surgeon, and the diagnosis of bilateral cleft lip, alveolar process, hard and soft palate was confirmed (Fig. 1, 2). The diagnosis of congenital facial defects was made prenatally, at the 20th week of pregnancy using an ultrasound scan. From the anamnesis it is known that the child was born from a second pregnancy, a second term birth. Birth weight – 3000 g, height – 52 cm, Apgar score – 8/8 points. The peculiarities of the newborn's appearance were noteworthy: dry skin, sparse eyelashes, unusual hair structure (“doll hair”). When examining the limbs, syndactyly of the II, III, IV fingers of the left hand, syndactyly of the II and III fingers of the right hand were determined. Based on the available phenotypic signs (CCGN in combination with pathology of the distal extremities, signs of ectodermal dysplasia), an assumption was made that the child has EEC syndrome.

Our assumptions were confirmed after a conversation with the patient’s mother. Upon examination, the woman was found to have scars on her upper lip and palate after undergoing cheilorhinoplasty and uranoplasty. The hair is light, dry, there is pathology of the hands. According to the mother, the eldest child in the family also has a cleft lip and palate, dry skin, and is seen by an ophthalmologist with frequent conjunctivitis. Based on the obtained phenotypic data and medical history, it was suggested that the EEC syndrome is hereditary. It is interesting to note that the diagnosis of EEC had not been previously established, and the woman was not informed about the likelihood of having children with this pathology.

In the hospital, patient T. was examined, a multislice computed tomography (MSCT) of the facial skeleton was performed (Fig. 3), a consultation was held, and a treatment plan was drawn up. Due to the large volume of defects in the upper jaw, a decision was made on two-stage surgical treatment. The first stage was an osteotomy of the vomer to reduce the protrusion of the premaxillary bone (Fig. 4), and the second was a one-stage bilateral cheiloplasty. The immediate result of the operation is shown in Fig. 5. Thus, based on the medical history and phenotypic characteristics, the patient was clinically diagnosed with EEC syndrome. The child is referred for genetic counseling. It is planned to continue treatment and rehabilitation at the Department of Pediatric Dentistry and Orthodontics of Sechenov University.

Clinical example No. 2

Patient M., 8 years old, the brother of patient T, was brought to the clinic. The child underwent a series of surgical interventions to eliminate a bilateral cleft lip, hard and soft palate. The portrait resemblance to his mother was noteworthy. The examination revealed the following phenotypic features: light hair, dry, sparse eyelashes, skin pigmentation in the periorbital region, dry skin of the extremities, syndactyly of the first and second toes of the right foot (Fig. 6-8). There are scars in the area of ​​the upper lip, hard and soft palate after repair of a bilateral cleft (Fig. 9). Mental development corresponds to the age norm; he studies at school. It has been suggested that the patient has hereditary EEC syndrome. To confirm ectodermal dysplasia, we performed the Minor test, which showed a decrease in the number of sweat glands (Fig. 10, 11). A treatment plan was drawn up, the patient underwent plastic surgery of the defect of the hard palate (Fig. 12), further treatment and rehabilitation are planned at the Department of Pediatric Dentistry and Orthodontics of Sechenov University.

results

Both patients were clinically diagnosed with EEC syndrome for the first time. A plan for further treatment and rehabilitation has been drawn up, taking into account the pathogenesis of the disease. Recommendations are given to parents on caring for children with this syndrome.

Discussion

EEC syndrome is quite rare in the practice of a doctor of any specialty. This leads to difficulties in making a diagnosis, and the condition is more often described as “multiple malformations.” Treatment of individual clinical manifestations included in the EEC syndrome (cleft lip and palate, dermatitis, adentia, xerostomia, lacrimal atresia), without combining them into a single nosological form, can be lengthy, expensive and unsuccessful, since it does not take into account the pathogenesis of the disease. When treating pathological processes in isolation, outside the syndrome, it is impossible to significantly improve the quality of life of patients, since therapeutic measures are palliative in nature. The result of a comprehensive examination and treatment of this group of patients should be an improvement in the quality of life and social adaptation. Parents should have information about the specifics of caring for a child with EEC syndrome (use of moisturizers, prevention of overheating, monitoring oral health and timing of teething).

A genetically confirmed diagnosis of EEC syndrome allows one to predict the birth of children with this pathology. Thus, in the case of a de novo mutation, the probability of a child being born with EEC syndrome is 1%, and with the hereditary nature of the disease it reaches 50%. In the latter case, the method of choice is the use of assisted reproductive technologies, for example, the use of donor eggs or sperm.

conclusions

When examining patients with CGN, attention should be paid not only to the condition of the oral cavity and face, but also to phenotypic characteristics (condition of the limbs, skin, hair, lacrimal ducts). The combination of CCRN with limb pathology can become a predictor of EEC syndrome, as shown in clinical examples.

Early detection of EEC syndrome allows you to draw up a correct treatment plan for the child, taking into account the pathogenesis of the disease. It is advisable to treat these patients in a multidisciplinary children's hospital with the possibility of consultations with specialized specialists. Informing parents who are carriers of EEC syndrome about the characteristics of this disease will make it possible in the future to predict the birth of children with this pathology and, if necessary, to use reproductive technologies.

CONFLICT OF INTEREST. The authors of the article have confirmed that there is no conflict of interest to disclose.

CONFLICT OF INTERESTS. Not declared.

Literature/References

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  2. Kasimovskaya N. A., Shatova E. A. Congenital cleft lip and palate in children: prevalence in Russia and in the world, groups of risk factors // Issues of modern pediatrics. 2020; 19 (2): 142-145.
  3. Meshcheryakova T.I. Analysis of the genetic causes of the development of congenital cleft lip and/or palate. Diss... cand. honey. Sci. M., 2015. 117 p.
  4. Beryl Benacerraf. Ultrasound of Fetal Syndromes. 2008. pp. 225-228.
  5. Barrow L.L., van Bokhoven H., Daack-Hirsch S. et al. Analysis of the p63 gene in classical EEC syndrome, related syndromes, and non-syndromic orofacial clefts // J. Med. Genet. 2002; 39 (8): 559-566.
  6. Andreeva E. N. EEC syndrome. Possibilities of prenatal diagnosis and features of medical genetic counseling // SonoAce Ultrasound. 2015; 27: 26-33.
  7. Vasiliev Yu. A., Redko A. N., Gulenko O. V., Udina I. G. Detection of congenital cleft lip and palate during prenatal ultrasound examination in the Krasnodar region // Russian Dental Journal. 2017; 21 (4): 190-193. [Vasil'ev Yu. A., Red'ko AN, Gulenko OV, Udina IG Prenatal ultrasonic detection of congenital clefts of lip and palate in Krasnodarskii krai // Rossiyskii stomatologicheskii zhurnal. 2017; 21 (4): 190-193.]
  8. Galonsky A. A., Radkevich A. A., Shushakova V. O. Ectodermal dysplasia: characteristic clinical signs and methods of dental rehabilitation // Siberian Medical Journal. 2011; 2:21-27.
  9. Celli D., Manente A., Grippaudo C., Cordaro M. Interceptive treatment in ectodermal dysplasia using an innovative orthodontic/prosthetic modular appliance. A case report with 10-year follow-up // Eur J Paediatr Dent. 2018; 19 (4): 307-312.

A. A. Mamedov1, 2 , ORCID: 0000-0001-7257-0991, S. A. Parshikova1, 2 , ORCID: 0000-0003-1029-9264, Yu. O. Volkov1, 2 , ORCID: 0000-0002- 2169-9717, L. A. Mazurina1, 2 , ORCID: 0000-0003-0841-1843, G. Ma1 , ORCID: 0000-0002-1135-6796, [email protected] H. Go1 , N. V. Gorlova1 , ORCID: 0000-0001-8537-3970,

1 Federal State Autonomous Educational Institution of Higher Education First Moscow State Medical University named after. I.M. Sechenov Ministry of Health of Russia ; 119991, Russia, Moscow, st. Trubetskaya, 8, building 2 2 GBUZ Children's City Clinical Hospital No. 9 named after. G. N. Speransky Department of Health ; 123317, Russia, Moscow, Shmitovsky pr-d, 29

Information about authors:

Mamedov Adil Askerovich , Doctor of Medical Sciences, Professor, Head of the Department of Pediatric Dentistry and Orthodontics, First Moscow State Medical University named after. I. M. Sechenov Ministry of Health of Russia; 119991, Russia, Moscow, st. Trubetskaya, 8, building 2; maxillofacial surgeon, State Budgetary Healthcare Institution Children's City Clinical Hospital No. 9 named after. G. N. Speransky Doctor of Health; 123317, Russia, Moscow, Shmitovsky pr-d, 29;

Parshikova Svetlana Aleksandrovna , candidate of medical sciences, assistant at the Department of Pediatric Dentistry and Orthodontics, First Moscow State Medical University named after. I. M. Sechenov Ministry of Health of Russia; 119991, Russia, Moscow, st. Trubetskaya, 8, building 2; maxillofacial surgeon, State Budgetary Healthcare Institution Children's City Clinical Hospital No. 9 named after. G. N. Speransky Doctor of Health; 123317, Russia, Moscow, Shmitovsky pr-d, 29;

Volkov Yuri Olegovich , Candidate of Medical Sciences, Head of the Department of Maxillofacial Surgery, Children's City Clinical Hospital No. 9 named after. G. N. Speransky Doctor of Health; 123317, Russia, Moscow, Shmitovsky pr-d, 29; Associate Professor, Department of Pediatric Dentistry and Orthodontics, First Moscow State Medical University named after. I. M. Sechenov Ministry of Health of Russia; 119991, Russia, Moscow, st. Trubetskaya, 8, building 2;

Lina Adilevna Mazurina , Candidate of Medical Sciences, Associate Professor of the Department of Pediatric Dentistry and Orthodontics, First Moscow State Medical University named after. I. M. Sechenov Ministry of Health of Russia; 119991, Russia, Moscow, st. Trubetskaya, 8, building 2; orthodontist at the Department of Maxillofacial Surgery, Children's City Clinical Hospital No. 9 named after. G. N. Speransky Doctor of Health; 123317, Russia, Moscow, Shmitovsky pr-d, 29;

Ma Gopei , graduate student of the Department of Pediatric Dentistry and Orthodontics, First Moscow State Medical University named after. I. M. Sechenov Ministry of Health of Russia; 119991, Russia, Moscow, st. Trubetskaya, 8, building 2;

Guo Hao , graduate student of the Department of Pediatric Dentistry and Orthodontics, First Moscow State Medical University named after. I. M. Sechenov Ministry of Health of Russia; 119991, Russia, Moscow, st. Trubetskaya, 8, building 2; [email protected]

Gorlova Natalya Vyacheslavovna , applicant for the Department of Pediatric Dentistry and Orthodontics, First Moscow State Medical University named after. I. M. Sechenov Ministry of Health of Russia; 119991, Russia, Moscow, st. Trubetskaya, 8, building 2;

Information about the authors:

Adil A. Mamedov , Dr. of Sci (Med), Professor, Honored Doctor of the Russian Federation, Academician of the International Academy of Informatization, Head of the departament of Pediatric Dentistry and Orthodontics FSAEI HE The First Sechenov Moscow State Medical University of the Ministry of Health of the Russian Federation ; 8, b. 2 Trubetskaya str., Moscow, 119991, Russia; maxillofacial surgeon of the Department of Oral and Maxillofacial Surgery, Moscow state budgetary healthcare institution “Children's City Clinical Hospital No. 9 named after GN Speransky, Moscow City Health Department"; 29 Shmitovsky Ave., Moscow, 123317, Russia;

Svetlana A. Parshikova , MD, Assistant professor of the department of Pediatric Dentistry and Orthodontics, FSAEI HE The First Sechenov Moscow State Medical University of the Ministry of Health of the Russian Federation; 8, b. 2 Trubetskaya str., Moscow, 119991, Russia; maxillofacial surgeon of the Department of Oral and Maxillofacial Surgery, Moscow state budgetary healthcare institution “Children's City Clinical Hospital No. 9 named after GN Speransky, Moscow City Health Department"; 29 Shmitovsky Ave., Moscow, 123317, Russia;

Yury O. Volkov , MD, Associate professor of the departedament of Pediatric Dentistry and Orthodontics FSAEI HE The First Sechenov Moscow State Medical University of the Ministry of Health of the Russian Federation; 8, b. 2 Trubetskaya str., Moscow, 119991, Russia; Head of the Department of Maxillofacial Surgery Moscow state budgetary healthcare institution “Children's City Clinical Hospital No. 9 named after GN Speransky, Moscow City Health Department"; 29 Shmitovsky Ave., Moscow, 123317, Russia; [email protected]

Lina A. Mazurina , MD, Associate professor of the department of Pediatric Dentistry and Orthodontics FSAEI HE The First Sechenov Moscow State Medical University of the Ministry of Health of the Russian Federation; 8, b. 2 Trubetskaya str., Moscow, 119991, Russia; orthodontist of the Department of Oral and Maxillofacial Surgery, Moscow state budgetary healthcare institution “Children's City Clinical Hospital No. 9 named after GN Speransky, Moscow City Health Department"; 29 Shmitovsky Ave., Moscow, 123317, Russia;

Guopei Ma , postgraduate of department of Pediatric Dentistry and Orthodontics FSAEI HE The First Sechenov Moscow State Medical University of the Ministry of Health of the Russian Federation; 8, b. 2 Trubetskaya str., Moscow, 119991, Russia;

Hao Guo , postgraduate of department of Pediatric Dentistry and Orthodontics FSAEI HE The First Sechenov Moscow State Medical University of the Ministry of Health of the Russian Federation; 8, b. 2 Trubetskaya str., Moscow, 119991, Russia;

Natalya V. Gorlova , postgraduate of department of Pediatric Dentistry and Orthodontics FSAEI HE The First Sechenov Moscow State Medical University of the Ministry of Health of the Russian Federation; 8, b. 2 Trubetskaya str., Moscow, 119991, Russia;

Cleft lip and palate as predictors of EEC syndrome: clinical observation / A. A. Mamedov, S. A. Parshikova, Yu. O. Volkov, L. A. Mazurina, G. Ma, H. Guo, N. V. Gorlova For citations: Mamedov A. A., Parshikova S. A., Volkov Yu. O., Mazurina L. A., Ma G., Guo H., Gorlova N. V. Cleft lip and palate as predictors of EEC syndrome: clinical observation // Attending doctor. 2021; 10 (24): 36-40. DOI: 10.51793/OS.2021.24.10.006 Tags: children, congenital facial pathology, autosomal dominant disease

Orthodontic treatment of cleft palate

In addition to surgery, the assistance of an orthodontist . Medical measures are carried out from the first days after the birth of children with cleft palates until adulthood, and, if necessary, throughout adult life.

Pre-teething period

The primary task is to provide nutrition to the baby so that milk does not enter the mouth and nose during feeding. The problem is solved with the help of specially designed nipples and obturators.

Already at 1–4 months, they begin to correct the shape of the upper jaw using a removable apparatus with a sliding screw. Subsequently, it is replaced with a retention plate that closes the cleft. Before veloplasty, the orthodontist makes a new protective plate for the hard palate.

Period of temporary occlusion

Before uranoplasty, the orthodontist must help the surgeon narrow the cleft of the hard palate. For this purpose, a plate with special devices - pelota - is used. They put pressure on the mucous membrane along the edges of the cleft, irritating it and thereby stimulating the growth of bone tissue.

From 5–6 years of age, the doctor needs to ensure that the jaws develop evenly: restrain the growth of the lower jaw and optimize the growth of the upper one. The first problem is solved by a cap with a chin sling, the second by orthodontic devices for correcting anomalies (activators and regulators).

Mixed dentition period

Before uranoplasty, the orthodontist makes the next protective plate, which is necessary during the recovery stage. After 1–1.5 months, it is replaced with a removable denture with missing teeth.

Replacing baby teeth with permanent ones is the most important time for an orthodontist. Morphological defects worsen, some teeth begin to tilt, overcrowding and crossbite appear. The doctor must diagnose and minimize all these negative changes in a timely manner. At the same time, measures for balanced jaw development are continued.

Period of permanent dentition

At this stage, multibonding systems ( braces ) begin to be included in the process of correcting the bite. Fixed structures help expand narrowed areas of the dentition and correct the position of individual teeth. The upper jaw is also retained: by this time it can already be expanded sufficiently, but the new position is still unstable and requires support. After completing hardware treatment, the teenager should undergo examinations by an orthodontist once every six months [2].

A cleft palate is a serious pathology, but with the coordinated and professional actions of specialists, the defect and its consequences can be completely eliminated. In the future, the person lives a full life, without experiencing discomfort while eating or communicating.

List of sources
  1. “Cleft palate and cleft lip”, interview with D. A. Grichanyuk, maxillofacial surgeon, Ph.D. // URL: https://www.the-village.me/village/city/people/271423-hirurg (accessed 09/02/2020).
  2. Special issues of orthodontics. Educational and methodological manual / Tokarevich I. V., Gorbatsevich N. A., Moskaleva I. V., Gorbatsevich D. V., Chernyavskaya M. V., Vasilenko E. P., Kolomiets E. G., Korneeva A. S., Minsk: BSMU, 2012. // URL: https://www.bsmu.by/downloads/kafedri/k_ortodont/3.pdf (access date 09/02/2020).
  3. Alexandrova L.I. Comprehensive assessment of the results of early preoperative orthopedic therapy taking into account dynamic indicators of the quality of life of children with congenital cleft lip and palate: dissertation. Ph.D. honey. Sciences: 01/14/14 - dentistry. Perm, 2022. // URL: https://www.psma.ru/index.php?option=com_mtree&task=att_download&link_id=218&cf_id=24 (date accessed 09/02/2020).
  4. Surgical methods of treatment of children with congenital cleft lip and palate: textbook / Chuikin S.V., Davletshin N.A., Averyanov S.V., Chuikin O.S., Ufa: State Educational Institution of Higher Professional Education "Bashkir State Medical University of Roszdrav" , 2011. // URL: https://library.bashgmu.ru/elibdoc/elib491.pdf (accessed September 2, 2020).

Features of caring for a baby with facial clefts

Carefully clean the nose using damp cotton swabs soaked in oil (sterile olive, sea buckthorn) or herbal infusions (chamomile).

With cleft lips and palate, part of the oral mucosa is in constant contact with air. This leads to the formation of cracks and crusts on the mucous membrane of the upper lip. Toilet your upper lip, especially in the cleft area: carefully remove pre-soaked crusts and treat the surface with sterile oil.

When walking in the cold season, use gauze masks on the area of ​​the wide cleft of the upper lip to warm the incoming air.

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