INTRODUCTION:

There are multiple investigations and case studies regarding step-by-step treatment of children with some anomalies in neonatal period^1-10.

Congenital and hereditary pathology of the maxillofacial area takes the leading place in pediatric maxillofacial surgery. The frequency of congenital malformations in the population is an important characteristic of its health status. Mortality associated with these malformations ranks 2nd-5th in the structure of child mortality. The congenital malformation of the maxillofacial area is detected on average in one of 500 newborns. In recent years, the number of such children has increased, with congenital clefts of the upper lip and palate being most common among them¹¹.

In the structure of fetal congenital malformations, cardio-vascular system anomalies occupy the leading place (50.8%), mitral valve diseases come second (13.8%), locomotor and musculoskeletal disorders – third (11.5%), multiple congenital malformations – fourth (6.0%), central nervous system malformations – fifth (5.76%), and cleft lip and palate – sixth (4.12%)¹².

MATERIALS AND METHODS:

The methods of synthesis and analysis, relevant literature study and statistic data systemizing, gathering data and their further scientific interpretation, generalization with accordance to the objectives of the investigation were used.

In the structure of surgical diseases of the maxillofacial area in children, congenital malformations constitute a significant group. Among all types of malformations of the maxillofacial area, the frequency of birth of children with cleft lip and palate is the highest, varying in different regions of the Russian Federation from 1:500 to 1:1000 newborns. According to the World Health Organization, the average frequency of this anomaly in the world is 1:700 newborns¹³.

Cleft palate, ranking 4th-7th in the structure of congenital anomalies, is one of the most important anatomical and functional disorders¹⁴.

Currently, the birth of a child with cleft lip and palate constitutes: 1 per 500-1000 newborns in Europe (depending on the country); 1:600 – in the United States; 1:588 – in Japan, and lowest in Africa among Negroids – 1:2440 newborns. According to H.J. Neumann, in Central Europe, the ratio is 1:500. He also notes that the number of patients with cleft lip and palate has increased two-fold over the past 40 years¹⁵.

In Belarus in the early 1980s (before the Chernobyl accident), the frequency of birth of children with cleft lip and palate was 1:1124 newborns. According to the children's clinic of maxillofacial surgery of the Minsk Medical Institute, the number of patients with cleft lip and palate who were under medical treatment increased from 61 people in 1988 to 110 people in 1998. This also testifies (although indirectly) to an increase in the number of patients with cleft lip and palate in Belarus¹⁵.

Dynamic analysis of the frequency of cleft lip and palate in the Republic of Sakha (Yakutia) over the past 13 years has revealed its cyclical fluctuations with peaks of increase in 2000, 2007 and 2012, when the frequency of cleft lip and palate was 1.90, 2.60 and 1.54 per 1000 newborns, respectively¹⁶.

In the Republic of Bashkortostan, the birth rate of children with congenital cleft lip and palate is higher than the national average¹⁷.

There is contradictory information about the frequency of congenital clefts of the upper lip and palate in the Republic of Kazakhstan. The ecological disaster of the Aral Sea region has had a negative effect on the health of the population, especially among women and children. It has been established that the frequency of congenital clefts of the upper lip and palate in the region is 1.1 per 1000, and in the city of Baikonur – 2.1 per 1000, or 1 child per 476 births. Another environmentally unfriendly region (Central Kazakhstan) has a similar pattern. In general, in the region, the frequency of congenital cleft lip and palate amounted to 1.1 per 1000, and in the Temirtau industrial region – 1 per 422¹⁰.

When studying the data of obstetric institutions in Semipalatinsk, some interesting indicators have been obtained. Various congenital malformations among newborns occurred in 1.7% of cases. However, according to children's autopsies, the number of congenital malformations reaches 14.6%, which is 8.6 times higher than that obtained from live births, with congenital cleft lip and palate in 79% of cases being associated with developmental diseases of other organs and systems, while in Almaty this number reaches 15.6%¹⁰.

The prevalence rate of congenital cleft lip and palate in the Republic of Georgia in 1981-1990 was 1.05 per 1000 among live births (1:952). During this period, the figure increased from 1:1053 to 1:840.

At the beginning of the 20th century in Ukraine, children with congenital clefts were born at a ratio of 1:1200. In 1960-1970, their number reached 1:1000, and in 2000-2002 – up to 1:800-1:900. In most European countries, this ratio is currently 1:600. In 2000, in 28 European countries, 9,222 children with congenital cleft lip and palate were born, and 7,605 primary surgical interventions were performed on the lip and palate¹¹.

Children with congenital malformations in Russia consist 1 to 12% of all newborns, and their number is constantly growing. According to Russian researchers, there has been an increase in the number of congenital anomalies, especially after 1986, as a consequence of the accident at the Chernobyl nuclear power plant. This, apparently, is associated with a real increase in the frequency of pathology and with improved accounting. Thus, according to statistical data⁸, in Moscow for 1979-1993, this pathology ranged from 0.60 to 1.17 cases per 1000 newborns. In the Volgograd region, the ratio was 1:745 newborns¹¹.

According to the Federal State Statistics Service, congenital anomalies (malformations), which include congenital cleft lip and palate, amounted to 2.0 per 1000 population in 2017^18,19.

Based on the literature, the percentage ratio of clefts of the upper lip and palate can be represented as follows: isolated clefts of the upper lip are found in 10-20% of cases, isolated clefts of the palate – in 20-25% of cases, and clefts of the upper lip and palate – in 50-70% of cases. This pathology is most common among boys (60-70%). However, isolated clefts of the palate are more common among girls (55-60%)¹⁵.

Children with congenital cleft lip and palate should be under medical supervision from the moment they are born. The way of their treating is the central objective of current investigation.

RESULTS AND DISCUSSION:

The main method of treating children with this pathology is surgery to correct the defect of the lip and palate – cheiloplasty and uranoplasty, and the problem of complex rehabilitation is relevant, multifaceted and quite complicated.

In order to provide successful rehabilitation assistance to a child, each of the narrowly focused specialists, such as maxillofacial surgeon, orthodontist, psychotherapist, otolaryngologist, dentist, speech therapist and social worker, requires complete information about the course of treatment and the stages of further complex rehabilitation.

The rehabilitation process takes place during the entire childhood age, and in a number of individuals – at an older age. However, it is advisable to divide it in accordance with the periods identified in pediatrics into 7 periods: 1) prenatal period; 2) neonatal period: from 0 to 1 month; 3) infancy period: 1 month-1 year; 4) toddler period: 1-3 years; 5) preschool period: 3-6 years; 6) primary school period: 7-11 years; 7) senior school period: 11-18 years.

Pregnant women need the consultation of an ecologist and a professional pathologist (if medically required) for planning treatment and preventive measures¹⁷.

The next most important is the neonatal period (from 0 to 1 month), including the organization of treatment, care and drawing up an approximate plan of complex rehabilitation during this period for a child with congenital cleft lip and palate.

Modern practice of the birth of children with congenital cleft lip and palate in the leading dental clinics of the Russian Federation and abroad is as follows: after a newborn with a defect in the maxillofacial area is born, an emergency notice must be sent to the Center for Maxillofacial Surgery during the first 3 days. After receiving an emergency notice, the head of the Center organizes an urgent visit of the dental surgeon to conduct a detailed examination and determine the extent of the necessary measures to ensure the adequate feeding of a child, as well as the orthodontist to settle the manufacture of an obturator according to the type of removable prosthesis. An exact clinical and anatomical diagnosis is established, and a plan for surgical and conservative treatment is determined. Then an in-depth examination by related specialists is conducted, congenital malformations of other organs and systems are identified, and concomitant somatic and neurological diseases are diagnosed.

The right to call specialists for emergency consultation belongs to:

· Chief obstetricians, neonatologists, pediatricians, dental surgeons and other top specialists of districts and cities;

· Heads of maternity, neonatological and surgical children's departments of medical institutions of districts and cities;

· At night and on weekends – doctors on duty at the specified medical units under the supervision of the heads of the respective departments.

Individual work is carried out with parents on teaching how to nurse a sick child to ensure optimal development, rational feeding and prevention of infectious diseases. Parents receive information about the child’s health, prospects and rehabilitation algorithm.

With a complete unilateral and bilateral cleft of the upper lip, the alveolar process, and the soft and hard palate, an early presurgical orthopedic preparation of the patient is carried out.

Then the toxicologist’s consultation is scheduled. As medically indicated, he examines the child’s urine and blood or the mother’s breast milk for ecotoxicants, and prescribes detoxification therapy for both of them. If there are concomitant malformations, severe somatic diseases or central nervous system diseases, the terms of surgical treatment are postponed, but surgical rehabilitation should be completed in no longer than 5-6 years, so that the child could go to a regular school. Children with Down syndrome, Little’s disease, cerebral palsy and other gross body anomalies, according to contraindications, can undergo surgery at a later date. The result of the work of specialists at this stage is an individual plan of preparing the patient for surgery, taking into account the severity of the malformation of the maxillofacial area, concomitant defects and the child’s somatic status¹⁷.

However, the neonatologist and the pediatrician are the first to examine such a newborn child. Their task is to correctly assess the child’s condition and to send him to doctor specialists in a timely manner¹⁸.

The neonatologist should know the features of feeding children with congenital cleft lip and palate. In this regard, one of the first and important stages of complex rehabilitation is the selection and provision of the optimal feeding method. Immediately after the birth of the child with a cleft, the maternity hospital employee is obliged to inform the head of one of the centers and the maxillofacial surgeon, who together with the orthodontist in the maternity hospital will determine the feeding method. This refers to the category of emergency care for a child with congenital cleft lip and palate¹⁰.

During the neonatal period, congenital cleft lip and palate cause severe functional disorders, often accompanied by teeth and occlusal abnormalities after teething. The mortality rate of such patients earlier reached 25%. The improper or inadequate care and feeding of the child, leading to inflammatory diseases of the respiratory tract, is one of the main causes of mortality among such children, most often from pneumonia. Currently, mortality significantly has decreased¹⁵.

The impaired respiratory function in newborns with cleft lip and palate is due to the fact that they have a mixed nasal-oral breathing type¹⁵.

The sucking function in children with congenital clefts of the upper lip and palate is impaired due to the lack or insufficient level of hermeticity in the oral cavity. As a result, the child does not take the mother’s breast or, during feeding, quickly gets tired and malnourished, which leads to a delay in the child’s physical development, malnutrition, rickets, etc.¹⁵.

The disturbance of the respiration and nutrition functions from the first days of life of children with cleft lip and palate leads to the fact that the concomitant morbidity (as a complication of congenital pathology) among these children is much higher than among healthy children, which is especially expressed in the first 6 months of the child's life¹⁵.

One of the priorities in the neonatal period is the organization of proper nutrition and care for children with cleft lip and palate. For children with cleft lip and palate, just like for other children, there are two best ways of feeding: breast feeding and bottle feeding. Feeding a child from a cup is not an option, and in the case of normal breast feeding or bottle feeding, there is no need to resort to this method²¹.

Breast feeding is certainly the best type of feeding for a healthy child, and even more so for a child with this pathology. But, unfortunately, children with cleft lip and palate in the maternity hospital are transferred to artificial feeding, which cannot be considered right. As a result of the mother’s psychological trauma caused by the birth of a child with such a pathology and due to the fact that the child was not held to the breast, this can lead to the development of the mother’s hypogalactia.

The early artificial feeding due to the impossibility of breast feeding reduces the body's resistance, which leads to growth inhibition and retardation. Children with this pathology from the first months are significantly different from their peers²².

For this reason, talking with the doctor in the maternity hospital about the importance of breast feeding, the real possibilities of correcting this congenital malformation, and the vitality and social acceptability of the child in the future can calm the mother and help maintain or extend the lactation period. In addition, in the first months of life, a more frequent feeding of these children, compared with healthy ones, is needed⁵. In this case, the doctor recommends that the mother should persistently latch her child to the breast and develop patience when feeding, as well as teaches how to express milk in order to maintain longer lactation.

On the one hand, the feeding method directly depends on the type of cleft lip or palate. On the other hand, it is known that naturally (enterally) a child (newborn) can be fed through the mother’s breast milk, as well as with the help of a nipple, a spoon or a stomach tube. Therefore, the task of the doctor is to achieve the most optimal physiological feeding method for the child, based on the specific pathology¹⁵.

In cases when breast feeding is not possible, nursing staff teach the mother how to feed the child with a nipple or spoon (a nipple should be long enough to close the defect and create the best conditions for sucking; there must be several holes in it; the most adapted are NUK nipples). If necessary, a disconnecting plate should be made. Together with the neonatologist, the dental surgeon develops recommendations on the feeding regimen of a child with congenital cleft lip and palate (feeding is allowed after 2-2.5 hours 8-10 times per day), conducts psycho-prophylactic work with the child’s mother, explains the features of congenital malformation, feeding and caring specificity, as well as informs about the possibilities of surgical and rehabilitation treatment, the need for dispensary observation and treatment in a specialized Center, genetic counseling. The dental surgeon gives the mother and nursing staff a booklet with the address and telephone number of the Center, consultation days and hours of admission, and the terms of the initial consultation of the particular child. Prior to discharge from the maternity hospital, the head nurse transmits the information about the newborn to the district children's consultation, where, on the basis of this information, the patronage of the newborn is recorded in the journal of the doctor’s call to the house. The district pediatrician and nurse visit the newborn on the first day after discharge from the hospital. In the first 3-5 days after discharge, the child should be examined by the head of the outpatient pediatric ward. Further observation is carried out according to an individual plan, and a diary of observations is kept. The pediatrician on site visits the child with congenital cleft lip and palate every week for the first month, and the nurse comes every day²³.

Dispensary observation of the child with congenital cleft of the upper lip and palate must begin from the moment of birth. The approximate scheme of the medical rehabilitation of children with congenital clefts of the upper lip and palate during the first month of life can be represented as follows:

· Registration of the child for dispensary observation in the center with the statement of the diagnosis and development of a plan of complex treatment, depending on the severity of the pathology;

· Tips for parents on feeding and child care;

· Prevention of respiratory diseases and chronic diseases of the upper respiratory tract;

· Orthodontic treatment (if medically indicated) with cleft palate, especially with through clefts, before cheiloplasty surgery;

· Documentation of disability from childhood.

According to the Order of the Ministry of Labor and Social Protection of the Russian Federation N 1024n dated December 17, 2015 "On the classifications and criteria used in the implementation of medical and social expertise of citizens by federal government institutions of medical and social expertise", this pathology is one of the criteria for determining the disability groups²⁴.

Unfortunately, the majority of medical workers (general practitioners, nurses) cannot give parents the right advice on this issue. Moreover, often in maternity hospitals, they immediately begin feeding the children through a tube, arguing that such children have no sucking reflex. It is an erroneous opinion, since the sucking reflex, under the action of which the muscles of the maxillary tissues are strengthened and the lower jaw moves forward, is unconditioned, and feeding through a tube leads precisely to its extinction^25,26.With long-term feeding through a tube, the mucous membrane of the esophagus is injured, which results in its inflammation and potential strictures. Therefore, the use of a tube should be short-term, only in children with Pierre-Robin syndrome. These children, due to the underdevelopment of the lower jaw and the large retroposed tongue, have significant difficulty in breathing, which is an additional aggravating circumstance for sucking (normally, the child breathes at the same time when sucking)¹⁰.

The most common well-known method of artificial feeding through a nipple can be used in almost all cases of clefts of the upper lip and palate. If the mother has enough milk, it can be expressed¹. Depending on the type of cleft, it is possible to choose the most physiological optimal feeding methods for each newborn with cleft.

Thus, the proper care and full feeding of children with congenital clefts of the upper lip and palate is the most important link in the preparation of these children for surgical interventions.

The article entitled "Policy on the Management of Patients with Cleft Lip/Palate and Other Craniofacial Anomalies" presents typical problems faced by parents of a child born with cleft lip and palate and other craniofacial anomalies, namely: early feeding and nutritional concerns, middle ear disease, hearing deficiencies, deviations in speech and resonance, dentofacial and orthodontic abnormalities, and psychosocial adjustment problems. The study also discusses parameters, standards and fundamental principles, identified as critical to optimal cleft/craniofacial care. However, these principles state that management of patients with craniofacial anomalies is best provided by an interdisciplinary team of specialists, but the role of nursing staff in organizing the care of patients with congenital cleft lip and palate and teaching parents how to care for children with congenital cleft lip and palate, especially at home, is not considered in detail^27,28.

According to Paul Martin, typical problems faced by parents whose children were born with cleft lip and palate are: ineffective airway clearance; imbalanced nutrition: less than body requirements; anxiety; deficient knowledge; compromised family coping; risk for injury.

CONCLUSION:

In Russia, the problems of cleft lip and palate are solved by medical specialists. It is advisable to delegate the powers of surgeons, dentists and pediatricians to specially trained nursing staff. Nursing staff in the neonatal period can teach mothers how to feed children with this pathology, discuss this issue with parents, provide the necessary medical literature, advise on care of a newborn with congenital cleft lip and palate, especially at home, as well as help parents to solve their psychological problems. Thus, there is a need to develop guidelines for the care and feeding of children with congenital cleft lip and palate, both for parents and nurses in the framework of the organization “Nursing and Rehabilitation Schools for Children with Congenital Malformations of the Maxillofacial Area”.

CONFLICT OF INTEREST:

The authors declare no conflict of interest.

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Received on 24.07.2019 Modified on 05.10.2019

Research J. Pharm. and Tech 2020; 13(3): 1441-1446.

DOI: 10.5958/0974-360X.2020.00263.2