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Ризаев Жасур Алимджанович
Шамсиев Жамшид Азаматович
Шамсиев Равшан Азаматович
Зайниев Сухроб Сабирович
Самаркандского государственного
медицинского института, Узбекистан
СОПУТСТВУЮЩИЕ ПОРОКИ РАЗВИТИЯ У ДЕТЕЙ С ВРОЖДЕННОЙ РАСЩЕЛИНОЙ
ГУБЫ И НЕБА
http://dx.doi.org/10.26739/
2181-0966-2020-1-11
АННОТАЦИЯ
На базе 2-клиники Самаркандского государственного медицинского института проведен анализ лечения 122 пациентов с
различными видами врожденных расщелин верхней губы и неба. В результате проведенных исследований выявлено, что
врожденные расщелины верхней губы и нёба встречаются в сочетании с аномалиями развития других органов. Основными
из них были- короткая уздечка губ и языка у 76 пациентов, низкий индекс массы тела у 97, деформация позвонка у 6,
косолапость у 19, врожденное плоскостопие у 5, врожденная дисплазия тазобедренного сустава у 2, дефекты развития
мочевыделительной системы у 19, пренатальная энцефалопатия у 46, врожденная мышечная недостаточность у 15 детей.
Анализ проведенного исследования показал, что для достижения высокого уровня медицинской помощи детям с данной
патологией требуется четкая и единая, взаимосвязанная тактика всех специалистов, участвующих в лечении ребенка с
врожденной расщелиной губы и нёба.
Ключевые слова:
врожденные расщелины верхней губы и нёба, пороки развития, лечение, дети.
Rizaev Jasur Alimjanovich
Shamsiev Jamshid Azamatovich
Shamsiev Ravshan Azamatovich
Zainiev Sukhrob Sabirovich
Samarkand State Medical Institute, Uzbekistan
ACCOMPANYING DEFECTS OF DEVELOPMENT IN CHILDREN WITH CONGENITAL CLEFT OF LIP AND
PALATE
ABSTRACT
An analysis of the treatment of 122 patients with various types of congenital clefts of the upper lip and palate was performed on the
basis of the 2nd clinic of the Samarkand State Medical Institute. As a result of our studies, it was found that congenital clefts of the
upper lip and palate are found in combination with developmental abnormalities of other organs. The main ones were short frenulum
of lips and tongue in 76 patients, low div mass index in 97, vertebral deformity in 6, clubfoot in 19, congenital flat feet in 5,
congenital dysplasia of the hip joint in 2, defects in the development of the urinary system in 19, prenatal encephalopathy in 46,
congenital muscle failure in 15 children. Analysis of the study showed that to achieve a high level of medical care for children with
this pathology, a clear and uniform, interconnected tactics of all specialists involved in the treatment of a child with congenital cleft
lip and palate is required.
Keywords:
congenital clefts of the upper lip and palate, malformations, treatment, children.
Ризаев Жасур Алимджанович
Шамсиев Жамшид Азаматович
Шамсиев Равшан Азаматович
Зайниев Сухроб Сабирович
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Самаркандского государственного
медицинского института, Узбекистан
БОЛАЛАРДА ЛАБ ВА ТАНГЛАЙ ТУҒМА ЁРИҚЛАРИ БИЛАН РИВОЖЛАНАДИГАН БИРГАЛИКДАГИ
НУҚСОНЛАР
АННОТАЦИЯ
Самарқанд давлат тиббиёт институтининг 2-клиникасида юқори лаб ва танглай туғма кемтикли 122 нафар беморни даволаш
таҳлили ўтказилди. Олиб борилган тадқиқотлар натижасида юқори лаб ва танглайнинг туғма битмаслиги бошқа аъзолар
ривожланишининг аномалиялари билан биргаликда кечиши аниқланган. 76 беморда тил ва лаб тутқичининг туғма
калталиги, 97 беморда тана вазнининг пастлиги, 6 холатда умуртқа поғонаси деформацияси, 19 холатда туғма маймоқлик,
ясси оёқлик -5, чанок-сон буғимининг дисплазияси-2, сийдик чиқариш тизимининг ривожланиш нуқсонлари-19, перенатал
энцефалопатия-46 ва мушакларнинг туғма етишмовчилиги-15 холатда кузатилди. Тадқиқот таҳлили шуни кўрсатдики,
ушбу патологияга эга бўлган болаларга юқори даражадаги тиббий ёрдам кўрсатиш учун касалликни даволашда иштирок
этадиган барча мутахассисларнинг аниқ ва ягона, ўзаро боғланган тактикаси талаб этилади.
Калит сўзлар:
клиникасида юқори лаб ва танглай туғма кемтиклари, туғма нуқсонлар, даволаш, боолалар.
To the most widespread of congenital defects of
development refers congenital cleft of upper lip and palate. The
birth rate of children with the fact of defect is composed of about
86% anomalies of jaw-facial area and 20-30% defects of
development of man [9]. Basically, clefts of lip and palate are
considered polygenic multifactoral diseases. They may occur as
isolated defect of development and was been one of the
symptoms of congenital syndromes (syndrome Van-der-Vud,
Pier Robin and others). Besides, in majority of sick children with
congenital clefts of lip and palate are occurred accompanying
congenital defects of development of other organs and system
(heart, lungs, kidneys and others), and it is also important to take
into consideration in forming plan of treatment.
Immediately, after birth of a child with these anomalies
of development in maternity hospital is established exact clinic-
anatomical diagnosis and determined the plan of surgical and
conservative treatment. In case of need child is moved to the
surgical department of newborns where is carried out
examination, revealed congenital defects of development of
other organs and system, diagnosed accompanying somatic and
nervous diseases [1,7,8]. In the presence of accompanying
defects of development, severe somatic diseases, diseases of
central nervous system the time of surgical treatment are
separated. Surgical rehabilitation must be finished not later 5-6
years with that estimation that the child can go to ordinary school
[1,7]. Children with Daun, Little, children cerebral paralysis and
other rough pathology of organism on contraindications may be
operated in later estimations [1,2,5]. Therefore, in the period new
birth is composed of individual plan of preparation of patient to
the surgical operation with taking into consideration of gravity
of defect of development in the maxillary-facial area
accompanying defects and somatic status condition of the child
[4,6,7,10].
Aim:
To reveal accompanying defects of development
(ADD) in children with congenital cleft of lip and palate.
Materials and methods
: For the examination and
correction 122 patients from them with congenital cleft of lip –
68(55.74%), palate- 13(10.66%), one-sided- 96 (88.07%), two-
sided- 13 (11.93%) patients, girls- 47(43.12%) and boys
accordingly-62 (56.88%) were in the clinic № 2 of SamMI
during 2004-2014. 54 patients from them complete cleft of
palate -52 (96.30%), with incomplete- 2 (3.70%), girls-19
(35.19%), boys – 35 (64.81%) were with non closure palate. In
primary referencing these children was carried out complete
clinic-laboratory examination including clinical, laboratory and
instrumental examinations of patients with examination of
qualified specialists such as jaw-facial surgeon, INT doctor,
geneticist, orthopedist, pediatrician in necessarily neurologist,
hematologist and orthopedist-traumatologist.
Carried out research was showed that the children with
congenital cleft of upper lip and palate accompanying defects of
development were registered in the area of head, neck,
extremities and trunk and were often combined [3,5].
The main of them were short frenum of lip and tongue
in 76 patients, low index of div weight in 97, deformation of
vertebra in 6, club-foot in 19, congenital flat-foot in 5,
congenital dysplasia of hip joint in 2, defects of development of
urinary system in 19, prenatal encephalopathy in 46, congenital
muscular wryneck in 15 children.
In the process of research the functions of kidneys were
established pyuria in 19 children with congenital cleft of upper
lip and palate. These patients were carried out special urological
examination in which was revealed congenital stricture of
prerenal (8) and pelvic area of urethra (6) was carried out
ultrasound examination of urinary system. On evidence of
excretion urography in 14 patients were revealed pyelcctasis, in
8 hydronephrolic transformation from the direction of urethra
obstruction. Cystic-urethral reflux II degree as manifestation of
congenital dysplasia stoma of urethra was established in 5
children.
In combination of pathology the child was examined by
particular specialists (neurologist, endocrinologist, cardiologist
and others), were carried out suitable recommendations, in the
case of need was carried out treatment. The children with
accompanying defects of development of extremities and
congenital muscular wryneck after training features of nursing
and feeding, examination of orthodontist and making of
obturator for closing of palate defects during feeding were
directed for treatment to the orthopedist-traumatologist before
cheilorhinoplasty that is till three-four months of age.
Psychoneurologist carried out medico-pedagogical
rehabilitating measures with parents and then with patients
about medicamental corrective therapy, psychotherapy. All
children without exception were examined by neurologist; in the
case of need they were taken electroencephalographically
examination. Children with encephalopathy got adequate
treatment till I stage of operation and further was carried out
prophylaxy, observation and the treatment of the specialist
[6,7.10]. After made diagnosis and type of hereditary
transmission(in the presence of the same) by geneticist were
prognosticated the degree of birth risk in proband or members
of his family of other children with CCL and P.
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It was carried out by the orthodontist ensuring full
feeding of child (obturators, disjunctive plastics and etc.), pre-
and post-orthodontic treatment, notice and treatment of
secondary deformation of tooth-jaw system, restoration of tooth
set. Periods of active observations by doctor-orthodontist are:
new born period, before and after operation, changeable bite
period, and further systematically observation till complete
formation of permanent bite [6,7,10,13]. Logopedist carried out
logopedic treatment before and after uranoplastics, training of
muscular structure of soft palate, pharynx wall, the development
of speech respiration, pre- and post-operative logopedic
treatment, development of phonemic
hearing, formation of
connected speech. Periods of active observation of logopedist
are periods in 1-2 years, 4-5 years and further 7-13 years. It has
been carried out estimation of somatic status by the pediatrician
individually chosen type of feeding and volume of nutrition,
evaluated micro flora of oral cavity, immune status, carried out
prophylaxy and in the case of need anemic treatment, rickets and
hypotrophy, realized preparation for surgical treatment during
the first year. These children were actively observed prophylaxy
by ENT doctor taking into consideration that the possibility of
hit of food from oral cavity to nasal and pharyngonasal cavity
may result in to the development of chronic rhinitis, eustachitis,
otitis and as consequence to the reduce of hearing.
Even in the absence of evident complaints to the
function
of
cardiovascular
system,
once-only
electrocardiographically research in 74 (60.66%) children with
congenital cleft of lip and palate was revealed disturbance of
automatism function, myocardial, because of which the patients
were examined by cardiologist and got preoperative corrections
of disturbances.
Success of surgical treatment in majority of patients
depended on timely conduction of complex preparation in
conditions of dispensary observation [7,12,14]. Surgical
treatment began from 3 months in satisfactory somatic and
neurological status.
Heading and coordinating the work of doctors-
consultants the surgeon-dentist determined data and volume of
surgical treatment, controlled timeliness referral children to the
consultation of particular specialists and carrying out
prophylactic treatment, together with them determined absolute
and relative contraindication to the operation. It shouldn’t forget
in decision of question operation dates and higher responsibility
of pediatrician giving permission to the surgical treatment. His
conclusion about contraindication or indication to the operation
of a child having accompanying CDD
or somatic diseases, the
pediatrician confirmed conclusions of particular specialists
(cardiologist,
nephrologist,
urologist,
neurologist,
pulmonologist, endocrinologist and others).
Absolute contraindication to the operation is
insufficiency of blood circulation IA and IIB degrees.
Relative contraindications are: sharp evident dystrophic
changes in myocardium, acute somatic and infectious diseases
and not earlier 2 months after recovery hypotrophy I-III degrees,
rickets I-III degrees in fastigium period, atopic dermatitis in the
stage of recrudescence, anemia with containing of erythrocytes
less 3.5x10
12
/l, hemoglobin less 120g/l, colored finding less
0.75, chronic diseases during 6 months after recrudescence,
chronic nephritis with manifestations of renal insufficiency,
dermatitis and eczema in the facial area, affection of mucousa.
Children with relative contraindications were carried out specific
treatment accompanying pathology (in the out-patient or profile
departments) and on favorable dynamic was allowed operative
treatment [7,10]. But in consideration of earlier address to the
specialized medical aid from the first days of the life and timely
diagnostics of accompanying defects of development, primary
cheilorhinoplasty were carried out at the age of 2-3 months, but
qualified surgical treatment of children with congenital cleft of
upper lip and palate was finished during 12-16 months of life.
Conclusions: Detected in children with cleft of upper lip
and palate external stigma of disembryogenesis- congenital
defects of development of extremities, kidneys and other
changes of organs have systemic character, progress which is
conditioned with dysplasia of connective tissue. For extension of
complex aid and provision complete rehabilitation of patients
with congenital cleft of lip and palate is necessary synchronic
work of specialists: geneticist, logopedist, hematologist,
otorhinolaryngologist, orthodontist, pediatrician, psychiatrist,
orthopedist, surgeon, for timely detection of accompanying
defects of development and diseases with the purpose of
conduction of full, active preoperative preparation and qualified
staged correction of all defects of development.
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