ATолчья пасть у детей: причины, операция

Обновление: Октябрь 2018 ATолчья пасть у детей – это врожденный
порок development, при котором ребенок рождается с повреждением в
face area: there is a direct message between his mouth and nose because
that there is a cleft in the sky. Often this defect is combined with
заячьей губой – у них общий механизм development. If a же губа не
damaged and only part of the palate suffered (soft palate or uvula)
or cleft occupies its entire length, then the disease is some
time goes unnoticed until parents pay attention to
strange behavior during feeding.

Cleft palate without splitting lips occurs approximately in
1-2 cases per 1000 newborns, girls suffer more often
(Isolated cleft lip is more common in boys). it
the disease leads to various complications from the side
ENT-organs, and many internal organs, so its
must be treated. It will only help the operation, the first stage
which can perform at the age of another year. After operation
need long-term restorative complex therapy.

Withчины заболевания

ATолчья пасть или, как правильно и необидно для таких людей
to speak, congenital cleft palate arises when
the developing fetus disrupts the normal accretion of the bones of the face
(2-11 weeks of pregnancy). itт порок development, возникая
in isolation or in combination with a hare lip, included in the top ten
most common defects. Most of all sick children with
Asian, Latin American “roots”.

Withчины, по которым появляется волчья пасть, многообразны.
it:

  1. Defective genes. The main “culprit” is considered to be the TBX-22 gene, but
    scientists also say that causing a cleft palate can and
    other parts of the DNA molecule: the MSX1 and MSX2 genes, TCOF, VAX1, NOG and
    many other. ATероятность передачи такого гена от одного из
    parents – only 7%.
  2. Insufficient provision of tissues of the developing fetus
    with oxygen. it бывает, если мать не бросила курить, принимать
    alcohol or continues a course of blood-lowering drugs
    pressure that is not resolved during pregnancy (and this is easy,
    because usually 2-6 weeks after conception, not every woman
    guessing about pregnancy). Also, fetal hypoxia will have
    place if pregnant has anemia, cardiovascular
    diseases if she developed severe toxicosis.
  3. Chemical factors:
    • work with pesticides;
    • lead exposure;
    • petrol;
    • nitric oxide;
    • herbicides;
    • defoliants;
    • organophosphate insecticides (for example,
      �”Dichlorvos”);
    • epoxides;
    • arsenic.
  4. Biological factors – diseases that manifested or
    worsened in the first trimester of pregnancy. Especially dangerous are sharp
    viral, bacterial and infections caused by protozoa:

    • rubella;
    • cytomegalovirus;
    • measles;
    • herpes simplex virus;
    • toxoplasmosis;
    • malaria;
    • chickenpox;
    • parotitis.
  5. Mental trauma, stress.
  6. Beats in the lower abdomen, surgery on the lower sections
    belly.
  7. Withем лекарств, которые могут вызывать уродства плода (то есть
    have a terratagenic effect):

    • sodium compounds;
    • anticonvulsant drugs;
    • anti-cancer agent Metrotrexat;
    • anti-acne based on vitamin A derivatives
      (Roaccutane, Aknekutan);
    • synthetic hormones of the adrenal cortex;
    • tranquilizer “Diazepam” (it is also called “Sibazon”,
      «Реланиум», «ATалиум»).
  8. Unbalanced diet with inadequate intake
    the body of folic acid, which is contained in fruits and greens,
    zinc, copper or manganese.
  9. Obesity.
  10. ATозраст старше 35 years old.
  11. Increased body temperature: due to infectious
    diseases, overheating in the sun, in the sauna or bath, work in
    hot shop or in the laundry.
  12. ATибрация на производстве, где работает женщина.
  13. Uterine fibroids, whose node presses on the developing fetus.
  14. ATещества, которыми женщина пыталась вызвать самопроизвольный
    miscarriage.
  15. Little and plenty of water.
  16. ATоздействие на беременную ионизирующего (например, для лечения
    cancer, or if it works in a nuclear power plant),
    рентгеновского излучения, УATЧ- и СATЧ-токов.
  17. Hypervitaminosis A combined with an increase in blood levels
    adrenal hormones (for example, at high levels of male
    sex hormones, what causes acne, and acne treatment
    preparations based on vitamin A).
  18. A long time from ovulation to the merger of the egg with
    sperm.
  19. AT 25% случаев причина расщелины нёба остается неясной.

Not always cleft palate with splitting lips or without it.
Often this facial defect is only one of the “kit” in the composition.
following syndromes:

  • ATан дер ATуда – когда ребенок рождается с заячьей губой и
    cleft palate, pitted on one or two lips;
  • Stickler: besides cleft palate and lips there is myopia and
    joint damage;
  • Lois-Ditz – with a combination of cleft palate, lips, uvula
    a large distance between the eyes and an aortic aneurysm;
  • Hardikar: cleft lip, palate plus hydronephrosis and impairment
    intestinal patency;
  • Patau – when in cells there are three thirteenth chromosomes.
    The syndrome is characterized by a large number of defects. development
    (нарушение development отделов мозга, пороки сердца и внутренних
    bodies), due to which most babies die in the first
    weeks and months of life.

However, scientists believe that the influence of genetic causes –
only 10-15%. The rest is due to external influences on
pregnant woman listed above.

For the occurrence of a cleft palate, one or more
these factors arose in the period from 2 to 11 week
fetal life just when it happens
facial structure formation. Namely, during this period the woman is not
suspects her pregnancy and does not limit admission
drugs, alcohol or continues to smoke.

How the palate works and why it is needed

The palate is a plate that separates the nasal cavity.
from the mouth, because they must perform different functions, and if
you can still breathe through your mouth, food getting into your nose is a very
dangerous.

The palate consists of two parts:

  • hard palate – the area formed by the bone (this bone is one
    parts of the upper jaw);
  • soft palate – a department located closer to the throat. is he
    consists of muscles, their tendons (aponeurosis) and glands.

How does face formation occur?

Once the egg has merged with the male gamete, it begins
its division. ATначале получившиеся клетки одинаковы, но со 2 недели
внутриутробного development зародыш становится двух-, а потом
three-layer, and each of these layers is different in structure and
functions: skin and hair are formed from one layer
plot, from another – internal organs, from the third – bones,
muscles, vessels at each site. And on the 4th week of intrauterine
development в области головы, где уже начинает развиваться мозг,
there are 5 protrusions of all three layers: an unpaired frontal appears
tubercle, 2 maxillary and 2 mandibular. is heи движутся навстречу
each other:

  • maxillary processes go to each other, but do not connect
    among themselves: the gap between them fills the process of the frontal tuber,
    which grows downwards. Using this plot and
    maxillary tubercles forming the outer part of the nose;
  • mandibular – to each other: so it turns out the lower jaw and
    underlip;
  • maxillary and mandibular processes, growing on the sides and
    connecting to each other, form the cheeks, the side of the upper
    jaws and lips.

From the outer layer is formed skin of the face, oral mucosa, enamel
teeth, glands that produce saliva. Middle layer becomes
progenitor of the bones of the face, the muscles of the face, as well as those under
enamel tooth parts.

At week 7, the nose is separated from the mouth by the “primary palate”, from
parts of the upper lip, teeth and front
hard palate. But from the 7th week the “secondary palate” is formed – from
palatal processes that grow from the inside
maxillary tubercles. At first they grow vertically, but when
the tongue appears shifted down, the palate begins to grow horizontally
and its parts are interconnected.

The process of face formation to the 11th week of life is ending. If a
connection of embryonic processes is broken, clefts appear
faces. This can happen in any one place, or
simultaneously in several and even all of them. For example, cleft
the palate is formed if the processes of the maxillary and
frontal tubercle. And if the maxillary and mandibular
processes, formed pathologically large mouth. To do this,
to any (or several) of teratogenic factors
подействовали на 2-11 неделе development ребенка (особенно если есть
genetic predisposition). Especially dangerous are considered
3-6 weeks of life.

Disease classification

Cleft palate can be:

ATид Which means Picture
Hidden Split only muscles of the soft palate, mucous membrane
whole
 волчья пасть скрытая
Incomplete Splintered only soft palate and partly hard  волчья пасть неполная
Complete The cleft is marked both on the hard and on the soft palate – to
cutter hole
 волчья пасть полная
Pass-through

Splintered and soft, and hard palate, as well as a portion of the upper
jaws where teeth grow.

Can be single or double sided

 волчья пасть сквозная

Symptoms of cleft palate

симптомы волчьей пастиIf a расщелина нёба у ребенка
affects only the soft palate and is not accompanied by a hare lip,
The disease is not immediately diagnosed. You can pay attention
only that the child eats strangely: he gets tired of sucking quickly,
periodically takes breaks, may refuse to eat.

If a расщелина неполная, родители узнают об этом с первых минут
life of the baby: he is breathing heavily, short-term sucking, while he
choked and milk excreted from the nose. With полной или сквозной
its cleft is already visible at first glance at the child, his breath
difficult and sucking impossible. Such a child may be
immediately after delivery in intensive care, which is associated with hit
amniotic fluid in the respiratory tract.

What is dangerous wolf’s mouth

Children with cleft palate are painful, since the air they breathe
passes into the oral cavity, where the “not provided” system for
moisturizing it and warming it. This leads to frequent occurrence.
заболеваний дыхательных путей и внутреннего ear.

Deformation of the palate also causes speech impairment: the sound received
when the vocal cords vibrate, it penetrates not only into the mouth, but also into
nose, gets nasal shade.

Often there are otitis because fluid and food get into
through the cleft of the palate into the auditory tubes. Due to chronic inflammation
ear deteriorates hearing until its complete loss. ATыход есть – это
installation on the eardrum drainage tubes through which
liquid will be released outside, and not stagnate.

The fluid can also get into the sinuses, which leads to
the development of sinusitis.

Due to a defect in the palate, sucking is difficult. AT результате ребенок
lacks nutrients and fluids, which leads to a delay
his physical (to a greater extent) and mental (to a lesser extent)
development. AT дальнейшем деформация зубов и нарушения прикуса
complicates the process of chewing, which exacerbates the situation and leads to
the development of diseases of internal organs. This requires
special devices for feeding, or installation of temporary
�“Artificial palate” – obturator.

ATозникают и проблемы непосредственно с зубами: их часто поражает
caries.

Cleft palate uncorrected in early childhood,
leads to the child’s social and psychological problems –
those relating to appearance. It affects the formation
self esteem, behavior and social skills.
This is especially true for girls, even small ones – age 3-5
years old. According to a survey of such children, they felt sadness, anger, fear
or alienation from their peers. AT подростковом возрасте
boys become aggressive, anxious or depressed.
Social life also suffers in girls who become
closed and can make a lot of mistakes in relation to
opposite sex.

Diagnostics

Determination of the presence of wolf mouth can be in the intrauterine
period, but at this stage of pregnancy (16-24 week), when abortion
can be performed only by strict indications, for example, if
кроме расщелины нёба имеются другие пороки development, опасные или
incompatible with fetal life (this sets out specifically
collected consilium). In addition, no sonologist (the one who
does ultrasound), even when examining the palate in Doppler mode, not
vouch for the accuracy of the diagnosis of “wolf mouth”. This diagnosis
set only after the birth of the child – on the basis of inspection
oral cavity.

If a у данной супружеской пары уже рождался ребенок с расщелиной
palate or lips recommended before planning the next
pregnancy apply for medical genetic
counseling.

Treatment

The only treatment for cleft palate is surgery. But up to a year
it can be performed only if there is a defect only soft
palate (the operation is called veloplasty): stitching a hard palate
повлечет за собой остановку его development, в результате разовьются
deformation of the skull.

AT любом случае, до проведения оперативного вмешательства нужна
preparing the child – so that he could gain weight, gain
nutrients not getting sick. For this you need to go through a few
stages.

Preoperative preparation

is heа заключается в:

  • psychotherapeutic assistance to the parents of the child;
  • organization of proper feeding;
  • prevention of colds;
  • ear disease prevention;
  • dental and speech therapy.

Psychotherapy for parents

is heа проводится для родителей ребенка, которые зачастую винят
yourself in what happened. ATрачи соответствующей специальности беседуют с
parents, explaining that such a defect is well corrected with
using the operation. If a есть необходимость, они прибегают к
various psychotherapeutic techniques.

Feeding organization

It is very important that food and drink do not get into the nose. For this
take measures corresponding to the degree of the defect. So, with small
Defects may apply or special nipples on the breast, or
special nipples that are put on the bottle (preferably all
to feed the baby with expressed mother’s milk, which contains
times more nutrients than mixtures) and will cover
cleft palate.

AT случае больших дефектов применяют зонд – трубку, которую
injected into the nose, and which will reach the stomach. Such a probe
installed only in the hospital, they are taught to use. Once in 3
weeks the probe needs to be rearranged to reduce the risk associated with
him complications. As soon as possible, such children need
set obturator – a plate that will cover
defect.

Prevention of colds and diseases of upper respiratory tract

Until the operation becomes possible, the child with
cleft palate:

  • must have a monthly ENT doctor every month when
    no complaints. With наличии выделений из носа, ушей, повышении
    body temperature – urgently contact the same doctor;
  • не должен ходить в сад при малейших признаках ОРATИ;
  • undergo tempering procedures;
  • on scheduled inspections, especially in the cold season, to come
    in mask;
  • monitor the purity of the nose of the baby – so there was no time left
    food residue.

AT некоторых случаях ЛОР-врач может порекомендовать установку
special tube, which will prevent infection mean
ear.

Dental care

Children with cleft palate need to start brushing their teeth from the moment
their appearance using a special silicone brush on the finger. AT
year you need to visit the dentist, if necessary – to hold
orthodontic correction using plates, braces or others
devices that advise the orthodontist.

Classes with speech therapist

From the age of two, children with cleft palate need periodic training.
speech therapist to learn the correct pronunciation of sounds and
breathing. On the recommendation of a speech therapist at home with the child are engaged
parents.

Operation

ATид вмешательства определяется видом дефекта. Usually with a wolf
two types of surgery are applied to the mouth: veloplasty (crosslinking of soft
palate) and uranoplasty (elimination of the defect of hard palate).

ATелопластика

It is recommended to perform with 8 months of the child’s life (you can do
in the period from 1 to 2 years), while he is still not trying to really speak:
a later surgery may result in
speech defects. It allows you to restore swallowing and breathing.
baby

It is an operation to suture a soft palate defect.
pre-harvested triangular fabrics taken from
mucous membrane of the oral cavity.

This intervention is considered not very traumatic, therefore
after 2-3 days you can enter the power supply instead of the probe supply
in the usual way. When this succeeds, the probe is removed, and the child
write out.

Uranoplasty

The purpose of such an operation is to lengthen the soft palate and narrow the middle
department of the pharynx. If the defect is large, the intervention is carried out in
several stages. The first stage can be carried out in 6 months.
Additional interventions are performed from 2 to 4 years.

In case of blind crevices, the first stage is performed in 3-5 years. With
through unilateral defects intervention is performed in 5-6
years old. If the defect is bilateral, usually you need 2 stages with a difference in
half a year

Optimal interventions up to 6 years, until the child
went to school. After 6 years cosmetology can be carried out.
surgeries and cosmetic manipulations (for example, removal of scars
laser). It happens that the complete elimination of the cleft palate ends
only 18 years old.

Uranoplasty состоит из таких этапов:

  • cuts on both sides of the cleft with detached mucous
    shells;
  • a Z-section is applied, with the help of which the sections are joined
    – the defect is closed;
  • cutting off the nasal mucosa from the hard palate with
    moving her back;
  • stratification of muscle tissue from the sides of the pharynx and moving it to
    middle;
  • stitching on the nasal mucosa, then on the muscles and
    nasal mucosa;
  • stitching on hard palate areas;
  • treatment of the palate antiseptic;
  • overlaying the plate on the palate to protect against infection.
    The plate is attached with a bandage, which is fixed on the head
    operated on.

After operation

After the stage of the operation, the child for some time
(on average, about 3 weeks) is in the hospital. First 2-3
days you need to comply with bed rest, and about 10 days to comply
silence. Pain medication, antibiotics,
every 2-3 days spend bandaging, make sure that not
there was an accumulation of mucus, saliva and blood in the region of the palate (to
then it did not fester).

From 5 days, massage of the seam is prescribed.
antiseptic: the index finger of the parent or the child
stroking and rubbing, then perform zigzag movements
along the mucous membranes in the place where the hard palate is connected to
soft You will also need to do some exercises,
aimed at providing anatomical conditions for development
speech. It is painful to perform such exercises, therefore parents should
follow the correctness and regularity of its implementation.

After discharge home parents need to contact the clinic,
where will appoint rehabilitation measures:

  • classes with a speech therapist, including improved articulation,
    development of soft palate, nose breathing;
  • performing special exercises;
  • speech therapy massage;
  • physiotherapy;
  • feeding with an orthodontic nipple with a slit.

Assess how a wolf’s mouth will look before and after surgery
possible only after 3-4 weeks, when the swelling subsides
reconstructed tissue. The scar on the face will shrink back in
2-3 months, and later it will be possible
correct by laser.

Complication of operations

Such situations may occur as complications of the operation:

  1. seam divergence;
  2. flap necrosis;
  3. the formation of coarse or keloid scars;
  4. softness of the palate;
  5. shortening of the soft palate.

Contraindications to surgery

Both uranoplasty and cycle plastic are contraindicated in:

  • congenital heart defects – before their correction;
  • other severe malformations of internal organs;
  • anemia;
  • pneumonia.

Автор: Кривега Мария Салаватовна врач-реаниматолог

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