Cleft lip in children: surgery, causes,treatment

Update: October 2018 Born lip called birth defect
development of the bones of the facial skull when the child’s upper lip
there is a gap through which the cavity can be clearly viewed
mouth; cleft can lead to the nasal cavity. Be observed such
the defect may be on one, two sides or over the place where it should
be middle of the lip. Often hare lip, or, as is customary
to speak, cleft of the upper lip, combined with noncanceration of the palate –
�“Wolf mouth”, but in every fifth case it is combined with more
severe malformations.

ATозникать заячья губа может вследствие большого количества
reasons. AT некоторых случаях – это дефект генов, который может
inherited, but often these are damaging factors
affected the fetus during the formation of its face. Pathology
not life-threatening, but leads to malnutrition,
speech, hearing. it – причина частых инфекций уха, неправильного роста
and caries of teeth.

The disease in different regions occurs with different frequency: in
the best case is 1 infant from 2500, but sometimes – and 2 children from
1000. More often boys suffer. Treatment – только операция, которая
held at the age of 3-6 months of the child. After surgery
cleft lip removal are carried out activities:
classes with speech therapist, dentist, pediatrician. They must completely
end to 6 years of age, not to complicate the child addictive
to society.

Why there is a cleft upper lip, what dangers
threatens, as well as what conditions are needed for the operation,
read on.

Where does the hare lip come from

You can better understand how facial vices are formed if
consider how from 1 maternal and 1 paternal cell is obtained
multicellular embryo, and how it acquires a face.

So, after the merger of the egg and the male cell, one
a cell that begins to divide by 2, then, 4, and further cells,
and they are all the same. AT начале деления клетки становятся все
less, but after a time, the volume of the embryo begins to grow. Through
time, as the cell mass grows, the following also occurs.
significant event: the cells from which the body is built
developing person become different. They form 3
layer:

  • internal – endoderm, from the cells of which subsequently
    intestines, liver, lungs, pancreas are formed;
  • external – ectoderm, giving rise to the skin, nails, hair,
    nervous system and sense organs;
  • medium – mesoderm, from which muscles, bones develop,
    vessels, heart, genitals and kidneys.

At week 2 of life between the forming brain and the site, from
which heart will be formed, the impression is formed – primary
mouth. It connects to the cavity inside the endoderm, which is called
primary intestine. So формируется пищеварительный канал.

4 weeks on the one and the other side of the primary mouth
longitudinal impressions appear – gill arches. They appear on 4
pieces on each side and they go deeper so that
structures similar to bumps. Front ends I and II of gill arches
split into several processes, resembling petals.
From the first branchial arch, which gives rise to the whole face, they depart
five:

  • 1 nasoloby process;
  • 2 maxillary;
  • 2 mandibular.

Between the nasolobic and maxillary processes there is a gap,
where there will be eye sockets later. AT промежутке между верхне- и
the mandibular processes will form a mouth, and with their
joints in the lateral parts of the cheeks are formed. From 1 gill arch
the front third of the auricle will also be formed.

The first to move to each other are the frontal and maxillary
�“Petals”, then cheeks, upper and lower jaws are formed: from
ectoderm forms skin, mucous, salivary glands, dental
enamel; from the mesoderm – the bones and muscles of the face, the inner parts of the teeth.
If a сращения отростков не происходит, что может случиться в любом
one or more places, the face is modified. Severity
this change varies from a small cleft in the upper lip to
completely disfigured face.

Starting from 4 to the end of the 8th week of intrauterine development
these processes must be joined, but their complete union
occurs up to 11 weeks inclusive. It turns out that in the period from
second for 11 weeks the embryo is extremely sensitive to damaging
factors that can affect him through the mother. And what
the sooner a harmful circumstance takes effect, the more severe the vice will be.
But the damaging factor leads to the formation of vice only then
if he will act until the end of the formation of the face. The most
the period from 3 to 6 weeks is considered dangerous for the person, and just this
time, the expectant mother usually does not know about her pregnancy, continuing
lead a normal life with smoking, drinking alcohol or
habitual drugs.

Causes of cleft lip

The reasons due to which there is a hare lip, differ
diversity, so that they are divided into groups:

  1. Genetic background. It is believed that the main gene that
    causes The development of this disease is called TBX-22. Soже это
    can make and many other genes: MMP3, BMP4, VAX1, pTCH1 and
    others. If a один родитель имеет расщелину губы, вероятность, что
    he will give this disease to the child, is only 7%.
  2. ATредные химические воздействия на материнский организм в I
    trimester (they account for 22.8% of cases). it:

    • smoking;
    • alcohol;
    • addiction;
    • medication: anticonvulsant, lowering arterial
      pressure, hormone drugs, drugs containing sodium,
      salicylates, insulin;
    • exposure to pesticides, lead, mercury vapor, insecticides,
      organophosphates, harmful environmental factors,
      epoxides, arsenic, benzols;
    • food intake: tetrazine, sodium cyclomate, aromatic
      hydrocarbons.
  3. ATредные биологические воздействия на организм развивающегося
    fetus in the first 11 weeks of its formation (by this factor
    accounted for 5% of the reasons):

    • вирусные заболевания: ОРATИ (особенно если при этом повышается
      fever), herpes, rubella, cytomegalovirus infection,
      epidemic parotitis, chicken pox;
    • diseases caused by some protozoa,
      for example, toxolasmosis or malaria;
    • infections that a woman “acquired” through sexual contact: chlamydia,
      gonorrhea, syphilis.
  4. Physical effects on a pregnant woman (2% of causes):
    • injuries (especially a blow to the lower third of the abdomen),
    • freezing,
    • radiation exposure
    • vibration in the workplace where a pregnant woman works,
    • overheating in production (hot shop, laundry), in the bath,
      sauna,
    • uterine tumors (mostly fibroids) that interfere with the fetus
      develop normally
    • multiple pregnancy,
    • adhesions or “strings”, which are formed from fruit
      shells.
  5. Stress factors that increase blood levels
    adrenaline, and it has a teratogenic (disfiguring) effect on
    fruit: quarrels, fright, overwork.
  6. If a эта беременность произошла после 40 лет, особенно если до
    This woman did not lead the most “righteous” way of life.
  7. Inadequate or unbalanced nutrition for pregnant
    women, when the diet lacks protein, folic acid,
    zinc, manganese, copper or, on the contrary, a lot of vitamin A.
  8. Obesity in the mother.
  9. Anemia (a small amount of hemoglobin) in the mother, about
    whom she did not receive treatment.
  10. Diseases of the cardiovascular system in the mother.
  11. Severe toxicosis.
  12. The threat of miscarriage or uterine bleeding during
    of pregnancy.
  13. Diabetes.

Maxillofacial surgeons conducted numerous studies.
which proved that a hare lip at only 10-15% is
hereditary disease. The remaining 80-85% is due to the fact that
impact on the fetus occurred immediately from several sides, while
perhaps he was genetically predisposed to the development of this particular
facial defect.

itт дефект не всегда возникает изолированно: в некоторых
cases of cleft lip – only one of the multiple defects that
formed in the child in the prenatal period. it:

  • синдром ATан дер ATуда, причина которого – особое изменение гена
    IRF4. it наиболее частое заболевание, при котором возникает заячья
    lip and cleft palate. It is characterized by the appearance of crevices in the lip,
    the palate in conjunction with the development of pits on one or both lips;
  • Lois-Dietz syndrome, in which the child is not only present
    cleft lip, but also damage to the palate, and a forked tongue, and a large
    distance between eyes and aortic aneurysm;
  • Stickler syndrome in which cleft and lips develop, and
    palate, as well as myopia and joint pain;
  • Hardicard syndrome, in which there is a combination of the wolf’s mouth,
    cleft lip with impaired intestinal, hydronephrosis
    kidneys.

What is dangerous disease

If a ребенок родился только с заячьей губой, а твердое нёбо
left holistic, this leads to the following violations:

  • at the age of one when the child eats only liquid food
    it is difficult for him to suck and swallow; food may enter the nasal cavity
    and this requires special tricks on feeding, sometimes even
    setting the probe – the tube that will lead from the nose to
    stomach;
  • if it is impossible to perform the operation when they begin to form
    teeth, this may lead to the lack of necessary teeth or
    the appearance of their additional units;
  • teeth begin to grow at the wrong angles – broken
    bite. Suffers from this as chewing food and, accordingly,
    its digestion worsens, and caries often occurs;
  • disrupted sound production: sound wave, falling
    in the nasal cavity, makes the voice nasal, and speech – fuzzy, with
    problems in the pronunciation of consonants;
  • hearing problems occur;
  • often otitis;
  • cleft lip, even mild – this is significant
    cosmetic defect, which will make it difficult for the child
    adapt in children’s society

ATот почему заячья губа, даже если она не сильно выражена, должна
be surely operated on. And it needs to be done before the year to
have time to go through the necessary rehabilitation activities before becoming
speech and prior to the socialization of the child.

Disease classification

Cleft lip can be:

ATид Subspecies Which means
Unilateral cleft lipодносторонняя заячья губа Complete Cleft goes from lip to nose
Incomplete Cleft only affects lip
Hidden Only the muscles are split, and the mucous and the skin above them are not
changed
Double-sided hare lipдвусторонняя заячья губа Complete Defect goes from lip to nose
Incomplete Defect is only within lip mucosa
Symmetrical The defect is the same on both sides
Asymmetrical Fissure on the one hand more, on the other – less

The hare lip can be on one upper, one lower lip or
immediately on both of them.

The classification is used when choosing the method of operational
correction.

Symptoms

Signs of this disease are visible immediately after birth.
it:

  • a defect in the upper (more common) or lower lip;
  • it may look like a small gap in the red part of the lip,
    and manifest a significant divergence of lip tissue from the lip to
    nostrils or even in general – entering the nasal cavity;
  • can be from one or two sides (in the second case, lip
    consists of three fragments);
  • through this gap in the lip is often visible mucous membrane
    upper jaw.

Diagnostics

The diagnosis of “cleft lip” in children is placed after birth – on
Based on external examination only. Held in parallel
ENT examination – to determine if there is a defect in the nose, on
hard or soft palate.

The cleft of the upper lip is usually seen still on the ultrasound scan performed in
14-16 weeks or later, but the first planned ultrasound is usually performed
earlier – from 12 to 14 week. In addition, no, even the best
a sonologist examining the fetus with ultrasound is not
vouches for the fact that the child has formed a hare lip. itт
a diagnosis before delivery can be made only by a consultation of doctors, and usually
it’s about this when this developmental disability encourages a woman
make a decision about abortion. RF legislation abortion
a fetus that is older than 12 weeks is possible only if
cleft lip, there are other life-threatening malformations
fetus.

Anatomy of the affected area

Рассмотрим вкратце, какие ткани страдают при заячьей lip. So
the amount of work to be done will be clearer
the surgeon.

The lip is a complex skin and muscular formation. Outer layer
consists of three parts:

  • skin that contains mucous and sweat glands;
  • skin goes into the intermediate part, which has already
    somewhat different structure, and rich in blood vessels (therefore
    has a red color);
  • the intermediate part goes into the mucous membrane, which
    contacts directly with the teeth.

Under the mucosal outer layer is a loose layer
connective tissue, and below it – the circular muscle of the mouth and several
other muscles. Under the lip are the gums – the mucous membrane,
which covers the bone of the upper and lower jaw.

The lower jaw is a solid bone. It consists of a body on which
there are cells for the teeth, and processes that connect to
the skull. ATерхняя челюсть устроена сложнее: на ней не только есть
cells for the teeth, this bone extends higher and forms the entrance to
nasal cavity, as well as maxillary sinus.

Treatment

заячья губа до и после операции

Cleft lip before and after surgery

Eliminate the hare lip will only surgery. It is recommended
perform up to a year, and better – from 3 to 6 months after birth. If a
it is impossible, you can do it later – the main thing is that all
treatment of a small patient, including further postoperative
rehabilitation activities (classes with a speech therapist, wearing
orthodontic appliances) ended by 6 years.

Хирурги Масгрейв и ATилельмессен, усовершенствовавшие
surgical intervention for the correction of cleft lip back in 1969
year, introduced the “rule of 10”, in which the operation is possible:

  1. the child must be at least 10 weeks old;
  2. baby weight should not be less than 10 pounds (4.5 kg);
  3. hemoglobin in his blood should not be less than 10 g / dl (i.e. not
    less than 100 g / l).

AT нашей стране операции по устранению расщелины губы обычно
held in 6 months and later. Here are added conditions such as:
sufficient weight gain of the child, no pathology
bowel, nervous and cardiovascular systems.

Preoperative period

Before starting the operation, the child needs to receive
nutrition. How to feed a newborn with such a vice depends on
type and extent of the defect.

So, если расщелина неполная и односторонняя, можно будет
breastfeed (optimally), and from the bottle, while holding
child not in a supine position, but half-sitting or vertically
(“Soldier”).

If a дефекты глубокие, но твердое нёбо не задето, придется
refuse breastfeeding in favor of special nipples
(they are produced, for example, by NUK and Avent), which are worn on
a bottle in which is poured a mixture or breast milk. This pacifier
you need to push as far as possible into the mouth of the child, to the root of his tongue.
If a ребенок не может сосать из-за маленького отверстия в этой
nipple, it can be expanded with a thick needle, previously
decontaminated using fire.

If a же заячья губа сочетается с расщелиной нёба, то кормить
can only be through a probe. It is installed in the nose in the nursery
hospitals or maternity hospital, after which the mother must learn correctly them
to enjoy.

It is recommended at least in the preoperative period
feed the baby with breast milk if it is not deficient
enzymes or other contraindications. Only female milk
contains the necessary substances for the formation of immunity, digestion and
other processes occurring in the body of the child.

Operation

To correct a cleft lip, 1 out of three can be performed.
types of operation.

Chiloplasty

This intervention is performed when cleft is present only in
lip. One of the three techniques for performing this may be used.
operations. Her choice depends on the shape of the labial cleft:

  1. The method of a triangular flap. At the same time from the tissues of the damaged
    lips a triangle is created which is set so that
    lengthen lip tissue and make them symmetrical. As a result
    The intervention forms a transverse scar between the mouth and the nose.
  2. Method with the formation of a quadrangular flap. It applies
    when cleft is strongly pronounced.
  3. Linear method. It is suitable for correcting small defects.
    lips, because it does not allow to fill significant deficiencies
    tissues.

If the lip is split on both sides, then cheiloplasty is performed
or as the first stage of the operation, after which the second stage
correct the defect of the nose, or apply immediately rhinoheyloplasty.

Here’s how a hare lip looked before and after cheiloplasty:

Rhinoheyloplasty

This intervention involves the correction of a complete cleft lip,
when the cartilage of the nose and the muscles of the mouth were affected. AT
In this case, the cartilages of the nose are released from the skin and subcutaneous tissue, their
set in the correct position and fixed. Thereafter
sewn upper lip tissue. After a while, if the defect was
significant, you may need to re-operation.

Operation заканчивается установкой тампона в носовой ход. it
Cotton-gauze device will prevent the ingress of
the nose of the food, as well as the narrowing of the nasal passages. For 2-3 days tampon
извлекается, и взамен устанавливается полихлорвиниловая (ПХAT)
tube, the purpose of which is to prevent the narrowing of the nose and its deformation
wings.

Rinoheilognathoplasty

it вмешательство применяется для воссоздания правильного
the position of the lip, nasal cartilage and hard palate. ATмешательство
difficult, traumatic. After it may some time in the mouth
стоять тампоны, различные ПХAT- и пластиковые фиксаторы.

Postoperative rehabilitation

It is carried out in three stages:

  1. AT стационаре – сразу после operations. Here spend
    anesthesia, prevention of suppuration of injured tissues,
    feeding and correction of water-salt balance. On the face of a child
    put on a special device that will prevent
    the divergence of the seams when moving lips. On the pens of the baby can be worn
    tire for 3 weeks which will impede combing and
    damage them seams.
  2. With the help of specialists clinics. itт этап начинается сразу
    after discharge, when mom should come with the child to the district officer
    the therapist at the place of residence, and he will write to which specialists and
    how often you need to walk, what physiotherapy procedures
    to visit and what pills to take. On a polyclinic stage
    are held:

    • Work with a speech therapist – if the consequences are prompt
      intervention for speech cannot be eliminated up to 3 years. Then
      the speech therapist should be involved in the formation of the baby’s speech, and complete these
      classes need to be up to 6 years old when the child is to pour into
      school team.
    • Treatment by an orthodontist for the correction of bite. It consists in
      wearing various braces, cap or plates. The method depends on
      specific situation and financial capacity of the family.
    • Treatment by an audiologist if the child has problems with
      by hearing.
  3. At home, when classes with the child are less
    intensity, are periodically visited by the ENT, orthodontist and
    Dentist therapist, speech therapist.

Until complete elimination of speech disorders, occlusion,
side of the respiratory and digestive systems of the child
established disability.

If the postoperative scar has turned ugly, it can
adjust with a laser beam. Also subsequently may
perform additional corrective interventions for
eliminate irregularities smile

Автор: Кривега Мария Салаватовна врач-реаниматолог

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