“Once a doctor at a children’s clinic wrote us a diagnosis of “cleft lip, cleft palate,” I told her: “You seem to be a doctor, why do you write so illiterately? I’m not diagnosing you with “chicken brains.” After that, I tore out the page and moved on to another doctor. Stand up for your children, you must do this” (from correspondence on the forum).
Unfortunately, in our country, facial clefts are diagnosed in 1 out of 500-1000 newborns and almost always cause parents to feel guilty and fearful. In fact, facial clefts have been successfully operated on for a long time.
Colloquially, a cleft palate is often referred to as a “cleft palate” and a cleft lip as a “cleft lip.” However, in the professional world this is not accepted - confirms the head of the 1st surgical department of the St. Vladimir Children's City Clinical Hospital, Doctor of Medical Sciences, Professor Dmitry Yurievich Komelyagin .
– We never call our patients and their diseases that way. People are very upset to hear this. These features are called "congenital unilateral cleft lip, alveolar ridge and palate" and "congenital bilateral cleft lip, alveolar ridge and palate".
Facial clefts are divided into median and lateral. The most common clefts are median cleft lip and cleft palate. Incomplete clefts are called non-through clefts - they can involve only the uvula, or the uvula and the soft palate or partially the hard palate and end behind the incisive foramen.
Clefts in which the gap from the hard palate extends to the alveolar process and upper lip are called complete, or through.
“Why did this happen to me?!”
Dmitry Yuryevich Komelyagin
- Dmitry Yuryevich, there is a myth that facial clefts are a problem for children from disadvantaged families.
- No! We have many families with prosperous, beautiful parents whose children are faced with such a problem. The risk factor, rather, will be the fact that the parent himself was a carrier of this pathology in childhood. We have such families. Let's say we once operated on a girl with a cleft lip, she got married and gave birth to a child with exactly the same pathology, they came to us again. We had families in which the second and even third child was born with a similar pathology. And we successfully operated on all of them.
– How many years have you been operating?
– The department was founded 26 years ago. We have been operating for 26 years now. Facial clefts are a multifactorial problem, and it is still difficult to identify specific causes. It is known that the cause may be intrauterine fetal hypoxia. What caused this hypoxia? Anything. Stress? Maybe. Mother's infection? Taking medications? Yes, it's possible. But no one can tell you what exactly.
Today, the most substantiated theory is considered to be that the cause of facial clefts is a combination of hereditary factors and adverse environmental influences.
Mutation of several genes entails increased sensitivity to bacteria in the environment. In this case, the cleft may be caused by factors such as medications, x-rays, infections, and even stress and noise. As a result of their exposure, the fetus experiences a lack of oxygen during the few hours it takes for the face to form, and then the fused tissues suffer.
Carmit Bachar
Source: @therealcarmit
The American singer and dancer was one of the vocalists of The Pussycat Dolls. She left the team in 2008. Looking at her attractive appearance, it is difficult to believe that she was born with a defect.
In fact, she was diagnosed with both a cleft lip and a cleft palate at birth. To correct the defect, she underwent several operations. At the beginning of her career, one of the producers turned her down because of the scars on her face. He harshly told her that with such an appearance, the path to show business was closed.
However, Carmit did not give up. She was successful, removed all the scars, and now they are not visible at all in photographs.
Something went wrong…
Around the 4th-6th week of fetal formation, the paired median nasal protrusions fuse. They fuse to form the incisive bone, the philtrum of the upper lip, and the tip of the nose. The lateral elements of the upper lip originate from the paired maxillary processes. If the tissues do not heal for some reason, a cleft lip appears. It usually originates at the junction between the central and lateral parts of the upper lip on both sides.
The secondary palate begins to develop around the eighth week of pregnancy, after development of the primary palate has completed. The paired palatal folds are initially separated by the developing tongue. As the mandible grows, the tongue moves forward and the palatine folds move downward and assume a more horizontal position. If something goes wrong, the paired palatal folds cannot move down and medially - a cleft appears in the palate.
The secondary palate usually develops before the 12th week. Therefore, diagnosis of congenital facial clefts using ultrasound (ultrasound) is optimally carried out at 11–12 weeks of pregnancy (this is when the formation of the baby’s face ends).
A cleft lip can be diagnosed by a specialist if the fetus's face is positioned correctly. Diagnosing the palate using ultrasound is more difficult. However, an experienced doctor can record an atypical movement of the fetal tongue in the lateral projection. In some cases, specialists cannot identify this defect until childbirth if the child covers his face with his arm or leg.
D.Yu.Komelyagin : “We will soon open a consultation center for pregnant women in the St. Vladimir Children's City Clinical Hospital on the basis of the department for newborns, which, among other things, will see maxillofacial surgeons. And if, say, during an ultrasound examination the baby was diagnosed with a facial malformation, the mother will be able to come straight to us for a consultation. We will calm her down, tell her how this or that defect can be cured, explain what awaits her, and most importantly, we will prove that this is not a reason to abandon the child; with the right treatment tactics, you can live a full life even with facial defects.”
Tutankhamun had a cleft palate. This conclusion was reached two years ago by a group of scientists who examined his mummy. The research was led by the Secretary General of the Supreme Council of Antiquities of the Arab Republic of Egypt, Zahi Hawass. Tutankhamun is not the only person whose facial clefts did not prevent him from achieving success and fame.
The face of famous artist Joaquin Phoenix also seems to bear traces of a facial cleft. However, the artist’s biographers claim that the scar on his lip is not a mark from the operation, but a congenital “mark”. However, whether it is a hidden cleft or a cleft at all is not so important. More importantly, the scar from it did not prevent Joaquin from making a brilliant film career.
The same scar on his lip (also visually similar to an operated cleft) did not prevent Cheech Marin from becoming a popular American film artist, Denis Dorokhov from becoming a famous KVN player in Russia, and Denis Lebedev from becoming a world boxing champion.
Andrey Makarevich
Source: @mashinavremeniofficial
The leader of the group “Machine of Time” does not talk about his defect, but in childhood photos the scar above his lip is noticeable. As he grew older, he decided to disguise it with a mustache, and it was a great decision. Now few people realize that Makarevich has a cleft lip.
The future singer spent his childhood in a small communal apartment in the center of Moscow. He entered a music school and took a piano class, but quickly dropped out. He created the group with friends in the 9th grade, and it was she who brought him fame.
When can cleft surgery be performed?
Forms of cleft palate: A – Cleft uvula of the soft palate;
B – Cleft soft palate; B – Cleft of the soft and partially hard palate; D – Complete lateral cleft of the soft and hard palate; D – Complete bilateral cleft of the hard and soft palate D.Yu. Komelyagin : Different clinics adhere to different rules and different timing of the operation. We operate on cleft lip at the age of four months (more precisely, from four to six months) depending on the situation. If the child is hypotrophic, low weight, with low hemoglobin, then it is better to postpone the operation. Should I operate on a cleft before four months? Such an operation is painstaking work; it is important that the baby’s tissues with which the surgeon will work are already mature. In addition, the lungs and heart must mature so that the baby can safely endure anesthesia and the postoperative period. There's no point in rushing. Unless, of course, nothing threatens the child’s life.
Often, facial clefts are signs of one or another syndrome with associated complications (Stickler syndrome, Lowes-Dietz syndrome, Hardikar syndrome, Treacher-Collins syndrome, etc.), including Pierre Robin syndrome, characterized by underdevelopment of the lower jaw and recessed tongue. A child with this syndrome simply cannot breathe normally or breathes only in a certain position (on his side, on his stomach), because the tissues are displaced and block the airways.
Of course, in such cases, early operations are justified. This is what is called “operations for vital reasons” (D.Yu. Komelyagin and the team of the 1st surgical department have a patent for the invention of a compression-distraction device that makes it possible to successfully treat children with Pierre Robin syndrome - Ed.).
– What should parents of a child born with a cleft pay attention to?
- For all. Conduct a full examination. And after that, build a treatment plan together with specialists. Let's say, if he has a heart defect, of course, first of all, you need to deal with the defect, and then treat the cleft.
But if there are no other, more important factors, we recommend surgery for a cleft lip at the age of four months, and a cleft palate at the age of one and a half years. It is more difficult to treat an alveolar cleft. Previously, we proposed to operate on her at twelve years old, now we start at eight. For treatment, bone grafting is performed, that is, the patient’s own bone is used (say, from the lower jaw, tibia) or artificial bone (bone graft).
An important point in the treatment of alveolar cleft is orthodontic preparation. That is, first the orthodontist achieves the correct position of the upper jaw fragments relative to each other and relative to the lower jaw, and then the surgeons begin the correction.
If the operation is successful, patients then return for follow-up visits every six months. The surgeon and orthodontist monitor the formation of scars, the upper jaw, and whether there is any deformation of the nose. Sometimes you have to do surgical correction again. But it is important to make these decisions together with your orthodontist. If the growth of the upper jaw is not complete, surgery can be harmful.
In addition to the maxillofacial surgeon, you may need help from: neurologist, otorhinolaryngologist, orthodontist, dentist, speech therapist, audiologist, phonopedist, psychologist
Mikhail Boyarsky
Source: @boyarskiy_official
The signature mustache made the actor’s image recognizable. Many viewers will not be able to remember what Mikhail looks like with a clean-shaven face. It turns out that Boyarsky is using a mustache to disguise his scar after correcting the cleft lip he was born with.
Despite the defect, the artist was not deprived of the attention of the opposite sex. Before his marriage, he was a ladies' man. Many women could not resist his charm.
Now, depending on the severity, a cleft lip can be eliminated using cheiloplasty using local anesthesia.
Anatomy of the affected area
Let's take a brief look at what tissues are affected by a cleft lip. This will make it clearer the amount of work that the surgeon has to perform.
The lip is a complex musculocutaneous formation. The outer layer consists of three parts:
- skin, which contains mucous and sweat glands;
- the skin passes into the intermediate part, which has a slightly different structure and is rich in blood vessels (therefore has a red color);
- the intermediate part passes into the mucous membrane, which is in direct contact with the teeth.
Beneath the mucocutaneous outer layer is a loose layer of connective tissue, and below that is the orbicularis oris muscle and several other muscles. Below the lip are the gums, the mucous membrane that 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 teeth, and processes that connect to the skull. The upper jaw is more complex: it not only has cells for teeth, this bone continues higher and forms the entrance to the nasal cavity, as well as the maxillary sinus.
Where does cleft lip come from?
You will be able to better understand how facial defects are formed if you consider how a multicellular embryo is formed from 1 maternal and 1 paternal cell, and how it acquires a face.
So, after the fusion of the egg and the male cell, one cell is formed, which begins to divide into 2, then 4 and then cells, all of them identical. At the beginning of division, the cells become smaller and smaller, but after time the volume of the embryo begins to grow. Over time, as the cell mass grows, the following significant event occurs: the cells from which the body of a developing person is built become different. They form 3 layers:
- internal - endoderm, from the cells of which the intestines, liver, lungs, pancreas are subsequently formed;
- external - ectoderm, giving rise to skin, nails, hair, nervous system and sensory organs;
- middle - mesoderm, from which muscles, bones, blood vessels, heart, genitals and kidneys develop.
At 2 weeks of life, a depression forms between the developing brain and the area from which the heart will be formed - the primary mouth. It connects to a cavity within the endodermis called the primitive gut. This is how the digestive canal is formed.
At week 4, longitudinal impressions appear on one and the other side of the primary mouth - gill arches. There are 4 of them on each side, and they deepen so that structures similar to mounds are obtained. The anterior ends of the I and II gill arches bifurcate, forming several processes resembling petals. From the first gill arch, which gives rise to the entire face, there are 5 of them:
- 1 nasofrontal process;
- 2 maxillary;
- 2 mandibular.
Between the nasofrontal and maxillary processes there is a gap where the eye sockets will later be. In the space between the maxillary and mandibular processes, the mouth will form, and when they join in the lateral sections, the cheeks are formed. The anterior third of the auricle will also form from 1 gill arch.
The frontal and maxillary “petals” move towards each other first, then the cheeks, upper and lower jaws are formed: the skin, mucous membranes, salivary glands, and tooth enamel are formed from the ectoderm; from the mesoderm - bones and muscles of the face, internal parts of the teeth. If fusion of the processes does not occur, which can happen in any one or several places, the face changes. The severity of this change varies from a small cleft in the upper lip to a completely disfigured face.
Starting from the 4th to the end of the 8th week of intrauterine development, these processes must unite, but their complete fusion occurs until the 11th week inclusive. It turns out that in the period from the second to the 11th week, the embryo is extremely sensitive to damaging factors that can affect it through the mother. And the sooner the harmful circumstance takes effect, the more severe the vice will be. But a damaging factor leads to the formation of a defect only if it acts until the formation of the face is completed. The period from 3 to 6 weeks is considered the most dangerous for the face, and it is at this time that the expectant mother usually does not know about her pregnancy, continuing to lead a normal lifestyle with smoking, taking alcohol or taking the usual medications.
What diseases do oral and maxillofacial surgeons treat?
It would be a mistake to assume that maxillofacial surgery today is limited only to the correction of congenital malformations of the face. The range of problems solved within the framework of maxillofacial surgery is quite wide.
- Firstly,
inflammatory diseases of the maxillofacial area: abscesses, phlegmon, osteomyelitis of the jaws. - Secondly,
odontogenic and non-odontogenic benign neoplasms of the bones of the facial skeleton and soft tissues of the face (papillomas, fibromas, lipomas, etc.). - Thirdly,
all kinds of defects and disorders of the formation of the dental system and bite.
A special problem in pediatric maxillofacial surgery is the elimination of the consequences of childhood injuries, the sad statistics of which are only growing over the years in all regions of Russia.
What are the most common maxillofacial injuries in children?
Injuries to children in the maxillofacial area can be roughly divided by periods of their lives.
At first, the baby is completely dependent on the parents and can only be injured due to their oversight (for example, falling out of the cradle). Then the child expands his living space: he begins to walk. Therefore, closer to the age of one year, the most common injuries to the soft tissues of the face are due to a fall from one’s own height. By the age of one and a half years, children begin to run, they are actively teething, and as a result of falls or other injuries, damage to soft tissues can already be accompanied by dental injuries of varying severity.
Then a child of 3–4 years old begins to ride a scooter and bicycle, which is fraught with fractures of the bones of the axial and facial skeleton.
Schoolchildren not only often get injured at home, but also become victims of careless handling of pets: cats and dogs, unfortunately, often bite in the nose and mouth. This leads to quite pronounced and extensive defects, and subsequently to deformations of soft tissues, the elimination of which sometimes requires multi-stage plastic surgeries with transplantation of soft and integumentary tissues.
And finally, teenagers between 15 and 18 years of age are more likely to get into road accidents, man-made disasters, come into contact with firearms and explosives, and we have to observe the tragic consequences of severe combined and combined injuries.
Yuri Olegovich Volkov
maxillofacial surgeon, candidate of medical sciences, head of the department of maxillofacial surgery of the Children's City Clinical Hospital named after. G.N. Speransky in Moscow
How dangerous is the disease?
The anomaly affects the aesthetics of appearance and is accompanied by additional problems in the process of life, the main ones:
- Difficulty eating.
- If the problem is not eliminated before the first teeth erupt, then the problem manifests itself in the absence of some teeth or the appearance of extra ones. Over time, the bite becomes disrupted, causing the child to be unable to chew food normally. Tooth sensitivity appears and the risk of caries formation increases.
- Speech function disorder. If there is a cleft, normal pronunciation of individual sounds is impossible.
- The risk of developing otitis media due to hearing impairment increases.
It is difficult for a child to adapt to society. He experiences psycho-emotional stress due to his appearance.
Symptoms
Signs of this disease are visible immediately after the birth of the child. This:
- defect in the upper (usually) or lower lip;
- it can either take the form of a small gap in the red part of the lip, or manifest itself as a significant divergence of the lip tissue from the lip to the nostrils, or even even extending into the nasal cavity;
- may be on one or both sides (in the second case, the lip consists of three fragments);
- through this gap in the lip the mucous membrane of the upper jaw is often visible.
