Cleft palate, cleft lip - everything is fixable

Layout of congenital facial clefts: A – Transverse facial cleft;
B – Median cleft of the upper lip; B – Oblique facial cleft; D – Lateral cleft of the upper lip; D – Cleft lip We continue the series of publications devoted to neurosurgical diseases. The project was prepared jointly with the MBOO for helping children with neurosurgical diseases “He Needs You.” We want these “terrible” diagnoses not to put pressure on parents and not to take away their hope. In our publications, the best specialists and experienced parents will talk about ways to cure and overcome, give recommendations and show that life can go on with any, even such complex, diseases. “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.

Reasons for the development of the defect

Why are people born with a cleft lip? The formation of the maxillofacial system is influenced by many factors. The development of the defect can be caused by such unfavorable reasons as:

Genetic predisposition. If one of the child’s parents suffered from this defect, the probability of its occurrence in the fetus is about 7%.
Adverse chemical effects on the body of a pregnant woman at the beginning of pregnancy. Such influences include smoking, drinking alcohol and drugs, taking medications and dietary supplements, unfavorable environmental conditions, and eating food contaminated with chemicals.
Viral and bacterial diseases, as well as STDs.
Adverse physical effects on the body of the expectant mother, for example, injuries, hypothermia and overheating of the body, exposure to radiation.
Oncological diseases (in particular, a tumor developing in the uterine area).
Multiple and late pregnancy (over 40 years).
Stress and emotional distress.
Obesity.
Severe toxicosis in the 1st trimester.
Poor nutrition during pregnancy, insufficient intake of vitamins and minerals.
Diabetes mellitus of the expectant mother.
Anemia and diseases of the cardiovascular system.
Complicated pregnancy, accompanied by uterine bleeding, threat of miscarriage.

Read about methods for diagnosing Down syndrome in a child here.

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.

Facial anatomy

Lips are skin-muscular formations that are located around the entrance to the oral cavity on the anterior surfaces of the jaws. They are divided into upper and lower, and together they form the oral cavity.

Lips are formed in several layers:

cutaneous - numerous subcutaneous blood vessels are visible through it, giving the lips a pink color;
loose connective tissue - it contains a large number of choroid plexuses, sebaceous glands and nerve fibers;
muscular - it is predominantly represented by the orbicularis oris muscle; when various parts of its fibers contract, the lips can close, press against the teeth and move forward, and also when the fibers contract, the oral fissure opens; This layer also includes some facial muscles, with the help of which the lips can change position, thereby expressing various human emotions and feelings;
mucous - it lines the inner surface of the lips and on their outer surface passes into the skin layer. The zone of this transition is called the lip border, which, in turn, has a bright red color due to the highly translucent vessels. A large number of excretory ducts of the salivary glands of the upper lip emerge onto the surface of this layer.

The upper jaw is a fairly massive paired bone that is involved in the formation of the oral cavity, nose and eye sockets. Its front surface is covered by the upper lip. The body of the upper jaw is represented by a hollow bone with a rather large air sinus, which is called the maxillary (or maxillary). It has a connection with the nasal cavity through a wide opening.

There are also four bone processes in the upper jaw:

frontal - participates in the formation of the nasal cavity, fused with the frontal bone;
palatine – takes part in the formation of the hard palate;
alveolar - equipped with dental cells designed to attach eight teeth;
zygomatic – fuses with the zygomatic bone.

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

Characteristics of the pathology

Cleft lip in a child - photo:

A cleft lip is usually called a congenital defect of the bones of the maxillofacial system , manifested in the form of a cleft in the upper lip. The cleft can have different sizes, most often through it you can see the oral cavity.

In some cases, the depth of the cleft is quite significant; it can reach the nasal cavity.

The defect can be unilateral (the cleft appears on the right or left side), or bilateral (appears on both sides at once), in most cases there is a cleft that occurs in the middle of the upper lip.

Differences from cleft palate

Cleft lip is often accompanied by other malformations of the maxillofacial apparatus. The most common cases are when, along with a cleft lip, a child also has a cleft palate - a more serious problem that provokes serious problems with breathing, swallowing, and speech.

The differences between these two ailments lie in the location of the defect.

Thus, with a cleft lip, the pathological process involves the soft tissues of the maxillofacial system, and in some cases, the bone tissue of the upper jaw.

The cleft palate is a cleft located in the area between the nasal cavity and the palate .

How dangerous is the disease?

The defect, in addition to aesthetic problems, is accompanied by such phenomena as:

Difficulty swallowing.
Dental disorders . If the problem is not eliminated before the baby's first teeth begin to emerge, this may lead to the absence of some teeth, or, conversely, to the appearance of extra ones.

Over time, the child’s bite becomes disturbed, and this is fraught with problems such as impaired digestion of food (since the child cannot chew it well), tooth sensitivity, and a tendency to form caries.

Speech impairment . The child cannot pronounce certain sounds correctly, and his voice becomes nasal.
Hearing impairment, possible otitis media.
Difficulties in adaptation . The child experiences psychological problems related to his appearance.

Diagnostics

It is not difficult to make a diagnosis after the birth of a child; all that is required is a visual examination of the newborn.

In this case, the child will also need consultations with an ENT doctor.

This is necessary in order to determine whether there are any other problems (for example, cleft palate, abnormalities in the structure of the nasal cavity).

Signs of a cleft lip can be recognized in the prenatal period. This can be done using an ultrasound as early as 14 weeks of pregnancy. However, in order to finally confirm the diagnosis, you will need to gather a medical consultation.

This is very important, since this pathology is the basis for termination of pregnancy.

Of course, the decision in this case is made only by the woman herself, but at the legislative level, abortions at this stage in the presence of pathology are permitted (in normal cases, artificial termination of pregnancy is prohibited after 12 weeks).

Methods for correcting cleft lip

The cleft lip defect is completely eliminated using the following plan of basic surgical interventions and additional treatment.

Performing primary cheiloplasty eliminates a cosmetic defect and restores damaged integrity.
Primary rhinocheiloplasty is performed when it is necessary to correct a cleft lip defect associated with an abnormal arrangement of the cartilage of the nose and the muscles of the mouth.
Rhinocheilognatoplasty - solves problems of lip integrity, eliminates deformations of the nasal cartilage, and allows the formation of a muscular frame in the mouth area. A technically very complex operation, it is performed only in the absence of severe deformities of the nose, lip and facial skeleton.
Speech therapy treatment is necessary to restore speech function.
Cosmetic plastic surgery is used to eliminate residual defects of treatment.

The results of the operations can be assessed after 12 months.

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.