Premature closure of cranial sutures in children. The growth of the cerebral skull. Points of ossification of the skull

A.V. Lopatin, S.A. Yasonov, Department of Oral and Maxillofacial Surgery, State Institution "Russian Children's Clinical Hospital" of Roszdrav

Almost any doctor of the course of general anatomy remembers the existence of specific forms of the skull, such as scaphocephalic (extended in the anteroposterior direction) and brachycephalic (enlarged in width). But rarely does anyone recall that the unusual shape of the skull in a child in many cases is a sign of premature infection of the cranial sutures.

Of course, all doctors are familiar with the term craniostenosis - a premature healing of the cranial sutures, leading to nonspecific damage to the brain due to insufficient expansion of the cranial cavity during the period of the most active brain growth. When the question arises about how craniostenosis is treated, few remember about the possibility of surgical excision of prematurely overgrown sutures and only a few know about the existence of the two-flap craniotomy method.

Meanwhile, according to international statistics, premature closure of one of the sutures of the skull (isolated craniosynostosis) occurs in approximately one in 1000 children. It is interesting to note that the same frequency is typical for children with a cleft lip. At the same time, it is not difficult for anyone to diagnose cleft lips, because every doctor has undoubtedly seen such patients. Whereas almost none of the general practitioners can remember whether he has ever seen a child with premature scalp sutures.

The Road to Calvary

Craniosynostosis is a premature infection of one or more sutures of the skull, leading to the formation of a characteristic deformation of the head. Thus, already in the maternity hospital, a child with suspected craniosynostosis can be isolated from the total mass of newborns and sent for further examination. In practice, unfortunately, at this stage, all the deformities of the skull found in children are regarded by doctors as features of the postpartum configuration of the head and they are not given due attention. During the neonatal period, the shape of the skull is also not given much importance. The usual response of a pediatrician to parents' concerns: “… It's okay, he is gaining weight well, jaundice is gone, and the head is so much because it lies on its side. When he starts walking, everything will disappear. "

Psychomotor development of children lags behind, deformities of the skull do not spontaneously disappear, some deformities become less noticeable, hiding under the hair, others are mistakenly regarded by doctors as other diseases, and still others recede into the background in the presence of more obvious violations of the functions of organs and systems. Often, babies with craniosynostosis are consulted by genetics, and often a group of diseases is correctly established and even a direct genetic syndrome is assumed. Despite this, a few of these patients are admitted to specialized clinics for treatment. The vast majority of untreated children have reduced intelligence and become disabled. Due to the unusual shape of the skull, the proportions of the face are disturbed, and by the period of puberty, such children more often than others have difficulties in social communication and even suicidal attempts are possible.

Parents gradually stop paying attention to slight deformities of the skull, and if a child has a pronounced facial deformity, pediatric surgeons explain to them that correction of cosmetic defects is carried out only at the age of 16.

Based on the foregoing, we can conclude that in our country there is practically no qualified assistance for children with premature infection of one or more of the sutures of the skull. At the same time, all over the world, over the past forty years, great attention has been paid to the treatment of children with congenital deformities of the skull, and the developed methods of surgical treatment make it possible to eliminate brain compression and significantly improve the appearance of children with craniosynostosis already at the age of three to six months. The main reason for this gap is the lack of available information on the features of diagnosis and treatment of congenital skull deformities in children. In order to slightly correct the existing situation, we propose to consider the most general issues of diagnosis and treatment of craniosynostosis.

Diagnostics

The main sutures of the cranial vault are sagittal, coronary, lambdoid and metopic (Fig. 1). With premature overgrowth of the bone suture, compensatory bone growth occurs perpendicular to its axis (Virchow's law). As a result, a characteristic deformation appears. Let us describe the most common forms of craniosynostosis.

Sagittal craniosynostosis

Premature fusion of the sagittal suture leads to an increase in the anteroposterior size of the skull with overhanging frontal and occipital regions and to a decrease in its width with the formation of a narrow oval face (Fig. 2). This type of deformity is called scaphocephaly, or scaphoid skull. This is the most common disease among the total number of isolated synostosis (50-60%). The characteristic shape of the skull is visible from birth. When examining the head from above, the retraction of the parietal regions is noticeable, this gives a sensation of a circular constriction of the cranial vault at the level or slightly posterior to the auricles. The large fontanelle is clearly defined, and its size does not differ from the norm. The presence of a bone ridge, palpable in the projection of the sagittal suture, is considered characteristic.

: a - child before and b - after elimination of scaphocephaly.

Metopic craniosynostosis

The rarest representative of the group of isolated craniosynostosis is metopic craniosynostosis, or trigonocephaly, which makes up 5-10% of their total number. Despite this, this disease is, perhaps, most often recognized as a congenital deformity of the skull due to the characteristic clinical picture.

With early closure of the metopic suture, a triangular deformity of the forehead is formed with the formation of a bone keel extending from the glabella to the greater fontanelle. When looking at such a skull from above, a clear triangular deformation is visible with the apex in the nasal region. In this case, the upper and lateral edges of the orbits are displaced posteriorly, which gives the sensation of turning the plane of the orbits outward and decreasing the interorbital distance (hypotelorism). The deformity of the forehead is so unusual that children with trigonocephaly are often examined by geneticists and are observed as carriers of hereditary syndromes accompanied by a decrease in intelligence. Indeed, trigonocephaly is considered an integral part of such syndromes as Opitz, Oro-facio-digital syndrom, and some others. It is also true that many syndromic diseases lead to a delay in intellectual development, but their frequency is so low, and the clinical picture is so characteristic that it is not necessary to classify all children with only metopic synostosis as a risk group for the development of mental disability.

Unilateral coronary craniosynostosis

The coronal suture is located perpendicular to the median axis of the skull and consists of two equal halves. So, with the premature infection of one of its halves, a typical asymmetric deformation is formed, called plagiocephaly. The view of a child with plagiocephaly is characterized by a flattening of the superior orbital edge of the orbit and the frontal bone on the side of the lesion with compensatory overhang of the opposite half of the forehead (the baby seems to be frowning on one side of the face). With age, the ipsilateral flattening of the zygomatic region and the curvature of the nose in the same direction begin to appear more clearly. At school age, the bite deformity is associated with an increase in the height of the upper jaw and, as a result, a displacement of the lower jaw on the side of the prematurely closed suture. In severe cases, there is even a compensatory swelling of the occipital region from the side of synostosis. Disturbances on the part of the organ of vision are represented most often by unilateral strabismus. Plagiocephaly is more often than others regarded as a feature of the postpartum configuration of the head. But unlike the latter, it does not disappear in the first weeks of life, but, on the contrary, progresses with age.

Thus, the shape of the skull plays a huge role in the correct diagnosis.

Of the instrumental diagnostic methods, the best is computed tomography with three-dimensional remodeling of the image of the bones of the cranial vault and face. This examination helps to identify concomitant brain pathology, to confirm the presence of synostosis in the case of an isolated injury, and to establish all interested sutures in the case of polysynostosis.

Treatment

The most active period of brain growth is considered to be the age of up to two years. Thus, from a functional point of view, craniostenosis can be prevented by early surgical treatment. The optimal age for surgery for craniosynostosis can be considered the period from 3 to 9 months. The benefits of treatment at this age include:

• ease of manipulation with the thin and soft bones of the skull;
• facilitating the final remodeling of the skull shape by a rapidly growing brain;
• more complete and faster healing of residual bone defects.

If the treatment is performed after five years, it is doubtful that it will lead to a significant improvement in brain function. To a greater extent, the operation will be aimed at eliminating the deformation of the head.

The main feature of modern surgical treatment is not only an increase in the volume of the skull, but also the correction of its shape and combined deformity of the face during one operation.

Once again, we draw the attention of our readers to the fact that it is better to play it safe and send a child with a skull deformity to a specialist than to skip the pathology. The Department of Oral and Maxillofacial Surgery of the Russian Children's Clinical Hospital has accumulated extensive experience in the diagnosis and treatment of these conditions in children. Little patients are admitted to the department from all regions of Russia. In order to get an appointment with a specialist, you need to go to the hospital's advisory department. It is desirable to have a referral from the regional health department. If the child really needs surgical help, a plan of examination is outlined and a voucher for hospitalization is issued, which indicates the date of admission to the hospital and a list of all necessary documents. The length of stay in the clinic is 34 weeks on average. Children can be in the ward with one of the parents. After the treatment, all children up to the age of 18 are under the supervision of the doctors of department 1.

Summarizing the above, we note once again: in our country there is a vast stratum of patients who, due to the low awareness of doctors about the modern possibilities of diagnosing and treating craniosynostosis, do not receive adequate assistance. Meanwhile, the diagnosis of such conditions is quite simple and possible already in the early stages. Timely qualified assistance to children with craniosynostosis allows in the first months of life to eliminate not only the functional deficit, but also to correct the accompanying cosmetic deformity.

Andrey Vyacheslavovich Lopatin, Head Department of Oral and Maxillofacial Surgery, State Institution "Russian Children's Clinical Hospital" of Roszdrav, Professor, Dr. med. sciences

Sergey Aleksandrovich Yasonov, doctor of the Department of Maxillofacial Surgery, State Institution "Russian Children's Clinical Hospital" of Roszdrav

1 More detailed information can be obtained on the website: www.cfsmed.ru

There is a definite connection between the growth of the cerebral skull and the appearance of digital impressions on the inner surface of his bones, although the mechanism of their occurrence is still not fully understood. They are first detected at the age of 1.5-2 years in the region of the parietal bones, then in the occipital area and only by 7-8 years in the frontal area. Digital impressions reach their maximum severity in puberty, they do not change until 35-40 years old, and after 40 years they gradually smooth out. After 15 years, the severity of these anatomical formations in various parts of the cerebral skull is as follows: occipital, temporal, parietal, frontal; respectively as 10: 7: 7.

Some observations testify that digital impressions are more pronounced in children with delayed mental development, and their absence is an important symptom of impaired osteogenesis and is usually accompanied by cortical atrophy. In persons over 50 years of age, digital impressions persist only in 2-2.5% of cases, which reflects a decrease in the level of bone transformation processes.

The large fontanelles close during the first 1.5-2 years of postnatal period... Their longer existence may be a sign of such a common childhood disease as rickets, and in more serious cases, an increase in intracranial pressure.

Sutures of the cerebral skull gradually narrow - in a newborn, their diameter is 10-11 mm, and in a child of 7 years - about 2 mm. However, bone-forming activity at the edges of the seams continues for a very long time - up to 25-30 years on average, gradually decreasing in intensity after 20 years. Therefore, although by the end of the second year of a child's life, his cerebral skull is formed essentially in the same way as that of an adult, the growth of the skull, however, continues.

Complete closure of the gap cranial sutures marks the end of the physiological growth phase of the skull bones. On the inner surface of the skull, the sutures are closed earlier than on the outer, sometimes with a difference of 7 to S years. In this case, calcification of the edges of the seam is not an indicator of its obliteration. According to G. Friedman, R. Shapiro and A. Janzen, the closure of the suture occurs in several of its sites and does not always correspond to age. It is only known that in men the sutures are obliterated earlier and more evenly.

Closing the sutures of the cerebral skull, according to R. Shapiro and A. Janzen, occurs at the following age: sagittal suture - at 22-26 years old, coronary - at 29-30 years old, lambdoid - at 26-45 years old, parietomastoidal - at 30-40 years old, sphenotemporal - even by the age of 60. Individual mild fluctuations in the level of bone formation and the duration of the existence of sutures create different shapes of the skull, and premature closure of all sutures can lead to the development of pathological forms of the brachycephalic type of skull.

Unlike the rest of the bones facial and cerebral parts of the skull, its base is formed by bones of cartilaginous origin. Ossification of the latter begins as early as the 3rd month of the prenatal period from two growth centers in the basisphenoidal and occipital regions and later the emerging third center in the presphenoidal zone. Synchondroses are formed near the centers of ossification, which are homologues of the epiphyseal growth zones and represent the last rudiments of primordial cartilage in the human skeleton. Before the birth of a child, the indicated zones of ossification merge and only layers of synchondrosis remain between them.

To the moment birth the main growth occurs in the basisphenoidal and basicoccipital areas. During the first year of life, another growth center appears in the nasal septum - the mesoetmoidal one. With the beginning of its function, the crista galli and the upper half of the nasal septum ossify. The duration of the existence of this growth center is not exactly known. As pointed out by N. Ford, filed by various anatomists, the fusion of the specified growth center with the center located in the main bone occurs at the age of 12-25 years. The cartilaginous layers between the mesozmoidal growth center and the surrounding bones of the facial and cerebral skull (frontal, lateral ethmoid bone masses) begin to gradually ossify at 2-6 years of age.

Thus, a small child at the base of the skull there are four growth centers: spheno-occipital and sphenomesoetmoidal synchondrosis and cartilaginous layers between the ethmoid and frontal bones and in the frontal bone itself.

The skull of a newborn baby is made up of six separate bones: two parietal, two temporal, occipital, and frontal. Between them are thin layers of fibrous elastic tissue - cranial sutures. Areas where several bones of the skull join are called fontanelles or soft spots.

Sutures are necessary so that during the passage through the birth canal, the bones of the skull can partially overlap. This makes childbirth easier and the risk of brain damage to the newborn is reduced. The closure of fontanelles occurs during the first year of a child's life.

In 1 out of 1000 children, intrauterine or early overgrowth of cranial sutures is found - craniostenosis. Pathology is more common in boys. There are three forms of it:

• monosynostosis (the most common type) - one suture is closed - metopic, sagittal, coronary or lambdoid;
• polynostosis - more than two sutures are overgrown,
• pansynostosis - more than three sutures are missing.

Craniostenosis in newborns can be an isolated anomaly or part of a syndrome (20% of cases) and be combined with other malformations.

Causes

The reasons for the development of complete or partial craniostenosis have not been reliably established. The development of pathology is based on a violation of the formation of the bone tissue of the skull, which begins at the end of the first month of intrauterine development. The failure is assumed to be due to one of the following factors:

• hormonal imbalance;
• intrauterine infections (flu, herpes, rubella);
• genetic pathologies (a defect in the gene encoding fibroblast growth factor receptors);
• squeezing the fetal head in the uterus.

Normally, by the fifth month of pregnancy, the fetal skull bones should be well formed and separated by sutures. If craniostenosis occurs, the functioning of the brain is disrupted and ophthalmic pathologies develop.

Symptoms

The severity of the symptoms of craniostenosis depends on how many sutures are closed and at what time. With intrauterine growth, the clinical picture is more pronounced.

The main external manifestation of pathology is the deformation of the skull, which occurs due to compensatory bone growth perpendicular to the affected seam (Vikhrov's law). The peculiarities of the deformation are determined by which seams are prematurely closed. There are three types of pathology:

• scaphocephaly - overgrowth of the sagittal suture, which divides the head in the parietal part into the right and left halves;
• plagiocephaly - closure of the coronary suture, which runs perpendicular to the sagittal suture from ear to ear;
• trigonocephaly - closure of the metopic suture dividing the forehead into two equal parts;
• brachycephaly - overgrowth of the lambdoid and coronary sutures.

Sagittal craniostenosis (scaphocephaly) is the most common type of disease. The width of the skull is reduced, its length in the longitudinal (anteroposterior) direction is increased. The occipital and frontal bones overhang. The temples seem to be depressed. The child's face has a narrow oval (scaphoid) shape. Neurological symptoms are uncommon for scaphocephaly.

Plagiocephaly can be frontal (frontal) or occipital, as well as unilateral or bilateral. The most commonly observed unilateral frontal coronary craniostenosis. In this case, the skull is deformed asymmetrically. On the one hand, the forehead thickens, the orbit and eyebrow are raised, the auricle protrudes, the nose is bent, the zygomatic region is flattened, the jaw is shifted, and so on. Morphological changes occur as the child grows up. Strabismus develops.

Brachycephaly is characterized by an increase in the transverse diameter of the skull and a decrease in its height. Supplemented with ophthalmological and neurological signs.

Trigonocephalus is accompanied by a wedge-shaped protrusion of the forehead forward - a bony keel is formed, going from the large fontanelle to the glabella. The deformity is combined with hyptellorism and visual impairment.

Other possible signs of craniostenosis (usually manifest after 12 months):

• hydrocephalus;
• intracranial hypertension;
• headache;
• nausea, vomiting;
• convulsions;
• delayed intellectual development;
• meningeal symptoms.

Diagnostics

Craniostenosis is diagnosed based on:

• collecting anamnesis and analyzing symptoms;
• visual examination of the patient's skull and craniometry (measuring its parameters using a tape);
• feeling fontanelles;
• neurological and ophthalmological examinations;
• fundus examination to assess intracranial pressure;
• radiography;
• CT or MRI of the brain.

These methods make it possible to reveal the absence of cranial sutures, intracranial hypertension, ophthalmic pathologies, changes in the structure of the brain and other disorders.

Craniostenosis is differentiated from deformities of the skull, which did not arise due to synostosis of the sutures. In addition, the purpose of the diagnosis is to determine the accompanying developmental defects of the child.

Treatment

How is craniostenosis in a child treated? The main method of therapy is surgery. Its purpose is to enlarge the cranial cavity and give it an anatomical shape by dissecting the fused sutures or remodeling the bones of the vault using distraction devices.

The operation for craniostenosis is preferably carried out at 6-9 months. Up to 2 years of age, there is an active growth of the brain. Therefore, intervention at an early age allows achieving regression of all pathological manifestations, including eliminating the deformation of the cranial and facial bones. The effectiveness of the surgical treatment of craniostenosis is evidenced by photographs after the operation.

After 2-3 years, surgery has practically no effect on the functions of the brain and visual analyzer. The result is an improvement in the appearance of the child.

It is possible to do without surgery for craniostenosis if isolated scaphocephaly is observed without neurological disorders.

Forecast

The consequences of craniostenosis depend on the severity of the deformities and the number of affected sutures. An isolated form of pathology has a favorable prognosis, subject to surgical intervention. Without surgery, craniostenosis in adults leads to complications such as:

• persistent pronounced change in the shape of the skull;
• headache;
• periodic seizures;
• strabismus.

Syndromic craniostenosis is usually associated with disability.

Prevention

The main directions of prevention of craniostenosis:

• correction of disturbed hormonal levels in a pregnant woman;
• prevention of the development of intrauterine infections;
• medical genetic counseling.

The decisive role in the formation and subsequent development of the skull belongs to the brain, teeth, masticatory muscles and sensory organs. In the process of growth, the head undergoes significant changes. In the course of development, manifest age, sex and individual characteristics of the skull... Let's take a look at some of them.

Newborn

The baby's skull has a specific structure. Connective tissue fills the spaces between the bone elements. Newborns are completely absent sutures of the skull. Anatomy this part of the body is of particular interest. There are 6 fontanelles at the junction of several bones. They are covered with connective tissue plates. There are two unpaired (posterior and anterior) and two paired (mastoid, wedge-shaped) fontanelles. The frontal is considered the largest. It has a diamond shape. It is located at the point of convergence of the left and right frontal and both parietal bones. Due to the fontanelles, it is very elastic. When the head of the fetus passes through the birth canal, the edges of the roof overlap in tiles. Due to this, it decreases. By the age of two, as a rule, sutures of the skull. Anatomy previously studied in a rather original way. Medieval physicians applied hot iron to the fontanel area for diseases of the eyes and brain. After scar formation, doctors used various irritants to induce suppuration. So they believed that they were opening the way for the accumulating harmful substances. In the configuration of the seams, the doctors tried to make out the symbols and letters. Doctors believed that they contained information about the fate of the patient.

Features of the structure of the skull

This part of the body in a newborn is distinguished by the small size of the facial bones. Another specific feature is the fontanelles mentioned above. In the skull of a newborn, traces of all 3 unfinished stages of ossification are noted. The fontanelles are the remnants of the membranous period. Their presence is of practical importance. They allow the bones of the roof to move. The anterior fontanelle is located in the midline at the site of the junction of 4 sutures: 2 halves of the coronal, frontal and sagittal. It overgrows in the second year of life. The posterior fontanelle has a triangular shape. It is located between the two in front and the scales of the occiput bone in the back. It overgrows in the second month. In the lateral fontanelles, wedge-shaped and mastoid are distinguished. The first is located at the site of convergence of the parietal, frontal, temporal scales and the greater wing of the sphenoid bones. It grows in the second or third month. The mastoid fontanelle is located between the parietal bone, the base of the pyramid in the temporal lobe and the occipital scales.

Cartilaginous stage

At this stage, the following age-related features of the skull are noted. Cartilaginous layers are found between separate, non-fused elements of the base bones. The sinuses are not yet developed. Due to the weakness of the musculature, the various muscle ridges, tubercles and lines are poorly expressed. For the same reason, associated, in addition, with the lack of chewing function, the jaws are underdeveloped. Hardly ever. In this case, the lower jaw consists of 2 non-fused halves. Because of this, the face protrudes little forward relative to the skull. It is only 1/8 of it. At the same time, in an adult, the ratio of the face to the skull is 1/4.

Displacement of bones

Skulls after birth are manifested in the active expansion of the cavities - nasal, cerebral, oral and nasopharynx. This leads to a displacement of the bones surrounding them in the direction of the growth vectors. The movement is accompanied by an increase in length and thickness. With marginal and superficial growth, the curvature of the bones begins to change.

Postnatal period

At this stage, they manifest themselves in the uneven growth of the facial and brain regions. The linear dimensions of the latter are increased by 0.5, and the first by 3 times. The volume of the brain region doubles in the first six months, and triples by the age of two. From the age of 7, growth slows down, in puberty it accelerates again. By the age of 16-18, the development of the vault stops. The base increases in length up to 18-20 years and ends when the sphenoid-occipital synchondrosis closes. The growth of the facial section is more continuous and even. Bones grow most actively around the mouth. Age features of the skull in the process of growth, they are manifested in the fusion of parts of the bones, separated in newborns, differentiation in the structure, pneumatization. The relief of the inner and outer surfaces becomes more contoured. At an early age, smooth edges form on the seams, by the age of 20, serrated joints are formed.

Final stages

By the age of forty, obliteration of the sutures begins. It covers all or most of the connections. In old and old age, osteoporosis of the cranial bones is noted. Thinning of the plates of the compact substance begins. In some cases, thickening of the bones is observed. Jaw atrophy becomes more pronounced in the facial region due to the loss of teeth. This leads to an increase in the angle of the lower jaw. As a result, the chin comes forward.

Gender traits

There are several criteria by which a male skull differs from a female one. These signs include the severity of roughness and tuberosity in the areas of muscle attachment, development of the occipital protuberance, protrusion of the upper jaw, etc. The male skull is more developed in comparison with the female. Its outlines are more angular due to the severity of roughness and tuberosity in the areas of attachment of the chewing, temporal, occipital and cervical muscles. The frontal and parietal tubercles are more developed in women, in men - the glabella and the brow ridges. in the latter, the lower jaw is heavier and larger. In the area of ​​the lower edge and corners of the inner part of the chin, tuberosity is clearly pronounced. This is due to the attachment of the digastric, masticatory and pterygoid muscles. The shape of the human skull also differs depending on gender. In men, a sloping forehead is noted, which passes into a rounded crown. Often there is an elevation in the direction of the swept seam. The forehead of women is more vertical. It passes into a flat crown. In men, the eye sockets are lower. They are usually rectangular in shape. Their upper edge is thickened. In women, the eye sockets are located higher. They are close to an oval or round shape with sharper and thinner upper edges. On the female skull, the alveolar process often protrudes forward. The nasal angle in men is clearly expressed in most cases. On the female skull, the frontal bone to the nasal bone passes more smoothly.

Additionally

The shape of the human skull has no effect on mental capacity. According to the results of numerous studies of anthropologists, it can be concluded that there is no reason to believe that the size of the brain region prevails in any race. Bushmen, pygmies and some other tribes have slightly smaller head sizes than other people. This is due to their small stature. Often, a decrease in head size can be the result of poor nutrition over the centuries and the influence of other adverse factors.

Craniostenosis is understood as premature infection of the cranial sutures, which contributes to the limitation of the total volume of the skull, its deformation and provokes development.

The disease is diagnosed with a frequency of 0.5-1 cases per 1000 newborns, mainly in boys.

Pathology can act in isolation or in combination with abnormalities in the development of other organs and systems (as a component of the Apert, Pfeiffer syndrome), often accompanied by impaired development of the brain.

Etiology and pathogenesis of the disorder

Sutures are thin layers of fibrous tissue located between the cranial bones and forming fontanelles (“soft spots”) when they merge. They allow the skull to change its configuration as the child passes through the birth canal, thereby preventing traumatism during childbirth.

Normally, fontanelle closure occurs by the end of the first year of a child's life. With their premature overgrowth and early ossification of the seams, a picture of craniostenosis is observed, the severity of which directly depends on the moment of onset of the disease.

If the sutures are closed in the prenatal period, the deformation of the skull will be significant, if after birth it will be less pronounced.

Pathogenetically, the main role in the development of the disease belongs to impaired blood circulation and metabolism in bone tissue, which can be triggered by the following factors:

• genetic mutations and hereditary conditions;
• infections transferred in utero (rubella, flu, herpes, etc.);
• the impact on the fetus of unfavorable environmental factors (bad habits of the mother, household and industrial toxins, radiation, taking certain medications);
• mechanical compression of the fetal head in the uterine cavity;
• giving a wrong position to a newborn, immobility in infancy, limitation of motor capabilities;
• birth injuries and diseases suffered in the first months of life.

Varieties of abnormal development

Depending on the number of sutures involved in the pathological process, craniostenosis is distinguished:

• simple(closing only one seam),
• combined(overgrowth of several),
• complicated(all cranial sutures are involved).

In addition, the disease is subdivided into the following forms:

The nature of the clinical picture

Closing only one suture is usually a cosmetic defect.

With a combined lesion, the clinical picture is much brighter, it exhibits a significant deformation of the skull characteristic of each type of craniostenosis and neurological symptoms (due to an increase in intracranial pressure, which is associated with a disproportionately slow growth of the skull, insufficient for the volume of the brain):

Eye symptoms present:

• bilateral exophthalmos (bulging eyes);
• stagnant changes in the retina;
• decreased or loss of vision.

Mental disorders are variable, lethargy or increased excitability may appear, mental development, as a rule, is slowed down.

Establishing a diagnosis

To make a diagnosis, it is necessary to carry out a comprehensive comprehensive examination. In addition to clarifying complaints and anamnesis of the disease (the time of the onset of the main symptoms, their progression), it is important to collect the obstetric anamnesis of the mother (especially the course of pregnancy, diseases suffered during this period, harmful effects, complications in childbirth).

A complete examination of the child is mandatory, the structure of the skull, the shape and size of the head are studied in detail, the degree of closure of the sutures and fontanelles is assessed by palpation.

Neurological examination reveals cerebral and focal signs of brain damage (pathological reflexes, nystagmus, gaze paresis, etc.).

The ophthalmologist assesses the degree of visual disturbances (congestive changes in the fundus, edema of the optic nerve head, its atrophy, exophthalmos). Genetic research helps to establish the hereditary nature of the disease.

In addition, a number of instrumental diagnostic methods are performed to determine the further tactics of patient management:

Given the peculiarities of childhood, most invasive studies are carried out under drug sedation. This allows you to avoid excessive stress in the child and get a more diagnostically significant result.

Modern surgery

With premature closure of the sutures, mainly surgical treatment is used, the main purpose of which is to increase the volume of the skull and create favorable conditions for the normal development of brain structures.

There are a huge number of surgical techniques depending on the type of craniostenosis - linear craniotomy, flap bilateral, circular, resection of the cranial vault and others.

The technique of the operation consists in performing a soft tissue dissection and bone grafting. During the intervention, skull defects are corrected and intracranial hypertension is eliminated.

It is believed that the operation is advisable until the age of three, when the most intensive formation of brain structures in a child is noted. Completed in a timely manner, it allows you to prevent compression of the brain structures and almost completely levels the previous symptoms of the disease, including a gross cosmetic defect.

Surgical treatment is not indicated in the case of:

A relative (temporary) contraindication is an acute disease or chronic in the acute stage, purulent infections of the skin, recurrent inflammation of the upper respiratory tract.

In the postoperative period, a course of physiotherapeutic procedures and symptomatic drug therapy is shown, as well as long-term observation.

Forecast and prevention

Early diagnosis and timely surgical intervention can prevent the development of serious consequences. In this case, the prognosis for the life and normal development of the child is favorable.

Loss of vision, mental retardation, constant headache and gross deformation of the skull complicate the course of the pathology and lead to disability.

For prevention purposes, it is shown:

• medical and genetic counseling for couples from risk groups;
• limiting the impact on the body of a pregnant woman of unfavorable external factors;
• quitting smoking and other bad habits;
• prevention and timely treatment of diseases of the expectant mother and newborn.

And most importantly, if an infant develops symptoms of concern, parents need to urgently consult a specialist. Timeliness of treatment will allow avoiding the terrible consequences of craniostenosis and will give the child the opportunity for a normal life.