Premature closure of cranial sutures in children. Craniosynostosis, or premature fusion of the bones of the skull Frontal crest in a child

21.02.2022

Craniostenosis is a pathology that is characterized by premature fusion of several sutures on the skull. Code according to the international classification of diseases ICD-10: Q75.1. If the cranial joints are overgrown in a child at too early an age, this will provoke a violation of the growth of the cranial bones, as well as a deformation of the facial structure of the bones, in particular, the orbits.

At the same time, as the child grows, other bones in the skull will increase in a compensatory manner. At the moment, the reason why the connections in the skull close prematurely is unknown, but doctors have found that this disease is not inherited.

In some cases, craniostenosis is part of a syndrome that can affect several organ systems at once. At the same time, the probability of having a second child in the family who is diagnosed with craniostenosis is extremely low.

Causes of the disease

Among the causes of this disease are:

problems in the development of the child's skull in the first trimester of its bearing;
the presence of genetic mutations;
diseases that affect the fetus inside the womb. These include such diseases of the mother: colds, and others.

Types of disease

There is such a classification of craniostenosis according to the order of prevalence:

fusion of the sagittal suture. A symptom of this type of disease is the elongation of the skull. The head with the sagittal variety of the disease takes on a narrow, elongated shape;
fusion of the metopic suture. This craniostenosis causes pathologies in the structure of the skull, contributing to the development of a triangular protrusion in the frontal region. The distance between the eyes when splicing this connection decreases;
fusion of a coronal or coronal suture. With this type of pathology, the head looks asymmetrical. If there is a thickening of the frontal bone when the connection is closed, then the patient may have asymmetrically located eyes and ears;
fusion of two coronal connections. In this case, the head looks wide in front and flat on the side;
fusion of the lamboid junction. In this case, the patient's head takes a diamond shape. There is also a thickening of the occiput.

The sagittal suture in a child is most often observed in clinical practice. Therefore, parents are advised to know all the symptoms of this disease in order to notice its development in time and seek help.

Symptoms of the disease

Craniostenosis, in addition to deformation of the structure of the bones of the skull, has other symptoms:

changes in the appearance of the face;
premature fusion of the fontanel;
the presence of bumpy protrusions along the seams;
complaints of headaches;
vomit;
memory impairment;
sleep problems (presence of insomnia, night awakenings);
slowdown in development.

Deformation of the bones of the skull can take the following forms:

narrow skull;
broad forehead;
enlarged eye sockets;
reduction of the lower jaw.

Craniostenosis in a child can be treated in the Department of Surgery for Children and Adolescents.

Forms of the disease

Craniostenosis differs depending on the joint that has grown together. There are such forms:

scaphocephaly. This pathology develops when the sagittal suture is fused together. The sagittal connection divides the skull into right and left halves, so its premature fusion causes significant deformations;
brachycephaly. The transverse diameter of the head is increased, and the height of the skull is reduced. It develops in a newborn and an older child when the frontal bone is fused with the temporal;
trigonocephaly. When the metopic suture (a continuation of the sagittal suture) is fused, the forehead protrudes forward in the form of a wedge;
microcephaly. In this case, the skull of a child or a newborn will be noticeably reduced in size. It can develop if all the seams are overgrown.

Treatment of the disease

Craniostenosis requires surgical treatment, but compensated craniostenosis does not require surgery. The main goal of treating a child in the surgical department is to eliminate, which will help to avoid serious consequences, which include loss of vision, as well as mental retardation from peers.

The main goal pursued by the operation is to increase the volume of the cranium, creating favorable conditions for the development of the brain in the future. When a disease is detected in a child under 3 years of age, it is important to promptly begin to treat it, since the greatest brain development is observed during this period of the child's life. Incisions in soft tissues, which involve surgical intervention, should be made very carefully so as not to cause profuse bleeding.

Among the surgical treatment methods are the following:

linear type craniotomy. It is carried out at an early age, while the soft tissues are cut parallel to the fused junction. Also, a surgical operation for this disease involves cutting out the periosteum;
circular craniotomy. It helps eliminate hypertension and is suitable for children over 3-5 years old. In this case, the soft tissues that cover the cranium are cut. Soft tissues near the arteries at the temples are not dissected. The operational process lasts in 2 stages, while the second part of it must be completed in two weeks;
fragmentation. With this type of surgical intervention, surgeons cut the cranial vault. It is carried out for older children, as it has a rather large trauma;
bilateral flap craniotomy. With the help of it, the disease is treated in a decompensated form.

Craniostenosis requires immediate medical attention. Identification of it in the initial stage and the performed surgical procedures can completely remove all symptoms.

Most often, manifestations of craniosynostosis begin as a deformity in the first months of life. In addition to a cosmetic defect, craniosynostosis can lead to an increase in intracranial pressure and has an adverse effect on the development of the child.

normal development of the skull

Scaphocephaly is premature fusion of the sagittal suture.
Plagiocephaly - premature fusion of the coronal suture.
Trigonocephaly - premature fusion of the metopic (frontal) suture.
The terms brachycephaly, oxycephaly, and turriccephaly are used to refer to various forms of synostosis in which different sutures of the skull are affected.

Cause of craniosynostosis

Manifestations of craniosynostosis

Diagnosis of craniosynostosis

Treatment of craniosynostosis

Free consultation on treatment in Moscow and abroad

Craniosynostosis, craniostenosis. Operations to change the shape of the skull in infants.

What is craniosynostosis?

Craniosynostosis, craniostenosis is a rare pathology, which is characterized by a violation of the structure of the skull and skeleton of the child. In children with this pathology, there is a violation of the structure of the facial and brain skull (for example, a narrow skull, narrow forehead). Also, the child may have pathologies of the skeletal structure, for example, fusion of several vertebrae, long and thin fingers and toes, some joints may develop incorrectly, which leads to limitation of movement.

The skull consists of bony plates, which are connected by sutures. Such stitches, as the child's brain grows, begin to increase, and eventually harden. With craniosynostosis, these sutures do not heal properly, and therefore the child's head has an unusual shape.

The severity of the disease depends on which part of the skull is damaged, and how many sutures have grown together incorrectly.

Most often, with this disease, only one suture is damaged and, as a rule, this sagittal suture that runs along the skull. In this case, the child's brain develops normally, however, the head acquires an irregular shape.

When fusion several seams the baby's brain can't grow properly. Moreover, pressure is gradually built up on the child's brain, which can cause blindness and convulsions, retardation in growth and development. If a child has several sutures of the skull that have grown together incorrectly, then he will have multiple pathologies of the face and skull.

Causes of craniosynostosis

Doctors still cannot say with complete certainty what exactly is the cause of the incorrect fusion of the bones of the skull. In 20% of cases, this disorder is hereditary, the child may have one pathological gene from the mother or father.

Most often, this pathology is transmitted genetically in an autosomal recessive manner. However, some scientists believe that this disorder can occur spontaneously, due to the mutation of certain genes, and can be transmitted in an autosomal dominant manner.

Some cases of this pathology can be attributed to the fact that the child during pregnancy lay incorrectly. Other reasons: smoking and drinking in large quantities by the mother during pregnancy.

Manifestations of craniosynostosis.

The most important manifestation of this pathology is the unusual shape of the head, which can be from birth or begin to change in the first months of a child's life, which may be the only sign of craniosynostosis.

In addition, there may be symptoms such as:

* the child does not play;

* sleep apnea at night.

In severe cases, there may be blindness, defects of various kinds, and developmental delays.

Diagnosis of craniosynostosis

Diagnosis is based on the fact that the shape of the child's skull is of an unusual shape; moreover, the child stops growing and developing. Parents and the doctor may notice that the baby's skull shape is abnormal or shortened after birth. Even if a child has a pathological head shape, this does not mean at all that he has this disease. An unusual head shape can occur for a number of other reasons.

During the examination, the doctor will measure the diameter of the child's head. If the shape of the child's head does not change over time, then an X-ray examination or tomography is required, which will give the most accurate answer. Moreover, the specialist will study the child's medical history. It is possible that this disease is transmitted genetically.

In Israel, this procedure is carried out in Tipat Khalyav at each visit, the data is entered into a computer on site and checked against a schedule corresponding to the age of the child. Every month the baby is examined by a doctor in Tipat Freebie, even if his condition does not cause any alarms. If necessary, a referral for examination is issued.

Treatment of craniosynostosis

The usual treatment for an abnormally shaped head in infants is to perform skull surgery.

Remember! Only at the age of up to six months, an operation to change the shape of the skull makes sense - in the future, the changes are cosmetic in nature. In addition to the operation, you may be offered the option of wearing a helmet for 23 hours a day for a child, or courses of physiotherapy, lifestyle changes for the child.

In our clinic, operations of any degree of complexity are performed. When contacting our Top Ichilov clinic, you will receive an answer within two working days, so even with urgent requests you have the opportunity to arrive for treatment as soon as possible. We provide all the necessary documents for the visit.

Fusion of the bones of the skull in children

NEUROSURGERY - EuroMedicine.ru - 2007

Craniosynostosis is a pathology caused by premature overgrowing or congenital absence of one or more cranial sutures, leading to deformity.

With craniostenosis, compression of the brain occurs due to craniosynostosis.

Premature fusion of the bones of the skull can often occur even before the birth of the child, as a result of which the skull cannot expand in certain directions and take the normal shape necessary for the growing brain. Depending on which cranial sutures fuse first, the skull may be elongated from front to back, wide and short, pointed (acrocephaly (oxycephaly or turricephaly)) or asymmetrical.

normal development of the skull

In an adult, the skull normally consists of 28 bones. The flat bones that form the cranial vault are interconnected by sutures.

At birth, a baby's skull is made up of 45 individual bone elements. As they grow, many of these elements fuse into single bones (for example, the frontal bone, which at birth consists of two parts).

The bones of the cranial vault are initially divided into areas of dense connective tissue. At birth, these areas consist of fibrous tissue and are mobile, which is necessary for the newborn and further development. Over time, areas of connective tissue ossify.

There are two types of classification of craniosynostosis: according to clinical manifestations and, accordingly, the affected cranial suture.

Scaphocephaly is premature fusion of the sagittal suture.

Plagiocephaly - premature fusion of the coronal suture.

Trigonocephaly - premature fusion of the metopic (frontal) suture.

The terms brachycephaly, oxycephaly, and turriccephaly are used to refer to various forms of synostosis in which different sutures of the skull are affected.

The cause of craniosynostosis is still not completely known, but the genetic theory of the development of craniosynostosis is currently considered the main one. Similar genetic disorders of the development of the skull occur in three syndromes: Cruson, Apert and Pfeiffer.

In addition to the most visible deformation of the head and face, problems with the respiratory tract can be noted in craniosynostoses, especially in the syndromic form of craniosynostosis. Due to the underdevelopment of the upper jaw, such patients have difficulty in nasal breathing. At night, they may have periods of "apnea" - holding their breath. This affects not only their development, but also behavior and speech. With increased intracranial pressure in children, there is a chronic headache, mental disorders develop, visual impairment. As the child develops, the visible deformation of the skull negatively affects his social adaptation and self-esteem.

Special mention should be made of conditions similar to craniosynostosis. This is the so-called positional plagiocephaly. It is a flattening or deformation of certain areas of the skull. Although there may be some superficial similarity to craniosynostosis, it is important to distinguish these conditions. The fact is that positional plagiocephaly does not require surgical treatment. The treatment of this pathology is simple and consists in a certain laying of the child's head during sleep; in some cases, a special cranial "bandage" may be needed, put on the child's head in order to correct the shape of the skull.

Diagnosis of this disease is, first of all, in the examination by a doctor, as well as in the X-ray of the skull. In addition, methods such as computed tomography and magnetic resonance imaging are used.

Craniosynostosis is usually treated surgically. It consists in separating prematurely fused sutures and recreating the shape of the skull. Often, cosmetic problems require joint treatment with a dentist.

Surgical treatment consists of making a zigzag incision from one ear to the other through the top of the head. The use of this type of incision is due to the fact that the scar remaining after such an incision allows the hairline to look more natural. Leroy clamps are used to stop bleeding in this incision. After the incision, the skin flap (scalp) is folded back. There are holes in the skull. Through these holes, the bones of the skull are sawn, as it were, which makes it possible to divide it into several parts. After the surgeon has reconstructed the shape of the skull, the parts of the skull are put back (usually in a different configuration) and connected together with absorbable sutures, plates and screws. The plates and screws are made of special polymers that dissolve after a year to form water and carbon monoxide. Often, demineralized bone grafts are used to repair a bone defect after surgery. After removing the hemostatic clamps, the skin incision is sutured.

Currently, there are new methods for the treatment of craniosynostosis using endoscopic techniques. They are characterized by significantly less blood loss, swelling, pain, and hospital stay. However, this method of treatment is indicated only for children younger than 6 months. For older children, more intensive treatment is needed.

Craniosynostosis, causes, symptoms and treatment of pathology

Happiness is impossible without health

Especially when it comes to our long-awaited babies. All mothers carefully inspect their treasure during daily care, and any change in the appearance of the baby is of great concern. The greatest fear appears when we see the presence of problems associated with the development of the head and brain of our baby.

But know - any diagnosis made by doctors is not a sentence! When the baby is still very small, all violations can be corrected with the right approach. The more we know, the more effective our actions will be.

This terrible word craniosynostosis

In other words, the earlier fusion of the sutures of the skull. At birth, a child has 45 bone cranial elements. They are mobile and connected by a dense fibrous tissue. This instability allows the skull to expand as the brain grows and develops. As the baby grows older, the bone elements grow together.

But in some cases, overgrowing occurs ahead of time, and the head takes on an unusual shape. This is craniosynostosis (or craniostenosis). A fairly rare pathology (according to statistics, it occurs in 1 out of 2000 children). Most often, boys are affected by the disease.

When your baby's head becomes strangely shaped, frequent inexplicable vomiting appears, hearing goes down, eyes become inflamed. He ceases to please you with a good mood, cries for a long time and does not want to play - these may be symptoms of craniosynostosis.

What does he threaten

In addition to external changes, the disease can lead to big problems in the normal development of the little man and the appearance of many concomitant diseases:

Pathology of the structure of the entire skeleton (fusion of the vertebrae).
Improper development of the joints, which leads to disability.
Difficulty in nasal breathing (night sleep apnea, in which the baby temporarily stops breathing).
Hydrocephalus.
Lagging behind in physical and psychological development.
Strabismus, blindness, tremor.
Underdevelopment of the jaws and eye sockets.

Craniosynostosis leads to such severe complications in extreme forms, when several areas of the skull are fused. There are several degrees of the disease, which depend on the number and type of closed sutures.

Types of pathology

Scaphocephaly (sagittal craniosynostosis). Earlier closure of the sagittal or sagittal suture (it connects the left and right parietal region). The most common disease (40-60% of all cases). With scaphocephaly, the baby's head takes on a laterally flattened shape. The back of the head and forehead bulge.

Brachycephaly (coronary craniosynostosis). Fusion of the coronal suture (it is located across the entire head from ear to ear). The height of the crown increases, the occipital region protrudes, and the forehead is slanted. There is a type of pathology when the coronary and sagittal sutures are fused (oxycephaly or agrocephaly). The head in this case acquires a conical shape.

Trigonocephaly (metopic craniosynostosis). Closure of the metopic suture (frontal). A rare form of craniostenosis, it occurs in 10% of cases. The head of the baby becomes triangular, in the form of a drop, the distance between the eyes decreases.

Turriccephaly. Fusion of the coronal (the junction of the frontal and two parietal plates) and sagittal sutures. The skull is shaped like a clover leaf. The temporal bones bulge, the remaining parts narrow.

Diagnostics

Finally, such a diagnosis can be made only after the studies:

X-ray (contrary to popular belief about its harmfulness, the amount of radiation that the baby will receive is minimal and not dangerous to health).
Computed tomography of the brain (CT). Layered scan of the skull and brain. The procedure takes only a few minutes.
Magnetic resonance imaging (MRI). The most accurate research method. Images are obtained by applying electromagnetic waves. Longer in impact (about 20 minutes), so the baby can be anesthetized.

After conducting such studies, make sure that the baby receives the necessary vitamins and often walks in the fresh air. This will remove the possible consequences of the procedures.

Why does craniosynostosis occur?

In most cases, the fault lies with defective genes and hereditary pathologies (Cruzon, Pfeiffer and Apert syndromes). Also, various intrauterine diseases of the unborn child are also dangerous.

Treatment of craniosynostosis

Endoscopy. This method is used only if your baby is not yet 6 months old. This is a gentle method with the least tissue damage.
Operation. During the operation, the fused sutures are separated and the correct shape of the skull is recreated. In addition to skull surgery, consultation and treatment with a dentist is necessary. After the operation, the scar on the baby's head will be almost invisible and completely hidden under the hair.

After the operation, your little one will need to wear a special helmet every day and undergo physical therapy for a while.

And the most important. Don't panic! Our children are very sensitive and react to any change in the mental state of their mother. They should feel strength and protection from us, and not see tears and upset eyes. With timely treatment, your favorite miracle is waiting for a full recovery and the most wonderful life!

There are a number of conclusions about the dangers of washing cosmetics. Unfortunately, not all new mothers listen to them. 97% of shampoos use the hazardous substance Sodium Lauryl Sulfate (SLS) or its equivalents. Many articles have been written about the impact of this chemistry on the health of both children and adults. At the request of our readers, we tested the most popular brands.

The results were disappointing - the most advertised companies showed the presence of those very dangerous components in the composition. In order not to violate the legal rights of manufacturers, we cannot name specific brands. Mulsan Cosmetic, the only company that passed all the tests, successfully received 10 points out of 10 (read). Each product is made from natural ingredients, completely safe and hypoallergenic.

If you doubt the naturalness of your cosmetics, check the expiration date, it should not exceed 10 months. Approach carefully to the choice of cosmetics, it is important for you and your child.

Journal headings

Craniosynostosis is characterized by premature fusion of one or more cranial sutures, often resulting in an abnormal head shape. This may be the result of primary abnormal ossification (primary craniosynostosis) or, more commonly, a brain growth disorder (secondary craniosynostosis).

The disease often occurs in utero or at a very early age. It lends itself exclusively to surgical treatment, although a positive outcome is not possible in all cases.

Classification of craniosynostosis and the causes of its development

Normal ossification of the cranial vault begins in the central region of each cranial bone and extends outward to the cranial sutures. What shows the norm?

When the coronal suture separates the two frontal bones from the parietal bones.
The metopic suture separates the frontal bones.
The sagittal suture separates the two parietal bones.
The lambdoid suture separates the occipital bone from the two parietal bones.

The main factor that holds back the untimely fusion of the bones of the skull is the ongoing growth of the brain. It is worth emphasizing that the normal growth of each cranial bone occurs perpendicular to each suture.

Simple craniosynostosis is the term used in situations where only one suture is fused prematurely.
The term complex or junctional craniosynostosis is used to describe premature fusion of multiple sutures.
When children showing symptoms of craniosynostosis also suffer from other body deformities, this is called syndromic craniosynostosis.

Primary craniosynostosis

With premature fusion of one or more sutures, skull growth may be limited by perpendicular sutures. If several sutures are fused while the brain is still changing in size, intracranial pressure may increase. And this often ends with a series of complex symptoms, up to death.

Varieties of primary craniosynostosis (premature fusion)

Scaphocephaly - swept suture.
Anterior plagiocephaly - first coronal suture.
Brachycephaly - bilateral coronal suture.
Posterior plagiocephaly - early closure of one lambdoid suture.
Trigonocephaly - premature fusion of the metopic suture.

Secondary craniosynostosis

More often than in the primary type, this type of pathology can lead to early fusion of the sutures due to primary failure of brain growth. Since the growth of the brain controls the distance of the bone plates from each other, the disorder of its growth is the main cause of premature fusion of all sutures.

With this type of pathology, intracranial pressure is usually normal, and there is rarely a need for surgery. Typically, the lack of brain growth leads to microcephaly. Premature closure of the suture, which does not pose a threat to brain growth, also does not require surgical intervention.

Intrauterine space restrictions may play a role in premature fusion of the fetal skull sutures. This has been demonstrated in cases of coronal craniosynostosis. Other secondary causes include systemic disorders affecting bone metabolism such as rickets and hypercalcemia.

Causes and consequences of early craniosynostosis

Several theories have been proposed for the etiology of primary craniosynostosis. But the variant with the etiology of a primary defect in the mesenchymal layers of the skull bones has become the most widespread.

Secondary craniosynostosis usually develops along with systemic disorders

These are endocrine disorders (hyperthyroidism, hypophosphatemia, vitamin D deficiency, renal osteodystrophy, hypercalcemia and rickets).
Hematological diseases that cause bone marrow hyperplasia, such as sickle cell disease, thalassemia.
Low growth rates of the brain, including microcephaly and its underlying causes, such as hydrocephalus.

Causes of syndromic craniosynostosis are genetic mutations responsible for class 2 and 3 fibroblast growth factor receptors.

Other important factors to consider when studying the etiology of the disease

Differentiation of plagiocephaly, which is often the result of positional fusion (which does not require surgery and is quite common) from fusion of the lambdoid suture, is an extremely important aspect.
The presence of multiple adhesions is suggestive of a craniofacial syndrome, which often requires diagnostic expertise in pediatric genetics.

Symptoms of craniosynostosis and diagnostic methods

Craniosynostosis in all cases is characterized by an irregular shape of the skull, which in a child is determined by the type of craniosynostosis.

Rigid bone ridge, well palpable along the pathological suture.
The soft spot (fontanelle) disappears, the child's head changes shape, the sensitivity in these areas is usually changed.
The baby's head does not grow in proportion to the rest of the body.
Increased intracranial pressure.

In some cases, craniosynostosis may not be noticeable until several months after birth.

Elevated intracranial pressure is a common feature of all types of craniosynostosis, with the exception of some secondary pathologies. When only one suture is fused prematurely, increased intracranial pressure occurs in less than 15% of children. However, in syndromic craniosynostosis, where multiple sutures are involved, an increase in pressure can be observed in 60% of cases.

If a child suffers from a mild form of craniosynostosis, the disease may not be noticed until patients begin to experience problems due to increased intracranial pressure. This usually occurs between the ages of four and eight.

Symptoms of increased intracranial pressure

They begin with persistent headaches, usually worsening in the morning and at night.
Vision problems - double vision, blurred vision, or impaired color vision.
An unexplained decrease in the child's mental abilities.

If a child complains of any of the above symptoms, a pediatrician should be contacted as soon as possible. In most cases, these symptoms will not be caused by increased intracranial pressure, but they should definitely be studied.

vomiting;
irritability;
lethargy and lack of reaction;
puffy eyes or difficulty seeing a moving object.
hearing impairment;
labored breathing.

A close examination of the skull makes it clear that its shape does not always confirm the diagnosis of craniosynostosis. In such cases, a number of visual examination methods are used, for example, an x-ray of the skull.

Radiography is performed in several projections - anterior, posterior, lateral and top. Prematurely fused sutures are easily identified by the absence of connected lines and the presence of bony ridges along the suture line. The sutures themselves are either not visible, or their localization shows evidence of sclerosis.

A cranial computed tomography with 3D projection is generally not required for most infants. The method is sometimes performed when surgery is being considered as the next step in treatment or if radiographic findings are inconclusive.

Pathology correction methods, possible complications and consequences

In the last 30 years, modern medicine has developed a deeper understanding of the pathophysiology and treatment of craniosynostosis. At present, surgery generally remains the main type of treatment for correcting skull deformities in children with fusion of 1-2 sutures resulting in an ugly head. Children with microcephaly, which is often seen in mild craniosynostosis, usually do not require surgery.

When drawing up a therapeutic regimen, specialists must take into account a number of points.

In patients with microcephaly, the cause of this disease should be studied.
At the first visit, the circumference of the head in the longitudinal direction is measured and further monitoring of changes is carried out. The physician should ensure normal brain growth in patients with primary craniosynostosis.
Regular monitoring for signs and symptoms of increased intracranial pressure should be made.
If there is a suspicion of increased intracranial pressure, then neurosurgical counseling is very appropriate.
To preserve visual function in patients with increased intracranial pressure, additional ophthalmological consultations should be carried out.

Surgical treatment is usually planned for increased intracranial pressure or to correct a deformity of the skull. The operation is usually performed in the first year of life.

Conditions for surgery

If the shape of the head does not change for the better at the age of two months, then the anomaly is unlikely to change with age. Early intervention is indicated if children may be candidates for minimally invasive surgery. It is worth noting that the deformity is more noticeable in the chest period, and it may become less obvious with age.
As the child grows, he has more hair, the visible manifestations of the anomaly may decrease.
Indications for surgical correction of craniosynostosis depend on the age, general condition of the child and the number of prematurely fused sutures.
Surgical treatment of a cranial or craniofacial deformity is performed in children aged 3-6 months, although the variety of approaches varies among surgeons.

Surgery in infants can result in relatively large blood volume losses. Accordingly, minimally invasive surgical techniques should be considered. One of the promising is the use of intraoperative tranexamic acid. Patients with indications for surgical correction of craniosynostosis were subjected to pretreatment with erythropoietin and tranexamic acid, which allowed them to maintain lower volumes of blood loss.

Other features of surgery

Surgery in infants over 8 months of age may be associated with reduced skull growth.
Infants diagnosed with syndromic craniosynostosis should be operated on as soon as possible.
The results of surgery are best when performed on infants under 6 months of age.
Patients with corresponding facial defects may need a stepwise approach - surgical treatment by the efforts of an entire team of multidisciplinary specialists.
In some cases, molding helmets may be required, which are used in especially severe cases.

Anomaly Chiari (Chiari)
Spina bifida (non-fusion of the arches of the spine)
1. Diastematomyelia
2. Myelomeningocele
encephalocele
Arachnoid cysts
Anomaly Dandy-Walker (Dandy-Walker)

craniosynostoses

Sagittal (scaphocephaly)
Coronary unilateral (anterior plagiocephaly)
Coronary bilateral (brachycephaly)
Metopic (trigonocephaly)
Lambdoid (posterior plagiocephaly)
Multiple synostosis (oxycephaly)

Pediatric neuro-oncology:

Medulloblastoma
Astrocytoma
Craniopharyngioma
PNET
Germinoma and GCT
ependymoma
Epidermoid/dermoid

Formerly called "craniostenosis", but this is not entirely true from the point of view of pathogenesis, because not everyone "craniosynostosis" leads to "craniostenosis". The essence of the pathology lies in the early (most often prenatal) closure of one or more sutures, which leads to gross deformities of the skull. In addition, craniosynostoses involving multiple sutures can lead to intracranial hypertension.

Epidemiology: the incidence is ~0.6 per 1000 live births.

Diagnostics:

The presence of a specific deformity of the bones of the skull (not to be confused with the so-called “positional” deformities)
Palpation of the area of the alleged altered suture reveals a characteristic “roll”
There is no “enlightenment” typical for the suture area on the craniogram
Immobility of bones with light pressure in the zone of "synostosis"
3-D CT reconstruction helps to clarify the “roughness” of the deformity and the degree of involvement in the process of the base of the skull (the picture shows an example of a neglected (at the time the child was 6 years old) right-sided plagiocephaly with left-sided “compensatory” bulging in the left frontal region)
Treatment: in most synostoses, the main problem is a “cosmetic” defect. This problem can only be solved by surgery. Only in cases of “multi-suture” craniosynostosis (oxycephaly) is it also about resolving problems with intracranial hypertension. The purpose of the operation is to correct the cosmetic deficit, the optimal timing is 6-12 months. child's life. At a later date, the situation becomes “neglected” and requires a much more extensive (i.e., more traumatic for the patient) operation. It should be noted that the cosmetic result of “late” operations is worse.

Forecast: in the vast majority of cases, it is possible to achieve a “good” and “excellent” cosmetic result when the operation is performed at the optimal time. The risk of neurological problems is minimal. The main intraoperative complication is massive (for an infant) blood loss associated with the “volume” of the operation in a small patient. Modern surgical and anesthetic techniques make it possible to successfully control this problem. In rare cases (with the most severe deformities and late requests for help), several operations are required in order to achieve optimal correction.

This is the most common type of “single-suture” synostosis. It arises due to premature closure of the sagittal suture and the resulting limitation in the growth of the skull “to the sides”. The head gradually assumes a “navicular” shape with a protruding forehead and occiput and a “long, narrow, keeled” parietal region, as if flattened from the sides. In 80% it is observed in boys.

Occurs as a result of premature closure of both coronal sutures and is characterized by a wide and flat forehead. Brachycephaly is common in multi-suture and syndromic synostoses (eg, Apert's syndrome). Surgical treatment is "bilateral frontal extension"(similar to anterior plagiocephaly) .

d.Metopic synostosis (trigonocephaly)

Leads to the appearance of a "keeled" deformity in the frontal region along the midline, which is accompanied by a "triangular shape of the head." Many of these children have abnormalities in chromosome number 19 and mental retardation.

e. Lambdoid (posterior plagiocephaly)

The most important in the diagnosis is the differentiation of "lambdoid synostosis" and the so-called. "positional occipital deformity". However, in most cases, such a differential diagnosis is not easy to make. Here are just some of the causes of "positional occipital deformity":

A sedentary child who constantly lies on his back

Abnormal position of the head due to "torticollis", anomalies of the cervical spine

- "special" laying the child on his back during sleep (this trend has been widespread since 1992 to prevent the so-called "sudden death of newborns").

Problems with the position of the fetus during pregnancy

Diagnosis is easily made by visual examination. The head has a "diamond-shaped" shape, with the ear displaced anteriorly on the side of the synostosis (figures).

Treatment: in 80% of all cases, conservative measures are sufficient to correct the deformity in the occipital region (changing the position of the child, using special corrective "helmets"). If conservative treatment is unsuccessful, surgical correction of the deformity is indicated. The optimal terms of the operation are 6-18 months of the child's life.

f. Multiple synostosis (oxycephaly)

By name it is clear that with this pathology there is a premature closure of several sutures at once. The head at the same time has a characteristic "tower" shape with underdeveloped sinuses and eye sockets. In this form of synostosis, there are always signs of increased intracranial pressure.

Treatment: only surgical, the operation is not only cosmetic, but also a functional goal - normalization of intracranial pressure and, accordingly, brain development.

Yu.V.Kushel, PhD, neurosurgeon, Burdenko Research Institute of Neurosurgery

Parents of a newborn, as a rule, know that the baby has a fontanel on the head, or, as it is often called, a soft crown. Many of them are convinced that the fontanel in infants is a weak and vulnerable place that should not be touched once again. Is it true? Is there one fontanel in newborns? What is it for? Let's figure it out.

FEATURES OF THE STRUCTURE OF THE SKULL OF THE FETUS AND NEWBORN

The fontanelles of a newborn, and there are several of them, can be safely called “devices” given by nature to facilitate the process of the birth of a baby. During the passage of the fetus through the birth canal of the mother, its head can be deformed (in the good sense of the word) and take on the forms corresponding to the birth canal. This makes childbirth easier for both the baby and the mother.

Such anatomical "mutual understanding" is possible due to the structural features of the fetal skull. It consists of the same bones as the skull of an adult. But the bones of the baby's cranial vault are highly elastic and are interconnected by a kind of shock absorbers - non-ossified connective tissue areas.

These are sutures and fontanelles:

frontal or metopic suture - between the frontal bones;
coronal or coronal suture - between the parietal and frontal bones;
swept or sagittal suture - between the parietal bones;
occipital or lambdoid suture - between the occipital and parietal bones;
left and right scaly sutures - between the parietal and temporal bones;

anterior or large fontanel - a rhomboid membranous plate, from the corners of which the frontal and sagittal sutures, the left and right parts of the coronal suture depart;
posterior or small fontanel in children - a triangular depression at the intersection of the occipital and sagittal sutures;
left and right wedge-shaped fontanelles - at the junction of the coronal and scaly sutures;
left and right mastoid fontanelles - at the junction of the lambdoid and scaly sutures.

In a healthy full-term newborn, of all the listed membranous structures of the skull, only the large fontanel (anterior) and, in rare cases, the posterior small fontanel, are determined. And all the seams and other fontanelles are closed. In premature newborns, some sutures between the bones of the skull and lateral fontanelles may remain partially open.

The connective tissue membrane that forms the fontanelle in children resembles a tarpaulin in its density. Therefore, it is extremely difficult to violate its integrity. Bathe the baby calmly, if necessary, use a comb, play with the baby, give him a massage and do not be afraid to damage the fontanel.

The anterior children's fontanel is shaped like a rhombus. The doctor measures the size of the fontanel not along the diagonals of the rhombus, but along the lines connecting the midpoints of its opposite sides.

The sizes of a large fontanel in babies born at term vary from 2x2 cm to 3x3 cm. In premature babies, not only the fontanel is larger, but the areas of the skull sutures adjacent to it remain open.

Normally, the anterior children's fontanel is on the same level with the frontal and parietal bones surrounding it, or sinks quite a bit. Looking closely, you can see how the fontanel pulsates. With strong crying and anxiety of the baby, he may swell a little.

In the first year of a child's life, the brain grows quite rapidly in size. Due to the elasticity and compliance of the fontanel, the cranium does not interfere with the growth of the brain.

In addition, the fontanel in the baby performs the function of thermoregulation. When the child's body temperature rises through the large fontanelle, excess heat is released by the membranes of the brain, that is, they cool naturally. Therefore, never swaddle a feverish baby with his head and do not wrap his head in hats and scarves.

In a full-term baby, the size of the fontanel located behind, provided that it is not completely closed, is so small that the tip of the finger barely fits into the triangular recess.

In a healthy baby born at term, only the anterior large fontanel remains open. But as the bones of the skull grow, its size gradually decreases, and it closes.

For the age at which the anterior children's fontanel is completely closed, the norms are not strictly defined. In most newborns, this happens by 12, and sometimes by 18 months. But even if the fontanel is overgrown, and the child is not a year old, you should not worry. In a healthy baby, this may be a variant of the norm, which the pediatrician will certainly tell you about.

The posterior fontanel is usually not even determined by the time of birth. If you managed to find it, don't worry. It usually happens like this: by the time the anterior fontanel overgrows, there is no trace of the posterior one for a long time. It closes by 1.5–2 months.

FELLOW - SIGNAL LIGHT

As parents of a newborn baby, you should monitor the condition of the children's fontanel and tell the local pediatrician about all changes. If you notice that your baby's fontanel is pulsating strongly, or it seems to you that he has a very small fontanel (for example, you know the norm for its size), do not be silent, but share your observations with the doctor.

For neonatologists and pediatricians, the fontanel in the baby is a kind of signal beacon. He is the first to react to any trouble in the head of a newborn. By too early or very late overgrowth of the fontanel, the pediatrician may suspect a serious illness.

If at birth the anterior fontanelle is very small or completely absent, first of all, doctors exclude microcephaly and craniostenosis. In the first case, the child has all parts of the body of normal size, and the head (cranial skull and brain) is significantly behind in development. Microcephaly is often a manifestation of severe chromosomal diseases such as Patau syndrome, Edwards syndrome, etc.

With craniostenosis, the sutures between the bones of the skull heal early, and the fontanelles close. Because of this, the head is deformed, the brain cannot grow normally, intracranial pressure increases with all the symptoms that follow from this.

The pressure inside the skull can be so high that the fused bones begin to separate again.

If a woman, being pregnant, ate a lot of foods containing calcium (cheese, milk, cottage cheese, etc.) and was fond of multivitamin preparations, her child's fontanel may close early. A lack of calcium in the body can cause late overgrowth of the fontanel.

If a full-term baby has a very large anterior fontanel and an open posterior fontanel, he is examined for hydrocephalus (hydrocephalus) and congenital hypothyroidism (thyroid insufficiency). Doctors exclude or confirm intrauterine hypoxia, birth trauma and intrauterine infections, in which the size of the fontanel can also be above average.

The fontanel in children reacts to an increase (ICP) with tension and bulging.

ICP in infants increases with the following diseases and conditions:

congenital diseases (hydrocephalus, etc.);
brain infections (purulent meningitis, etc.);
volumetric formations in the cranial cavity (hematomas, tumors, etc.);
perinatal encephalopathy;
thrombosis of the sinuses and veins of the brain in severe infections, blood diseases, etc.

Important:if the bulging of the fontanel appeared immediately after the child received an injury (of the head and not only), immediately contact a medical facility or call an ambulance at home.

Be sure to pay attention not only to the bulging, but also to the sunken fontanel, which serves as an indicator of the degree of dehydration of the body. With intestinal infections due to vomiting and diarrhea, with neuroinfections due to repeated vomiting, dehydration develops very quickly. A child in such a situation requires urgent medical attention.

Note: with meningitis, the fontanel first swells due to an increase in ICP, and then, due to the loss of fluid by the baby's body, it sinks.

If a pediatrician or neurologist directs your baby to measure intracranial pressure, do not refuse this study. The procedure is absolutely safe and painless, but its results are quite informative. They will help the doctor make the correct diagnosis and promptly prescribe treatment for the baby, if necessary.

The fontanel in babies is a kind of "window" through which you can "look" inside the skull and brain of the baby.

Therefore, infants with access through the fontanel perform some diagnostic and therapeutic manipulations, such as:

subdural puncture under local anesthesia;
puncture of the ventricles of the brain to measure the pressure of the cerebrospinal fluid, study its composition and subsequent ventriculography;
puncture-free measurement of ICP using special tonometers;
two-dimensional echoencephalography and sonography - ultrasound studies;

radioisotope scintigraphy.

Zaluzhanskaya Elena, pediatrician

Main symptoms:

hand atrophy
Atrophy of the forearm
Elbow joint hypertrophy
Skull deformity
short neck
Developmental disorder
Inability to move the shoulder
Limitation of hand movements
Neck restriction
mental retardation
Decreased performance

Synostosis is the process of fusion of some parts of a bone or two bones located between each other. There are many varieties of this condition. It is noteworthy that some of them do not have a pathological basis.

There are quite a few reasons for the occurrence of such a disorder, and all of them will differ depending on the type of pathology. In some cases, the predisposing factors are completely harmless.

The disease has a characteristic external symptomatology, expressed in the fusion of several bones or other types of deformation. An experienced specialist will be able to identify the pathology already during the first examination, however, to determine the form of the disease, several instrumental examinations and consultations of specialists from different fields of medicine will be required.

The treatment regimen will directly depend on the etiological factor and the form of the course of the disease.

The International Classification of Diseases does not allocate a single meaning for such a disorder. From this it follows that the ICD-10 code will differ depending on the type of pathology.

Etiology

Currently, clinicians do not know the mechanism of development and the causes of the congenital form of the disease in a child. However, doctors say that the main role is played by the hereditary factor. This suggests that if one of the parents is diagnosed with a similar pathology, then the probability of having a sick child varies from 50 to 100%.

A large number of pathological cases of synostosis are associated with intrauterine anomalies in the structure of the skeleton. But in some cases, such a process may be secondary, that is, acquired.

Sources of such a disorder can be:

a wide range of injuries associated with a violation of the integrity of bone tissue, as well as cartilage or periosteum;
the presence in the medical history of such an ailment as;
the course of degenerative-dystrophic processes with localization in the spine;
ailments such as;
dysfunction of the endocrine system.

From this it follows that synostosis can be both an independent disease and develop against the background of the lack of timely treatment of other ailments. In addition, some types of pathology can be combined with other anomalies.

In some cases, synostosis in children and adults may be the result of surgery. Sometimes the disease does not have a pathological basis, but is a completely normal phenomenon, for example, fusion of the pelvic bones.

It is extremely rare that the source of the disease cannot be identified.

Classification

Depending on the main cause of the occurrence, synostosis is divided into:

congenital- is caused by intrauterine anomalies of the structure, namely such conditions as hypoplasia or aplasia of the connective tissue between the fused bones. In such cases, radioulnar synostosis is often diagnosed, cranial sutures are less often fused;
acquired- often the result of diseases that affect the bones;
physiological- does not have a pathological basis and occurs during the maturation of the body. Normally, synostosis is formed in adolescence, but under the influence of certain diseases, this process can accelerate or, conversely, slow down. Such ailments include hypergonadism, eunuchoidism and Kashin-Beck syndrome;
pathological;
artificial- created during surgery. This is necessary to eliminate extensive structural bone defects, as well as to prevent the occurrence of false joints. Most often, this kind of synostosis is created between the tibia;
post-traumatic- based on the name, it is formed against the background of a violation of the integrity of the bone, which occurred against the background of an injury. In the vast majority of situations, the bones of the lower leg, forearm and adjacent vertebrae are deformed.

According to the place of localization, the fusion of bones has several types, for example:

Symptoms

The clinical signs of synostosis will differ depending on which variety the disease belongs to. For example, the synostosis of the bones of the forearm or the radioulnar type of the disease is expressed in the following signs:

atrophy of the hand and forearm, which can very rarely be combined with a condition such as hypertrophy of the elbow joint;
the impossibility of shoulder movements, which is fraught with a decrease in working capacity and the presence of pronounced problems with self-service;
limitation of brush movement.

Coronary synostosis is expressed in:

Symptoms of damage to the vertebrae by synostosis:

shortened neck;
limited mobility of the upper regions of the spine;
deterioration in hair growth or their complete absence in the back of the head;
spina bifida;
decreased skin sensitivity;
increased location of the scapular zone;
the presence of pterygoid folds in the scapular region.

In addition, this type of disease is often accompanied by anomalies in the development of the organs of the reproductive system, fingers and toes, ribs and kidneys, respiratory system and face.

For any kind of disease, a aching character is characteristic. The localization of pain will be the zone of bone deformation.

Diagnostics

Despite the fact that synostoses have pronounced symptoms, a definitive diagnosis will require the implementation of hardware examinations.

However, in any case, primary diagnostics should be carried out, which is aimed at the work of the clinician with the patient. Among these measures it is worth highlighting:

study of the patient's medical history and life history - to identify the nature of the pathology;
a thorough physical examination - aimed at both identifying the underlying defect and concomitant symptoms;
a detailed survey of the patient - the need for it is that the doctor receives complete information about the nature of the course of the disease.

Diagnosis of synostosis does not imply the implementation of laboratory tests of the patient's blood, urine and feces, since such tests are not of diagnostic value.

Instrumental methods for establishing the final diagnosis include:

Such a diagnosis can be performed by an orthopedist or traumatologist, but in addition, consultations from specialists from other fields of medicine, in particular, cardiology, endocrinology, neurology and surgery, will be needed.

Treatment

The treatment regimen directly depends on the type of the course of the disease.

Synostosis of the bones of the forearm and the radioulnar type of the disease are treated with:

corrective plaster bandages;
therapeutic massage of the injured hand;
ensuring the position of supination with the forearm;
surgical removal of synostosis, but only after ten months of conservative therapy.

Treatment of synostosis of the skull involves only surgical intervention, which is performed in the first years of the patient's life.

The deformation of the vertebrae and coccyx is eliminated by using:

physiotherapy procedures;
therapeutic massage;
analgesics.

The question of surgery is decided on an individual basis for each patient.

In the treatment of synostosis of the ribs, restorative methods prevail, but with a pronounced deformation, medical intervention is indicated. When diagnosing metopic synostosis, the only treatment is a surgical procedure.

Prevention and prognosis

There are no specific preventive measures aimed at preventing synostosis. The general rules should only include:

proper management of pregnancy;
early detection and treatment of pathologies that can lead to the development of the underlying disease;
counseling with a geneticist before a couple decides to have a baby
regular complete medical examination.

Despite the fact that the disease is difficult to treat, it has a favorable prognosis. After surgery and with intensive exercise therapy in the postoperative period, it is possible to achieve 100% restoration of the functioning of the area affected by the pathology. Nevertheless, untimely therapy can lead to disability of the patient.

In any case, the outcome depends on the degree of damage and deformation of the bones.

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