Craniostenosis is a serious disease that is characterized by deformation changes in the bones of the skull in newborns. This pathology occurs on average in 1 child in 2000 infants.
The disease is characterized by premature fusion of cranial sutures, which prevents the full development of the brain, the formation of nerve endings and blood vessels. According to statistics, craniostenosis most often affects boys, and this pathology is extremely rare in newborn girls.
Deformation changes in the skull of this type are detected in the first days after the birth of a child, or are determined by a pediatrician at a later stage of the baby’s development. The severity of the symptoms of the disease depends on the type of craniostenosis and its severity.
Early diagnosis of the disease makes it possible to develop an effective treatment regimen to restore the natural shape of the child’s skull and eliminate prematurely formed sutures.
The maximum therapeutic effect is achieved if the sick infant received qualified medical care no later than 2-3 months. own life.
At later stages of development of a newborn, the risk of complications and negative consequences caused by surgical intervention, as well as compression of the centers of the brain, optic nerve and great vessels that provide blood supply to the tissues, increases.
Kinds
Craniostenosis in newborns is classified into separate types, based on the nature of the deformation changes in the infant’s skull, as well as the nature of the origin of the disease. The table below lists the main types of this disease, and also describes in detail the characteristics of the pathological process.
| Types of craniostenosis | Distinctive features of the disease |
| Congenital | Congenital craniostenosis is characterized by deformation of the skull and fusion of its sutures even at the stage of intrauterine development of the fetus. This pathology occurs during the first 12 weeks of pregnancy. The nature of the origin of the disease is not fully understood, but most scientists from all over the world are inclined to believe that it is a genetic failure. In this case, the child is already born with a deformed skull, which is diagnosed by doctors in the first 1-2 hours after birth. Moreover, most pathologies do not have a hereditary connection, since there were no previous cases of such a disease in the families of parents of newborn children with craniostenosis. |
| Acquired | Acquired craniostenosis is a disease that occurs after the birth of a child. This pathology is a complication of surgery on the bones of the skull and can be caused by a difficult birth or traumatic brain injury. The main danger of acquired craniostenosis is that it is difficult to diagnose in the early stages of pathological fusion of cranial sutures. In most cases, negative changes in the skull are manifested by concomitant symptoms associated with dysfunction of the brain, organs of vision and hearing. Pathology of this type is successfully treated if therapy is carried out on a child whose age is not older than 5-6 years. |
| Syndromic | Craniostenosis of the syndromic type is a more severe disease of the skull bones, which is characterized not only by deformation changes in the shape of the head, but also by the appearance of concomitant developmental anomalies. In most cases, this type of disease has a congenital etiology. Infants with syndromic craniostenosis have an insufficient number of fingers and toes, are born with a cleft lip or palate, and suffer from heart defects or other abnormalities of the internal organs and musculoskeletal system. Treatment of syndromic type craniostenosis requires restoration of the physiologically correct shape of the head, as well as elimination of associated injuries. |
| Trigonocephaly | Trigonocephalic craniostenosis is a disease characterized by early fusion of exclusively metapic sutures of the skull. As a result of this pathological process, the child's head is characterized by a triangular bulge in the frontal lobe. At the same time, the occipital segment of the skull retains its natural shape without signs of negative changes. Children with trigonocephaly suffer from impaired vision and intraocular pressure. |
| Brachycephaly | Craniostenosis of the brachycephalic type occurs due to early overgrowth of the lambdoid and coronal sutures of the skull. As a result of this pathological process, an increase in the transverse diameter of the newborn baby's head occurs. Upon visual inspection, it seems that the baby’s skull is 2-3 times larger than its physiologically natural size. |
| Scaphocephaly | Craniostenosis of the scaphocephalic type is one of the most severe pathologies in this category. This disease occurs due to premature fusion of the sagittal suture of the skull. In this regard, the head narrows on the sides, but at the same moment a voluminous bulge is formed in its front and back parts. Scaphocephalic craniostenosis can be complicated by functional disorders of the brain, the tissues of which experience severe compression and do not receive sufficient blood supply. |
Types of craniostenosis in children
Craniostenosis in children is a pathological condition of the skull bones, the type of which is determined based on the results of a diagnostic examination of the infant. The disease can be diagnosed in the first hours after the birth of a child, or it manifests itself at a later stage of its development.
Early detection of the disease and correct diagnosis allow for effective surgical treatment with minimal risk of complications.
Etiology and pathogenesis of the disorder
Sutures are thin layers of fibrous tissue located between the cranial bones and, when fused, form fontanelles (“soft spots”).
They allow the skull to change its configuration as the child passes through the birth canal, thereby preventing trauma during childbirth. Normally, the fontanelles close by the end of the child’s first year of life. With their premature healing and early ossification of the sutures, 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 provoked by the following factors:
- genetic mutations and hereditary conditioning;
- infections suffered in utero (rubella, influenza, herpes, etc.);
- exposure of the fetus to 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 an incorrect position to a newborn, immobility in infancy, limitation of motor capabilities;
- birth injuries and diseases suffered in the first months of life.
Stages and degrees
Craniostenosis in children is a disease that is divided according to the severity of deformation changes in the skull, as well as the possible presence of symptoms of various kinds of complications.
Mild degree
Children with mild craniostenosis have signs of cranial deformation, but with impaired development of only a separate segment of the bones. For example, the presence of an abnormal bulge exclusively in the frontal or occipital part of the head, without signs of severe compression of the brain.
In this case, there are completely no symptoms of complications associated with dysfunction of other organs and elements of the musculoskeletal system. Mild craniostenosis responds well to surgical treatment with minimal risk of complications.
Average degree
Moderate craniostenosis is a pathological condition of the bones of the head when several sutures of the skull fuse together. The child has simultaneous deformation of the forehead and the back of the head.
A more detailed examination reveals the first signs of dysfunction of the organ of vision, hearing, and cerebral circulation. The lack of qualified treatment leads to further progression of the disease and the development of irreversible complications.
Severe degree
Severe craniostenosis is characterized by total deformation of the skull bones with impaired brain function. Children with a similar diagnosis suffer from cramps of the lower extremities, sudden loss of consciousness, a rapid decrease in visual and hearing acuity, and disturbances in the functioning of the central and peripheral nervous system.
A child with severe craniostenosis needs urgent surgery to restore the anatomically correct position of the skull bones. Otherwise, such children will face lifelong disability or death.
Symptoms
Craniostenosis in children has the main diagnostic sign, which is detected based on the results of a preliminary examination of the child. This is a deformed head that has an unnatural and bizarre shape.
This disease is also accompanied by the following symptoms:
- increased intracranial pressure, which progresses as compression of the brain tissue increases;
- the child is bothered by nausea with periodic vomiting;
- there is a severe headache;
- there is a sudden appearance of convulsions of the lower extremities, and the occurrence of epileptic seizures cannot be excluded;
- visual acuity worsens, and a more detailed examination reveals atrophic changes in the optic nerve;
- certain areas of the lining of the brain become inflamed, which resembles meningeal symptoms;
- the child has difficulty hearing, which is due to compression of the nerve endings responsible for processing and transmitting environmental sounds;
- cerebral circulation and coordination of movements are impaired;
- signs of dizziness appear (this symptom can be reported by children over 3 years of age who have received an acquired form of craniostenosis, but have not yet undergone surgical treatment).
The most severe types of deformation changes in the bones of the skull can cause the development of mental disorders. The child may be significantly retarded in mental and physical development, the consequences of which cannot be eliminated even after a successful surgical operation.
Symptoms of craniosynostosis
Clinically, craniosynostosis manifests itself from the moment the child is born. All forms are characterized by plagiocephaly and early closure of the large fontanel (normally this occurs at 12-18 months). Only with polysynostosis or concomitant hydrocephalus can it remain open until 3 years of age. Also, with craniosynostosis, an increase in intracranial pressure is often observed, which can be manifested by neurological disorders: anxiety, intense crying, nausea and vomiting, sleep disturbance, loss of appetite, positive Graefe's symptom, convulsions.
Each form of the disease has characteristic clinical features. Craniosynostosis of the sagittal suture (scaphocephaly or scaphoid skull) is characterized by an increase in the anteroposterior size of the child’s head with insufficient width. The elongation of the skull, “indentation” of the temporal regions, “overhanging” of the forehead and occipital part, narrowing of the face and its acquisition of an oval shape are visually determined. Palpation reveals a bone ridge above the passage of the sagittal suture. At an early age, mental development may be delayed.
Overgrowth of the lambdoid suture is most often unilateral and manifests itself as flattening of the occipital region. It is a difficult form to diagnose, since plagiocephaly is almost invisible under the hair, and neurological disorders are minimal. As the patient grows older, the dynamics of the disease are practically absent.
Coronal or coronal craniosynostosis can be either unilateral or bilateral. Overgrowth of only one half of the suture is accompanied by a typical deformation of the child’s skull - flattening of the frontal bone and the upper part of the orbit on the affected side. At the same time, the opposite half “overhangs” compensatoryly. Over time, a curvature of the nose in the opposite direction, flattening of the cheekbones, malocclusion and strabismus develop. Bilateral coronary craniosynostosis is manifested by a wide, flat and high forehead with flattened orbital margins of the frontal bone, and rarely by tower deformation of the skull (acrocephaly). Neurological disorders are nonspecific and similar to other forms.
Nontopic craniosynostosis or trigonocephaly is characterized by the development of a triangular forehead with a bony keel extending from the glabella to the greater fontanel. Hypotelorism is also observed - posterior displacement of the orbits with a decrease in the interorbital space. Over time, there is some smoothing of the bone ridge and normalization of the shape of the forehead. In half of the cases, visual impairment and mental retardation occur.
Syndromic craniosynostosis is the rarest and most severe form. In addition to plagiocephaly, there is dysplasia of the bones of the facial part of the skull, which causes respiratory failure, eating disorders and vision pathology. It is characterized by synostosis of the coronal suture and, as a result, a brachycephalic shape of the child’s head. Hypoplasia of the bones of the upper jaw, protrusion of the eyeballs from the orbits, and hypertelorism also occur. Often there is a significant expansion of the fontanel and divergence of the sagittal suture. Without treatment, children develop severe mental retardation and often die during the first 12 months of life from acute respiratory viral infections complicated by pneumonia.
Craniosynostosis in all cases is characterized by an irregular shape of the skull, which in a child is determined by the type of craniosynostosis.
Main features
- A rigid bone ridge, easily palpable along the pathological suture.
- The soft spot (fontanelle) disappears, the child's head changes shape, and sensitivity in these areas is usually altered.
- 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.
Increased intracranial pressure is a common feature of all types of craniosynostosis, with the exception of some secondary pathologies. When only one suture fuses prematurely, increased intracranial pressure occurs in less than 15% of children. However, in syndromic craniosynostosis, where multiple sutures are involved, increased pressure can be observed in 60% of cases.
Symptoms of increased intracranial pressure
- They begin with persistent headaches, usually worse in the morning and at night.
- Problems with vision - double vision, blurred vision or impaired color vision.
- Unexplained decline in the child's mental abilities.
If your child complains of any of the above symptoms, you should contact your pediatrician as soon as possible. In most cases, these symptoms will not be caused by increased intracranial pressure, but they should definitely be investigated.
If left untreated, other symptoms of increased intracranial pressure may include:
- vomiting;
- irritability;
- lethargy and lack of response;
- swollen eyes or difficulty seeing a moving object.
- hearing impairment;
- labored breathing.
Upon closer examination of the skull, it becomes 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, a skull x-ray.
X-rays are performed in several projections - anterior, posterior, lateral and superior. Prematurely fused sutures are easily identified by the absence of connected lines and the presence of bone ridges along the suture line. The sutures themselves are either not visible, or their location shows evidence of sclerosis.
A 3D cranial CT scan is usually not needed for most infants. The technique is sometimes performed when surgery is being considered as the next step in treatment or when X-ray results are equivocal.
Without health, happiness is impossible
Vissarion Belinsky
Especially when it comes to our long-awaited babies. All mothers carefully examine their treasure during daily care, and any change in the baby’s appearance causes great concern. The greatest fear appears when we see the presence of problems associated with the development of our child’s head and brain.
But know that any diagnosis made by doctors is not a death 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.
Symptoms of craniosynostosis usually appear immediately after birth or several months later. The symptoms are as follows:
- skull deformation
- abnormal appearance of the fontanel or its absence in the upper part of the calvarium
- formation of a bone ridge at the site of fusion of a suture that closed too early
- abnormal growth of a child's head
Depending on the type of craniosynostosis, your child may have other symptoms:
- headache
- close or wide set eyes
- reduced learning ability
- vision loss
Doctors diagnose craniosynostosis during a physical examination. Sometimes a CT scan is used, which reveals the closure of the sutures in the child's skull. Genetic tests and physical characteristics help the doctor determine the presence of syndromes that contribute to the development of this disease.
Craniostenosis, in addition to deformation of the structure of the skull bones, has other symptoms:
- changes in facial appearance;
- premature fusion of the fontanel;
- the presence of tuberous protrusions along the seams;
- complaints of headaches;
- vomit;
- memory impairment;
- sleep problems (insomnia, night awakenings);
- slowdown in development.
Deformation of the skull bones
Deformation of the skull bones 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.
Reasons for appearance
Craniostenosis in children appears under the negative influence of external and internal factors. Pathology can manifest itself at the stage of embryonic development, or at a certain stage of the child’s independent life.
In this regard, the following reasons for the appearance of this disease are identified:
- damage to the fetus by dangerous bacterial and viral infections that cause embryonic developmental defects (in medical practice, this pathology is most often provoked by rubella, toxiplasmosis, spirochete pallidum, gonococcus);
- hereditary predisposition to premature fusion of the sutures of the skull, when such a disease is transmitted to the child along with genetic information from one of the parents;
- consumption of alcoholic beverages, drugs, smoking during pregnancy;
- difficult childbirth, which led to damage to the bones of the skull (similar situations arise if a woman has a physiologically too narrow pelvis and birth canal, and the obstetrician refused to use the cesarean section method);
- unsatisfactory environmental conditions caused by the presence of chemically hazardous or radioactive substances in food and water that negatively affect the intrauterine development of the fetus;
- traumatic brain injury that a child received as a result of a fall or impact on a hard surface;
- negative consequences and complications caused by brain surgery.
In the event that the sutures heal at the stage of intrauterine development, then the deformation changes in the skull are more pronounced. Pathology acquired after birth does not cause critical changes in the shape of the head.
The cause of craniostenosis is determined at the stage of diagnostic examination of the child. The nature of the origin of the disease does not affect the specifics of the organization of the therapeutic course.
Treatment of craniostenosis
Treatment of craniostenosis is mainly surgical. The primary goal of surgery is to relieve increased intracranial pressure and create conditions for further growth and development of the brain. In some cases, operations can eliminate a cosmetic defect in the skull and correct the shape of the head.
The cause of craniostenosis is premature mineralization of cranial sutures.
The optimal age for intervention is the early period – 3–9 months. After the third year of life, when the period of most active brain growth has ended, operations may be ineffective.
The following types of surgical interventions are used in the treatment of craniostenosis:
- linear craniotomy – indicated at an early age;
- circular craniotomy - more often used in older age;
- fragmentation of the cranial vault - indicated for multiple fusion of cranial sutures and only at an older age;
- flap bilateral craniotomy - indicated for severe cases of decompensated craniostenosis.
Source: lipetskmedia.ru
After surgical treatment, a control X-ray or computed tomography is performed to assess the degree of correction of the skull deformity.
Diagnostics
Diagnosis of craniostenosis begins with a preliminary examination of the child’s head. The doctor determines physiological changes in the shape of individual segments of the skull.
Then the patient is prescribed to undergo instrumental examination methods, the results of which make it possible to determine the severity of the disease, functional disorders of the centers of the brain and assess the performance of other internal organs.
Craniometry
Craniometry is a diagnostic technique that involves measuring the anthropometric parameters of a child’s skull. The obtained data are compared with standard indicators, which are the norm for children of the corresponding age.
During this diagnostic procedure, a centimeter tape, a cephalometer, and a special computer program based on Sp-CT examination are used.
Ultrasound of the abdominal organs
Ultrasound of the liver, kidneys and other internal organs, if a sick child has syndromic type craniostenosis. This examination method allows for timely identification of pathologies in the development of vital body systems. Especially if the child has signs of a dysfunctional disorder on the part of one or another internal organ.
Clinical examination of blood and urine
To obtain general information about the patient’s health status, the attending physician prescribes capillary blood donation from the ring finger, as well as morning urine. Then the obtained biological materials are amenable to clinical research.
ECG of the heart
Compression of the brain, increased intracranial pressure, impaired circulation, can provoke a painful condition of the heart. An ECG can detect a decrease in heart rate, as well as signs of arrhythmia.
Venography of blood vessels
This method of diagnostic examination makes it possible to determine the functional state of the great vessels that provide blood delivery to the cavity of the cranium.
Venography displays in detail the intensity of the outflow of venous blood, making it possible to confirm or refute suspicions of the presence of intracranial hypertension.
Spiral CT
This method of diagnostic examination of children with signs of craniostenosis detects a prematurely closed suture of the skull, and also determines the severity of the pathology with the possible presence of intracranial pressure. Information data obtained from the results of Sp-CT is used in the process of planning a surgical operation.
The total cost of a comprehensive diagnosis of a child suffering from craniostenosis, which is carried out in a private clinic, averages from 7,000 to 8,500 rubles. In a public hospital, these services are provided free of charge.
Treatment
Treatment of patients with K. can be either conservative or surgical. Patients with K. with well-compensated intracranial circulation do not need treatment. If there are transient signs of impaired intracranial circulation, then appropriate medications should be used with the introduction of certain restrictions on work schedule, diet, etc. In cases of prolonged decompensation of intracranial circulation, accompanied by the threat of loss of vision, the need for surgical intervention arises. If patient K. experiences decompensation of the “venous encephalopathy” type, then surgical interventions on the bones of the cranial vault are permissible - King fragmentation (dissection of the bones of the cranial vault into separate fragments), Bagdasar-Arseni operation (resection of the bones of the cranial vault within 1-2 cm along the sagittal, coronal, parietal, lambdoid sutures), Arendt's operation (use of dissection of the frontal bone to increase the capacity of the skull), etc. In case of decompensation such as cerebral ischemia, various surgical interventions are performed on the great vessels of the head, depending on the nature of the pathology detected in them during angiography .
When to see a doctor
A visit to the doctor should take place as soon as parents notice physiological changes in the shape of their child’s head. It is almost impossible to independently determine the premature fusion of the sutures of the skull. Therefore, children should only be examined by a specialized specialist.
A child with signs of craniostenosis should be examined by the following specialists:
- a neurologist to exclude dysfunctions of the central and peripheral nervous system;
- an ophthalmologist who examines the condition of the optic nerve;
- cardiologist to promptly detect a decrease in the rhythmic activity of the heart.
The first doctor who examines the child should be the local pediatrician. After performing a preliminary examination, he decides on the possible involvement of doctors of other profiles.
Prevention
In order to minimize the likelihood of developing intrauterine or acquired craniostenosis, it is recommended to follow the following rules of prevention:
- parents planning to conceive a baby should donate blood for genetic research to determine predisposition to deformation changes in the bones of the skull;
- women who are pregnant should not use drugs, alcohol, tobacco products and hormonal medications;
- before pregnancy, eliminate all foci of chronic infection that are present in the body;
- During the period of intrauterine development of the fetus, regularly undergo scheduled medical examinations.
A pregnant woman should monitor the quality of her diet, consume enough dairy products, meat, chicken eggs, fresh vegetables and fruits.
Treatment methods
The only effective treatment for craniostenosis in children of all ages is surgery.
Medications
Deformation changes in the bones of the skull caused by premature fusion of its sutures cannot be eliminated with the help of drug therapy.
Traditional methods
Previously, one of the common methods of alternative treatment for craniostenosis in newborns was tying the skull with dense tissue, which did not lead to disability and irreversible changes in brain tissue. Currently, therapy for this disease is carried out only in healthcare institutions.
Other methods
Treatment of craniostenosis is possible only with the help of surgery performed by a neurosurgeon. The main objective of this method of therapy is to expand the internal volume of the skull, as well as giving the bones an anatomically correct shape.
The surgical intervention scheme is developed individually, taking into account the external symptoms of each patient. If necessary, severely deformed areas of the skull are removed and modern plates made of polymer materials are installed in their place.
Throughout the entire period of the surgical operation, the child is under general anesthesia. The average duration of surgery is 3 hours.
Upon completion of medical procedures, the patient is transferred to the intensive care ward for 24 hours, where he is under round-the-clock medical supervision.
If there are no complications, the child is placed in the general hospital of the neurosurgical department. After 8-10 days the patient is discharged home.
The first examination after surgery should take place after 3 months. The child must undergo a spiral CT scan.
Forecast
In the case of timely surgical treatment and with an isolated form of craniostenosis, the symptoms of the disease may regress.
If left untreated, craniostenosis usually leads to disability due to vision loss, mental retardation, and other complications. An unnatural shape of the skull and a violation of the proportions of the face leads to a serious cosmetic defect that affects the child’s future life.
The incidence of craniostenosis is 1 case per 2000 newborns; in boys it is observed twice as often as in girls.
Possible complications
Failure to promptly consult a doctor to treat a newborn suffering from craniostenosis can lead to the development of the following complications:
- cerebral stroke;
- retardation in physical and mental development;
- dysfunctional disorders in the functioning of internal organs;
- inflammation of the cerebral cortex;
- complete or partial deafness;
- a significant decrease in visual acuity, or the onset of complete blindness;
- impaired coordination of movements;
- epileptic seizures and convulsions of the lower extremities;
- severe dizziness, excluding the ability to move independently;
- bedridden;
- the onset of death.
Craniostenosis, congenital or acquired, is a serious disease that is diagnosed in newborn children. The pathology is characterized by premature fusion of the sutures of the skull, which leads to deformation changes in the shape of the head.
The disease can occur in mild, moderate or severe form, disrupt cerebral circulation and the functions of the central nervous system, or be limited only to a visual defect in the structure of the skull. Treatment of this disease is possible only by performing surgery under sterile conditions in a neurosurgical department.
Early closure of the fontanelle or its size is smaller than normal
Early closure of the fontanel in a newborn child is not a sign of pathology. There is a misconception that due to the rapid fusion of cranial sutures, the development of brain tissue will be disrupted.
Even after the fontanel is closed, the soft parts of the skull still contain a sufficient amount of cartilage tissue in their structure, which ensures a high degree of elasticity of the frontal, occipital, parietal and temporal bones. In this case, there is no threat to the physical and mental development of the newborn child.
According to medical statistics in the field of pediatrics, in 1% of newborns, complete fusion of the skull sutures occurs at the age of 3 months. This indicates that the baby's body has sufficient amounts of calcium, phosphorus, vitamin D and other nutrients necessary for rapid and active growth.
A sharp decrease in the size of the fontanelles with the appearance of accompanying symptoms in the form of strabismus, convulsions, lack of response to external stimuli, loss of consciousness, indicates a progressive pathology. In this case, the child should be examined by a specialist.
