Ventriculomegaly is a pathology that affects the ventricles of the brain. The function of these parts of the organs is to maintain intracranial pressure and ensure normal blood circulation to the human cerebral cortex.
Most often, the disease occurs in unborn babies, less often in children of preschool and school age. Ventriculomegaly in adults develops in exceptional cases, since the disease is congenital.
Let's take a closer look at what ventriculomegaly is, the causes of its occurrence and how it is treated.
Causes and prerequisites for the development of ventriculomegaly in the fetus
According to medical research, ventriculomegaly of the lateral ventricles occurs due to the following reasons:
- Hereditary prerequisites. If any of the child’s close relatives (mother, father, siblings/cousins) suffered from a similar disease, the likelihood of its occurrence in the newborn increases to 25% compared to a baby in whom no one in the family had such a disease;
- Ventriculomegaly in the fetus often develops as a side disease along with another disease, such as Down syndrome;
- Pathology is provoked by intrauterine infections, as a result of which the ventricles of the brain enlarge.
What is the cause of the pathology
One of the reasons for the development of ventriculomegaly in the fetus is a viral infection in early pregnancy, TORCH syndrome. The disease can be triggered by an infectious lesion of the brain during the perinatal period (prenatal).
Rarely, the cause of the pathology is neonatal sepsis - blood poisoning in an infant during the first 28 days of life.
In many sources you can find information that in most cases the disease is diagnosed in children whose mothers are over 35 years old. This is argued by the high risk of genetic damage in a woman’s body. There are no clear WHO statistics (percentages) on this issue, and clinical practice refutes this statement. Children with ventriculomegaly are also born to women aged 20-28 years.
At the Detroit Medical Research Center (USA) from 1992 to 1994. Pregnant women were monitored during the prenatal period. The scientific data obtained are presented in the publication “Mild Isolated Ventriculomegaly: Associated Anomalies and Observations” Mark W. Tomlinson, Marjorie C. Treadwel, 1997. Cytogenetic analyzes were performed on women whose fetuses had enlarged lateral ventricles by 11-15 mm. In 10-12% of cases, a positive relationship was found between the disease and chromosomal abnormalities.
Factors leading to the development of pathology:
- multifunctional immaturity of the central nervous system;
- increased reabsorption (reabsorption) of bicarbonates by the kidneys, resulting in metabolic acidosis (blood oxidation);
- disturbance of fetal hemodynamics (blood movement along the vascular bed);
- perinatal hypoxia (intrauterine oxygen deficiency);
- injuries during labor.
The listed factors lead to the disease not only in infants, but also in children and adolescents under 18 years of age. Ventriculomegaly is extremely rare in adults.
The scientific publication “Neurosurgery and Neurology of Children” by L.V. Kuznetsova, 2007, contains data from numerous clinical studies. According to their results, 50-64% of patients have stable ventriculomegaly of unknown etiology (idiopathic).
The course of fetal ventriculomegaly in pregnant women after 35 years
Ventriculomegaly in the fetus most often develops if the child’s mother is over 35 years old. This happens for the following reasons:
- The risk of genetic disorders (for example, Down syndrome) increases;
- Intrauterine infections occur more often;
- A pregnant woman over 35 years of age is susceptible to complications while bearing a child.
Thus, if the mother is under 35 years old, ventriculomegaly in the child occurs only in exceptional cases.
Treatment
In infants, with the expansion of the gastrointestinal tract, the hemodynamics of blood vessels and anterior cerebral arteries are disrupted during the first 2-3 weeks of life, and the pH of the blood shifts to the acidic side. The child's body compensates for these phenomena. If compensatory mechanisms fail by the end of 4 weeks of life, Diacarb is prescribed.
A diuretic belongs to the group of diuretic drugs that are prescribed for epileptic seizures and disruptions in the dynamics of the cerebrospinal fluid. The drug is prescribed with caution, monitoring kidney function, as it has a nephrotoxic effect. Diacarb can increase blood oxidation.
Infants are prescribed antihypoxants to eliminate oxygen deficiency. Saturation of the brain with O2 improves the functionality of the central nervous system.
B vitamins have a positive effect on the immature nervous system, normalize metabolic processes at the cellular level, and synthesize hemoglobin. If the expansion of the gallbladder is caused by an infection, the woman is prescribed antibiotics.
Neurosurgical treatment is indicated for children if the disease progresses to hydrocephalus, when a large amount of fluid accumulates in the brain. If the condition of the fetus during gestation is extremely serious, there is a high probability of incompatibility with life, it is recommended not to give birth.
Diagnosis of lateroventriculoasymmetry of the brain
Ultrasound procedure of a child’s brain
Depending on the age of the patient, various methods for diagnosing pathology are used:
- The disease can be detected in the fetus starting from the 17th week of pregnancy. For diagnosis, the doctor performs an ultrasound of the mother’s abdominal cavity;
- Ventriculomegaly in an infant is confirmed after an ultrasound of the head;
- To diagnose an adult, an MRI of the brain is prescribed.
All procedures are aimed at examining the ventricles of the brain and identifying lateroventriculoasymmetry (violation of ventricular symmetry).
Diagnosis of hydrocephalus
Neurologists at the Yusupov Hospital conduct a comprehensive examination of patients with hydrocephalus. Computed tomography currently occupies a dominant position among methods for diagnosing hydrocephalus. The procedure is carried out to determine the size and shape of the ventricles, identify developmental anomalies and neoplasms, cysts.
Magnetic resonance imaging allows you to determine the shape and severity of hydrocephalus. Using this diagnostic method, the cause of cerebral hydrocele is determined. Neurosonography helps assess the degree of expansion of the ventricles of the brain. The procedure is used only when diagnosing the disease in children with an open fontanelle, since the skull blocks ultrasound. Neurosonography is used to diagnose hydrocephalus in utero.
Cisternography is a research method in which a radioactive substance is injected into the cerebrospinal fluid. It is used to clarify the type of hydrocephalus and determine the direction of cerebrospinal fluid flow. During an angiography, a contrast agent is injected into the arteries that supply blood to the brain. After some time, anomalies at the level of blood vessels and pathological processes are detected. Doctors at the Yusupov Hospital use innovative methods for diagnosing cerebral vascular diseases - magnetic resonance angiography, which does not require the administration of contrast agents. A neuropsychological examination involves conducting a survey to identify abnormalities in the functioning of the brain.
Severity of ventriculomegaly of the lateral ventricles
Doctors distinguish three degrees of severity of pathology:
- First: an increase in the ventricles of the brain to 11-12 mm. Patients are diagnosed with “ventriculodilation” - a slight expansion of the ventricles of the brain in newborns and preschool children;
- Second: enlargement of the ventricles up to 15 mm. Against this background, asymmetry of the lateral ventricles of the brain and impaired blood flow to the affected areas are often observed;
- Third: enlargement of the ventricles up to 20 mm. At this stage, irreversible changes occur in the structure of the brain.
In addition, the child may be diagnosed with “borderline ventriculomegaly,” which means an increase in the lateral ventricles by no more than 9 mm.
Ventriculomegaly 1st degree
At the first stage of development of the disease, there are no pronounced symptoms. Only a neurologist can detect it after diagnosis. Most parents notice that the child becomes excitable and irritable. Unfortunately, no other symptoms appear.
Moderate ventriculomegaly
The second stage of the disease is also called “moderate ventriculomegaly.” As a rule, it is combined with other ailments, for example, ventriculomegaly and agenesis of the corpus callosum often occur together. Also in this case, lateroventriculoasymmetry of the brain (asymmetry of the ventricles) occurs. The following symptoms are also observed:
- Periodic convulsions, similar to epileptic seizures;
- Increase in head size;
- The presence of bulging veins on the forehead;
- Slow physical development;
- Mental retardation.
Severe ventriculomegaly
In addition to abnormal expansion of the ventricles of the brain, the following symptoms are present:
- Increased intracranial pressure due to the asymmetric shape of the ventricles;
- Recurrent headaches;
- Speech disorders.
Additional diseases are often added to the pathology: cerebral palsy, Down syndrome or hydrocephalus.
Consequences of congenital ventriculomegaly
Perinatal mortality caused by severe intrauterine growth retardation, premature birth or intrauterine infection is 4%.
Also, in 4% of cases, newborns have various kinds of chromosomal abnormalities.
9% of newborns are born with vascular malformations of the brain.
In another 11% of cases, mental and physical developmental delay is diagnosed against the background of congenital ventriculomegaly, the severity of which can vary from mild to moderate severity.
Moreover, the risks of developing an unfavorable neurological outcome of ventriculomegaly for a female fetus are higher than similar risks for a male fetus.
Treatment of ventriculomegaly in the fetus
The disease is most often congenital, so it can be detected even before the birth of the child. To do this, the expectant mother undergoes an ultrasound of the abdominal cavity, based on the results of which the degree of pathology is determined. In the third degree, the woman is offered an abortion, since the risk of miscarriage is high.
In other cases, doctors begin searching for the cause of the pathology:
- A woman must be tested for infectious diseases;
- The fetus is checked for the presence of congenital pathologies (cerebral palsy, Down syndrome, etc.);
- If no abnormalities are found, the pregnant woman is prescribed potassium tablets. It saturates the fetus with oxygen, which improves its condition.
With ventriculomegaly in the fetus, the consequences depend on the promptness of treatment at the stage of pregnancy.
Why is ventriculomegaly dangerous?
Ventriculomegaly in a child can lead to the development of serious disorders in the functioning of the central nervous system and brain. In an isolated form, when the deviation is observed on its own, the prognosis is usually favorable.
Ventriculomegaly, the causes of which are genetic disorders, is often combined with abnormalities in mental development. In particularly severe cases, the child may not be viable. It is important not to skip an ultrasound during pregnancy, when it is already possible to detect many developmental disorders, in particular, ventriculomegaly. What is dangerous about this situation is that by starting the process, you can miss precious time.
Ventriculomegaly, caused by prolonged exposure to infection on the fetus, hypoxia during gestation or childbirth can cause consequences from various kinds of psychoneurological disorders to severe forms of cerebral palsy. Such consequences of ventriculomegaly are difficult to treat. It has its effect, but requires a huge amount of time and effort from doctors and parents.
Treatment of ventriculomegaly in a newborn
With a mild degree of pathology, no therapy is prescribed: the mother only needs to carefully monitor the child’s condition and, at the slightest deviation from the norm, contact a neurologist.
The second stage is treated with medication: the child is prescribed drugs that accelerate processes in the brain:
- Vitamin B tablets;
- Diuretics are drugs that remove excess fluid from the body;
- Medicines to improve blood circulation.
To enhance the effect, it is recommended to walk with your child in the fresh air more often and perform minimal physical exercise with him.
Surgery
The third degree of pathology is treated surgically, since taking medications will not give any results. The asymmetric shape of the ventricles is corrected by performing ventriculo-peritoneal shunting.
This is an operation during which shunts are installed to remove excess fluid. After surgery, children recover quite quickly: insomnia disappears and appetite returns.
Treatment of hydrocephalus
Neurologists at the Yusupov Hospital take a differentiated approach to treating patients with hydrocephalus. In case of a regressed form of the disease, drug therapy is not used. To reduce cerebrospinal fluid pressure, patients are prescribed diuretics: Diacarb, mannitol, Lasix. Nootropics, venotonics and angioprotectors improve the functional activity of the brain.
For progressive hydrocephalus, neurosurgeons at partner clinics of the Yusupov Hospital perform shunt operations. If there is an obstacle to the outflow of cerebrospinal fluid, the space-occupying lesion is removed or the adhesions are cut. If a hematoma is present, it is removed surgically.
An innovative method of treating hydrocephalus is endoscopic surgery:
- endoscopic ventriculocisternostomy of the bottom of the third ventricle;
- endoscopic installation of a shunt system;
- septostomy;
- aqueductoplasty;
- ventriculocystocysternostomy;
- endoscopic removal of intraventricular brain tumor.
Endoscopic operations have a number of advantages compared to bypass interventions: they restore the physiological flow of cerebrospinal fluid, are less traumatic, and improve the patient’s quality of life.
Treatment with endoscopic methods
Endoscopic treatment of hydrocephalus, or dropsy of the brain, is a priority in neurosurgery, since this disease is common among both children and adults. Hydrocephalus of the brain occurs due to impaired absorption of cerebrospinal fluid into the venous system.
Endoscopic surgery
Endoscopic interventions for hydrocephalus of the brain are used to reduce intracranial pressure. Surgeries for dropsy are most effective in comparison with drug therapies, which help slow the progression of the disease, but do not eliminate it. Endoscopic surgery for hydrocephalus, the cost of treatment of which is determined by the severity of the disease, is divided into several types:
- Septostomy;
- Ventriculocisternostomy of the floor of the third ventricle;
- Ventriculocisternostomy;
- Installation of the shunt system endoscopically;
- Removal of intraventricular tumors endoscopically;
- Aqueductoplasty.
The most widely used is endoscopic ventriculocisternostomy of the bottom of the third ventricle. The main task of the surgeon with this technique is to create pathways for the outflow of cerebrospinal fluid from the ventricles into the cisterns of the brain, through which the cerebrospinal fluid is absorbed into the vascular walls, as in a healthy person.
Advantages of endoscopic operations
Endoscopic surgery can only be effective if it is performed by a good specialist. One wrong move by the surgeon during brain surgery can lead to irreparable, severe consequences. When shunts are installed, they are often blocked by blood clots, tumor cells, etc. During endoscopy, no foreign objects remain in the body, and accordingly, the development of such complications is excluded.
In severe cases, patients are given brain drains. Such an event is very dangerous, since the infection can easily penetrate the brain through the drainage. With endoscopic intervention this complication does not occur.
Bypass surgery
Many people who have been diagnosed with the disease wonder where to treat hydrocephalus: in a public medical institution or a modern clinic. Yusupov Hospital is equipped with the latest equipment. Doctors regularly improve their skills and master new treatment methods. The clinic staff ensures a comfortable stay for patients.
Shunt surgery for hydrocephalus in adults, the price of which varies depending on the characteristics of the disease, is performed safely and efficiently at the Yusupov Hospital. Installation of a shunt system ensures the removal of cerebrospinal fluid. The procedure lasts about 90 minutes and is performed under general anesthesia for patients of any age.
Bypass surgery is a safe intervention, but there are certain risks when using it, which the neurologist informs the patient about. For example, a reaction to anesthesia or bleeding may develop. Shunt surgery allows the patient to restore normal brain function.
Main factors in the development of the disease
Most often, ventricular enlargement develops as an independent disease.
But sometimes Turner, Down, Edwards syndrome, etc. are jointly diagnosed. Symptoms appear if a woman has various anomalies during pregnancy, and these do not necessarily have to be chromosomal diseases. Dilatation of the ventricles is caused by infectious diseases that a woman suffered while pregnant. The following provoking factors of ventriculomegaly are also identified:
- fetal hypoxia;
- intrauterine infections;
- hydrocephalus;
- heredity.
What exactly provoked the expansion of the ventricles can only be determined by a neurologist after a thorough examination.
Ventriculomegaly is more often detected in newborns if the mother’s age exceeds 35 years. After reaching this age mark, a woman may develop deviations that must be taken into account when conceiving a child:
- the risk of genetic disorders increases;
- the maternal chromosome may be mutated;
- oxygen starvation in the fetus doubles;
- the risk of infectious diseases increases.
Based on this, we can say that in women under 35 years of age, the risk of developing the disease in their unborn child is unlikely, and after that it increases sharply, two to three times.
Possible disorders and complications
According to research, 11% of children diagnosed with ventricular enlargement suffer from physical and psychological developmental delays. Symptoms may be obvious or subtle.
4% have chromosomal abnormalities. Another 4%, with a combination of fetal development pathology and ventriculomegaly, have a fatal outcome.
If a child is diagnosed with an isolated form of the disease, i.e. no other developmental defects or genetic mutations have been identified, the prognosis will be favorable, provided that treatment is started immediately from the moment the child is born.
Ventriculomegaly requires mandatory treatment and monitoring of the course of the disease over time; if this is not done, the child will develop neurological diseases and other consequences that will significantly affect his health and development.
