Causes and clinical manifestations of hydrocephalic syndrome

Babies are often diagnosed with hydrocephalic syndrome. In fact, hydrocephalic syndrome in infants is not such a common occurrence. What kind of pathology is this, and what does it threaten? What are its features?

Subsequent examination may not confirm hydrocephalic syndrome in a child. Even if the diagnosis is confirmed, do not be alarmed. The main thing is to have the information and follow all the doctor’s recommendations.

Hydrocephalic syndrome in adults has its own characteristics. It is provoked not by congenital, but by acquired pathologies of brain nutrition.

Peculiarities

All signs of hydrocephalic syndrome are associated with the fact that an excessive amount of cerebrospinal fluid accumulates in the head. It should be there, but the volume is important. When there is an excess, cerebrospinal fluid begins to compress brain tissue, causing disruption of their functioning.

Fluid in the brain accumulates during development in the womb. Normally, before the birth of a child, its amount decreases. If this does not happen, the baby may lag behind in development and will suffer from high intracranial pressure.

Parents should not panic if such a diagnosis is confirmed. We'll fix everything. Correction is possible, especially if the baby is not yet six months old. The main thing is not to delay treatment and strictly follow all recommendations.

Hypertension syndrome in newborns can provoke an increase in intracranial pressure, but this is not an independent diagnosis. This is a symptom. He appears and then retreats for a while. Such jumps can be caused by emotional stress, physical activity and even food intake. In this case, there is no need to treat ICP. It is dangerous only in advanced hydrocephalus.

It is important to determine the origin of HGS in each specific case. For correction, massage is used in combination with drug treatment (do not confuse it with the mentioned syndrome).

Forms of the disorder

The internal form of hydrocephalus is the accumulation of a large amount of cerebrospinal fluid

In medicine, there are several types of hydrocephalus:

• hypersecretory – excessive formation of CSF;
• aresorptive - impaired resorption of CSF
• occlusive – blockade of the CSF outflow tract;
• external – increase in the content of internal CSF;
• external - an increase in the amount of external cerebrospinal fluid.

Development mechanism

Hypertension syndrome develops differently in adults, newborns and children. In the womb, the child grows quickly, and the system that feeds the brain rapidly develops. At first, it feeds only through blood, then, as it develops, cerebrospinal fluid also joins. By the end of intrauterine development, not only blood, but also spinal nutrition should be formed.

Each of us in the sixth month of development in the womb had quite a lot of fluid in the head. This is due to the fact that the ventricles of the fetal brain are wider. Then they should narrow and return to normal before birth. Then the cerebrospinal fluid completely leaves the head.

If this does not happen or the process has slowed down, a diagnosis of “hypertensive syndrome in infants” is made.

Do not confuse hypertensive hydrocephalic syndrome with hydrocephalus. These are two different diagnoses. The latter has more serious consequences and develops differently.

Complications and prognosis

Complications of hypertensive-hydrocephalic syndrome are possible at any age:

• delayed mental and physical development;
• blindness;
• deafness;
• coma;
• paralysis;
• epilepsy;
• urinary and fecal incontinence;
• death.

The prognosis is most favorable for hydrocephalic syndrome in infants. This is because they experience transient increases in blood pressure and cerebrospinal fluid, which stabilize with age.

In older children and adults, the prognosis is relatively favorable and depends on the cause of HGS, the timeliness and adequacy of treatment.

Sources:

• Bitterlich L.R. On the issue of differential diagnosis, classification and differentiated treatment of hydrocephalus and hirdocephalic syndromes in children. - International Neurological Journal, No. 1 (79), 2021.
• Order on approval of the standard of specialized medical care for children with hydrocephalus. — Ministry of Health of the Russian Federation, 2013.
• Federal clinical guidelines for the provision of medical care to children with consequences of perinatal damage to the central nervous system with hydrocephalic and hypertension syndromes. — approved Union of Pediatricians of Russia February 14, 2015

Causes

There may be several reasons for this pathology in newborns. Most often this is:

1. infections;
2. complications during pregnancy;
3. brain damage;
4. prematurity;
5. abnormalities of brain development;
6. long stay without water (12 hours or more);
7. chronic diseases (mothers);
8. trauma during childbirth.

The diagnosis of “hypertensive hydrocephalic syndrome” is usually made only in the countries of the former USSR. Russia is no exception. In the West, it is considered a manifestation of certain brain pathologies.

During pregnancy, it is important to take all tests and monitor general blood counts. An analysis for toxoplasmosis will help prevent a crisis and identify the problem in time.

Acquired reasons:

• hematoma, abscess, tumor, brain cysts;
• foreign bodies;
• bone fragments that entered the brain during a skull fracture;
• intracranial hypertension;
• infections;
• metabolic disorders;
• cervical osteochondrosis;
• disorders after a stroke.

The listed reasons may indirectly affect the drainage of fluid from the brain area. The disease itself manifests itself in different ways - from mild to severe. Symptoms become especially pronounced if the disease is not treated.

Toxoplasmosis can provoke not only hydrocephalic syndrome. It leads to serious disorders of the central nervous system. Manifestations will not keep you waiting. The most dangerous thing is encephalopathy. This is a pathological condition of the brain in which nerve cells die and dystrophic changes occur. Brain functions are seriously impaired.

Hypoxia, prematurity (deep), and infections can disrupt the normal development of the fetus.

Childbirth itself is also extremely dangerous for the baby. Although this is a natural process, it often leads to complications and injuries. Childbirth can cause hemorrhages, injuries, and hypoxia. An external open or closed fracture or dislocation may even occur. All these reasons can provoke a serious imbalance between the process of production and absorption of cerebrospinal fluid. But it is he, along with blood, that nourishes the brain. It is important to sub-compensate for loads.

Sometimes after birth the baby has a residual syndrome. These are changes in brain tissue and functions due to mechanical trauma.

Etiology

All etiopathogenetic factors of HS are divided into two large groups - congenital and acquired.

Congenital causes of hydrocephalic syndrome include:

• Perinatal hypoxia,
• Intrauterine intoxication with alcohol or medications,
• cerebral ischemia,
• Birth head injuries
• Hemorrhage into the subarachnoid space,
• Intrauterine infection of viral or bacterial etiology,
• Congenital toxoplasmosis,
• Congenital malformations of the brain.

Among the acquired reasons:

1. Hematomas associated with head trauma,
   increased intracranial pressure due to hemorrhage under the lining of the brain
2. Tumors of various parts of the brain and optic nerve,
3. Infections – meningitis, cysticercosis, malaria, encephalitis,
4. Hypertension of unknown etiology,
5. Operations on the posterior cranial fossa,
6. Hypervitaminosis A,
7. Foreign bodies and neoplasms - abscesses and cysts in the brain,
8. TBI with the introduction of bone fragments into the brain,
9. Atherosclerosis of cerebral vessels, hypertension, stroke, diabetic macroangiopathy,
10. Metabolic disorders.

Idiopathic syndrome develops in cases where it is impossible to identify its cause.

There are risk factors that contribute to the development of pathology in newborns:

• toxicosis in a pregnant woman - preeclampsia, eclampsia,
• arterial hypertension in the expectant mother,
• drinking alcohol during pregnancy,
• childbirth up to 36 weeks,
• delayed birth - after 42 weeks,
• long stay of the fetus in the womb without water,
• multiple pregnancy,
• chronic maternal pathologies - diabetes mellitus, hypo- or hyperthyroidism, collagenosis,
• mother's age is more than 40 years,
• hereditary predisposition.

Liquor is actively produced by the choroid plexus of the ventricles of the brain, ependyma and meninges. Passive production of cerebrospinal fluid occurs as a result of changes in osmotic pressure and the movement of ions outside the vascular bed.

Liquor is absorbed by the cells of the arachnoid membrane, the vascular elements of the membranes of the brain and spinal cord, ependyma, parenchyma, and connective tissue fibers running along the cranial and spinal nerves.

Mechanisms providing liquor flow:

1. hydrostatic pressure drop,
2. the process of secretion of cerebrospinal fluid,
3. movement of ependymal villi,
4. cerebral pulsation.

The pathogenesis of hydrocephalic syndrome is based on the processes of obstruction and obstruction of the cerebrospinal fluid pathways with impaired reabsorption of cerebrospinal fluid.

Pathogenetic factors of the syndrome:

• Overproduction of cerebrospinal fluid in the brain,
• Accumulation of cerebrospinal fluid,
• Enlargement of the ventricles of the brain
• Penetration of cerebrospinal fluid into the medulla,
• Scar formation in brain tissue.

The amount of cerebrospinal fluid in the skull changes when the balance between its production and absorption is disturbed. The accumulation of cerebrospinal fluid in the ventricles of the brain occurs due to the formation of obstacles to its normal outflow. If left untreated, death can occur.

Symptoms

It is very difficult to determine visually whether these are symptoms of the syndrome or behavioral characteristics of the baby. Pathology manifests itself in different ways. The nature of the manifestations depends on the form, degree of the disease, and its cause. However, there are still signs that may be symptoms of HGS:

• irritability;
• anxiety;
• the child has poor sleep;
• frequent and prolonged cry of the baby;
• lethargy;
• low activity;
• drowsiness.

The severity of symptoms depends on the genesis of the disease. Many of them are the result of hypertension. Children with HGS may experience bulging of the eyes, Graeffe's sign (a noticeable white stripe forms between the upper eyelid and the pupil).

With severe development, the upper eyelid may half cover the eye (the “setting sun” symptom). Such children may develop strabismus and throw their heads back. Muscle tone can be either decreased or abnormally increased. This is especially noticeable in the leg muscles. The child may walk on tiptoes. Such symptoms should alert you. You need to immediately show the child to the pediatrician, and if necessary, he will refer you to a pediatric neurologist. Therapy must be comprehensive.

In children with this syndrome, reflexes (walking, crawling, grasping) are reduced. Such movement disorders often accompany HGS. Hypertensive abnormalities are often observed.

The listed symptoms also occur with other pathologies, for example, with perinatal encephalopathy (PEP). It often results from prolonged intrauterine hypoxia (oxygen deficiency).

PEP becomes the result of disruption of intrauterine development processes. The child can recover, but long-term developmental activities are needed.

It is important to exclude other diseases. With PEP, it is strictly forbidden to prescribe diuretics. They can cause heart problems and interfere with neuro-reflex processes. But for HGS and hypertension they are indicated.

A mandatory symptom of HGS is pathological changes in the size of the baby’s head circumference. She's growing too fast. In a month, 1.5 cm or more may increase. At the same time, the seams of the skull may swell, and the shape of the head itself changes.

Some people may have a naturally large head. This is a genetic feature, not a symptom of pathology. This is why it is important to do an ultrasound and not guess based on tactile and visual examination. If one of the parents has a large head, then the baby’s large head is not a pathology.

HGS does not manifest itself in newborns. In older children, HGS is often associated with infection or trauma. Characteristic symptoms:

• I often have a headache (the pain is throbbing, bursting or aching, more often happens in the morning). Localization – forehead, temples, brow ridges;
• nausea, vomiting;
• the child has difficulty lowering his head or raising his eyes;
• dizziness;
• may see double and consciousness may be impaired;
• Sometimes there are convulsions and even coma.

During a painful attack, the child may turn pale, be lethargic, and feel general weakness. He is bothered by a loud sound and the light seems bright.

Diagnostics

If symptoms of HGS occur, you should contact a pediatric neurologist. You may also need to consult a neurosurgeon and ophthalmologist. To make a preliminary diagnosis, anamnesis is collected and the baby is examined. Subsequently, diagnostic procedures are prescribed to confirm the diagnosis and find out the cause of the disease.

Diagnostic methods:

• ultrasound examination (ultrasound);
• radiography;
• magnetic resonance imaging (MRI);
• lumbar puncture (taking a sample of cerebrospinal fluid for laboratory analysis);
• echoencephalography.

Based on the results of the diagnostic examination of the child, further treatment is prescribed.

Diagnostic features

Hypertensive-hydrocephalic syndrome is very insidious. It is not easy to diagnose, especially at an early stage. Only a pediatric neurologist can make the correct diagnosis. He will determine the degree of pathology, its causes, changes in the tissue structure of the brain. Moreover, it is based on head ultrasound data. As they say, it is impossible to make such a diagnosis by eye, although many pediatricians are guilty of this. At the first signs of nervousness, poor sleep, or suspicion of increased intracranial pressure, doctors rush to announce the diagnosis of “hydrocephalic syndrome.”

By the way, in 95% of cases such an intuitive diagnosis is not confirmed again. It often turns out that this is not a disease, but the behavioral characteristics of a particular child. If the diagnosis is confirmed, the disease often has a moderate manifestation.

Even instrumental methods do not always help in making a diagnosis. If we are talking about a baby, it is important to monitor the dynamics of the increase in head circumference and check reflexes.

The following methods are also used:

• the condition of the fundus vessels is analyzed;
• neurosonography is performed;
• sometimes a puncture is made in the lumbar region to analyze the pressure of the cerebrospinal fluid (this method is considered the most reliable);
• CT scan;
• nuclear magnetic resonance.

Symptoms by age

Typical symptoms of hydrocephalic syndrome are visual disturbances

Symptoms are caused by intracranial hypertension and hydrocephalus.

General signs of HS:

• nausea;
• vomit;
• headache;
• personality changes or epileptic seizures.

However, in children and adults, the symptoms of hydrocephalic syndrome differ significantly.

In infants

In a child under one year old with hydrocephalic syndrome, decreased muscle tone, weak tremor, and a positive Graefe sign (“setting sun”) are noted.

There is an increased increase in head circumference (about 1-2 cm monthly). The baby's head looks disproportionately large.

Sometimes a baby with hydrocephalic syndrome exhibits swelling of the optic disc.

In children and adolescents

Symptoms of HS in adolescents and children;

• visual impairment (strabismus, loss of visual field), decreased visual acuity to the point of blindness;
• fine motor skills disorders;
• disturbances in concentration;
• personality changes, behavioral problems (anxiety, impatience, categoricalness), hypersensitivity to noise;
• respiratory, speech and swallowing disorders;
• endocrinological (metabolic) disorders;
• early puberty before 8 years of age (very rare).

In adults

Symptoms of HGS in adult patients:

• cephalalgia, vomiting, dizziness in the morning;
• visual impairment (diplopia - double vision);
• convulsive conditions;
• severe fatigue;
• restless behavior.

Symptoms of hydrocephalus with normal intracranial pressure:

• moderate gait disturbances;
• dementia and/or personality changes (forgetfulness, increased irritability);
• hydrocephalic cry;
• urinary and fecal incontinence.

If the diagnosis is confirmed

If ultrasound and neurosonography confirm HGS, you will need to:

• treat the baby with medication;
• visit a massage therapist regularly;
• postpone routine vaccinations for a while.

The healing power of massage should not be underestimated. A competent massage therapist can work miracles. It is indispensable in the treatment of HGS. Replacement therapy is also important. It compensates for the insufficient output of cerebrospinal fluid. It is important to treat not the symptoms, for example, high intracranial pressure, muscle tone disorders, poor sleep, but the cause. Specific treatment methods should be selected by a neurologist - pediatric or adult.

How does hydrocephalus of the brain manifest in children?

In newborns, the cranial bones are still mobile in the fontanelle area and easily diverge, so the main symptom of hydrocephalus is a head circumference of 50–70 cm - this is almost 2 times more than normal. The proportions are disturbed, the forehead is enlarged, the skin around the fontanel does not pulsate. In the subcompensated stage with the absence of visual signs, pathology is detected only during examination of the brain, since the symptoms are nonspecific.

Important: the earlier childhood hydrocephalus is detected, the higher the chances of stopping its development and successful treatment.

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Up to a year

Even at the subcompensated stage, pathology in infants clearly makes itself felt. The nervous system and brain structures are imperfect, so the child’s general condition is serious. The main manifestations of hydrocephalus in infants include:

• disproportionately large head with an enlarged forehead;
• bulging fontanel;
• a clear pattern of swollen veins on the temples, forehead and back of the head;
• lag behind the norm in weight gain, attempts to crawl, sit, walk;
• poor sleep, tearfulness, irritability;
• breast refusal or sluggish sucking, frequent belching;
• strabismus and other visual disorders;
• the head does not hold up - it falls back;
• convulsions, loss of motor reflexes, vomiting (with a rapid transition from the subcompensated stage to the decompensated stage).

Over a year old

A doctor can identify pathology in the subcompensated stage in a child at 1.5–2 years of age or older during examination, since the symptoms here are less striking and are mainly manifested by neurological disorders. If hydrocephalus was not detected at birth, deviations in head size may not occur. The clinical picture is formed by the following signs of the subcompensated stage of pathology:

• morning headaches;
• increased excitability;
• poor appetite;
• lethargy;
• decreased hearing, vision;
• muscle hypertonicity;
• impaired coordination of movements;
• slow mental development;
• involuntary urination;
• The child does not sit down himself, but maintains the position.

Methods for detecting pathology at the stage of intrauterine development

Throughout pregnancy, the doctor prescribes ultrasound examinations to the expectant mother several times to monitor the development of the fetus. To identify pathology, an important parameter is the size of the head and the correspondence of these indicators to standard values, especially at 16–20 weeks. With an abnormally rapid increase in numbers, they speak of deviations in the development of the nervous system and the accumulation of cerebrospinal fluid, but an absolutely accurate diagnosis - whether it is hydrocephalus - cannot be made before the birth of the child.

Treatment

A neurologist selects the correct treatment regimen. In severe cases, the assistance of a neurosurgeon may be required. An ophthalmologist is often involved. He analyzes how full the vessels of the fundus are, whether they are spasming, etc. Such patients are treated in neurological departments or centers.

Treating a newborn

Traditional methods will not help here. Unqualified treatment can lead to disastrous consequences. Babies up to 6 months are treated on an outpatient basis. The following activities will be required:

• Treatment with diacarb. This is a diuretic. It reduces the production of cerebrospinal fluid. Enhances fluid removal.
• Using nootropics. These drugs stimulate blood supply to the brain (Actovegin, Piracetam, Asparkam).
• Treatment with sedatives (Tazepam, Diazepam).
• A professional massage is a must.

We treat older children and adults

When selecting methods and means of therapy, the doctor must take into account the specific cause of the syndrome. If it is a neuroinfection, treatment with antibiotics or antiviral drugs will be required. Injuries often require the help of a surgeon.

Prognosis, complications

Complications can develop at any age:

• deafness;
• blindness;
• developmental delay;
• paralysis;
• coma;
• epilepsy;
• fontanelle disorders;
• fecal and urinary incontinence;
• mortality.

The younger the child who gets sick, the better the prognosis. The pathology is most easily tolerated by children under 6 months. Their condition can quickly stabilize. Blood pressure, even if it fluctuates, quickly returns to normal.

For older children, the prognosis will be relatively favorable. It will be affected by the reasons, timeliness of treatment, and its adequacy.

Treatment of hydrocephalic syndrome

Treatment of hydrocephalic syndrome is carried out by neurologists and neurosurgeons with the involvement of ophthalmologists. Patients with HGS need to be observed and treated in a specialized neurological center.

Treatment in newborns

Children require outpatient or surgical treatment, depending on the cause. Main therapeutic measures:

• prescribing a diuretic drug - diacarb (reduces the production of cerebrospinal fluid and removes excess fluid from the body, primarily from the cranial cavity);
• it is possible to take nootropics - some experts claim that these drugs improve blood supply to the brain (Encephabol, Actovegin), but there are no convincing large-scale studies on this topic;
• for hyperexcitability, sedatives are indicated;
• massage.

Treatment for infants is quite long, taking several months.

Treatment of HGS in older children and adults

In adults and older children, therapy also depends on the cause of hydrocephalic syndrome. If it is the result of a neuroinfection, then appropriate antiviral or antibacterial therapy is carried out.

In case of traumatic brain injuries and tumors, surgical intervention is indicated. Diuretics and nootropics are also used.

Treatment in newborns

Treatment of HGS in newborns should be carried out according to a specific regimen.
The child must be registered with a neurologist until he reaches the age of 1 year (according to the testimony of a specialist, this period is extended). Drug treatment involves the use of drugs whose action is aimed at removing excess fluid. The most popular of this group of drugs is Veroshpiron , which is contraindicated for:

• hypersensitivity to the main components;
• Addison's disease;
• hyperkalemia;
• hyponatremia;
• renal, liver failure;
• Lactase deficiency, lactose intolerance.

Average price:

tablets (50 mg) - 80-90 rubles;

capsules (100 mg) - 240-260 rub.

In addition, for the treatment of HGS in newborns, it is necessary to use drugs that provide vascular tone. One such remedy is Aescusan, which has the following contraindications:

• hypersensitivity to the components of the drug;
• liver diseases;
• lactose intolerance;
• lactase deficiency;
• childhood.

Average price:

solution for oral use (1 pc.) - 160-175 rubles.

Additional therapy may include the use of sedatives, natural medicines and their infusions (valerian, mint, etc.).

Often the cause of the development of the syndrome is dysfunction of the nervous system.

In addition to the recommended drug treatment, mothers and fathers are advised to organize a normal diet for the baby, wakefulness and sleep, walks, eliminate the influence of external irritants and carry out the prevention of infectious diseases.

Treatment in adults

Treatment of the disease in adults is necessary, since advanced pathology poses a serious threat .
Therapy consists of taking diuretics: Triampur

Contraindications

• anuria;
• chronic renal failure;
• acute glomerulonephritis;
• hepatic coma, precoma;
• functional liver disorders;
• impaired electrolyte metabolism (hyperglycemia, hypercalcemia, etc.);
• pregnancy and lactation;
• allergic reactions to sulfonamides.

Average price:

tablets - 280-310 rub.

If the disease in adults is mild, then it is possible to do without drug therapy, but subject to several rules:

• it is important to normalize your water consumption;
• perform exercises aimed at normalizing blood pressure (selected by your doctor).

In rare cases, hypertensive hydrocephalic syndrome can threaten the patient's life. In such situations, surgical intervention is resorted to, during which shunts are implanted. With the help of such implants, excess fluid from the brain will be removed. This method of treatment leads to a gradual reduction in the symptoms of the pathology, and subsequently a complete cure.

Symptoms in children

Symptoms of the pathology in children appear after head and/or brain injury or infection. A characteristic manifestation is a migraine, which bothers the child, usually in the morning after waking up, a feeling of nausea and profuse vomiting, which does not bring relief to the baby. The pain is concentrated in the temples, in the forehead and brow ridges. The pain can be dull, aching, or bursting.

Children with HHS complain of the inability to lower their head down or raise their eyes, severe dizziness (younger children describe a condition such as “swinging objects”, swinging on a carousel).

During a headache attack, parents may notice paleness of the child's skin , weakness, and apathy. There is an irritable reaction to sound and light.

Due to increased muscle tone in the legs, children with the syndrome move, relying on the entire foot, and only on the toes. Also, hypertensive hydrocephalic syndrome in children is characterized by the presence of strabismus, lethargy, memory impairment and inattention.

More details about HGS in a child