Congenital hydrocephalus is an excessive accumulation of cerebrospinal fluid in the brain from birth. With this type of pathology, the ventricular systems of the brain and the subarachnoid space expand. This phenomenon is better known as dropsy of the newborn. However, according to ICD-10, congenital hydrocephalus is defined precisely as "hydrocephalus" and nothing else.
In this pathology, due to the cerebrospinal fluid, pressure on the brain increases, which leads to damage to the organ, the development of physical and mental disabilities. To avoid all this, it is necessary to start treatment as early as possible, and early diagnosis of the disease contributes to it.
According to statistics, every thousandth newborn suffers from congenital hydrocephalus. Pathology is often found in children born with abnormal development of the brain tube.
Another type of ailment is acquired hydrocephalus, which develops at any time after birth.
What is
Congenital hydrocephalus is caused by an abnormal accumulation of cerebrospinal fluid inside the brain. Liquor is produced by the choroid plexus of the brain. Moreover, the level of productivity depends on the time of day. Normally, a person contains about 150 cerebral fluid. It covers the spinal cord and brain, fills the ventricular system, which consists of four ventricles. CSF flows through the ventricles of the brain, exits into the subarachnoid cavity, where it is reabsorbed into the bloodstream.
CSF absorption and production are balanced processes. The liquid itself has special purposes. It acts as a shock absorber for the brain, providing protection against concussions and shocks. CSF also delivers the nutrients the brain needs to function properly.
Liquor provides oncotic and osmotic balance. CSF accumulates antibodies that protect the brain from bacteria and viruses. The fluid takes part in the regulation of blood circulation in the closed space of the cranial cavity, spinal canal.
When there is an imbalance between the production and removal of fluid, the expansion of the ventricles of the brain occurs. Due to their increase, pressure on the brain increases, which can lead to serious neurological damage to the organ.
International Classification of Diseases
Congenital hydrocephalus in the ICD-10 refers to the group of congenital anomalies, deformities and chromosomal disorders (Q00-Q99). This class includes a variety of congenital anomalies in the development of the nervous system, eyes, ears, neck, circulation, respiration, palate andlips.
In ICD 10, congenital hydrocephalus is included in the group of congenital malformations of the nervous system - Q00-Q07. This group includes a variety of cephalies, malformations, including congenital anomalies of the brain, spinal canal, and spinal cord. In ICD-10 CNS CM, congenital hydrocephalus is described in section Q03.
Types of pathology
Currently, there is no single classification of hydrocephalus. The reasons for diversity are polyetiology, diversity of morphological manifestations.
According to etiological properties, congenital hydrocephalus (ICD code 10 Q03) is divided into the following types:
- Hydrocephalus, in which normal cranial pressure is maintained. Usually, the pathology is observed in people over 50 years of age. In most cases, the cause of the pathological condition is not determined.
- Congenital hydrocephalus. It can occur due to congenital infectious pathologies suffered by the mother during pregnancy, with congenital developmental anomalies and a number of other reasons.
- Acquired hydrocephalus. Most often it develops as a complication of a stroke, brain tumors, after a head injury.
According to morphological features, hydrocephalus is occlusive and communicating. The latter is characterized by impaired absorption of cerebral fluid due to damage to certain structures.
According to the nature of the course, the pathology is divided into acute, subacute and chronic. The first type occurs before the development of gross decompensation. The acute form lasts up to threedays. Then comes the subacute stage, which develops for about a month. In a chronic course, pathological changes develop slowly, up to six months or longer.
Forms of illness in children
Congenital hydrocephalus (ICD-10 code above) has several degrees of severity. It can be moderate, in which the lateral ventricles dilate up to three centimeters, and the minimum thickness of the brain cape is more than two centimeters. When the ventricles expand to four centimeters, and the cloak narrows to one, they speak of severe dropsy. The critical form is characterized by an expansion of the ventricles to five centimeters or more and a narrowing of the cerebral cape to five millimeters or less.
Causes of a congenital type of illness
The most common cause of congenital hydrocephalus is stenosis of the Sylvian Falls, which connects the fourth ventricle to the third. It is not uncommon for the disease to occur due to intracranial hemorrhage under the epindima of the cerebral aqueduct, caused by an aneurysm of the cerebral vein. Most often, congenital hydrocephalus of the brain is of the closed type, i.e. occlusive.
In half of the cases of the disease in the anamnesis, infectious pathologies of the mother are detected. This is another cause of congenital hydrocephalus in a child. Examples of pathologies that cause dropsy are:
- Cytomegalovirus. This is one of the most common types of viruses transmitted transplacetally - it is transmitted through the placenta from mother to fetus. It puts the fetus at risk for abnormal development.
- Rubella. Pathology is not dangerous, but withinfection during pregnancy, especially in the early stages, there is a high probability of serious violations of the formation of the fetus.
- Pig. The disease refers to acute infectious pathologies in which the glands are affected, in particular, the parotid.
- Syphilis. The disease belongs to venereal. If a pregnant woman is infected in the early stages of pregnancy, pathology can lead to miscarriage, fetal growth retardation, and premature birth.
- Toxoplasmosis. The pathology is parasitic, caused by toxoplasma. With a congenital type of illness, an anomaly in the development of the child's nervous system occurs.
There are a number of other pathologies that lead to the development of congenital hydrocephalus of the brain.
Signs of illness
The main sign that suggests dropsy in a child is an increase in the size of the head. You may also experience the following symptoms:
- breathing disorder;
- frozen, prone to contraction of the muscles of the arms, legs;
- delayed development of some stages, for example, the child cannot learn to sit, crawl;
- bulging fontanelles, changing their shape;
- appetite disorder;
- refuse to move head;
- the scalp is thin, the veins are visible.
Congenital hydrocephalus in children can be manifested by a violation of the location of the pupil, it is close to the lower part of the eyelid. This symptom is known as the setting sun symptom. There may also be convulsions, vomiting.
Symptoms
If congenitalinternal hydrocephalus does not progress, then it does not have severe symptoms. Most often, in these children, the head circumference remains normal, other clinical symptoms are practically absent.
As dropsy progresses, there is a dynamic increase in head size. In premature babies in the first month of life, the circumference should not increase by more than one centimeter per week. With faster growth, doctors suggest dropsy.
Signs of disease progression are the following symptoms:
- swollen fontanelles;
- the head is deformed, the proportions of the skull are not respected, a pronounced predominance of the cerebral region;
- muscle tone impaired;
- convulsions;
- chin tremor;
- tendon reflexes.
At the moments of increased intracranial pressure, the child vomits, pulse quickens, breathing is disturbed. There is a lag in psychomotor development, the functions of the autonomic nervous system are disturbed, the function of the cranial nerves changes.
Diagnostic Methods
Different diagnostic methods are used to establish a diagnosis. They include: collection of complaints, anamnesis, evaluation of clinical data. In children, there is an increase in the head, nausea, vomiting, headaches. Newborns cry all the time because of pain. Instrumental diagnostic methods in the form of MRI, CT are mandatory, and an examination of the fundus is carried out. Often a consultation with a neurosurgeon is needed.
There are methods to detect congenitalhydrocephalus of the brain in utero. For this, the determination of alpha-fetoprotein in the blood serum, amniotic fluid, and karyotype is prescribed. An ultrasound examination is carried out. These methods allow you to determine the anomaly in the development of the nervous system, and the pregnant woman decides whether the pregnancy will be terminated. This approach helps to reduce the birth rate of children with abnormal development of the nervous system in many countries of the world.
This diagnostic technique is carried out among women at risk. These are all those whose work is associated with hard physical labor, who suffered from infectious diseases during pregnancy, who have a poor environmental situation, work in hazardous production.
After birth, the baby's head circumference is constantly measured. With a deviation from the norm, various tests begin to run. The simplest and most affordable method is to conduct diaphanoscopy - transillumination of the head with light. Ultrasound is mandatory, tomography can be prescribed. It makes it possible to see the cavity with liquid, assess its size, the severity of cerebral edema.
Ultrasonography
Fetal ultrasonography, neurosonography is the only primary diagnostic method that allows you to determine congenital hydrocephalus of the brain and monitor the condition of the ventricles, subarachnoid spaces. This technique is used not only for intrauterine examination, but also for examination of older children.
Craniography
Knowing how congenital hydrocephalus manifests itself, a craniographic method is performedexaminations. It helps to assess the bone tissue of the skull, the condition of the sutures, the shape of the base, and to see local changes. With different types and degrees of dropsy, a different location of the transverse sinus is observed.
Ophthalmoscopy
The vascular system of the brain and the eye are directly connected. With an increase in intracranial pressure, the outflow of blood through the veins of the fundus becomes difficult. This phenomenon leads to the formation of edema of the optic nerves, small hemorrhages in the retina. The presence of congestion in the fundus is a direct sign of high blood pressure.
Tests
When diagnosing, infusion-drainage tests are mandatory. They allow you to determine the quantitative parameters, elastic properties of tissues, liquorodynamics.
The test method is to drain the lumbar cistern and reduce the pressure in the sinuses. When infused with saline, there is a short-term increase in pressure in the sinuses, then it decreases. Calculations are carried out using special formulas to determine the fluid velocity, resistance and other parameters.
Testing helps determine if surgery is needed.
Treatment
The causes and treatment of congenital hydrocephalus are closely related. The doctor may prescribe surgery or choose a conservative method of therapy.
Conservative methods include drugs that reduce intracranial pressure, diuretics are selected.
Therapy also includes drugs that eliminate the inflammatory process,leading to the development of dropsy. It can be antibiotics and other medicines. With the progression of the disease, occlusions occur, surgical intervention is indicated.
In most cases, drug treatment does not give the desired results, which is the basis for surgical intervention. They are conventionally divided into several groups:
- Interventions to temporarily reduce ICP and neutralize cranio-cerebral imbalance.
- Surgical intervention to remove fluid to other body systems.
- Operations carried out to restore fluid circulation.
- Interventions to suppress CSF production.
- Surgical intervention to activate CSF sorption.
Surgical treatment involves removing the cause that led to the accumulation of fluid in the brain, performing a bypass operation. It involves the implantation of a silicone tube through which fluid is drained from the skull into another cavity.
Surgical treatment methods
In various periods of development, neurosurgery gave preference to CSF shunting operations. They allow for occlusion of the CSF pathways by shunting with implantation of the valve system. During surgery, a catheter is placed in the brain, through which excess cerebrospinal fluid is drained to other parts of the body. This may be a combination with the abdominal cavity, the chambers of the heart. The doctor inserts one end of the catheter into the ventricle of the brain,then creates a subcutaneous tunnel. Usually such a procedure gives positive results, after which it is not necessary to carry out repeated operations. However, complications such as infection or blockage of the shunt may occur.
Patients with dropsy walk with a shunt all their lives. If it is installed in childhood, then as they grow, several more operations are performed, in which the tube is replaced with a longer one.
In some cases, plastic aqueduct of the brain is shown. It is performed on patients with occlusion by endoscopic aqueductoplasty.
In patients, the rostral orifice of the cerebral aqueduct is covered with a thin and elastic connective tissue, which was formed as a result of hemorrhages and infections. Eliminating it is simple, but before this procedure, a thorough examination of the patient is carried out using tomography.
For the treatment of newborns in cases where massive subarachnoid, intraventricular hemorrhages occur, diuretics are combined with unloading punctures of the lumbar, ventricular region. When an intrathecal hematoma appears, a procedure is performed to remove it.
The drainage system allows you to normalize ICP, as well as control the dynamics of cerebrospinal fluid. This allows avoiding hypertensive CSF crises and postponing bypass surgery.
For children with low body weight, an additional subcutaneous reservoir can be installed, which allows you to repeatedly pump out cerebral fluid, control ICP.
In the treatment of progressive hydrocephalus, bypass surgery is performed. In 50% of cases, immediately after the installation of a shunt in children, the general condition returns to normal, which almost completely eliminates postoperative complications.
After surgery
A shunt is being placed to treat hydrocephalus. Most often, it remains for life. However, periodic medical monitoring of valve pressure may be required. A surgeon is watching him.
In addition, after bypass surgery, it is necessary to monitor the state of he alth by constantly visiting a doctor. This is due to the fact that each operation can be complicated. To avoid this, it is recommended to regularly undergo preventive examinations by a surgeon, a neurologist. These specialists will monitor the development of hydrocephalus and determine if the shunt can be removed.
Complications of dropsy
Complications of dropsy can be:
- hearing loss, visual impairment up to blindness;
- muscle weakness;
- disturbed carbohydrate, fat metabolism;
- there are violations of the respiratory, SS system;
- regulation of body temperature is disturbed.
When complications occur, the risk of death is high. To avoid complications, it is necessary to start treatment of pathology as early as possible. It allows you to achieve either a complete recovery or a stable remission.
With implanted shunts, people live their entire lives. If dropsy does not return after a few years, the shunt is removed. This outcome is considered a full recovery.
Complications after operations
Postoperative complicationsobserved in about 20% of cases, late - in 15%. Early complications are characterized by inflammatory changes, the formation of fibrinous clots.
Sometimes there are infectious complications in the form of bacterial meningitis, ventriculitis. Less common glomerulonephritis, endocarditis. With a prolonged course of ventriculitis, periventricular encephalitis occurs.
Complications can be caused by:
- infectious processes in postoperative wounds;
- shunt contamination with pathogen growth on it;
- necrotic complications.
Most often, non-infectious ventriculitis provoked by antiseptics occurs after ventriculostomy.
Hyperthermia, a feverish state may be due to the reaction of the hypothalamus to the heating of the light flux of the endoscope.
The rarest complications are bleeding leading to bulimia and diabetes insipidus.
Prevention
To prevent congenital hydrocephalus, a thorough examination of pregnant women is carried out, especially if they are at risk. Women are advised to follow the daily routine, eliminate stressful situations, overwork. Measures are also being taken to prevent infectious pathologies such as meningitis, encephalitis, syphilis and other diseases that lead to infection of the fetus, its abnormal development.
Newborns must undergo transcranial ultrasonography. This diagnostic procedure is done on the first day of life to exclude hemorrhages and congenital malformations of the central nervous system. Verificationintraventricular hemorrhages, neurosonographic monitoring is carried out every day.
When increasing ventriculomegaly, children should be in intensive care under constant supervision. In some cases, it is advisable to immediately conduct ventriculosubgaleal drainage in order to evacuate the CSF.
In pediatric surgery, the least traumatic method in the treatment of hydrocephalus is endoscopic ventriculostomy, which is performed on children older than one year.
Disability
In some cases, a child is assigned a disability group for congenital hydrocephalus. It is determined by the degree of loss of he alth. The status of a "child with a disability" is given for 1 or 2 years, or until the age of 18.
Hydrocephalus is included in the list of pathologies in which a child is given a disability before he reaches 16 years of age. However, in order to clarify which group will be given, it is necessary to pass a medical examination, at which doctors will make a conclusion about the state of he alth, existing disorders, developmental anomalies.
Timely treatment will allow the child to live, develop normally, without deviations from the norm. Even if he gets a bypass, there is a chance he will live a full life or make a full recovery.
Modern medicine has learned to help people with a variety of diseases, and dropsy is no exception.
