Congenital hydrocephalus is an excessive accumulation of cerebrospinal fluid since 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.
With this pathology, pressure on the brain increases due to cerebrospinal fluid, which leads to organ damage, the development of physical and mental disabilities. To avoid all this, it is necessary to begin treatment as soon 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
Congenital hydrocephalus is caused by 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, consisting of four ventricles. CSF flows through the ventricles of the brain, enters the subarachnoid cavity, where it is reabsorbed into the bloodstream.
The absorption and production of cerebrospinal fluid are balanced processes. The liquid itself has special purposes. It performs the function of a shock absorber of the brain, providing protection against concussion and shock. CSF also supplies the nutrients necessary for the brain to function properly.
Liquor provides oncotic and osmotic balance. In CSF antibodies accumulate that protect the brain from the penetration of bacteria and viruses. The fluid takes part in the regulation of blood circulation in a confined space of the cranial cavity, spinal canal.
When an imbalance occurs between the production and removal of fluid, the ventricles of the brain expand. 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 belongs to the group of congenital malformations, deformations and chromosomal abnormalities (Q00-Q99). This class includes a variety of congenital anomalies in the development of the nervous system, eyes, ears, neck, blood circulation, respiration, palate and lips.
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 malformations of the brain, spinal canal, and spinal cord. In ICD-10 CNS CNS, congenital hydrocephalus is described in section Q03.
Types of pathology
There is currently no unified classification of hydrocephalus. The causes of diversity are polyetiological, the diversity of morphological manifestations.
By etiological properties, congenital hydrocephalus (ICD code 10 Q03) is divided into the following types:
- Hydrocephalus, in which normal cranial pressure remains. Usually, pathology is observed in people older than 50 years. In most cases, the cause of the pathological condition is not determined.
- Congenital hydrocephalus. It can occur due to congenital infectious pathologies that the mother suffered during pregnancy, with congenital malformations and a number of other reasons.
- Acquired hydrocephalus. Most often develops in the form of complications of a stroke, brain tumors, after a head injury.
According to morphological characteristics, hydrocephalus is occlusive and communicating. The latter is characterized by impaired absorption of cerebral fluid due to damage to certain structures.
By 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 three days. Then comes the subacute stage, developing about a month. In a chronic course, pathological changes develop slowly, up to six months or longer.
Forms of the disease in children
Congenital hydrocephalus (ICD-10 code above) has several degrees of severity. It can be moderate, in which the lateral ventricles expand to three centimeters, and the minimum thickness of the brain cloak is more than two centimeters. When the ventricles expand to four centimeters, and the cloak narrows to one, they speak of pronounced dropsy. The critical form is characterized by the expansion of the ventricles to five or more centimeters and the narrowing of the brain cloak to five millimeters or less.
Causes of a Congenital Type of Illness
The most common cause of congenital hydrocephalus is stenosis of Sylvia Falls, which connects the fourth ventricle to the third. Cases of the occurrence of an ailment due to intracranial hemorrhage under the epindym of the brain water supply caused by aneurysm of the brain vein are not uncommon. Most often, congenital hydrocephalus of the brain is closed, i.e., occlusal.
In half the cases of the occurrence of the disease, a history of maternal infectious pathologies is 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 by the trans-acetal route - through the placenta it is transmitted from mother to fetus. He puts at risk the abnormal development of the fetus.
- Rubella. Pathology is not dangerous, but with infection during pregnancy, especially in the early stages, there is a high probability of serious violations of the formation of the fetus.
- Piggy. The disease refers to acute infectious pathologies in which the glands are affected, in particular, the parotid.
- Syphilis. The disease refers to sexually transmitted. When a pregnant woman becomes infected in the early stages of pregnancy, pathology can lead to miscarriage, fetal growth retardation, and premature birth.
- Toxoplasmosis. Parasitic pathology, caused by toxoplasma. With a congenital type of ailment, 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 symptom suggesting dropsy in a child is an increase in head size. The following symptoms may also occur:
- respiratory failure;
- congealed, prone to contraction muscles of the arms and legs;
- developmental delay of certain stages, for example, a child cannot learn to sit, crawl;
- bulging fontanelles, changing their shape;
- impaired appetite;
- refusal to move his head;
- the scalp is thin, 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 called the setting sun symptom. Convulsions, vomiting can also be observed.
Symptoms
If congenital internal hydrocephalus does not progress, then it does not have pronounced symptoms. Most often, in such children, the head circumference remains normal, and other clinical symptoms are practically absent.
With the progression of dropsy, a dynamic increase in head size is noted. In premature infants 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:
- fontanelles are swollen;
- the head is deformed, the proportions of the skull are not respected, a pronounced predominance of the cerebral region;
- muscle tone is impaired;
- cramps
- chin tremor;
- tendon reflexes.
At moments of increased intracranial pressure, the child experiences vomiting, the pulse quickens, and breathing is disturbed. There is a lag in psychomotor development, the functions of the autonomic nervous system are disrupted, the function of the cranial nerves changes.
Diagnostic Methods
Different diagnostic methods are used to establish the diagnosis. These include: collection of complaints, medical history, evaluation of clinical data. In children, there is an increase in head, nausea, vomiting, headaches. Newborns constantly cry because of pain. Instrumental diagnostic methods in the form of MRI, CT scan are necessarily prescribed, the fundus is examined. Consultation of a neurosurgeon is often necessary.
There are methods to detect inborn hydrocephalus of the brain in utero. For this, a determination of alpha-fetoprotein in the blood serum, amniotic fluid, karyotype is prescribed. An ultrasound examination is performed. These methods allow you to determine the abnormality of the development of the nervous system, and a pregnant woman decides whether the pregnancy will be interrupted. 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 infectious diseases during pregnancy, who have poor environmental conditions, work in hazardous work.
After birth, the baby's head circumference is constantly measured. With a deviation from the norm, various tests begin to be carried out. The simplest and most affordable method - conducting diaphanoscopy - shining the head with light. Ultrasound is definitely prescribed, tomography can be prescribed. It makes it possible to see the cavity with the liquid, to evaluate its size, the severity of cerebral edema.
Ultrasonographic study
Ultrasonography of the fetus, 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 examination method is performed. It helps to evaluate the bone tissue of the skull, the condition of the sutures, the shape of the base, and see local changes. With different types and degrees of dropsy, a different arrangement of the transverse sinus is observed.
Ophthalmoscopy
The vascular system of the brain and eyes are directly related. With an increase in intracranial pressure, the outflow of blood through the veins of the fundus is difficult. This phenomenon leads to the formation of edema of the optic nerves, minor retinal hemorrhages. 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 determining quantitative parameters, elastic properties of tissues, and liquorodynamics.
The test method consists in draining the lumbar cistern and reducing the pressure in the sinuses. During infusion with saline, a short-term increase in pressure in the sinuses occurs, then it decreases. According to special formulas, calculations are performed to determine the fluid velocity, resistance, and other parameters.
Testing helps determine the need for surgery.
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 that caused the development of dropsy. It can be antibiotics and other medicines. With the progression of the disease, occlusion occurs, surgical intervention is indicated.
In most cases, drug treatment does not give the desired results, which is the basis for surgery. They are conditionally divided into several groups:
- Interventions to temporarily reduce ICP and neutralize cranio-cerebral imbalance.
- Surgical intervention to remove fluid to other body systems.
- Operations performed to restore fluid circulation.
- Interventions to suppress cerebrospinal fluid production.
- Surgical intervention to activate the sorption of cerebrospinal fluid.
Surgical treatment involves the elimination of the cause that led to the accumulation of fluid in the brain, bypass surgery. It involves the implantation of a silicone tube through which fluid is drained from the skull into another cavity.
Surgical methods
At various periods of development, neurosurgery preferred liquor-guiding surgery. They allow the occlusion of the cerebrospinal fluid paths by shunt with implantation of the valve system. During surgery, a catheter is inserted into the brain through which excess cerebrospinal fluid is removed to other parts of the body. This can be a combination with the abdominal cavity, heart chambers. The doctor inserts one end of the catheter into the ventricle of the brain, then creates a subcutaneous tunnel. Typically, this procedure gives positive results, after it is not necessary to conduct repeated operations. However, complications such as infection or clogging of the shunt may occur.
Patients with dropsy go with a shunt all their lives. If it is installed in childhood, then as it grows, several more operations are performed in which the tube is replaced with a longer one.
In some cases, plastic plumbing of the brain is indicated. It is carried out for patients with occlusion by endoscopic aqueductoplasty.
In patients, the rostral mouth of the brain water supply is covered with a thin and elastic connective tissue, which was formed as a result of hemorrhages and infections. It is simple to eliminate it, but before this procedure, a thorough examination of the patient is carried out using tomography.
For the treatment of newborn children in cases when massive subarachnoid, intraventricular hemorrhages occur, diuretics are combined with unloading punctures of the lumbar, ventricular. When a subshell hematoma appears, a procedure is performed to remove it.
The drainage system allows you to normalize the ICP, as well as monitor the dynamics of the cerebrospinal fluid. This avoids hypertensive cerebrospinal fluid crises, postponing bypass surgery.
With low body weight, children can install an additional subcutaneous reservoir, which allows you to repeatedly pump cerebral fluid, control ICP.
In the treatment of progressive hydrocephalus, a shunt surgery is performed. In 50% of cases, immediately after the installation of the shunt in children, the general condition normalizes, which almost completely eliminates postoperative complications.
After operation
A shunt is used to treat hydrocephalus. Most often, he remains for life. However, periodic medical monitoring of valve pressure may be required. The surgeon watches him.
In addition, after shunting, it is necessary to monitor the state of health, 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, as well as determine whether the shunt can be removed.
Complications of dropsy
Complications of dropsy can be:
- hearing loss, visual impairment up to blindness;
- muscle weakness;
- carbohydrate, fat metabolism is disturbed;
- violations of the respiratory, SS system are observed;
- the regulation of body temperature is disturbed.
If complications occur, the risk of death is great. To avoid complications, it is necessary to begin treatment of the pathology as soon as possible. It allows you to achieve either full recovery, or persistent remission.
With implanted shunts, people live their whole lives. If after several years the dropsy does not return, then the shunt is removed. This outcome is considered a complete recovery.
Complications after surgery
Postoperative complications are observed in about 20% of cases, later - in 15%. Early complications are characterized by inflammatory changes, the formation of fibrinous clots.
Infectious complications sometimes arise in the form of bacterial meningitis, ventriculitis. Glomerulonephritis, endocarditis is less common. With a prolonged course of ventriculitis, periventricular encephalitis occurs.
The causes of complications may be:
- infectious processes in postoperative wounds;
- shunt contamination with the growth of the pathogen on it;
- necrotic complications.
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Disability
In some cases, the child is identified as a disability group with congenital hydrocephalus. It is determined by the degree of loss of health. The status of “disabled child” is granted for 1 or 2 years, or until reaching the age of 18.
Hydrocephalus is included in the list of pathologies in which a child is given a disability until they reach 16 years of age. However, in order to clarify which group will be given, it is necessary to undergo a medical examination, at which doctors will make a conclusion about the state of health, existing disorders, developmental abnormalities.
Timely treatment will allow the child to live, develop normally, without deviations from the norm. Even if a shunt is established for him, then there is a chance that he will live a full life or recover completely.
Modern medicine has learned to help people with a wide variety of diseases, and dropsy is no exception.