Spina bifida - what is it? Malformation of the spine

The developmental defect of the spine, which can be accompanied by the pathology of the development of the spinal cord, is Spina bifida. What is it, in what cases arises, to what violations in the body can result - the reader will find the answers to these questions in our article. However, in order to understand the clinical picture of the disease, it is also necessary to understand the concept of the central nervous system of man, the anatomical structure of the spine and the functions of the spinal cord.

The concept of the central nervous system

The central nervous system is the main part of the human nervous system. It is represented by the spinal cord and the brain and consists of neurons and their processes. The central nervous system is formed by three departments: lower, middle and higher. The lower and middle include the spinal cord, the medulla oblongata, the middle brain, the intermediate brain and the cerebellum. These components regulate the activity of individual organs, establish a connection between them, ensure the integrity of the functioning of the body. The upper section of the central nervous system is represented by the hemispheres of the brain and functionally ensures the interaction of the organism with the environment.

The main function of the central nervous system is the implementation of simple and complex reactions - reflexes. The central nervous system is formed in the first weeks of pregnancy from a special structure - ectoderm. With the wrong division and development of nerve cells, there is a defect in the neural tube, which subsequently leads to the development of pathologies of the spinal cord.

The spinal cord is the organ of the nervous system located in the vertebral canal. The conditional border between the spinal cord and the brain passes at the place of interposition of pyramidal fibers. Both the brain and spinal cord are enveloped in a shell consisting of three layers: solid, spider veins and vascular. From external injuries, the brain is protected by the cranium, and the spinal cord by the spine.

Structure of the spinal column

The spine is the basis of the human skeleton, it consists of vertebrae, between which the intervertebral discs are located - the cartilaginous layers. Functionally, the disks provide mobility of the spine. In total, a person has 33-34 vertebrae, they are conditionally combined into departments: cervical, thoracic, lumbar, sacral, coccygeal.

Each vertebra is formed by the body, arc and processes. The body and the arc make up the vertebral foramen, the appendages connect the vertebrae to each other, and also perform the function of fixing the muscles. In a healthy human body, the orifices of the vertebrae merge into a single vertebral canal, which, in fact, is the location of the spinal cord. But there are also pathological conditions, in which there is a gap in the bone - this phenomenon is called "sprain of the vertebrae".

The spinal cord provides the connection of the brain with the peripheral nervous system. The central canal is the cavity inside the spinal cord. The spinal cord, like the head, has a three-layered shell, between the layers of which there is a spinal fluid. The bones of the vertebrae and the outer shell of the spinal cord form an epidural space between them, filled with fat tissue and a venous network.

Spina bifida: what is it?

Spina bifida is a degeneration of the spinal column (incomplete closure of the spinal canal), which is often aggravated by another pathology - the development of the spinal cord. This defect begins to manifest in the early stages of pregnancy and is a violation in the formation of the neural tube. The neural tube is a structure from which, in later pregnancy, the embryo forms the central nervous system - the spinal cord and the brain. Numerous medical records of patients with Spina bifida give grounds to classify pathology into separate species in accordance with the severity of viral development:

• Conditionally light forms: hidden Spina bifida, meningocele, lipomeningocele;
• Severe forms: myelomeningocele; Myelomeningocele, complicated by hydrocephalus.

Spina bifida is detected during magnetic resonance imaging or neurosonography.

Statistics show that the disease occurs in 1-2 newborns out of 1000. Pathology is genetic, because up to 8% of repeated births ends with a diagnosis of Spina bifida. Doctors say that it often occurs in children born to women in their late teens. However, 95% of newborns diagnosed with a split spine appear in healthy parents who do not suffer from such a disease.

Light forms Spina bifida

Hidden Spina bifida (occulta) - the easiest form of the disease, in which there is underdevelopment, a small gap in the vertebrae. Most often occurs in the lumbar or sacral parts of the spine. This pathology extends only to the bone tissue of the spine and does not affect the spinal structures. The disease is moderately expressed and often does not manifest itself as symptoms that can disturb a person. Many patients with this pathology and do not suspect about their disease. A visually conspicuous sign of the disease in a newborn can be a bundle of hair, usually small; Fat pouch; Deepening or birthmark in the place of pathology.

Only in some cases (approximately in 1 out of 1000 patients with the latent form of Spina bifida) pathology leads to functional disorders in the work of the genitourinary system, the intestine, to the muscular weakness of the lower extremities, to the curvature of the spine. Usually in the patient's medical record the specific number of the vertebra of one of the departments of the spine, in which the pathology arose, is indicated. For example, the record "Spina bifida posterior S1" indicates problems with the first vertebra of the sacral spine.

Meningocele is a kind of splitting of the spine, in which, in addition to bone tissue dystrophy, the protective membranes of the brain can protrude or bulge in the form of a capsule through an existing gap. Often, the spinal cord, as well as nerve processes without pathologies or with small defects. In the overwhelming majority of cases, the capsule is covered with skin, which conditionally protects its contents. However, even this small defect requires surgical treatment to eliminate the cosmetic defect.

Lipomeningocele

Lipomeningocele - a form of Spina bifida, which is considered a pathology of a moderate nature. In medicine, this disorder is classified as a symbiosis of the resulting spinal hernias and lyme - benign tumors. This disorder in the formation of the neural tube occurs during the first 20 days of pregnancy. Lipomeningocele is a defect that can cause the development of neurological pathologies and leads to serious diseases of the genitourinary system, the intestines - the fat tissue exerts pressure on the spinal cord. Taking into account the medical history of patients with lipomeningocele, four groups of pathological manifestations of the condition can be conventionally distinguished:

• Without neurologic, pelvic and orthopedic disorders;
• Moderate paresis of legs, deformation of feet;
• Pronounced impairment of motor function, curvature of the spine, pelvic disturbances;
• Deformity of the extremities, severe pelvic pathology.

Therefore, the only and correct method of treating this pathology is an immediate early surgical intervention. Together with the operation to remove the fat bag can be plastic surgery of the hernia gate.

Myelomeningocele - a severe form of Spina bifida

The most severe form of pathology that occurs in 75% of all vertebral dystrophy cases is myelomeningocele (Cystica spina bifida). What it is? Brain hernia is an element of the spinal cord, which protrudes through the defect of the spine. Sometimes the capsule with the spinal cord is protected by the skin, in other cases the brain tissue, together with the nerve processes, comes out. With this form of pathology, neurological disorders are inevitable. The degree of such disorders will be determined by the severity of the defect of the spinal cord, as well as by the site of the spine in which the disorder has occurred. If the disease affects the final section of the spinal cord, the problems affect the genitourinary system and the intestine, paralysis of the lower extremities occurs.

Often, spinal hernias occur in the lumbosacral spine. However, in medical practice, there are cases when the pathology develops at the level of the thoracic or cervical spine. Unfortunately, the defect of the neural tube is most often accompanied by another violation - hydrocephalus or Chiari anomaly, the cause of which is a violation of the formation of cerebrospinal fluid inside the skull.

Hydrocephalus and Chiari anomaly

Hydrocephalus (edema of the brain) is a pathology resulting from the accumulation of cerebrospinal fluid in the ventricles of the brain. Cerebrospinal fluid - cerebrospinal fluid - is constantly circulating in the ventricular system.

The required volume of cerebrospinal fluid can be formed in two ways:

• To be produced by glandular cells of blood vessels in the ventricles of the brain;
• Formed as a result of sweating blood plasma through the walls of blood vessels.

Likvor circulates from the ventricles to the subarachnoid space of the brain and spinal cord. Intracranial and spinal liquor spaces in a healthy body constitute a single whole. When there is a spinal hernia, communicating hydrocephalus is formed. The accumulation of fluid leads to an increase in intracranial pressure, which leads to a decrease in vision, the appearance of convulsions, strabismus, weakness of the muscles of the upper and lower extremities. Such violations lead to severe neurological disorders, a decrease in intellectual abilities, and death.

The Arnold-Chiari anomaly is a pathology in which the amygdala of the cerebellum descends to the level of the first or second cervical vertebrae, thereby blocking the circulation of cerebrospinal fluid.

Surgical treatment of myelomeningocele

The greatest danger to the life of a newborn with myelomeningocele is the rupture of the hernial sac and infection into its contents. Infectious complications, decompensation of hydrocephalus - the main cause of high mortality of newborns who are diagnosed with Spina bifida. As soon as liquorrhea is detected - the course of the cerebrospinal fluid - an emergency surgical procedure is performed to remove the spinal hernia.

To date, the optimal method of eliminating these pathologies, successfully used throughout the world, is the intrauterine removal of spinal hernias. This type of surgery prevents the development of hydrocephalus and Chiari anomaly in small patients; Allows to avoid serious complications, life-threatening, and also the subsequent shunting operation. The above manipulations give the newborn a chance at a normal level of development and quality of life.

If timely intrauterine surgery is not performed for some reason, resort to a liquor-shunting operation that normalizes the amount of cerebrospinal fluid. Usually such an operation is carried out in two stages. The clinic of the spine is a medical institution with a narrow focus, in which, as a rule, many problems associated with spine pathologies help to solve.

Diagnostics

In modern medicine, Spina bifida is diagnosed in early pregnancy. Therefore, future parents have time to understand the diagnosis and psychological preparation for the birth of a child with features. On the other hand, they also have the time and the opportunity to decide on non-preservation of the fetus.

During pregnancy (at weeks 16-18), future mothers undergo screening tests based on blood tests. The essence of the method is to check the concentration of rudimentary protein, which in normal pregnancy is produced by the fetus in a small amount. If there is a defect in the central nervous system, the concentration of alpha-fetoprotein increases. There are special features in the test. Due to possible errors in deciphering the results of the analysis, you should carefully follow certain rules:

• To establish the most accurate timing of pregnancy;
• If pregnancy is prolific, it must be diagnosed in time.

Another method of diagnosis is prenatal ultrasound, carried out at certain periods of pregnancy, favorable for the detection of pathology. This method allows you to visualize the brain, spinal cord, vertebral column and make a timely diagnosis. However, it must be borne in mind that, with minor defects (hidden Spina bifida), the pathology may not be detected.

Amniocentesis - puncture of amniotic fluid is another method of diagnosis. It is performed by means of a large needle and only strictly under ultrasound control. This method is used in the case when the blood test showed an excess of the concentration of alpha-fetoprotein, and ultrasonic diagnostics of pathologies did not reveal. The liquid, like blood, is examined for the concentration of the embryonic protein, the excess of which indicates the presence of disturbances in the development of the spinal column.

Prevention

Of course, no medical specialist will guarantee that, if all the necessary precautions and the absence of common risk factors are observed, healthy children will have a healthy baby. Unfortunately, sometimes nature disposes differently than a person assumes. But in the power of future mothers and fathers make the maximum in order to reduce all possible risks to a minimum and reduce the likelihood of the birth of a child with the pathology Spina bifida almost to zero. We are talking about preventive measures.

One of the main measures to prevent a newborn from diagnosing "spina bifida" is careful planning of pregnancy. To do this, the potential mother and father before the conception must undergo a medical examination, without fail, go to a consultation with a geneticist. A woman during pregnancy is supposed to lead a healthy lifestyle, be wary of taking anticonvulsants. But the intake of folic acid in the required volume, on the contrary, is shown in this period. In many developed countries, this is the main point of the program recommended by the pregnancy organization for health.

It should be remembered that genetic inheritance is considered to be a risk factor for the onset of pathology; Transferred by a woman during pregnancy disease, for example, rubella; The impact of the environment - radiation, alcohol, drugs.

Main theses

So, answering the question "Spina bifida - what is it?", One can single out the main theses. Spina bifida - a pathology that occurs in utero, during the first weeks of pregnancy. The only way to avoid a baby's illness is to plan pregnancy, fulfill all the necessary prescriptions of doctors, a future mother to lead a healthy lifestyle, make up for the lack of folic acid in the body.

If pathology is still detected, delivery is performed only by caesarean section in order to exclude injury to the spinal cord during the passage of the fetus along the generic pathways.

To diagnose the disease, there is a whole range of methods - a blood test, ultrasound diagnosis, amniotic fluid research. If nevertheless the infringement is revealed and the diagnosis is put, it is necessary to spend surgical intervention.

An innovative method is the intrauterine elimination of pathology. There is also an operative intervention after the birth of the baby - during the first three days of his life. By the way, suspicions of Spina bifida in a newborn can cause: a hernia on the spine, birthmarks or depressions on one of the skin areas, an unusual bundle of hair. Certainly, the definitive diagnosis is established after additional research - CT, MRI.

Depending on the location of the affected area of the spine, as well as associated functional disorders, the disease is classified into lighter and heavier forms.

Light forms of Spina bifida do not usually lead to severe vices, patients with this diagnosis are socially adapted. In the case of severe malignancies - meningomyelocele - disability can not be avoided, even if the operation is successful.

Clinic of the spine is a kind of medical institution specializing in medical services to help patients with CNS disorders. Therefore, the diagnosis of Spina bifida is not a verdict. You can and should live with it.