Spina bifida - what is it? Malformation of the spine

The developmental defect of the spine, which canaccompanied by the pathology of the development of the spinal cord, - 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 partnervous system of man. 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 organism. 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 central function of the central nervous system is the implementation of simple andcomplex 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 boundary 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, arachnoid and vascular. From external injuries the brain is protected by the skull, the spinal cord - the spine.

Structure of the spinal column

The spine is the backbone of the human skeleton, consisting ofvertebrae, between which are located intervertebral discs - cartilaginous interlayers. 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 andprocesses. The body and the arc make up the vertebral foramen, the processes 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 "nezaraschenie bow of the vertebrae."

The spinal cord provides the connection of the brain withperipheral nervous system. The central canal is the cavity inside the spinal cord. The spinal cord, like the head one, 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 developmental dystrophyspine (incomplete closure of the spinal canal), which is often aggravated by another pathology - the development of the spinal cord. This vice 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, at later gestation, the embryo forms the central nervous system - the spinal cord and the brain. Numerous medical histories of patients with Spina bifida give the basis to classify pathology into separate species in accordance with the severity of viral development:

• conditionally mild 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 thatthere is a disease in 1-2 newborns out of 1000. Pathology is of a genetic nature, because up to 8% of repeated childbirth ends with a diagnosis of Spina bifida. Physicians argue that it often occurs in children born to women in their 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 easiestform 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 applies only tobone 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; fatty sac; a deepening or a birthmark in the place of pathology.

Only in some cases (approximately 1 in1000 patients with the latent form 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 - a kind of cleavagespine, in which, in addition to bone tissue dystrophy, the protective shells 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 sprouts 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 - the form of Spina bifida, whichis 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 fatty 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 distinguished:

• without neurologic, pelvic or orthopedic disorders;
• moderate paresis of the legs, deformation of the feet;
• pronounced impairment of motor function, curvature of the spine, pelvic disorders;
• deformity of the extremities, severe pelvic pathology.

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

Myelomeningocele - severe form of Spina bifida

The most severe form of pathology that occursin 75% of all vertebral dystrophy cases - myelomeningocele (Cystica spina bifida). What it is? Brain hernia is an element of the spinal cord that 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 inlumbosacral spine. However, in medical practice, there are cases when the pathology develops at the level of the thoracic or cervical spine. Unfortunately, the neural tube defect often accompanied by another violation - hydrocephalus or Chiari anomaly caused by a breach of the formation of cavities within the skull liquor.

Hydrocephalus and Chiari anomaly

Hydrocephalus (edema of the brain) -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 tosubarachnoid space of the brain and spinal cord. Intracranial and spinal liquor spaces in a healthy body form 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 newbornMyelomeningocele represents a 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 cerebrospinal fluid - an emergency surgical procedure is performed to remove the spinal hernia.

To date, the best methodelimination of 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 threatening life, 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 forfor whatever reason is not performed, 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 diagnosedin the early stages of 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), futureMoms undergo screening tests based on a blood test. The essence of the method is to check the concentration of rudimentary protein, which during 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 isprenatal 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 should be borne in mind that with minor defects (latent Spina bifida) pathology may not be detected.

Amniocentesis - puncture of amniotic fluid - anothermethod 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 a 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 giveguarantees that, with all the necessary precautions and the absence of common risk factors in healthy parents, a healthy baby will be born. Unfortunately, sometimes nature disposes differently than a person assumes. But in the power of future mothers and fathers to make the most to minimize all possible risks and reduce the likelihood of a child with Spina bifida pathology almost to zero. We are talking about preventive measures.

One of the main preventive measuresthe occurrence of a newborn diagnosis of "spina bifida" is a careful planning of pregnancy. To do this, a potential mother and father before conception need to 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 the risk factorsthe emergence of pathology is genetic inheritance; transferred by a woman during pregnancy, for example, rubella; the impact of the environment - radiation, alcohol, drugs.

Main theses

So, answering the question "Spina bifida - what is itis 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 when the fetus passes through the birth canal.

For the diagnosis of the disease there is a wholea spectrum of methods - the analysis of a blood, US-diagnostics, research of amniotic waters. If nevertheless the infringement is revealed and the diagnosis is put, it is necessary to spend surgical intervention.

The innovative method is intrauterineelimination 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 hollows on one of the skin areas, an unusual bundle of hair. Of course, the final diagnosis is established after an additional study - 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 usually do not lead tosevere sickness, 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 - a kind of medicalan institution specializing in medical services to help patients with CNS disorders. Therefore, the diagnosis of Spina bifida is not a verdict. With him you can and should live.

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