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Congenital spinal hernia is a rare but severe developmental anomaly. This spinal cord defect appears in only 0,1-0,03% of newborns, and about two-thirds of them remain disabled for life.
Modern means of prenatal diagnosis allow you to find out about the presence of spina bifida even during pregnancy. For this, ultrasound data and additional tests for alpha-fetoprotein are used. The final confirmation occurs during the study of amniotic fluid – amniocentesis.
Causes of spinal hernia
The question of the occurrence of a hernia of the spinal cord is not well understood. Because of this, various medical scientists call such factors that affect the likelihood of its occurrence:
Deficiency of vitamins, and above all, folic acid (vitamin B9). Recognized by most researchers as the main cause of the disease;
A complex of factors that provoke a variety of fetal anomalies: alcohol and other drugs during pregnancy, intoxication of the body, infection of the expectant mother with certain infections and parasites, etc .;
Pregnancy at an early age;
Наследственность.
The neural tube of the unborn child is formed in the first eight weeks of pregnancy. It is at this time that the impact of the above factors can provoke insufficient fusion of the posterior wall of the spinal canal. Because of this, one or more vertebrae are separated in the area of the spinous processes. In the resulting defect in the process of fetal development, hard spinal membranes, cerebrospinal fluid and even nerve roots can come out.
Forms of spinal hernia in newborns
Doctors divide different cases of spinal hernias according to their localization and structural features. The location of the defect plays a big role for the patient and the doctor treating him, since the severity of the symptoms and the complexity of treating the pathology depend on it.
The easiest type of splitting, in which there is no hernia as such, is called hidden (spina bifida occulta (Latin) – hidden spina bifida). In this case, a slight deformity of one of the vertebrae is diagnosed, which often does not bring inconvenience to the patient. In some cases, mild neurological symptoms appear, but without significant health consequences.
A more serious defect in the structure of the spine provokes hernial splitting. At the same time, a protrusion that extends beyond the skin integument is clearly visible. Most often it consists of the meninges and fluid. In the most severe cases, the roots and the spinal cord itself enter the hernia cavity.
A herniated spinal cord can belong to one of three types of localization:
In the cervical region – the rarest variant of a hernia. Affects the upper part of the spinal cord, which innervates the muscles of the neck, face and vocal cords. Accordingly, the coordinating abilities of these and all lower parts of the spine may be impaired, which affects the motor activity of both the upper and lower extremities, as well as the heart and lungs;
In the chest – happens more often than in the cervical, but still much less often than in the lumbar. Disorders of the neck, facial muscles and larynx are excluded, but, in addition to the limbs, the respiratory system and heart, as well as internal organs (stomach, spleen, liver, duodenum) are at risk;
In the lumbosacral region – the most common anomaly affecting the lower limbs, bladder and rectum, sometimes even the kidneys and genitals.
Regardless of where the split is located, the severity of the symptoms determines the degree of protrusion of the structures that make up the spinal cord.
According to it, pathology is classified as one of four forms of spinal hernia:
Meningocele – a mild form of the disease, which is characterized by penetration into the intervertebral defect exclusively of the spinal membrane. The spinal cord itself remains properly formed and capable;
Meningomyelocele – in addition to the shell, there is a protrusion of the substance of the spinal cord. The structure of the neural tube is broken, neurological symptoms appear;
Meningoradiculocele – in the hole formed by the deformed vertebrae, both the sheath and the roots of the spinal nerves extend beyond the body, although the neural tube remains in its place;
Myelocystocele – the most severe form of anomaly, in which the spinal tissues are stretched from the inside by cerebrospinal fluid (a special cerebrospinal fluid necessary to nourish the cells and tissues of the nervous system). As a result, the structure of the spinal cord is almost completely disrupted, which is difficult to restore and any treatment.
In addition to all the forms described above, in very rare cases, the most severe of the complications occurs, caused by a combination of a hernia with a tumor. Usually these are benign lipomas or fibromas fixed on the membranes, roots or internal structures of the spinal cord. The degeneration of the tumor into a malignant formation does not occur, since it is removed along with the protrusion during surgery, or death occurs before this moment.
Symptoms of spinal hernia
The manifestation of symptoms of spina bifida depends on the location, size and composition of the hernia. Of course, the most insignificant external signs and the complete absence of neurological manifestations are characterized by latent splitting. The only symptom of such a pathology is a small notch at the site of the defect.
In terms of neurological symptoms, about a hernia with a protrusion of only one membrane, the same can be said as about hidden splitting. The only difference is that a hernia is a mass formation, not a deepening. In some cases, this place is also marked by redness, shine or bluish coloration of thinned skin, as well as dark hairline.
Neurological symptoms are manifested in the case of meningomyelocele, meningoradiculocele and meningocystocele. They arise due to disturbances in the structure of the spinal cord, which delays, distorts or completely stops conducting nerve signals.
They belong to:
Lack of tactile and (or) pain sensitivity;
Paresis, paralysis and malnutrition of the lower and, in rare cases, upper limbs;
Dysfunction of the pelvic organs, in particular, the muscles of the bladder, anus and genitals.
Violation of the coordination of the work of the heart, lungs, organs of the digestive and endocrine systems is rare when it comes to a hernia in the cervical or thoracic spine.
The above symptoms lead to secondary complications:
Atrophy of paralyzed muscles, lack of their temperature regulation due to loss of skin sensitivity, swelling, trophic ulcers on the skin of immobilized limbs;
Flexion contractures (immobility of the joints, which eventually becomes irreversible);
Bedsores in the buttocks and lower back;
Incontinence of feces and urine.
Diagnosis of spinal hernia in newborns
Accurate diagnosis of a spinal hernia requires a complete physical examination, which begins with an anamnesis. In the case of contacting a doctor after childbirth, in which no pathologies were found, special attention is paid to age indicators during the period of manifestation of the symptoms of the disease (weakness in the limbs, signs of atrophy and difficult muscle activity).
Mandatory are such diagnostic procedures as:
Examination by a neurologist, during which an assessment of motor activity, checking the muscle tone of the limbs, detecting protrusion and fixing relevant medical indicators are carried out;
Transillumination is a special light scanning, thanks to which it is possible to detect and characterize the contents of the hernial protrusion;
Contrast myelography is an intravenous injection of a contrast agent that accumulates in certain parts of the spinal cord. Gives an idea of the structure of damage inflicted to the spinal cord during the formation of a hernia;
The study of layered images of magnetic resonance or computed tomography of the spine, clarifying the data on the violation in the spinal cord and making it possible to predict the further development of the pathology;
Consultation with a neurosurgeon to determine the feasibility of surgical intervention.
Treatment of spinal hernia in newborns
The only recognized treatment for spina bifida with spinal hernia is surgery. Surgical intervention in this case is required as early as possible, best of all – already in the first week of the patient’s life. It is possible to prevent a threat to the life and health of a newborn only with the complete removal of the hernial sac and the elimination of the defect between the deformed vertebrae.
In some cases, with mild forms of the disease (meningocele of small size, which does not cause disturbances in motor activity and sensitivity, as well as while maintaining normal skin), the operation must be postponed. Rushing surgery can result in damage to important spinal structures, causing the same neurological problems that need to be avoided. Any other vertebral hernia requires urgent mandatory surgery.
Postoperative Therapy
To undergo such an operation, any infant will need a long rehabilitation period. A newborn needs careful care, maintaining impeccable hygiene, therapeutic massages, physiotherapy, gymnastics and prevention of constipation. The list of conservative measures also includes the prevention of bedsores, training of the sphincters of the bladder and anus, the treatment of spinal deformities and correction with support corsets.
Usually recovery from a spinal hernia occurs in one of two ways:
After the removal of the meningocele and all the required rehabilitation procedures, the development of the child continues normally. In most cases, these children grow up capable of leading an active lifestyle.
Repair of meningomyelocele, meningoradiculocele and meningocystocele hernia can cause certain difficulties, both during the operation and after it. Proper observance of the requirements of the rehabilitation period helps to avoid violations of the motor activity of the limbs, but the same cannot be expected from the functions of the pelvic organs. Urinary and fecal incontinence can persist much longer than in normal children. In adult life, even with the preservation of external signs of health, infertility is found in women, and erectile dysfunction in men.
Prevention of spina bifida
Since spinal hernia is a congenital anomaly of development, the prevention of this disease is the elimination of its causes even before conception. The primary measure is to provide the unborn child with all the necessary microelements and vitamins. Even if the pregnancy is unplanned, the use of drugs and appropriate foods can be started after conception – the sooner the better. In the specific case of preventing spinal cord herniation, the emphasis is on vitamin B9 (folic acid). Interestingly, this substance can enter the body of the fetus not only from the mother, but also from the future father, since it is transmitted through the seminal fluid, while having a significant effect.
Any future mother should consult a gynecologist and consult about diseases that develop at the stage of fetal formation. The doctor should talk about ways to ensure a favorable bearing of the child. To begin with, most pharmaceuticals are stopped for at least the first eight weeks, while the neural tube forms in the embryo. Also, do not get carried away with cosmetics, the same applies to any household chemicals.