The norm of the location of the cone of the spinal cord. Spinal cord. Brain stem: medulla oblongata. Anatomical and physiological features of the spinal cord

Congenital cysts of the spinal canal

Congenital cysts of the spinal canal belong to the category of rare dysembryogenic disorders and occur in most cases in male patients. Spinal canal cysts in children can appear from an early age up to 20-30 years and older. Cysts are often localized in the thoracic spine and spread over several vertebrae.

Spinal cysts are very rare. In the domestic literature, there are 7 observations of an extramedullary cyst of the spinal canal (A. Tishen, 1968; L.V. Paramonov, E.I. Kogan, 1976; Melnikova V.P. et al., 1991).

The reason for the formation of cysts is considered to be a mechanical factor caused by the fact that in the places where the roots of the spinal cord exit, between the dura mater and the root, there is a space into which the arachnoid membrane is wedged and a cyst gradually forms. In the spinal canal there are places of least resistance, allowing, under certain situations, to form extradural cysts, and their increase can be explained by the valvular mechanism, and belongs to the congenital ones.

On the other hand, the formation of these cysts can be attributed to dysembryogenic phenomena. In some patients, a cyst of the spinal canal is combined with spina bifida occulta and diastematomyelia. With a sufficiently thorough examination of the walls of these cysts using an operating microscope, no messages with the subdural space are detected.

Multi-chamber cysts are described that do not communicate with each other and with the subarachnoid space.

Spinal canal cysts were clinically manifested by symptoms of slow compression of the spinal cord, its roots, and with the development of lower paraparesis, dysfunction of the pelvic organs. The diagnosis was based on data from MPT, CT, myelography, thermography. Only the operation reveals the true nature of the disease.

Characteristic of these cysts is an increase in symptoms with a change in body position, in particular, when taking a vertical position, while with tumors of the spinal cord, pain often increases in the supine position and decreases in the sitting position.

The goal of surgical treatment is to empty and excise the cyst. With a sharply thinned dura mater at the level of the removed cyst, it is enhanced by sewing in a fragment of the fascia (autograft). During surgery, the cyst is completely or partially excised.

Early surgical treatment gives very good results, and operations against the background of severe neurological symptoms do not achieve good results. Perineural cysts are more often located in the lumbar region and are manifested by radicular pain syndromes. Their diagnosis is difficult, it is established on the basis of CT, MRI, myelography and thermography.

congenital dermal sinus

Congenital dermal sinus is a developmental defect in the form of a canal lined with epithelium that extends from the skin into the spinal canal and connects its contents to the surface of the body.

Embryologically, this anomaly is formed between the third and fifth weeks of intrauterine life. Incomplete separation between epithelial ectoderm and neuroectoderm is thought to be a possible mechanism for the origin of the dermal sinus canal. In areas of late closure of the primary neural tube, there is more frequent detection of the dermal sinus.

If the skin part of the dermal sinus is found in the lumbosacral region, then it penetrates into the bone canal and, passing through several bone segments, reaches the cone of the spinal cord.

In the cervical and upper thoracic regions of the spinal canal, the sinus usually extends obliquely downward (distally) under the skin and penetrates into the bone canal one, two or more vertebrae below its skin opening. The cranial dermal sinus canal runs inside the spinal canal in a caudal direction. In all cases, the sinus enters the spinal canal through a defect in the neural arch or through a defect in the intervertebral ligament.

Depending on sex, the distribution of congenital dermal sinuses is equal or with a slight predominance of boys. This disease can run in families.

Clinically, congenital dermal sinuses present in a variety of ways. When examining the posterior surface of the patient's back, a funnel-shaped depression or a gaping skin opening of the sinus can be detected on the skin. At the same time, due to the insignificance of the cutaneous part of the sinus, it may not be detected. The skin opening of the dermal sinus can be combined with a zone of skin pigmentation, capillary hemangioma, a tuft of coarse hair. With inflammation of the dermal passage, the inflammatory product is separated through the skin opening, which often serves as a reason for contacting a medical institution.

Sometimes there may be accompanying symptoms. It includes infection of nerve structures secondary to the spread of microorganisms from the skin through the sinus and symptoms of concomitant formation, since in 50% of cases the dermal sinus is combined with congenital ectopic tumors or cysts. Depending on the involvement of various CNS structures, there may be various infectious processes, including ascess, meningitis, intramedullary and epidural abscess. When the inner surface of the bone canal is affected, symptoms of a spinal cord tumor are noted. Congenital tumors accompanying the dermal sinuses are epidermoids, dermoids, and teratoid tumors. As a rule, the inner end of the sinus is dilated and forms a dermoid or epidermoid cyst.

The malformation usually manifests itself in childhood. Quite often, the congenital dermal sinus is localized in the lumbosacral region. If the dermal passage ends blindly in the skin, then they speak of a pseudosinus. The pseudosinus rarely contains squamous elements and hair. However, when the pseudosinus becomes inflamed, a skin abscess occurs, and further spread of the infection can lead to an epidural abscess.

Among the local, accompanying anomalies, the most common is spina bifida occulta. The canal of the dermal sinus can pass both through the unclosed vertebral arch and through the interspinous ligaments. Hypoplastic or bifurcated spinous processes may be noted. Sometimes congenital dermal sinuses are accompanied by diastematomyelia.

Visualization of the malformation is possible by radiation methods and MRI. Spondylography can detect a vertebral arch cleft, expansion of the space between the vertebrae, thinning of one or more pairs of pedicles, or any other congenital pathology of the vertebrae. Myelography and postmyelographic CT indicate an intravertebral mass. The introduction of a contrast agent into the sinus is impractical, because of the possibility of infection.

On MRI, congenital dermal sinuses appear as low-intensity cords separating high-intensity subcutaneous fat. In some cases, it is possible to determine the place of its transition into the epidermis. With a small size of the congenital dermal sinus, it is difficult to detect it on MRI. And if the sine is located between two slices in the interval, then it may not be visible.

Congenital dermal sinuses in the thoracic or cervical spine are particularly difficult to identify due to the lack of subcutaneous fat. It is important to pay attention to the structures of the spinal canal and paravertebral areas where a tumor or abscess may be located, which are clearly visualized on MRI.

Treatment of this congenital malformation is surgical. It involves excision of the canal of the congenital dermal sinus. The operation is performed immediately after its discovery. Preventive surgery helps to prevent a future complication of the disease - suppuration. The entire canal, including its epi-, subdural parts, is subject to removal, reaching its final location along its course. If a dermoid or epidermoid cyst is found, the latter is excised, with preliminary evacuation of its contents. The operation is carried out using magnifying optics and microsurgical instruments.

Caudal Regression Syndrome

Caudal regression syndrome or caudal dysplasia, characterized by a spectrum of anomalies in the lumbar and sacral, coccygeal vertebrae and the corresponding segments of the spinal cord, and various combinations of anomalies occur at different stages of their development.

Vertebral defects range from asymptomatic absence of the coccyx to aplasia of the sacrum and coccyx, agenesis of the sacral, lumbar, and sometimes thoracic vertebrae and are almost always accompanied by severe, persistent neurological symptoms and underdevelopment of the muscular system, visceral anomalies at the level of the pathological process.

The etiology of caudal agenesis is thought to be multifactorial and depends on the neural tube defect.

It has been established that in some cases the syndrome of caudal regression is accompanied by diabetes mellitus in the mother. The birth of children with this defect is observed with a frequency of 1 per 100 mothers with diabetes mellitus. Among children with caudal regression, 14-19% are born to mothers with diabetes.

Caudal regression syndrome occurs in 1 case per 7500 births (of all forms of malformations). However, the most severe forms of this syndrome (total agenesis of the entire sacrum) are quite rare and account for 1 case in 20,000 - 100,000 births.

In a child with caudal regression syndrome, a pathoanatomical study shows high hypoplasia and dysplasia of the spinal cord in the region of the lumbar enlargement. The spinal cord abruptly breaks off at the level of L2 without signs of narrowing and transition into a cone, and atrophy of the corresponding spinal nerves is noted.

The clinic of the disease depends on the level of involvement of the spine and the combination with neurological disorders. Patients with no coccyx or distal sacrum may not suspect that they have a malformation. With partial agenesis of the sacrum, the ilium is connected to the sacrum without signs of anomaly, and gait in such patients is not disturbed. In total sacral agenesis, the ilium often forms a bony pelvic ring by joining together. These patients are often unable to stand or walk. If the iliac bones take a more vertical than normal position, then this leads to a dislocation of the hip joints. In an even more severe form of the malformation, the hip joints are flexed, abducted, rotated outward, and fixed, bringing the patient into the Buddha or Frog position.

With caudal regression syndrome, along with malformations of the pelvic bones and lower extremities, there is atrophy of the muscles innervated by the caudal spinal cord (segments L5 - S1 and S2 - S5): muscles of the gluteal region, legs and perineum.

The characteristic conical shape of the lower extremities is noted, as the muscles of the thighs often remain intact. Atrophy of the muscles of the buttocks leads to a characteristic flattening of the legs and shortening of the intergluteal gap. In more severe forms of the defect, a pronounced arching of the popliteal fossa is observed. In especially severe forms of the caudal regression syndrome, a "fusion" of the lower extremities is noted, described as "sirenomelia" (from the Greek: "siren" - a sea woman-demigoddess) or "mermaid". In this case, the limbs are rotated and soldered along their outer surface. In this case, one of the limbs does not bend, but unbends.

Neurological symptoms in caudal regression syndrome are most often represented by motor disorders, less often in combination with sensory ones. It is always accompanied by urinary incontinence and rarely feces.

The caudal regression syndrome, in the vast majority of cases, is combined with such malformations of the spinal cord and spine as: spina bifida occulta, hemivertebrae, myelomeningocele, fixed spinal cord syndrome, diastematomyelia.

On spondylograms, the level of the end of the spinal column is quite clearly revealed. Spondylograms and CT can reveal concomitant bone defects (non-fusion of the vertebral arch, stenosis of the spinal canal, etc.).

The level of termination of the spinal column and spinal cord is best detected on MRI. The spinal cord usually ends one vertebra above the level of the malformed vertebra. In patients with caudal regression syndrome, the terminal part of the spinal cord is often bulbous or curved. Stenosis of the central canal of the spinal cord, shortening and thickening of the terminal filament, and changes in the subarachnoid space are often observed with this malformation. Well identified on MRI.

Surgical treatment of stenosis of the dural sac, fixed spinal cord, extra- and intradural space-occupying formations, diastematomyelia and other malformations of the spinal cord and spine can significantly reduce the neurological deficit and improve the impaired functions of the pelvic organs in a patient with caudal regression syndrome and thereby greatly facilitate social rehabilitation patient.

Spinal arteriovenous malformations

Spinal arteriovenous malformations (AVMs) are malformations of the vascular system of the spinal cord and spine. In the embryonic development of the vascular system, direct fistulas are formed between arteries and veins, which are present in human life.

Spinal vascular malformations are classified according to many criteria, including location, histology, angiographic variants, and etiology.

The difficulty in classifying spinal vascular malformations lies in their extreme rarity, which among spinal volumetric formations occurs only from 3% to 11%. Vascular malformations tend to be more frequently located on the dorsal surface of the spinal cord and in its more caudal part, due to the embryonic development of arterial anastomoses on the dorsal surface of the spinal cord during the 3rd and 4th weeks of embryogenesis.

The most common spinal location of vascular malformations is their subpial localization. Many of these malformations may be limited to the surface of the spinal cord, but increased pressure in dilated draining veins may present with dilated intramedullary veins. Arterial and venous aneurysms are common and may be the cause of subarachnoid hemorrhage. The juvenile form of vascular malformations is characterized by a vascular nucleus lying in the spinal cord and the substance of the latter is found inside the interstitium of the vascular formation.

ABMs can be the cause of many complications. Subarachnoid hemorrhage occurs in 30% of cases. The highest frequency of subarachnoid hemorrhages occurs in AVMs with a large arteriovenous shunt compared to malformations with slow shedding.

Extradural and subdural hemorrhages in vascular malformations are rare. Spinal cord hemorrhage is a fairly common complication of intramedullary vascular malformations. There may be compression of the spinal cord and nerve roots due to the mass effect of the dilated veins of the vascular malformation. As a result of the phenomenon of stealing, ischemia and infarction of the spinal cord can develop. This leads to the development of chronic progressive radiculomyelopathy (known as the Foyks-Alad syndrome).

Currently, the main methods for diagnosing AVMs of the spinal cord are MRI and selective angiography.

Differentiation of morphological and histological types of vascular malformations using MRI is not possible; only the localization of the malformation and its effect on the spinal cord can be assessed. The location of the vascular malformation provides some information about the degree of shunting. Different layers can be affected: vertebral, extradural, intradural, subpial, arachnoid and intramedullary. AVMs can occur in isolation or involve different layers, including even the skin. In 20% of cases, at the same level as vertebral vascular malformations, cutaneous vascular malformations are observed.

Vertebral angiomas affecting the vertebral bodies are most often found in the middle and thoracic spine. These malformations may extend into the extradural space. Extradural malformations are relatively common, accounting for 15-20% of all vascular pathology of the spine.

MRI features of extradural and vertebral malformations usually refer to dilated extradural veins. A small core of vascular malformation is usually not detected. Dilated veins are present as a signal similar to that from extradural adipose tissue. An increase in the volume of the spinal cord at the level of the vascular malformation, usually at the level of the cone, seems to be due to venous congestion.

The myelographic picture in AVM of the spinal cord is characterized by the presence of tortuous, worm-shaped defects in the filling of the sub-arachnoid space on direct and lateral myelograms. However, these defects in the filling of the subarachnoid space in AVMs may be similar to the myelography pattern in arachnoiditis.

The most important in the diagnosis and treatment of AVMs is selective angiography (SCA), which largely influences the decision on the choice of method for treating the disease.

Angiographic signs of AVMs of the spinal cord are highly variable. Afferent arteries can be multiple and single, depart from one or two sides. Receiving blood flow from one or more arteries, a group of vessels passes into the venous system of one or more vessels, thus forming malformations of various sizes, from simple fistulas to large common aneurysms. The blood flow in the AVM is accelerated due to the absence of the capillary phase of blood circulation in them.

Among all CNS malformations, the treatment of spinal AVMs is the least developed. Currently, open microsurgical interventions and transvasal embolizing operations are used. Often, specialists in the treatment of spinal AVMs use only the method that they know. In recent years, both open microsurgical methods for the treatment of spinal AVMs and methods of transvasal interventions have been significantly improved.

The introduction of microsurgical techniques, preoperative diagnostics and intraoperative monitoring significantly increases the chance of successful surgical treatment of this pathology. Surgical tactics in spinal AVMs depends primarily on the features of their structure, relative position, and connection with the vessels of the spinal cord.

Primary spinal teratomas

Primary teratomas of the spinal canal, in contrast to sacrococcygeal teratomas, are extremely rare volumetric formations.

The discussion of spinal teratomas is complicated by the variety of terms under which they have been reported and the confusion between the diagnosis of this pathology and other nosologies such as enterogenic cysts. Teratoma is characterized by a degree of progressive growth, in contrast to enterogenic cysts, dermoids, and epidermoids, which contain heterotopic tissues but do not have neoplastic growth. The usual components of teratoma are: elements of the skin, teeth, nervous tissue, respiratory and gastrointestinal mucosa, glandular tissue.

Structural differences distinguish dermoids, epidermoids, and enterogenic cysts from teratomas. Teratomas contain tissue components derived from all three embryonic layers and differ in this from epidermoid and dermoid tissues, which include only tissues of ectodermal and mesenchymal origin. In addition, it should be noted that teratomas usually differ from enterogenic cysts in that the latter arise exclusively from the anterior fetal gut or endoderm.

If epidermoids, dermoids and enterogenous cysts are simple volumetric formations that arise secondarily due to a violation of the location of normally developed somatic cells, then teratomas, on the contrary, can be attributed to true neoplasms due to abnormal development resulting from a violation of the location of several multipotent terminal cells in the early embryonic development. Teratomas contain elements that have no embryological connection with the nervous system. To date, their origin remains unclear, although there are a number of theories in this regard.

Clinical symptoms of teratoma can occur at any age. More often they appear in childhood, although they can occur throughout life.

Most often, teratomas are localized at the level of the lower thoracic and lumbar spine. In the spinal canal, they are located mainly on the dorsal surface of the spinal cord. Teratoma can be combined with such malformations of the spinal cord and spine as: spinal hernia, block-like fusion of the vertebrae, lipomyelomeningocele, non-fusion of the vertebral arches, scoliosis, diastematomyelia.

In relation to the spinal cord and its membranes, in most cases, the teratoma is intradural extramedullary. In 50% of cases, this volumetric formation is connected through a pedicle to the spinal cord or attached to the spinal cord. Macroscopic examination of the tumor tissue revealed cystic areas of white, yellow or chocolate color.

Clinically, the disease is manifested by local pain in the area of ​​the teratoma, followed by the addition of conduction disorders caused by compression of the structures of the spinal cord by the tumor. The course of the disease acquires, as a rule, a relapsing character, but it can also be progressive. The resulting exacerbation of the pathological process is often due to an increase in the secretory activity of the glandular elements of the tumor.

Identification of teratoma is possible with the help of radiation diagnostic methods. Spondylograms show thinning of the plates and pedicles of the vertebrae. In addition, spondylograms revealed concomitant bone anomalies of the vertebrae (non-closure of the vertebral arch, fusion of vertebral elements, etc.), CSF tests show a picture of a complete block of the subarachnoid space of the spinal cord. Myelography usually reveals compression of the spinal cord by a teratoma.

Surgical treatment of teratomas. This formation is sometimes very tightly attached to the spinal cord and surrounding structures. Sometimes it can be easily removed, even with intramedullary localization. In some cases, total removal is possible. Teratoma is usually a benign tumor, and surgical treatment leads to a positive result. The situation is much more complicated when this formation turns out to be malignant.

epidermoids and dermoids

Epidermoids and dermoids are heterotopic formations from skin elements. They are not true neoplasms. Epidermoid and dermoid cysts in 20% of cases are combined with the dermal sinus, usually isolated and localized in the posterior hemisphere of the dural space of the spinal cord. Approximately in 20-40% of cases they are introduced into the spinal cord and are intramedullary, about 67% - intradural, extramedullary.

Dermoids and epidermoids may arise from congenital remnants, local extension of the dermal sinus, or implantation of viable epidermal elements at the time of puncture or surgery. Inattentive incorporation of epidermal tissue during closure of a myelomeningocele or other dysraphic conditions can lead to epidermoid formation.

Epidermoids are cystic formations lined with a membrane from the superficial (epidermal) layers of the skin. Viable cells form a thin capsule containing dead, keratinized epithelial cells.

Dermoids are uni- or multilocunar cystic formations. They are lined with epithelium, which contains all layers of the skin: epithelial and mesenchymal components, as well as additional skin organs: sweat glands, hair follicles. As with epidermoid cells, viable cells form a thin capsule. The dermoids, however, contain not only the lowered epithelium, but also the sebaceous substance, hair. Pathological differentiation of epidermoid and dermoid can be difficult, as only small areas of the cystic wall may have fine structures, such as hair follicles, that characterize the mass as a dermoid. Microscopically, the dermoid is smooth, white, and less bright and shiny than the epidermoid. Both types of cysts present with similar symptoms. In both cases, the symptoms are due to the presence of a volumetric formation in the dural space of the spinal cord. The level of motor and sensory disturbances may be minimal compared to the size of the anatomical lesions. Epidermoids are congenital and may present in young children, but they often manifest in adult patients. They grow extremely slowly. The average age of clinical manifestation is 10 years or more, with a slight predominance in males. Clinically, dermoids usually appear before the age of 20. In those cases when the epidermoid or dermoid is localized in the region of the cauda equina, it causes irritation of the nerve roots of the spinal cord and its membrane. As a result of irritation of the roots of the cauda equina and the dura mater, a pronounced pain syndrome occurs in combination with a spasm of the muscles of the lumbar region. This is due to the resulting arachnoiditis due to exposure to the outflowing contents from the dermal or epidermal cyst. In a number of patients, the clinical course of the disease acquires a relapsing character, and then it is rather difficult to establish the correct diagnosis. Verification of the disease can occur by chance during a lumbar puncture.

Epidermoid and dermoid cysts can be combined with other malformations of the spinal cord and spine, most often with spina bifida occulta, skin stigmas. Epidermoids are more often localized at the level of the thoracic spinal cord intramedullary. The epidermoid, as a volumetric formation located in the spinal canal, can cause erosion of the legs and scalloped vertebral bodies.

Dermoids clinically manifest as volumetric formations. However, unlike the epidermoid, the dermoid has a denser consistency, which leads to more frequent erosion of the pedicles and scalloped vertebrae. In both types of pathology, liquorodynamic tests in 80-90% of cases reveal a complete block of the subarachnoid space of the spinal cord. On myelograms, an epidermoid or dermoid cyst looks like a smooth ovoid or leaf-shaped formation.

On MRI, the epidermoids are visualized with great difficulty, since they have a signal intensity equal to or slightly higher than that of the cerebrospinal fluid. An increase in the signal may be due to the presence of protein components in the epidermoid cyst, or due to the absence of CSF pulsation in it. Due to the lack of informativeness of MRI, in the detection of an epidermoid cyst, it is necessary to use myelography, as so far the most reliable method for diagnosing this disease.

Dermoids on MRI are characterized by the presence of a hyperintense signal. The intensity of the signal depends on the relative proportions of epithelial inclusions and the sebaceous component. In some patients, the dermoid is characterized by a signal intensity approaching the subcutaneous adipose tissue. In these patients, a differential diagnosis with lipoma should be made. In cases of calcium deposition in the cyst, a hyperintense signal occurs, which to a certain extent facilitates the diagnosis of the pathological process. Epidermoids and dermoids are benign, encapsulated formations. Their total removal leads to a cure for the patient. When removing these formations, care should be taken to ensure that the contents of the cyst do not enter the surgical wound during the operation, as this can cause aseptic arachnoiditis of the spinal cord. In some patients, the removal of a pathological formation does not lead to a significant improvement, but protects the patient from further progression of neurological symptoms.

Latent spinal dysraphism (SSD) is a diverse and heterogeneous group of malformations of the spine and spinal cord; includes such nosological forms as fixed spinal cord syndrome, spinal lipomas, dorsal dermal sinus, spinal cord splitting, etc. They are united only by the fact that they do not have a violation of the integrity of the skin over the defect of the vertebrae.

The true frequency of SJS is unknown., since its manifestations are not as obvious as with spinal hernias. The first symptoms are often delayed, progress slowly, and an MRI of the spinal cord is necessary to establish an accurate diagnosis.

The symptomatology of SJS is variable and, as a rule, is not associated with a specific form of damage. All manifestations of SJS can be divided into:
skin
bone
orthopedic
neurological
dysfunction of the pelvic organs

Skin stigmas are most often located in the midline in the lumbosacral region. in newborns, they occur in 3% of cases, and in patients with SJS - in 70%.

Skin manifestations include (often a combination of several stigmas is diagnosed):
hypertrichosis
capillary hemangioma
dermal sinus
retraction of the skin
subcutaneous lipoma
tail appendage (pseudo-tail), etc.

Bone manifestations of SJS are diverse:
anomalies of the vertebral arches - defects, splitting. misdevelopment
vertebral body anomalies
vertebral disc anomalies
expansion of the spinal canal
malformations of various parts of the spine
posture disorders - scoliosis, kyphosis, lordosis
anomalies in the development of the sacrum - agenesis, dysgenesis, deviation
bone spike along the midline of the vertebra, etc.

Orthopedic disorders include:
leg deformities
asymmetry of the legs and feet
asymmetry of the buttocks
change in gait

Pelvic disorders:
neurogenic bladder
urinary tract infections
urinary incontinence

Neurological symptoms depend on age; most frequent:
lower monoparesis and paraparesis
decreased tendon reflexes
gait disturbance
decreased sensation in the legs and, as a result, the appearance of painless wounds of the lower extremities
pain in the legs and lower back
spasm, etc.

Diagnostics SJS primarily depends on the alertness of pediatricians to this pathology. Thus, the presence of skin stigmas in the lumbosacral region in a child, the appearance and progression of neurological symptoms, orthopedic and neurological disorders should be the reason for an MRI study in order to search for hidden spinal dysraphism.

The symptomatology of SJS is usually associated with the syndrome of a fixed spinal cord and progresses gradually as the child grows.

Fixed spinal cord (FSM)

FSM is characterized by an abnormally low (below the level of L1 - L2) location of the cone of the spinal cord.

The term is often used in Russian-language literature tethering syndrome, which is derived from the English tethered cord syndrome. Less often you can find the name "stretched spinal cord syndrome" (SPCS), which is more correct in essence, since in young children the spinal cord is already fixed, but not yet stretched.

SFCM may be an independent disease and result from an abnormally thickened filament terminal or be a manifestation of other SJS conditions.

With SFSM, there is a gradual violation of blood circulation in the lower parts of the spinal cord as a result of its tension. Being pathologically fixed, the spinal cord cannot follow the growing spine.

SFSM underlies the symptoms of most latent malformations of the spinal cord. This explains the polymorphism and non-specificity of the clinical manifestations of SJS.

Frequency of symptoms in SFMS, (%)
gait disorders and lower paraparesis - 93
muscle atrophy, shortening of the lower limbs, deformity of the knee joints - 63
sensory disturbance - 70
pelvic disorders - 40
pelvic disorders as the only symptom - 4
pain in the lower back, legs, feet - 37
kyphosis and scoliosis - 29

Treatment patients with SFSM only surgical. The purpose of the operation is to free the spinal cord. So with a thickened terminal thread, it is identified and crossed. In other cases, the technique of the operation depends on the form of latent spinal dysraphism.

Split spinal cord

The malformation is characterized the presence inside the spinal canal of a bone, cartilage or fibrous septum or strand, which completely or partially separates the spinal cord lengthwise into two halves (hemichord).

There is some confusion in terminology. Previously, diastematomyelia and diplomamyelia were distinguished. In the first case, the splitting of the spinal cord was incomplete and the hemichords had only one pair of ventral and dorsal roots, and in the second case, two pairs each (i.e., complete doubling of the spinal cord and roots).

According to the Pang classification currently accepted, two types of malformation are distinguished:
type I malformation- there are two hemichords separated by an osteocartilaginous bridge, each of which is located in its own dural sac
type II malformation- both halves of the spinal cord are enclosed in one dural sac and separated by a fibrous septum

Symptoms in this malformation are determined by a fixed spinal cord, which is pathologically held by a bone, cartilaginous or soft tissue spike, cord or bridge. Skin stigmas (most often hypertrichosis) are detected in 90% of patients.

The split spinal cord is clearly visible on MRI, but the level of the septum is best determined using CT, which visualizes bone structures.

Treatment surgical. The purpose of the operation is to remove the bridge between the two hemichords and thereby free the spinal cord.

Improvement of neurological functions or their stabilization occurs in 90% of operated patients.

Spinal lipomas

Spinal lipomas (SLs) are the most common form of occult spinal dysraphism.

They fall into three main groups:
intradural lipomas
conus lipomas of the spinal cord
filament terminal lipomas

Intradural lipomas are extremely rare. They are located, as a rule, on the dorsal surface of the spinal cord and soldered to it. Their main localization is the thoracic and cervical spinal cord. Their localization in the lumbosacral region can cause a fixed spinal cord syndrome (SFCS).

Lipomas of the conus of the spinal cord- the most frequent and clinically significant group. Lipomas are located under the skin in the lumbosacral region, penetrate intradurally through a defect in the thoracolumbar fascia, clefts of the vertebral arches and dura mater and fuse with the cone of the spinal cord, fixing it at the lower lumbar level.

There are such forms of lipomas of the cone of the spinal cord as:
lipomyelocele - the spinal cord lies inside the canal, but ends in the thickness of the subcutaneous lipoma
lipomyelomeningocele- subcutaneous meningomyelocele in combination with lipoma
lipomyelocystocele- in rare cases, the terminal parts of the spinal cord are everted outward by cystic enlargement of the central canal

More than 90% of patients have skin stigmas of SJS - subcutaneous lipomas, angiomas, local pilosis, dermal sinus, fossa or pseudotail. Sometimes they are multiple. Frequent neurological symptoms - loss of sensitivity in the sacral dermatomes. In 30% of patients, pelvic disorders, various deformities of the lower extremities, scoliosis in 10% are noted. Symptoms slowly progress.

Filum terminal lipomas located intradurally, infiltrate the terminal thread and lead to pathological fixation of the spinal cord. Symptoms are usually absent.

In terms of examination, there should be consultations with a neurosurgeon, neurologist, urologist and orthopedist.

Treatment surgical. The main goal is to free the spinal cord to prevent the progression of symptoms, as well as to achieve a good cosmetic effect, since the subcutaneous lipoma tends to increase with age. The operation is indicated after the child reaches the age of 2 months. after surgery, the symptoms regressed in 19%, stabilized in 755. Deterioration - in 6%.

Dorsal dermal sinus

Dorsal dermal sinus it is a fistulous tract lined with epithelium, resulting from a defect in the closure of the neural tube, which can reach the subarachnoid space of the spinal cord. Most often located in the lumbosacral spine. A characteristic feature of the dermal sinus is the presence of a small skin opening around which there is redness or hypertrichosis in this area. Having a direct connection with the skin, the dermal sinuses serve as a conductor of infection and a source of development of meningitis, epidural and subdural abscesses. The manifestation of the dermal sinus, combined with a heterotopic tumor, is observed already in the first years of life with pronounced symptoms of recurrent meningitis, pus discharge from the fistula, and neurological disorders.

The main diagnostic method is an MRI of the lumbosacral spine. The introduction of a dye or contrast agent into the duct in order to study its anatomy is contraindicated due to the risk of infectious complications.

Treatment- surgical excision of the fistulous tract and revision of the subdural space, if it penetrates under the dura mater.

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Abnormally low position of the cone of the cauda equina with shortened and thickened filaments or intradural lipoma (other formations, such as lipoma extending through the dura or diastematomyelia are considered as independent diseases). Most often observed with MMC. In the case of MMC, the diagnosis must be made clinically, since virtually all patients have radiographically fixed SM.

Clinical manifestations: complaints and symptoms are given in Tables 6-15.

Table 6-15. Complaints and symptoms in fixed spinal cord syndrome

* high incidence of scoliosis or kyphosis due to the inclusion of data from the Hoffman series

Patients with myelomeningocele

If a patient with MMC has progressive scoliosis, increasing spasticity, worsening gait (in those who could walk before), or worsening urinary disorders:

It is always necessary to make sure that the existing shunt is working and that ICP is normal.
. if there is pain, then it should be considered that there is a fixed CM until proven otherwise
. the patient should be considered to have syringomyelia until proven otherwise
. may result from compression of the brainstem (symptomatic Chiari malformation type 2) requiring decompression of the PCF

Scoliosis with a fixed spinal cord

Progressive scoliosis can be observed in combination with a fixed SM. Early release of the SM can lead to a reduction in scoliosis, but it must be done while the scoliosis is not yet pronounced. In cases where SM release was performed with scoliosis ≤10º, neurological improvement was observed in 68% of cases, and in the remaining 32% of cases, stabilization of symptoms was observed. At the same time, if the scoliosis was severe (≥50º), ∼16% of cases worsened.

Tethered spinal cord syndrome in adults

Although in most cases the syndrome of fixed SM becomes apparent already in childhood, there are reports of cases in adults (by 1982, ∼50 were published). Comparison of children's and adult forms, see table. 6-16.

Tab. 6-16. Comparison of pediatric and adult forms of fixed spinal cord syndrome

Diagnostics

X-ray: low-lying cone (below L2) and thickened terminal thread (normal diameter of 2 mm is considered pathological). NB: the apparent diameter of the terminal filament on CT/myelogram may vary depending on the concentration of CM.

It is difficult to differentiate a fixed SM from a low-lying SM (in which the terminal filament usually has a normal diameter).

Preoperative diagnostics

Preoperative cystometrography is highly recommended, especially if the patient has urinary control (postoperative changes in bladder function are not uncommon, possibly as a result of tension on the lower fibers of the cauda equina).

Treatment

If the only abnormality is a shortened and thickened filament terminal, a small laminectomy of the lumbosacral region can be limited to the division of the filament terminal after its identification.

If a lipoma is found, it can be removed along with the terminal filament (if it easily separates from the nervous tissue).

Distinctive features of the terminal thread

The terminal thread differs from the nerve roots by the presence of a characteristic convoluted vessel on the surface. Also, under a microscope, the filament looks much whiter than the nerve roots, and stripes are visible on it. NB: intraoperative electrical stimulation and EMG recording of the anal sphincter are more accurate methods of identification.

outcomes

With MMC, it is usually impossible to eliminate the fixation of the SM for good. As a child with MMC grows, 2-4 operations may be required to eliminate fixation of the SM. However, once growth stops, SM fixation may no longer be a problem. In cases where fixation has been eliminated in early childhood, symptoms may recur later, especially during the period of intensive growth.

adult form: surgical release of the fixation usually provides good pain relief. However, it does not help restore bladder function.

Greenberg. Neurosurgery

The spinal cord is the central organ in the nervous system. It consists of special fibers that are located in the spine and nerve cells. The spinal cord is a long cylinder. The cerebrospinal region consists of a gray substance that surrounds a white substance. Spinal cord injury can occur due to many different factors. Such a disease, as well as damage to any other part of the spinal cord, can provoke serious disorders of the motor and autonomic systems.

Symptoms

Syndromes and symptoms can be very different, it depends on what level of development the disease is at, and which of the substances was affected. The gray matter is the nerve cells in the spinal canal, and the white matter is the processes of such nerves.

When the spinal cord is damaged, the following symptoms appear:

• There is a violation of the motor function of the limbs.
• There are pain in the lower back and neck.
• The sensitivity of the skin is impaired.
• Urethral incontinence occurs.
• The sensitivity of the joints and muscles is lost, atrophy may occur.
• In some places, the skin temperature may rise.
• There is pain in the muscles.

Spinal cord disease can lead to paralysis, cause serious and irreversible consequences, so when the first symptoms appear, you should consult a specialist doctor. He will conduct a complete and comprehensive examination of the body, determine whether there is a lesion of the spinal cord and at what level of development it is, as well as which of the departments is damaged. Then the doctor will prescribe the necessary course of treatment.

Syndromes

Briefly, several syndromes can be distinguished depending on their location. For this, a table is provided that briefly describes the syndromes of spinal cord injury:

Location of damage	Syndrome
Damage to the anterior horns	Paresis occurs, that is, partial paralysis of the body and limbs, pain in the muscles and joints, which appears in connection with the affected segments.
Damage to the posterior horns	There is a disorder of skin sensitivity.
Side edge area	On the affected side, central paresis occurs, and on the opposite side, pain and temperature sensitivity appears, sometimes the localization of the affected area may change.
Back edge area	From the side of the affected area, articular-muscular feelings are lost, they become much lower than the level of damage, tendon reflexes decrease.
Half lesion of the spinal cord	On the part of the affected area, central paresis occurs and joint-muscular feelings are lost, on the opposite side, pain and temperature sensitivity disappears. Half of the spinal cord is completely damaged.
Complete defeat	The sensitivity of the skin is completely lost, disorders in the pelvic region occur, cervical tetraparesis, thoracic, or paresis at the lumbar level occurs.

Damage levels

There are several levels of spinal cord injury:

1. Craniospinal disorder.
2. Syndrome of lesions of the upper cervical segments.
3. The cervical region thickens.
4. Thoracic injury.
5. Thickening of the lumbar region.
6. Epiconus of the spinal cord.
7. Cone failure.
8. Cone and epicone.
9. Damage to the horse's tail.

The first level is due to the fact that the spinal cord is affected mainly due to the presence of tumors or any injuries. Often such a lesion occurs in the spinal region or inside the posterior foramen. In this case, the following symptoms are typical:

• Pain occurs in the back of the head or neck, less often in the spine or limbs.
• Tetraparesis of a mixed type appears, mainly in the extremities.
• There is a partial disorder of sensitivity.
• Breathing is disturbed due to irritation of the respiratory apparatus in the medulla oblongata.
• The cranial nerves are affected.
• There is a violation of the functioning of the pelvic organs, possibly urinary incontinence or, conversely, the accumulation of urine in the human body.

When the upper cervical segment is affected, the following symptoms may occur: a complete violation of sensitivity, which is below the affected level, is possible; radicular symptoms, hiccups may occur; paralysis occurs.

The next level of thickening of the cervical region is characterized by the fact that lower and upper paraplegia occurs, all types of sensitivity and the urethra are completely violated.

When the thoracic region is affected, paraplegia of a spastic nature may occur, sensitivity that is below the affected level may be impaired, the functioning of the urethra may be impaired, and various reflexes may be disturbed, for example, vegetative.

With a thickening of the lumbar region, lower paraplegia occurs, the sensitivity of the lower extremities decreases, and the efficiency of the urinary system is impaired.

The epiconus of the spinal cord is a transverse injury to the spine, which over time can develop into a more serious disease and disrupt the integrity of the bone marrow. With such damage, the following symptoms develop:

• In men, an erection may completely disappear or be disturbed.
• The functioning of the pelvic organs slows down (in this regard, there is a delay in feces or urine).
• There are symmetrical paresis on the feet.

The defeat of the cone is characterized by the fact that people completely lack the anal reflex, erection, impotence, and the functioning of the urinary system is disrupted.

In the cone and epiconus syndrome, the above symptoms, which are related to the cone and epiconus syndrome, are combined into a single whole. In addition, the trophism of the buttocks may be disturbed.

The last level of development of spinal cord injury is the cauda equina or "roots". At this stage, the functioning of the pelvic organs is disturbed, pain in the pelvic region appears, more often - with a horizontal position of the body, the sensitivity of the lower extremities completely disappears.

Violation of the locomotor apparatus

With the defeat of the spinal cord, in any case, sick people have a violation of motor functions. It can be complete and be called "spinal cord paralysis" or partial and be called "spinal cord paresis". In the case when four limbs are damaged, a violation of motor functions is called "tetraplegia" or "tetraparesis", depending on the degree and level of damage. If only two limbs were affected, then the movement disorder is called “paralegia” or “paraparesis”, which are also affected by the level and degree of damage.

Movement disorders are almost always symmetrical - on the right side and on the left. But there are some exceptions, for example, when the ponytail is damaged or stab wounds were inflicted. In another way, it can be expressed that when the damaged area is a point.

There are several levels, but the most critical is the defeat of the cervical vertebra, because respiratory arrest can occur - the diaphragm. Accordingly, it can lead to death. Those injuries that are below this level can only cause a disorder of the respiratory system, in which case, if you notice the disease in time and provide first aid, you can save a person's life.

Loss of sensation

When the disease affects the spinal cord, there is a loss of sensation in the limbs. If the disease does not affect the spinal cord, but passes externally above it, then the sensitivity of a person gradually decreases, and then may disappear completely. At the same time, pain and temperature sensitivity may decrease, sometimes there may be a slight tingling sensation, a feeling that goosebumps are “running”, and even limbs may become numb. The degree and level of sensitivity reduction depends entirely on the individual characteristics of the person, the structure of his body and the level of damage to the spinal cord.

Vegetative system

With damage to the spinal cord, vegetative disorders occur, these include:

• Increased or decreased skin temperature.
• Increased sweating.
• Too dry skin in a certain area.
• The tissue trophism is disturbed (ulcers are formed).
• There is a delay in feces or diarrhea.
• Failure of the genitourinary system, that is, incontinence or difficulty emptying the urinary canal.
• Poor bowel and stomach function.
• Useful enzymes are produced with minimal intensity.

These symptoms indicate that a person had a failure of the autonomic system, which means that the spinal cord was affected or affected.

Pain

Pain in this disease is an integral part, they are almost always present. They occur in the middle of the back, which means that there is a compression of the spinal region. If pain is disturbing in the region of the upper extremities, then the cervical nerve was pinched, pain in the lower extremities is a sign of the development of osteochondrosis, which could appear due to an injury or tumor in the lumbar region. In order to better determine what disorders may appear when any of the departments is affected, you should consult a specialist doctor. With the help of diagnostics, the cause of pain will be established, and then eliminated.

Diagnostics

When the first symptoms appear, you should contact a specialist doctor so that he conducts a comprehensive examination of the body, reveals the cause of the violation, the level of its development. Diagnosis of the patient is carried out by various methods, such as:

• radiography;
• CT scan;
• myelography;

Thanks to x-rays, you can see fractures, bruises and other factors that could affect the damage to the spinal cord. All methods allow you to determine the location and level of development of damage that can adversely affect the patient's health.

Treatment Methods

When providing medical care, the following measures must be taken:

• Apply a bandage to the patient after injury.
• Give the victim as much fresh air as possible.
• Provide the patient with as much space as possible, free him from tight clothing or foreign objects.

If there is a suspicion that the injury is in the cervical region, the patient must be placed on a stretcher, a roller should be placed under his head, and a cotton collar should be applied to his neck. Be sure to lay some kind of bedding before laying the patient on a shield or stretcher. The main thing is that no folds are formed on it, because the patient may develop bedsores, and quickly enough.

After that, before the arrival of medical workers, you can give the victim a tablet of Analgin or some other pain medication. Then wait for the ambulance to arrive.

Medical method of treatment

Medical work is first introduced to the victim:

• Glucocorticoid hormones.
• Diuretic drugs such as furosemide.
• Neuroprotectors.

Then specialist doctors conduct a complete examination of the human body, identify the cause of the violation, the level and location of the damage. Based on this, further treatment is carried out in accordance with the individual characteristics of the person and his body structure.

Surgical intervention

This method of treatment is used in the event that drug treatment has not had an effective result. If a sick person has a cancerous formation, the operation is performed without fail. In the presence of a benign formation or injury, surgical intervention is used only when there are severe pain sensations that cannot be overcome with the help of pain medications. With instability of the spine, and this threatens with a general deterioration in the physical condition of a person, an operation is also performed.

Care of affected people

Care of victims requires special care. It is important to remember that it is often necessary to change the position of the patient's body in order to avoid the occurrence of bedsores. It is necessary to use special linings that are placed under the coccyx, lower back and heels. A massage is a must. If the victim is conscious, then breathing exercises should be performed. After consultation with a doctor and the absence of contraindications, you can begin to bend and unbend the limbs in the joints.

Conclusion

So, the spinal cord is the center in the human nervous system. It is responsible for the supply of impulses to the entire body. At the slightest violation of the spinal cord, there may be serious consequences associated with the motor apparatus and autonomic functions. There are several levels of development of the disease that are directly related to the disease of the spinal cord. Each level is assigned certain symptoms. Basically there is a violation of the functions:

• vegetative system;
• urethra;
• stomach;
• intestines.

In addition, the motor function of a person, his 4 or 2 limbs is disturbed, the sensitivity of the skin is significantly reduced. When the first symptoms appear, you should immediately contact a specialist doctor who will conduct an examination and prescribe the necessary course of treatment.

At the moment, there are several methods for diagnosing a patient, which allow you to almost immediately determine the cause of the lesion of the spinal cord, see fractures, bruises and their location.

Treatment is with medications or surgery (in certain situations, or if medical therapy has not helped).

When providing first aid, you should remember that if everything is done on time and correctly, you can save a person's life. Most disorders that affect the spinal cord can be fatal. When caring for the sick, you need to change their position more often, do massage and breathing exercises. This way you can help the victim recover as quickly as possible.

Fixed spinal cord syndrome- a rather rare pathology and is often not diagnosed until the appearance of neurological syndromes, pain and MRI tests. Other names for this pathology of the spine:

• Terminal thread tension syndrome (tethering syndrome)
• Syndrome of the fixed (stretched) spinal cord (FSM),
• Syndrome of a rigid trailer, or terminal, thread.
• Sometimes in the Russian-language literature the term tethering syndrome is also used (from the English tethered cord syndrome - the syndrome of the "tied" spinal cord).

What does "fixed spinal cord" syndrome mean?

Tethered spinal cord syndrome is a condition in which your spinal cord becomes attached to your spine. The most common site for this lesion is the lumbar spine.

Typically, your spinal cord "floats" in the cerebrospinal fluid at the center of your spine. This allows him to move freely and also protects him from damage.

Causes of a fixed spinal cord

There are several reasons for this deviation:

• Chiari defect, when the brain enters the upper part of the spinal canal
• spina bifida, or structural defects in the lower part of the spinal cord
• injury or damage to the lower back, resulting in injury to the spinal cord
• scar tissue after surgery
• tumors or cysts in and around the spinal cord

Risk factors

Fixed spinal cord syndrome affects both children and adults. Approximately 2 out of 1000 people are born with this pathology, but, as mentioned above, sometimes the disease develops as a result of the appearance of scar tissue, as well as the growth of a tumor or cyst in the spinal cord.

Symptoms

The symptoms of tethered cord syndrome can vary greatly. In addition, the symptoms in children often differ from those that appear in adults.

Symptoms in children may include:

• Various disorders, tumors, hair growth, dimples, or discoloration of the skin on the lower back
• Pain in the legs or lower back
• Difficulty walking
• Bed-wetting
• Leg and foot deformities

In 70% of children, the symptom begins to appear at 4 years. Symptoms always worsen with age, with growth, and some neurological damage may be irreversible.

In adults, these symptoms are similar but may be more severe due to the increased strain on the spinal cord over time.

Diagnostics of the fixed spinal cord syndrome

If tethered cord syndrome is suspected, magnetic resonance imaging (MRI) of the lower spinal cord is done.

Methods of treatment of the fixed spinal cord syndrome

If tethered cord syndrome causes only minor symptoms or is asymptomatic, your doctor may recommend observation instead of surgery. However, surgery may be indicated even for minor symptoms, as lost neurological functions cannot be restored even after surgery.

Operation

In severe cases, the only way to relieve symptoms is surgery. It involves opening the lower back to eliminate the affected area. Neurosurgeons usually separate the spinal cord from its surrounding structures using complex surgical techniques, working under a powerful microscope.
In cases where a recurrence of the fixed spinal cord syndrome occurs after the initial operation, it can be performed again. In addition, surgery is sometimes performed to remove a vertebra from the spine. This shortens the length of the spine and can relieve tension in the spinal cord, as well as relieve pain symptoms. This procedure is usually performed for patients in whom the disease often relapses.

What is the prognosis after the operation?

The operation can remove the fixation of the spinal cord, relieve neurological symptoms and prevent their worsening, as well as get rid of other symptoms, such as pain, that are not neurological in nature. However, symptoms such as weakness and numbness in the legs that result from nerve damage are often irreversible.

Important! Only here, at the American Institute of Spinal Surgery in Cyprus, an innovative treatment is performed in the field of spinal cord injury. This therapy showed clinically significant results - more than half of the patients noted a significant improvement in neurological symptoms, another quarter noted a noticeable improvement, the rest of the patients said that there was a slight improvement in their condition.

Relapse of this disease occurs in some cases, it is characterized by the return of old symptoms. In these circumstances, options for reoperation may be considered.