Fixed spinal cord is a rare pathology characterized by limited mobility of the spinal cord in the spinal canal. The disease is more often congenital in nature and is associated with intrauterine violation of the proportion of development of the spinal cord and spine, as well as with post-traumatic and post-inflammatory cicatricial-proliferative changes that lead to fixation of the spinal cord in the caudal region.
Normal development of the spinal cord
At the 3rd month of intrauterine development, the spinal cord occupies the entire length of the spinal canal. Then the spine is ahead in growth. At birth, the level of the final section of the spinal cord corresponds to the level of the 3rd lumbar vertebra. At the age of 1-1.5 years, the spinal cord ends at the level of the 2nd lumbar vertebra in the form of a pointed cone. The atrophied part of the spinal cord continues from the top of the conical point and is attached to the 2ndcoccygeal vertebra. Throughout the spinal cord is surrounded by meninges.
Spinal cord fixation
The spinal cord is fixed more often in the lumbosacral region, stretched, and metabolic and physiological disorders of neuronal formations occur. Neurological symptoms develop in the form of a violation of sensitivity, a decrease in motor activity, pathology of the pelvic organs, etc.
The term was first voiced by Hoffman et al. in 1976. A group of children was studied (31 cases). Identified sensory and movement disorders, which were accompanied by urination disorders.
Causes of a fixed spinal cord
Any process that can fix the spinal cord and limit its mobility can cause the syndrome:
- Fatty tumors of the lumbosacral region.
- Dermal sinus - the presence of a communicating canal of the spinal cord with the external environment using a fistulous tract. It is a congenital developmental anomaly.
- Diastematomyelia is a malformation in which a cartilaginous or bone plate extends from the posterior surface of the vertebral body, dividing the spinal cord in half. In parallel, rough scarring of the terminal thread occurs.
- Intramedullary volumetric education.
- Syringomyelia is the formation of cavities inside the spinal cord. Accompanied by pathological growth of the glial membrane.
- Cicatricialdeformation of the terminal thread.
- Fixation of the spinal cord may be secondary and appear at the site of surgical intervention on the spinal cord. Seen after myelomeningocele repair.
Clinical picture and diagnosis of the syndrome
Based on a thorough general clinical examination of the patient, neurological examination and supplemented by a set of instrumental studies. To expand the range of diagnostic search, neonatologists, pediatricians, surgeons, neurologists, neurosurgeons, orthopedists, urologists, infectious disease specialists are involved.
The age of the child determines the set of diagnostic measures.
Case history
In newborns, the anamnesis is poor and requires the expansion of diagnostic methods. In older children, there is a violation of gait, muscle weakness in the legs, sometimes there is a difference in the muscle mass of the limbs, one of which seems thinner. There are disorders of urination in the form of urinary incontinence. In adolescence, skeletal deformity may appear, children suffer from frequent urinary tract infections, and this is due to a decrease in bladder tone.
The most common symptom of the disease is unrelenting pain in the groin or perineum, which can spread down the spine and into the lower extremities. Often a pain symptom is accompanied by a violation of the sensitivity and tone of the lower limb.
Inspection
In the lumbar region you can find: a tuft of hair ("tailfawn"), median nevus, local hypertrichosis, dermal sinus, subcutaneous fatty tumor. These signs are stigmas of dysembryogenesis.
Skeletal deformities often occur (scoliosis, kyphosis, asymmetry of the pelvic bones, progressive deformities of the feet), which occur in a quarter of patients.
Most important is the neurological examination, which reveals early signs of fixed brain syndrome in children. Characterized by paresis of the lower extremities of varying degrees. In children under the age of 3 years, it is difficult to determine the depth of paresis, but it can be judged by the volume of movement disorders (there is no spontaneous activity). Outwardly, there may be signs of atrophy of the muscles of the limbs and the gluteal region, but if the child is overweight, this may hide the lesion. Violation of sensitivity manifests itself in the form of its decrease or absence on the lower extremities, the perineum and buttocks. These children are prone to burns due to loss of sensation.
Disorder of the pelvic organs in the form of various urination disorders (urine leakage after emptying the bladder, false urges, involuntary urination), violations of the act of defecation.
Instrumental methods of diagnostics
Supplement the data of the anamnesis and clinical examination.
- X-ray. It is the initial research method for suspected lesions of the spine and spinal cord. Inadequate in terms of the study of soft tissue formations.
- Ultrasonography. A valuable method for diagnosing the disease in children under 1year.
- MRI. The most sensitive method that allows you to study the structure of spinal pathology in detail.
- Computed tomography is informative only in cases where it is necessary to visualize the deformation of the skeleton before the upcoming operation.
- Urodynamic testing (cytometry, uroflumetry, electromyography). Performed for the diagnosis and postoperative control of children with fixed spinal cord syndrome.
Treatment of a fixed spinal cord in children
Therapy of fixed brain syndrome in children is handled by a tandem of specialists. Children undergo a complete examination every 3 months during the first year of life and every 6 months until adulthood. Thereafter, the survey is carried out annually. Family members are warned that if their general condition worsens, if neurological disorders appear, if urination and defecation are disturbed, they should consult a doctor unscheduled.
The main method of treatment is neurosurgical. The earlier treatment is started, the more effective the result. The essence of the surgical intervention is defixation of the spinal cord.
Results of fixed spinal cord surgery in children:
- regression of pain syndrome (65-100%);
- improvement in neurological status (75-100%);
- regression of urological disorders (44-93%).
Orthopedic disorders require additional surgical correction, for example,fixation of the corrected spinal deformity with a metal structure.
A high risk of poor outcome is observed in children with malformations of the divided spinal cord, lipomyelomeningocele. Such patients most often develop secondary fixations of the spinal cord at the sites of surgical intervention.
Conclusion
Symptoms of a fixed spinal cord in children can be detected in utero during a routine ultrasound. These data make it possible to prepare a born child for a planned operation at an early age.
