Spinal dysraphisms are congenital abnormalities of the spine and the spinal cord. The reason for the occurrence of these diseases is disorders in the womb during the development of the child. Many diseases and infections the mother and the baby have, or bad habits such as alcohol and smoking may be the cause of these pathologies. When these pathologies are detected, surgery is recommended to prevent various types of paralysis and sometimes infections that may develop later on. Since the urinary, bladder and kidney problems are observed at a high rate in patients with spinal dysraphism, patients who receive this diagnosis should be examined by pediatric nephrology and/or pediatric urology physicians. Spinal dysraphisms may be closed and open.
In this case, there is a gap in any part of the spine. While the cerebrospinal membrane and fluid are present in the sac that is observed in case of meningocele, the sac in case of meningomyelocele also contains the spinal cord tissue. Therefore, some of the patients are born partially or fully paralyzed. Since the skin is open and the risk of infection and death due to this infection is high in cases of meningocele and meningomyelocele, surgery is recommended without further delay. As pathologies such as hydrocephalus and Chiari malformation (cerebellum prolapse) are observed together with high rates, it may be necessary to scan these patients and, if necessary, to intervene in these pathologies. These conditions are called encephalocele and encephalomyelocele when they are encountered in the brain.
In this pathology, a pathological pathway, tunnel, from the skin to the spinal cord is found. With this connection, infection can be carried from outside of the skin to the spinal cord.
In split spinal cord syndrome, the spinal cord is split in half with bone or soft tissue bands. This condition develops when the baby is in the womb. Since the growth of the spine and spinal cord is not at the same rate after the baby is born, the bone or soft tissue curtain that splits the spinal cord into two can cause tension and damage in the spinal cord over time. It is usually seen with the pathology of tethered filum terminale.
In tethered cord disorder, the soft tissue band that originates from the lowest segment of the spinal cord and connects the spinal cord to the bottom of the spinal canal in the spinal cord (sacrum) becomes thicker than normal, stretching the spinal cord. Normally, the spinal cord terminates at the level of the first lumbar spine (L1), but in this case the spinal cord terminates at a lower level. Strokes, urination and defecation disorders can be seen as a result of this stretching, lack of blood circulation and lack of oxygen in the spinal cord.
Although the clinical examination is often diagnostic in these pathologies (especially in meningocele and meningomyelocele), magnetic resonance imaging (MRI) may be required in patients considering that some of these pathologies may be concurrent or an additional and different pathology may be observed. While MRI shows the spinal cord and soft tissue bands in great detail, Computed tomography (CT) usually shows bone structures in patients with diastematomyelia in a detailed manner. Since some of these patients already have hydrocephalus and Chiari syndrome or these may develop later, they need to be examined and monitored with brain MRI. In addition, urodynamic tests may be required to examine the urinary function of patients whose urinary problems are not apparently emergent.
The treatment for these diseases is surgical intervention, follow-up is not typically recommended.
In cases of meningocele and meningomyelocele, the skin and the sac should be repaired by surgical treatment. Because the risk of infection is high in cases where the skin is exposed, surgical treatment should be performed promptly.
In the dermal sinus tract, the tube extending from the spinal cord to the skin should be removed, repaired and closed.
In split spinal cord syndrome, the bone or soft tissue curtain that divides the spinal cord into two is removed, and the membrane around the spinal cord (dura mater) is combined to move freely in a single compartment with the growth of the spinal cord.
In the tethered cord syndrome, if the cause of the stretching is the thick filum terminale at the lower end, it may be necessary to remove it.
Patients who are operated for these pathologies should be strictly followed-up for urinary function and urinary tract infections. It should be noted that adhesions may develop in patients who undergo surgery, that may cause gait disorders, different types of paralyses and urinary problems, therefore the patients should be followed-up with this in mind. It should also be noted that hydrocephalus may develop especially after large meningocele and meningomyelocele operations.
