Spinal Congenital Anomalies

Spinal dysraphisms are congenital anomalies of the spine and 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 of the mother and the baby have, 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. Since the urinary, bladder and kidney problems are observed at a high rate in patients with spinal dysraphism, the patients receiving this diagnosis should be and need to be examined by pediatric nephrology and/or pediatric urology physicians. Spinal dysraphisms may be closed and open.

Meningocele and meningomyelocele

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 followed in meningocele, the meningomyelocele also contains the spinal cord tissue in the sac. 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 waiting. 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 in the brain.

Dermal sinus tract

In this pathology pathological pathway, tunnel, from the skin to the spinal cord is found. With this connection, infection from the skin to the spinal cord can be carried.

Diastematomyelia (separated spinal cord syndrome)

In separated 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 when the baby is born, the bone or soft tissue curtain that divides the spinal cord into two can cause tension and damage in the spinal cord. It is seen with the pathology of tethered phylum terminale most often.

Tethered cord (phylum terminale)

Tethered cord is a thick tissue band that connects the spinal cord to the bottom of the spinal canal in the spinal cord (sacrum), which emerges from the lowest end of the spinal cord and becomes thicker than normal, stretching the spinal cord. Normally, the spinal cord should end up at the level of the first lumbar spine (L1), but in this case the spinal cord ends up below. Strokes, urination and fecal disorders can be seen as a result of stretching, lack of blood build up and lack of oxygen of the spinal cord.

Symptoms

  1. The appearance of swelling in the back and waist and sac is usually observed in meningocele and meningomyelocele.
  2. In children, on the back and especially in the waist, if there is abnormal hairing, spine and spinal cord development anomaly, ie, closed spinal dysraphism should be investigated under the skin that appears quite normal.
  3. Nevus and spottings on the back.
  4. Different types of paralysis in the legs (weaknesses).
  5. Gait disorders.
  6. Urinary problems occur as a result of disorders in the bladder, kidneys and urine function.

Diagnosis

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 these pathologies may be together or an additional and different pathology may be observed. While MRI shows the spinal cord and soft tissue bands in detail, computed tomography (CT) usually shows bone structures in patients with diastematomyelia. Since some of these patients have hydrocephalus and Chiari syndrome or develop later, they need to be examined and followed by brain MRI. In addition, urodynamic tests may be required to examine the urine function of patients whose urinary problems are apparently not started.

Treatment

The treatment of these diseases is surgical intervention, usually, follow-up is not recommended.

In cases of meningocele and meningomyelocele, skin and sac should be repaired by surgical treatment. Because the risk of infection is high in cases where the skin is open, surgical treatment should be done immediately.

In the dermal sinus tract, the tube extending from the spinal cord to the skin should be removed and repaired and closed.

In separated 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 spinal cord is stretched down to the thick filum terminal, this may require surgical intervention by cutting that.

Follow-up

Patients who are operated for these pathologies should be strictly followed-up for urinary tract infections and urine. It should be kept in mind that adherence may develop in the patients who are operated and in this case gait disturbance, different types of paralysis and urinary problems should be kept in mind and patients should be followed-up in this direction. It should also be noted that hydrocephalus may develop especially after large meningocele and meningomyelocele operations.