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Diastematomyelia is a congenital spinal condition where tissue grows within the spinal canal, dividing the spinal cord in two. It can occur with other spinal abnormalities and is often diagnosed during prenatal ultrasound. Symptoms vary and treatment can be surgical or conservative.
Diastematomyelia is a congenital spinal condition in which bone, cartilage, or fibrous tissue grows within the spinal canal, dividing the spinal cord in two and then more often reconnecting below the lesion. The condition is often progressive and can occur in conjunction with spina bifida or other congenital spinal abnormalities. Most people are diagnosed with this congenital condition during prenatal ultrasound or baby doctor visits. Symptoms vary; they may include tethering of the spinal cord or fixation of the spine with tissue attachments, which stretch the spine and result in various neurological conditions. Treatment can be surgical or conservative depending on the symptoms.
The exact cause of diastematomyelia is unknown; Scientists think that early in gestation, an embryo develops two spinal canals somewhere between the ninth thoracic level and first sacral level of the spine. Below the lesion, the spinal columns can recombine, but not always. Diastematomyelia can occur in conjunction with other congenital spinal anomalies including spina bifida, butterfly vertebra, hemivertebra, or kyphoscoliosis. Girls are more often affected than boys.
Diagnosis of diastematomyelia can be made during prenatal ultrasound if the technician is able to visualize the back of the fetus. After birth, the baby’s back is regularly checked for any abnormalities. In infancy, a telltale symptom of this condition is a patch of hair, dimple, or other stigmata at the spinal separation. Confirmation of this condition can be done with screening MRI and postmyelography CT. The scan gives the doctor a very detailed picture of the bone and will reveal any associated pathology.
Ligation or fixation of the spinal cord may be responsible for the neurological symptoms in diastematomyelia. Children may develop leg weakness, lower back pain, or incontinence. Foot and spinal deformities such as scoliosis can also occur. Adults often exhibit sensorimotor problems, bladder and bowel incontinence, impotence, pain, and symptoms affecting the autonomic nervous system. The condition can be progressive with worsening symptoms over the course of the patient’s life.
There are two approaches to treating diastematomyelia: surgery and observation. Patients with worsening neurological symptoms can be treated surgically. During minimally invasive microsurgery, bone or fibrous tissue is removed and the dural sacs are repaired. The best candidates for surgical treatment are young people who have had less severe neurological symptoms for a shorter period of time. Asymptomatic patients or those with stable symptoms can be treated conservatively with periodic visits to a neurologist. If symptoms develop or worsen, surgery may be needed.
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