Myelodysplasia (Spina Bifida)
Myelodysplasia--also known as spina bifida--is a congenital birth defect that causes lesions of the spinal cord and canal. The lesions of spina bifida are divided into three categories:
- Spina bifida occulta
- Occult spinal dysraphic syndromes, including tethered spinal cord syndrome
- Myelomeningocele or spina bifida aperta
Spina Bifida Occulta
Spina bifida occulta occurs in about 25 to 30 percent of the general population. Children with spina bifida occulta have lesions on their spinal bones, and the spinal canal is open because the bones do not close during development. This type of spina bifida generally has no symptoms, and needs no treatment.
Occult Spinal Dysraphic Syndromes
Occult spinal disraphic syndromes, including tethered spinal cord, have a lengthy list of symptoms. They can include the following:
- Spinal lipoma (lipomyelomeningocele): Fatty tumors on the back that extend into the spinal canal
- Diastematomyelia: Splitting of the spinal cord into two separate spinal cords. The point in the spinal cord is often tethered by a bone or a fibrous band that goes front to back through the split spinal cord.
- Other tethering lesions, including thick filum terminale or thickened cord at the base of the spinal cord
You can usually see these symptoms under the skin covering the spine.
All of these lesions are best treated with surgery, which is done in order to untether the spinal cord. When a child has a large fatty tumor, surgery may also include reducing the size of the fatty tumor in order to release the spinal cord. Often, our expert pediatric surgeons are able to perform these surgeries with no injury to existing nerve function.
Before surgery, patients usually have an MRI scan in order to determine where the fatty mass is located. We can also perform diagnostic tests to assess urodynamics--or the child's bladder function. These tests can determine the urgency with which a surgery needs to be performed.
Myelomeningocele--or spina bifida aperta--is easily recognized at birth by the open sac on a child's back. Often, the diagnosis is made before birth by giving the mother a blood test and a prenatal ultrasound. Once born, a child with myelomeningocele must be operated on quickly in order to avoid spinal fluid infection. This operation is generally done within 24 to 48 hours of birth.
Children born with myelomeningocele may have other problems including hydrocephalus--which requires a shunt in more than 90 percent of all myelomeningocele children.
As patients born with myelomeningocele grow, an interdisciplinary team of specialists will oversee their developmental progress. The University of Chicago Medicine Comer Children's Hospital has an interdisciplinary myelodysplasia center in order to best address the needs of these children. This team includes:
Our experts will survey your child's development to make sure that leg function remains unchanged and later problems, including tethered spinal cord and difficulty with bladder function, are avoided.