A wide range of conditions can affect the spinal cord as it is developing. There are two main categories of spinal cord abnormalities:
- Spina bifida aperta / myelomeningocele – an opening in the skin exposes an abnormal spinal cord. Surgery is typically required within the first 2-3 days after birth in order to reduce the risk of infection. Children with this form of spina bifida require a complete evaluation, as it can often be associated with hydrocephalus and/or a Chiari malformation.
- Spina bifida occulta / occult spinal dysraphism (OSD) – more commonly, spinal cord abnormalities are covered by intact skin, but can be associated with skin pits, atypical dimples, lipomas, or unusual hair patterns. Spinal cord abnormalities may therefore be suspected based on certain types of skin changes. These abnormalities have the potential to “tether” the spinal cord to the surrounding tissues. This puts tension on the end of the spinal cord as the child grows taller. “Tethered cord syndrome” refers to the clinical signs and symptoms that may result from tension on the end of the spinal cord. These may include:
- Pain: both low back pain and leg pain, usually brought on by physical activity and relieved by rest
- Orthopedic deformity: usually a spine or leg deformity that may differ between the right and left sides of the body
- Neurologic findings: including weakness or sensory loss
- Autonomic findings: including bladder issues (urgency, frequency, infection) and severe, chronic constipation
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How are spinal cord malformations diagnosed and treated?
A spinal ultrasound (during the first 3 months of life) or an MRI may confirm the presence of a spinal cord malformation and guide surgical management.
Surgery relieves the tension on the spinal cord in order to preserve neurological function. Most children are discharged within a few days of surgery, but long-term follow-up is often recommended.
Coordinated through our Tethered Cord/Spina Bifida Program, Connecticut Children’s neurosurgeons work closely with a team of multidisciplinary specialists from urology, orthopedics, gastroenterology, and physical therapy to provide highly specialized care for children affected by spinal cord malformations.