Congenital Malformations of the Head and Spine

Congenital Malformations

Pediatric neurosurgeons can correct a variety of malformations of the bone and soft tissue of the head and spine, including neural tube defects such as spina bifida.

Each year, one in a thousand babies are born with spina bifida (meningomyelocele), a malformation of the bones (vertebrae) and/or skin surrounding the spine that can lead to serious infections, problems with bladder and bowel function, hydrocephalus and paralysis. In most cases, surgical correction of the neural tube defect can prevent such complications.

Other congenital problems that may be treated by a pediatric neurosurgeon include:

  • Chiari malformation, a condition in which portions of the base of the spine protrude into the upper spine, where they may compress the brain or spinal cord. Left untreated, this condition can lead to neck pain, hoarseness, upper-respiratory tract infections, and progressive weakness of the arms and legs. Chiari malformation also may block the flow of cerebrospinal fluid (CSF), leading to hydrocephalus. Treatments often focus on removing portions of the bone and soft tissue to relieve pressure on the spinal cord and brain, as well as providing new pathways to drain CSF. Encephaloceles is a condition similar to chiari malformation that affects the passageway between the nose and the front or back of the head.
  • Encephaloceles can lead to infections and hydrocephalus. Surgical treatment of this condition involves removing bone and soft tissue or drainage of CSF.
  • Tethered spinal cord, the attachment of the spinal cord to surrounding tissue. Failure to detect a tethered spinal cord can lead to a sudden catastrophic injury during childhood or adolescence, such as paralysis. Tethered spinal cord can be diagnosed through the detection of certain skin abnormalities along the midline of the back. Diagnosis may be confirmed by magnetic resonance imaging (MRI), and surgery is usually indicated to prevent neurological damage.