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NEUROLOGICAL DISORDERS

Spinal Anomaly and Spinal Cord Tumors

Spinal anomaly and spinal cord tumors are rare conditions in children that require extensive expertise to diagnose and treat. Children’s Hospital of Orange County is a leader in diagnosing and treating these conditions. The multidisciplinary team at The CHOC Neuroscience Institute offers patients access to the latest treatment and surgical approaches – providing care in a compassionate, patient-centered environment.

Spinal Anomaly

There are several types of spinal anomalies commonly seen in children:

Tethered spinal cord

Tethered spinal cord is one of the most common congenital abnormalities of the spine, occurring in one to two children per 1,000 live births. In this condition, the end of the spinal cord does not develop properly, becoming attached to the surrounding tissues. As a result, it cannot move freely or ascend during the phase of rapid growth.

Seventy percent of patients experience neurological symptoms by age 4. These include reduced leg strength, loss of sensation in the legs, bladder incontinence or low back pain. The remaining 30% of patients remain free of symptoms (asymptomatic) well into adulthood. They may eventually experience similar neurological symptoms as well as sexual dysfunction.

Sometimes, this disease can be diagnosed by the detection of characteristic skin changes at the base of the spine (e.g. fatty mass, birthmark, skin defect, deep dimple). A MRI can provide a definitive diagnosis. Early detection is key in treating this disease because longstanding or progressive neurological symptoms may prove irreversible.

Treatment involves surgical release of the spinal cord through an incision. It is imperative that the surgeon have expertise in this surgery as the corrective procedure is often complex.

Myelomeningocele a.k.a. Spina Bifida

The most common serious spinal cord anomaly is an open neural tube defect referred to as myelomeningocele, commonly known as spina bifida, affecting approximately 1 in 1,000 children born throughout the world. The congenital defect occurs early in the development of the embryo between the 20th and 28th day of development. This is well before most women even realize that they are pregnant. This condition is thought to be a result of folate deficiency in the mother’s diet. Unfortunately, most women do not know they are pregnant until after this crucial time period has past, which is why it is imperative that women take multivitamins prior to becoming pregnant. Between the 20th and 28th day of fetal development, a piece of tissue that will one day become the spinal cord must develop a groove and two folds that connect to form a tube. Myelomeningocele occurs when this tube fails to close. Babies affected with myelomeningocele do survive and are usually born with a back defect that is visible at the time of delivery. Myelomeningocele is commonly associated with the development of hydrocephalus – increased cerebrospinal fluid and pressure within the brain -- as well as with a malformation of parts of the brain, called Chiari malformations.

Symptoms of a myelomeningocele may include leg weakness and numbness, and inability to control the bowel and bladder. While some children with this condition will have minimal signs of these types of problems, others can be severely affected with complete paralysis of the lower extremities and impaired mental development. All patients with myelomeningocele will require continuing assistance from a skilled team of doctors and therapists throughout their lives.

Infants with myelomeningocele must have a repair of their open neural tube. If this operation is not done, the child may experience a serious infection that could harm his or her developing brain. After this repair operation, many children require the insertion of a shunt to treat hydrocephalus that is usually associated with myelomeningocele.

Older children with myelomeningocele may need evaluation of urologic function and regular visits to an orthopedic surgeon, who can determine whether they need to undergo surgery to correct deformities of the spin and lower extremities. Children with myelomeningocele also benefit from early physical therapy and occupation therapy to improve function. Patients can find this multidisciplinary expertise at CHOC. Neurosurgeons also closely follow these patients to detect the early signs of spinal cord tethering, including new weakness in the legs and feet, and changes in bowel or bladder function. They may also participate in management of spasticity and with assisting with orthopaedic procedures.

Occult Dysraphisms

Occult dysraphisms involve many different problems including:

  • Simple sinuses, which are tracks leading from the skin into the spinal canal, can lead to serious infections.

  • Complex combinations of spinal cord and fat tissue, known as lypomyelomeningoceles, may tether spinal cord.

  • Bony spikes that intrude between two halves of the spinal cord, known as diastematomyelia or split cords, can lead to tethered cord.

  • A spinal cord that ends in a flair of abnormal tissue rather that in the usual tapered tip.
Unlike myelomeningocele, which may require surgery quickly, surgery in patients with spina bifida oculta may be delayed to allow the infant to grow. It is recommended that this operation be done within the first 3 months to 6 months of age.

Spinal Cord Tumor

Tumors in the spinal cord are not as common as brain tumors, accounting for 15% of nervous system tumors. The majority of spinal cord tumors in children grow very slowly, with only about 10% presenting as aggressive and rapid-growing. If a tumor develops in the spinal cord, symptoms could take months or even years to develop. They include severe pain along the spine, numbness, weakness, incontinence or difficulty walking.

The best means of identifying a spinal cord tumor is with MRI. If a spinal cord tumor is detected, it can be treated with surgery and/or a combination of adjuvant treatments like radiation. It is vital that spinal cord tumors be treated as early as possible to avoid any further functional damage. Weakness or neurological impairment occurring prior to surgery may not significantly improve after surgery. This emphasizes the need for early diagnoses.

At the CHOC Neuroscience Institute, our experts have extensive experience in the diagnosis and treatment of spinal cord anomaly and spinal cord tumors. They work closely with families to ensure highly individualized, highly compassionate care.

Diagnosis and Treatment of Spinal Anomaly and Spinal Cord Tumors

At the CHOC Neuroscience Institute, our experts have extensive experience in the diagnosis and treatment of spinal cord anomaly and spinal cord tumors. They work closely with families to ensure highly individualized, highly compassionate care.

The best means of identifying a spinal cord tumor is with MRI - measurement of the brain’s electrical activity, generating a detailed picture of the brain’s structure.

If a spinal cord tumor is detected, it can be treated with surgery or radiosurgery. It is vital that spinal cord tumors be treated as early as possible to avoid any further functional damage. Once weakness or neurological impairment occurs prior to surgery, it does not significantly improve after surgery.

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It is important to remember the health information found on this website is for reference only not intended to replace the advice and guidance of your healthcare provider. Always seek the advice of your physician with any questions you may have regarding a medical condition. If you think you may have a medical emergency, call your physician or 911 immediately.
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