What’s spinal dysraphism?

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Spinal dysraphism is a congenital defect of the neural tube that can cause a range of symptoms from mild to severe. Open spinal dysraphism can result in meningocele or myelomeningocele, which can severely impair the central nervous system. Diagnosis can occur while the fetus is still in the uterus, and treatment may include surgery, physical therapy, and medications. Closed spinal dysraphism, such as spina bifida occulta, may not cause physical symptoms but can lead to complications later in life.

Spinal dysraphism is a congenital defect of the neural tube, the structure that precedes spinal cord development in fetuses. In normal development, the neural tube bends together precisely to form the vertebral column and secure the spinal cord. Problems can arise if the neural tube never closes completely or seals together incorrectly. Symptoms in infants and growing children can range from very mild and barely noticeable to debilitating and disfiguring. Depending on the severity of your symptoms, you may need a multifaceted treatment plan that includes medications, surgery, and physical therapy to promote proper growth and development.

Open spinal dysraphism occurs when the neural tube fails to close completely. There are several varieties of open spinal dysraphism, the most common of which is the development of a meningocele in the lower or mid back. A meningocele is basically an opening in the spine where membranes, cartilage, and cerebrospinal fluid leak out. The spinal cord is usually kept intact in the presence of a meningocele, so it can continue to develop without major complications to central nervous system function.

A less common but much more serious complication of open spinal dysraphism is the growth of a myelomeningocele. A large cap in the spine makes room for a membrane-lined sac to protrude outward, breaking up the skin and muscle tissue. The myelomeningocele sac may contain cartilage, cerebrospinal fluid, and fragments of the spinal cord and nerve endings. The condition can severely impair the future development and functioning of the central nervous system. A child may not have control over motor movements or bladder and bowel functions. In extreme cases, disabling weakness in the legs or even permanent paralysis can occur.

In most cases, open spinal dysraphism can be diagnosed while the fetus is still in the uterus. Ultrasounds and blood tests may reveal abnormalities in spine development in the second or third trimester. A child born with open spinal dysraphism typically needs to have immediate surgery to try to realign the spinal cord and vertebrae. A shunt may need to be placed in the brain to drain excess CSF into the abdominal cavity. Ongoing physical therapy, medications to control pain, and follow-up cosmetic surgical procedures may be needed throughout the patient’s life.

Spinal dysraphism can also be a more subtle problem where the neural tube closes nearly properly and internal structures do not protrude from the spine. Spina bifida occulta is the most common form of closed spinal dysraphism and may or may not cause physical symptoms and health complications. Most children born with spina bifida occulta develop normally despite minor defects and do not require special care. Occasionally, there may be an indentation in the lower spine that is prone to excessive hair growth. Patients may be at risk for complications later in life, such as scoliosis, difficulty bending or twisting, and a herniated disc that may require surgery.




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