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Holly Gilmer MDMyelomeningocele is a birth defect that occurs when the vertebrae do not fully form and remain open allowing the spinal cord to protrude. The condition is also known as a type of spina bifida. Exposure of the spinal cord leads to varying degrees of sensory damage, incontinence, disability and weakness. More advanced conditions that can occur as a direct result of myelomeningocele can include:

  • paralysis
  • kidney and liver damage
  • scoliosis
  • hip dislocation
  • tethered spinal cord
  • Chiari malformation
  • hydrocephalus

As the most common type of spina bifida, myelomeningocele affects as many as 1 out of every 800 infants. Many children diagnosed with myelomeningocele also have accompanying hydrocephalus, a buildup of fluid in the brain. The exact cause of myelomeningocele is unknown. However, low levels of folic acid during early pregnancy are thought to be related to this birth defect as folic acid is essential for proper brain and spinal cord development. Also, families with a child with myelomeningocele, or moms with a defect themselves, are at higher risk for having future children with the same condition.

Signs and Symptoms

The most obvious sign in newborn babies is a sac sticking out of the mid to lower back that contains elements of the spinal cord due to the lack of closure of the vertebrae and skin. Other symptoms can include:

  • partial or complete lack of sensation
  • weakness of the hips, legs or feet of a newborn
  • abnormal feet or legs, like clubfoot
  • hair at the back part of the pelvis
  • dimpling of the sacral area
  • midline skin lesions on the back like moles, pits or hemangiomas


Diagnosis

Although prenatal screening can be done to help diagnose the condition, myelomeningocele is sometimes seen after a child is born. A neurological examination may show that a child has inferior nerve-related function below the area of the defect. Diagnostic testing can also include x-rays, ultrasound, CT and MRI.

Treatment

After a baby is diagnosed with a myelomeningocele, surgical repair is the usual recommendation. It is ideal to have the procedure as quickly as possible as recommended by the neurosurgeon because most infants will require careful handling to reduce damage to the exposed spinal cord. Neurosurgical repair involves draining excess fluid and closing the opening in the spinal cord, which is necessary to protect the contents of the tissue inside. Children with accompanying hydrocephalus may also need surgery for placement of a ventriculo-peritoneal shunt to drain the extra fluid. A patient’s recovery from surgery and their residual weakness or paralysis depends on the amount of exposed neural tissue prior to surgery. Children with myelomeningocele can lead relatively normal lives given the proper treatment and clinical guidance. Recovery and physical capabilities depend on the number and severity of abnormalities and associated personal and environmental factors. Many children with the disorder have normal intelligence and can walk, usually with assistive devices. If learning problems develop, early educational intervention is helpful.

 

Content Courtesy of: Beaumon tHealth System

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