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Common name for myelomeningocele, it is a congenital disorder (birth defect) where the backbone and spinal canal do not close before birth. This allows the spinal cord and the covering membranes to protrude out of the child's back. Myelomeningocele is one of the most common birth defects of the brain and spinal cord (central nervous system).

Spina bifida includes any congenital defect involving insufficient closure of the spine. Myelomeningocele accounts for about 75% of all cases of spina bifida and may affect as many as 1 out of every 800 infants. The rest of the cases are most commonly spina bifida occulta (where the bones of the spine do not close, the spinal cord and meninges remain in place, and skin usually covers the defect) and meningoceles (where the meninges protrude through the vertebral defect but the spinal cord remains in place).

The cause of myelomeningocele is unknown. However, folic acid deficiency is thought to play a part in neural tube defects such as myelomeningocele. The disorder appears to be more common in families; if a child is born with myelomeningocele, subsequent children in that family have a higher risk than the general population. A viral cause or trigger has been theorized because there is a higher incidence of the defect in children born in the early winter months. Research also indicates possible environmental factors such as radiation.

Protrusion of the spinal cord and meninges damages the spinal cord and nerve roots, causing a decrease or lack of function of body areas controlled at or below the defect. Symptoms are related to the anatomic level of the defect. Most defects occur in the lower lumbar or sacral areas of the back (the lowest areas of the back) because this area is normally the last part of the spine to close. Symptoms include partial or complete paralysis of the legs, with corresponding partial or complete lack of sensation, and may include loss of bladder or bowel control. The exposed spinal cord is susceptible to infection (meningitis).

Other congenital disorders may also be present in the child, usually disorders of the spinal cord or the musculoskeletal system such as hydrocephalus (which may affect as many as 90% of children with myelomeningocele), syringomyelia, hip dislocation, or similar disorders.

Symptoms

  • Visible sac-like protrusion on the mid to lower back of a newborn not translucent when a light is shone from behind the sac
  • Weakness of the hips, legs, or feet of a newborn
  • Spina bifida occulta may be indicated by a tuft of hair at the sacral area (back part of the pelvis) dimpling of the sacrum

    Treatment The goals of initial treatment are to reduce the amount of neurologic damage caused by the defect, to minimize complications such as infection, and to aid the family in coping with the disorder. Follow-up neurologic testing as the child gets older helps to guide rehabilitation.

    Early surgical repair of the defect is usually recommended, although surgical repair may be performed later to allow the infant to tolerate the procedure better. Before surgery, the infant must be handled carefully to reduce damage to the exposed spinal cord. This may include positioning, protective devices, and modifications in the methods of handling, feeding, bathing, and caring for the infant. Antibiotics may be used aggressively to treat or prevent meningitis, urinary tract infections, or other infections.

    Gentle downward pressure over the bladder may aid in drainage of the bladder. In severe cases, indwelling or intermittent bladder drainage catheters may be needed. A diet high in fiber, and bowel training programs, may improve bowel function. Orthopedic intervention or physical therapy may be needed to treat musculoskeletal symptoms. Other neurologic losses are treated according to the type and extent of the loss of function. The goal of these interventions is to minimize future disability and maximize functioning.

    The defect of myelomeningocele can usually be surgically corrected. With treatment, length of life is not severely affected. Neurologic damage is often irreversible.

  • Spi"na bif"i*da (?). Med. [L., cleft spine.]

    A congenital malformation in which the spinal column is cleft at its lower portion, and the membranes of the spinal cord project as an elastic swelling from the gap thus formed.

     

    © Webster 1913.

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