Congenital disorders of the spine
Congenital deformities of the spine are caused by anomalous vertebral development in the embryo. Minor malformations of the spine are seldom apparent and often are identified only on routine chest films. The more severe congenital malformations that result in progressive scoliosis is even less common than are idiopathic scolioses. Congenital anomalies of the spine may be simple and benign, causing no spinal deformity, or they may be complex, producing severe spinal deformity or even cor pulmonale or paraplegia.
Spina bifida (cleft spine) is a birth defect affecting the spinal column. Spina bifida progresses from a cleft or split like opening, in the back part of the backbones (the spinal vertebrae). In more severe cases, it involves the spinal cord. Spina bifida is the most common of a group of birth defects known as neural tube defects, which affect the central nervous system (brain and spinal cord).
Types of Spina bifida
There are 3 types of spina bifida.
- Spina bifida occulta: "Occulta" means hidden, and the defect is not visible. Spina bifida occulta is rarely linked with complications or symptoms. Spina bifida occulta is usually discovered accidentally when the person has an x-ray or MRI for some other reason. The prevalence of occulta is not known, but it is probably the most common type of spina bifida.
- Meningocele: The membrane that surrounds the spinal cord may enlarge, creating a lump or "cyst." This is often invisible through the skin and causes no problems. If the spinal canal is cleft, or "bifid," the cyst may expand and come to the surface. In such cases, since the cyst does not enclose the spinal cord, the cord is not exposed. The cyst varies in size, but it can almost always be removed surgically if necessary, leaving no permanent disability. This is an uncommon type of spina bifida.
- Spina bifida cystica (myelomeningocele): This is the most complex and severe form of spina bifida. Spina bifida cystica usually involves neurological problems that can be very serious or even fatal. A section of the spinal cord and the nerves that stem from the cord are exposed and visible on the outside of the body. Or, if there is a cyst, it encloses part of the cord and the nerves. This condition, which was documented 4000 years ago, accounts for 94% of cases of true spina bifida.
Causes of Spina bifida
The cause of myelomeningocele is unknown. However, low levels of folic acid in a woman's body before and during early pregnancy are thought to play a part in this type of birth defect. The vitamin folic acid (or folate) is important for brain and spinal cord development.
Also, if a child is born with myelomeningocele, future children in that family have a higher risk than the general population. However, in many cases, there is no family connection.
Some theorize that a virus make play a role, since there is a higher rate of this condition in children born in the early winter months. Research also indicates possible environmental factors such as radiation.
Symptoms of Spina bifida
Symptoms of Spina bifida include:
- Loss of bladder or bowel control
- Partial or complete lack of sensation
- Partial or complete paralysis of the legs
- Weakness of the hips, legs, or feet of a newborn
- Other symptoms may include:
- Abnormal feet or legs, such as clubfoot
- Build up of fluid inside the skull (hydrocephalus)
- Hair at the back part of the pelvis called the sacral area
- Dimpling of the sacral area
Treatments for Spina bifida
- Genetic counseling may be recommended. In some cases where severe defect is detected early in the pregnancy, a therapeutic abortion may be considered.
- After birth, Spina bifida surgery to repair the defect is usually recommended at an early age. Before surgery, the infant must be handled carefully to reduce damage to the exposed spinal cord. This may include special care and positioning, protective devices, and changes in the methods of handling, feeding, and bathing.
- Children who also have hydrocephalus may need a ventricular peritoneal shunt placed. This will help drain the extra fluid.
- Antibiotics may be used to treat or prevent infections such as meningitis or urinary tract infections.
- Most children with Spina bifida will require lifelong treatment for problems that result from damage to the spinal cord and spinal nerves.
- Gentle downward pressure over the bladder may help drain the bladder. In severe cases, drainage tubes, called catheters, may be needed. Bowel training programs and a high fiber diet may improve bowel function.
- Orthopedic or physical therapy may be needed to treat musculoskeletal symptoms. Braces may be needed for muscle and joint problems.
- Spina bifida surgery is performed in severe cases.
- Neurological losses are treated according to the type and severity of function loss.
- Follow-up examinations generally continue throughout the child's life. These are done to check the child's developmental level and to treat any intellectual, neurological, or physical problems.
- Visiting nurses, social services, support groups, and local agencies can provide emotional support and assist with the care of a child with a myelomeningocele who has significant problems or limitations.
- Difficult delivery with problems resulting from a traumatic birth, including cerebral palsy and decreased oxygen to the brain
- Frequent urinary tract infections
- Loss of bowel or bladder control
- Permanent weakness or paralysis of legs