Spina Bifida

The severity of spina bifida depends on the type and the part of the spinal cord and nerves involved. Some children may have very mild or no symptoms, while severe forms can cause problems with movement, sensation, bowel and bladder control, and other neurological functions.

Types of Spina Bifida

1. Myelomeningocele

Myelomeningocele is the most serious type of spina bifida. In this condition, a fluid-filled sac comes through an opening in the baby's back. A damaged part of the spinal cord and nerves is present inside the sac.

Myelomeningocele may cause moderate to severe disability. The child may have difficulty moving the legs, loss of sensation in the legs or feet, and problems controlling bowel and bladder functions.

2. Meningocele

In meningocele, a fluid-filled sac comes through an opening in the baby's back, but the spinal cord is not present inside the sac. Nerve damage may be mild or may not be present.

Therefore, meningocele usually causes fewer disabilities than myelomeningocele.

3. Spina Bifida Occulta

Spina bifida occulta is the mildest type and is sometimes called hidden spina bifida. A small opening or defect is present in the spine, but there is usually no visible opening on the baby's back.

The spinal cord and nerves are usually normal. Many people do not know that they have spina bifida occulta until late childhood or adulthood. It usually does not cause disability.

Etiology and Risk Factors

The exact cause of spina bifida is not completely known. Genetic and environmental factors may contribute to its development. Certain conditions can increase the risk of neural tube defects in the baby.

1. Folate Deficiency: Folate is important for DNA and RNA synthesis and is essential for normal development of the baby. Folate is also involved in the formation of red and white blood cells.
2. Family History of Neural Tube Defects: A history of spina bifida or another neural tube defect in the parents can increase the risk. The risk may also increase if a previous pregnancy was affected by a neural tube defect.
3. Medications: Some medicines, particularly certain anti-seizure medicines, may increase the risk of neural tube defects. Medicines used during pregnancy should therefore be reviewed by a doctor.
4. Diabetes: Poorly controlled diabetes during pregnancy may increase the risk of spina bifida. Diabetes-related complications may affect fetal development.
5. Obesity: Maternal obesity is associated with an increased risk of neural tube defects.
6. Hyperthermia: High body temperature during the early weeks of pregnancy may increase the risk of neural tube defects.

Signs and Symptoms

Symptoms of Myelomeningocele

1. Open spinal canal, usually in the middle or lower part of the spine.
2. Membranes and spinal cord may come outside the back in an exposed or skin-covered sac.
3. Weak or paralysed leg muscles.
4. Seizures may occur.
5. Deformed feet.
6. Uneven hips.
7. Scoliosis or a curved spine.
8. Problems with bowel movement and bladder emptying.

Symptoms of Meningocele

1. Small opening in the back.
2. A sac may be visible at birth.
3. Membranes may come through the opening in the vertebrae into the sac.
4. The spinal cord usually develops normally.

Symptoms of Spina Bifida Occulta

1. Small opening or defect between the vertebrae.
2. No visible opening outside the body.
3. No fluid-filled sac outside the body.
4. Small birthmark or dimple on the back.
5. A small group of hair on the back.
6. An area of extra fat on the back.

Diagnosis

Severe cases of spina bifida can often be diagnosed during pregnancy or shortly after birth. Spina bifida occulta may remain undiagnosed throughout a person's lifetime because it can cause few or no symptoms.

1. AFP Test: A high level of alpha-fetoprotein (AFP), a protein produced by the developing baby, in the mother's blood during pregnancy may indicate a neural tube defect.
2. Ultrasound: Ultrasound examination during pregnancy is used to obtain images of the fetus and can help identify abnormalities of the spine.
3. Amniocentesis: A needle is inserted into the uterus to withdraw a small amount of amniotic fluid. The fluid may be tested for AFP and other markers.

Sometimes a hairy patch of skin or a dimple may be seen on the baby's back at birth. X-ray, MRI, or CT scan may be performed when more detailed imaging of the spine and surrounding structures is required.

In some cases, spina bifida remains undiagnosed until after birth because prenatal care was not received or the affected area was not clearly visible on routine ultrasound.

Complications

1. Mobility Problems: Problems with sensation and muscle control depend on the severity of spina bifida and the nerves involved. Severe cases may cause paralysis.
2. Orthopedic Complications: Myelomeningocele may be associated with scoliosis, abnormal growth, dislocated hips, bone and joint deformities, and muscle contractures.
3. Bowel and Bladder Problems: Nerves controlling the bowel and bladder may be affected, causing problems depending on the location and severity of the spinal defect.
4. Hydrocephalus: Hydrocephalus is the accumulation of excess fluid in the brain.
5. Shunt Malfunction: Some children with hydrocephalus require a shunt. Problems with the shunt can cause complications and require medical attention.
6. Chiari Malformation Type II: This condition involves downward displacement and elongation of parts of the brainstem and may cause breathing and swallowing problems. It commonly occurs in babies with myelomeningocele.
7. Meningitis: Infection involving the meninges can occur, particularly when there is an open spinal defect.
8. Tethered Spinal Cord: After surgical closure of the defect, the spinal cord may become attached to surrounding scar tissue. This condition is called tethered spinal cord.
9. Sleep Disorders: Sleep problems and episodes of apnea may occur in some children with spina bifida.
10. Skin Problems: Reduced sensation in the lower extremities can cause injuries or wounds to remain unnoticed.
11. Latex Allergy: People with spina bifida have an increased risk of developing latex allergy.

Management

Management depends on the type and severity of spina bifida. Severe spina bifida can cause permanent nerve damage, so treatment focuses on preventing further complications, improving mobility, maintaining bowel and bladder function, and supporting the child's development.

Children with severe spina bifida may need training for mobility and bowel and bladder management. Some children may require intermittent catheterization for urinary drainage. They may also require repeated surgeries and assistive devices such as braces, crutches, or wheelchairs.

Nursing Management

1. Risk for Infection

The risk of infection is increased when the meningeal sac is open or not covered by normal skin, particularly because of its proximity to the rectal opening.

1. The nurse should take measures to prevent infection.
2. The infant should be positioned to prevent contamination by urine and stool.
3. The defect should be cleaned carefully with sterile saline as prescribed and covered with sterile dressing.
4. Similar sterile care should be provided to the operative site after surgery.
5. Contamination of the lesion with stool should be prevented.
6. Good peripheral and general hygiene should be maintained.
7. Antibiotics or urinary tract antiseptics should be administered as prescribed.
8. Adequate fluid intake should be maintained according to the child's condition and medical advice.

2. Risk for Trauma

The thin membranous sac covering the spinal cord is delicate and can be easily injured.

1. The nurse should protect the delicate spinal lesion from trauma.
2. The infant should be handled carefully.
3. The infant should be placed in a prone or side-lying position as prescribed.
4. Protective devices should be used around the sac when required.
5. Routine nursing activities such as feeding and comforting should be modified to protect the lesion.

3. Risk for Impaired Skin Integrity

Reduced sensation in the lower extremities, urinary and bowel incontinence, and restricted movement can increase the risk of skin breakdown.

1. The nurse should prevent skin damage caused by continuous dribbling of urine and stool.
2. The perianal area should be kept clean and dry.
3. Diapers should be avoided when they may contaminate the spinal defect, until the defect has been repaired or adequately healed.
4. Soiled diapers should be changed promptly.
5. Passive range-of-motion exercises should be performed as indicated.
6. Muscle stretching should be carried out when indicated.
7. The hips should be maintained in slight to moderate abduction and the feet in a neutral position.

4. Risk for Impaired Parenting

Spina bifida is a chronic condition and parents may need considerable education and emotional support. Parents should be encouraged to participate in the child's care.

1. The nurse should educate parents about home care, including positioning, feeding, skin care, and range-of-motion exercises.
2. Parents should be taught appropriate catheterization techniques when catheterization is required.
3. The family should be taught to recognize signs of complications and seek medical assistance when required.
4. Parents should be encouraged to stimulate and support the infant in achieving age-appropriate developmental tasks within the limits caused by the disability.

Conclusion

Spina bifida is a neural tube defect that develops before birth and can range from a mild condition such as spina bifida occulta to a severe condition such as myelomeningocele. Early diagnosis, appropriate surgical management, rehabilitation, prevention of complications, and proper nursing care are important for improving the child's quality of life.

Nurses have an important role in protecting the spinal lesion, preventing infection and skin damage, supporting mobility and bowel and bladder care, educating parents, and helping the child achieve maximum possible independence.