Spina bifida is one of the most common spinal cord defects in children and can be dangerous for the child if neglected; it is treated in Turkey by elite doctors.
What is spina bifida?
Spina bifida is a congenital malformation characterized by the defective formation and closure of the spinal cord during the embryonic stage. The term is derived from Latin.
Spina bifida occurs when the neural tube fails to close completely during fetal life, resulting in the incomplete formation of the spinal cord and its closure to the structures that protect it (vertebrae), leaving part of the spinal cord and nerves exposed and uncovered by the spine and more at risk of external trauma.
Spinal cord nerve injury can cause serious and irreversible complications such as permanent disabilities, so it is important to protect the spinal nerves as soon as possible.
This deformity is most commonly seen in newborns, with one in 2,500 newborns globally affected.
This ratio varies according to geographical regions and different races, so this deformity is more common in whites than in blacks.
Spina bifida types
The three types of spina bifida are:
Myelomeningocele
The most serious and important type of spina bifida malformation is when a section of the spinal cord and nerves migrate through the opening into the child’s back.
It is seen as a cyst in the child’s back that contains cerebrospinal fluid, part of the spinal cord, and nerves.
Without adequate protection of the nerves and spinal cord, there is a high risk of neurological damage that can cause moderate to severe irreversible disabilities in children.
Meningocele
In this pattern, the membranes (meninges) and cerebrospinal fluid migrate through an opening in the child’s back, forming a cyst, but the spinal cord and spinal nerves do not migrate with them.
This pattern is less serious than myelomeningocele because the spinal cord and nerves do not migrate into the cyst.
Spina bifida occulta
The least serious and most common type of spina bifida is called latent or hidden spina bifida because it is often asymptomatic, rarely diagnosed in late childhood, and is often diagnosed incidentally when an image is taken for other reasons.
In this pattern, two or more vertebrae do not fuse, forming a small opening through which the meninges or spinal cord do not pass, and the skin on the child’s back is normal (no cyst).
This article will focus on myelomeningocele as it is the most important form of spina bifida.
Symptoms of spina bifida
Myelomeningocele, the most serious type of spina bifida, is often associated with symptoms of spinal cord nerve damage.
Depending on the location of the spinal cord incision and the degree of nerve damage that has occurred, spina bifida can cause a range of associated symptoms and complications, including:
- Difficulty walking in a baby
- Bladder and bowel control issues
- Foot and ankle deformities (club foot)
- Loss of sensation in the lower limb
- Some children develop breathing problems and paralysis of the lower limbs
Myelomeningocele is almost always accompanied by another malformation known as Arnold-Chiari malformation. This malformation causes fluid to accumulate in the brain and cause hydrocephalus, which can pose a serious risk to the child and may cause intellectual disability or even death.
Spina bifida can be asymptomatic in milder cases. Often, symptoms of spina bifida occulta are not apparent and are discovered incidentally when patients are scanned for other reasons.
Diagnosis of spina bifida
The abnormality can be detected during pregnancy or after birth.
Diagnosis during pregnancy
The diagnosis can be made through tests performed on the expectant mother to detect fetal neurological abnormalities, including:
Blood tests
The alpha-fetoprotein (AFP) titer is a protein produced by the fetus, and some of it crosses into the mother’s blood through the placenta. If the protein titer is high in the mother, this could indicate a malformation and neurological disease in the fetus, but a high titer is not necessarily associated with a neural tube defect.
Echocardiography
It is the optimal test for prenatal diagnosis of fetal cleft palate malformation, as the examiner can see the malformation during the second trimester (weeks 18 to 24) of pregnancy.
Amniotic fluid biopsy
Suppose the examining physician suspects that the baby has spina bifida. In that case, he will order an amniotic fluid biopsy for confirmation, but the difficult complications associated with the biopsy cannot be ignored, such as the possibility of miscarriage.
Postpartum Diagnosis
In some cases, the disease is not diagnosed until after the baby is born, as a hairy area or what looks like a birthmark is noticed on the baby’s back, so the doctor orders an X-ray of the spine or an MRI to see the spine and spinal cord and determine whether or not there is an injury.
Spina bifida Treatment in Turkey
In the case of myelomeningocele, surgery should be performed as soon as possible to cover the nerves and spinal cord and prevent nerve damage as much as possible, and currently, in advanced centers, surgery is performed on the fetus while in the mother’s womb before birth for fear of the effect of amniotic fluid on the fetus and the occurrence of spinal cord injuries.
Prenatal surgery
After the fetus is diagnosed with spina bifida, it is determined if the mother is a candidate for this type of surgery.
Doctors perform the surgery by making a horizontal incision in the mother’s abdomen and then using an Echo to obtain a clear view of the location of the fetus and determine the location of the cleavage. Then, an incision is made in the uterus to reach the fetal back, and then the defect is repaired by removing the cyst, if any, and closing the opening in the back with the surrounding tissues.
This complex surgery has risks for both the baby and the mother, as it can rupture the mother’s uterus and lead to a miscarriage of the fetus.
This operation requires the cooperation of doctors from different specialties and meticulous precision in centers equipped for it.
Postnatal surgery
The baby is born naturally at the normal time of birth and is then placed in the neonatal care unit, where the neurosurgeon monitors the baby after birth and assesses his/her condition.
Baby scans and an MRI are ordered to evaluate the spina bifida deformity. Surgery is often performed within the first or second day of birth when the opening in the baby’s back is closed with surrounding tissue and skin under general anesthesia.
After the surgery, the baby returns to the NICU for evaluation. Often, the baby stays in the hospital for about one to three weeks after the surgery for observation, and this procedure is considered less risky than the prenatal procedure.
One of the risks of this procedure is the risk of damage to the spinal cord nerves by amniotic fluid during pregnancy due to delaying surgical intervention until after birth.
Currently, spina bifida is treated in Turkey by a team of specialized doctors in surgical centers equipped with the latest technology.
Spina bifida surgery success rate
Clinical research suggests that prenatal cleft palate surgery has better outcomes for the baby but carries a higher risk for the mother.
By age 3, 42% of children who had prenatal surgery could walk independently, compared to 21% of children who had surgery after birth.
Complications of spina bifida and how to treat them
Pediatric spina bifida can cause complications that affect the child’s quality of life, such as partial or permanent physical disabilities as well as hydrocephalus, which can have serious intellectual consequences on the child’s life.
Hydrocephalus treatment
A buildup of cerebrospinal fluid in the head can cause increased pressure in the brain, leading to brain problems and disabilities that may be fatal. The child needs an operation to drain the fluid, in which the doctor places a shunt from the brain to the abdomen. If the shunt becomes blocked, it is replaced, or as the child grows, he may need a new shunt that is longer than the old one.
Physiotherapy
Physical therapy is an important means of helping spina bifida patients’ legs move so that they can live independently and not need medical devices such as wheelchairs.
The main goal of physical therapy is to prevent the development of muscle weakness in children. There is an important relationship between spina bifida and walking.
Treating bladder and bowel problems
Urinary incontinence is a common symptom in children with spina bifida. We can help these patients by giving medications that help relax the bladder to store more urine, or we can perform a bladder enlargement operation to ease the suffering of children and their families.
To treat constipation, laxatives can be given to help empty the bowels.
Causes of spina bifida
The underlying cause of this deformity is unknown, but there are causes or risk factors that may play a role in the development of this deformity in mothers of spina bifida babies:
Folic acid (vitamin B-9) deficiency
Folic acid deficiency can increase the risk of various neurological malformations, whether at the level of the brain or spine, as it is necessary for the development of the neural tube in the fetus.
Having a family history of neural tube defects
The risk of spina bifida in infants is higher if the same mother had a previous pregnancy with spina bifida.
Medications taken during pregnancy
Some medications taken by pregnant women may increase the risk of neural tube defects in their fetus, such as medications taken for epilepsy (carbamazepine and valproic acid), so pregnant women should consult their doctor about medications taken during pregnancy and replace these medications with medications that are safe for the fetus.
Pregnant women with uncontrolled diabetes
Pregnant women with untreated diabetes have an increased risk of fetal injury.
Obesity
The risk of neural tube defects is higher in women who are overweight (body mass index 30< BMI).
Preventing spina bifida
Folic acid is the main step in prevention, and it is important to avoid neural tube defects. Pregnant women are given a dose of 400 micrograms, and women who plan to have children should be made aware of the need to take the drug throughout pregnancy and eat a healthy diet containing folic acid.
Studies have shown that taking folic acid reduces the risk of congenital neural tube defects; mothers of spina bifida children who have a previous fetus with the disease or any of the neural tube-related diseases should take folic acid in double doses due to the increased risk of infection for the next fetus.
Certain foods, such as vegetables, fruits, egg yolks, rice, and cereal products, are rich in folic acid.
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