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Spina bifida

Spina bifida

Spina bifida is a neural tube defect that affects almost 3,000 pregnancies each year. Although its onset has begun to decline, spina bifida appears in 7 out of 10,000 pregnancies. This defect is manifested by the incomplete development of the spine and the membranes that cover it. The term 'spina bifida' comes from Latin and means the breaking or opening of the spine.

The spina bifida manifests itself at the end of the first month of pregnancy, when the two parts of the spine of the embryo cannot be united, leaving a free area. In some cases, the spine or other membranes may push through this area. This defect can be detected before the baby is born and can be treated immediately.

source photo: stanfordchildrens.org

Types of spina bifida

The causes of spina bifida are still unknown, some evidence suggests that some genes have not evolved, but in most cases there are no common connections. Severe fever during pregnancy can increase the chances of the baby developing with spina bifida. Pregnant women suffering from epilepsy and taking valproic acid to calm their seizures may have increased the risk of the baby having spina bifida.

"The term spina bifida means a malformation, a defect of the spine comprising several lesions of different severity and prognosis, which requires an adapted therapeutic but always surgical behavior.

According to the importance of this defect and of the associated injuries, we distinguish several lesion degrees:

- hidden spina bifida;
- dermal or epidermal fistulas;
- meningocele;
- myelomeningocele.

The occult spina bifida represents an elemental lesion, visible only on the x-ray, in the form of a partial defect of the posterior vertebral arch. In the absence of neurological symptomatology and associated skin signs, no therapeutic sanction is required.

N. R. Hidden spina bifida is the mild form of spina bifida (hidden means hidden). Most children with such a defect never have health problems in this regard, and spinal cord injury is often unaffected.

Dermal or epidermal fistula performs communication between the dural membrane and the skin, crossing a more or less discrete bone defect.

The fistula can be reduced to a fine communication in which we find fibrous elements, epidermoid or dermoid residues, or it is cloazoned, creating, as the case may be, a dermal or epidermal fistula, a true pilonidal cyst or a small "pocket" with LCR (cerebrospinal fluid). which corresponds to a minor stage of true meningocele.

These fistulas require surgical treatment, the problems being different depending on the existence or not of a complete communication with the dural sheet. The risk of treatment is recurrence, which occurs if the lesion has not been completely excised.

Meningocellus is a hernia of the dura mater and the arachnoid (membranes that cover the spinal cord) along a more or less widespread bone defect (depending on how many vertebrae are defective). It occurs at all levels of the spine, but especially in the lumbar-sacral region.

This table can reach a significant volume and is covered by the skin with a variable thickness. Often we find an adjacent lipoma (fat) that participates in the volume of the protrusion and, by amplifying it, can mask the exact importance of the underlying meningeal hernia.

The surgical treatment of such an injury is simple, the only difficulty being that it can result from the excessive volume of the pouch leading to considerable skin sacrifice or subsequently requiring an internal derivation for secondary hydrocephalus.

The myelomeningocele is a more complex malformation, which interests as well as the preceding one or more vertebrae, the meningeal sheaths and always the spinal cord and its roots. It is presented under different aspects:

- The myelomeningocele with an open medullary area has a round central area, red, bloody, with the superior central orifice, through which cerebrospinal fluid flows. Peripherally, there is a transition zone, covered with a thin skin, which makes the transition to the normal dermal area. The vertebral blades are missing and the medullary canal is opened posteriorly.

- Cystic myelomeningocele is the most common; the lumbar median tumor formation is large. It is covered with a thin skin and has liquid content; the membrane of the coating can be cracked easily which exposes to the loss of CSF and meningitis.

The meningeal sac contains besides the CSF, nerve threads or medullary substance that adhere in the form of plaques to the envelopes of the sac.

- The covered myelomeningocele is a rarer and less serious variant, being completely covered with normal skin.

In operable cases, the meningeal sac is excised, keeping the nerve elements (as far as possible) that are restored to the medullary canal; the meninges are closed by the suture; The closure of the canal is done with dura mater and muscular aponeurosis in the vicinity, after which a coating with healthy skin obtained by flap-plastic operations is attempted.

N. R. Myelomeningocele is the most severe form of spina bifida.

Neurological manifestations of myelomeningocele

- Partial or complete motor disorders of the lower limbs are common. Hip dislocation and cramped legs are common.

- The paralysis of the lower limbs is of a flaccid type, rarer in spastic form.

- There are areas without sensitivity, not only at the level of the limbs, but also in the spinal region and perineum.

- Major neurological disorders with partial or complete paralysis exist for anal sphincter and bladder.

- Hydrocephaly is associated in at least 80% of myelomeningocel cases.

The malformation consists of engaging the cerebellar tonsil in the medullary canal thus compressing the foramen magnum and preventing compression by leaking the CSF. By increasing the amount of cerebrospinal fluid, the brain is compressed and there are different degrees of delay.

If the child is born with such disorders (as a result of prenatal irritative spinal cord injury), the chances of recovery are practically nil. However, if these disorders occur after the surgery by cutting the nerve threads (inevitable in the applied surgical technique), there is a degree of recovery through medical gymnastics.

Regarding the aesthetic aspect and the postoperative scar, they differ from case to case, depending on the magnitude of the defect and the possibilities that the neurosurgeon had at the time of surgery. For the purpose of retouching them and possibly a reintervention (as recommended by the specialist), it would be advisable to contact the neurosurgeon. "

Dr. Dana Paduraru, Specialist in pediatric surgery - "Grigore Alexandrescu" Children's Emergency Clinic Hospital

Diagnostic

Parents who are expecting a child can find out if the baby has spina bifida following prenatal tests. The alpha-fetoprotein (AFP) test performed between weeks 16-18 of pregnancy, measures the amount of alpha-fetoprotein that the fetus produces. The doctor will find out the amount of it in the mother's blood. If there are high levels, usually the test is repeated because often the tests with very high levels are false. If the next test, the level will be as high, it will be researched again and thoroughly and the diagnosis will be made.

Folic acid deficiency has also been linked to spina bifida, and researchers believe that many cases could be avoided if pregnant women took a folic acid supplement every day during the first trimester of pregnancy.

Sources of folic acid can be found in:

- eggs;
- Orange juice;
- dark green vegetables.

But also an important source of folic acid can be found in the multivitamins recommended by the doctor during pregnancy.

Symptoms of spina bifida

Children born with the form of a hidden spina bifida do not show any signs or symptoms. Spinal cord does not penetrate the skin, but there may be a sign of birth, a blemish.

But in other forms of spina bifida, there are obvious signs. Children who are born with meningocele have a fluid-filled pouch, which is visible on the back. The sack is usually covered by the skin, and its size can vary from the size of a grape to the size of an orange.

Children who are born with myelomeningocele have a mass in the form of a pouch that emerges from the back, but may not be completely covered with skin. In some cases, the nerves of the spine are exposed, a child with hydrocephalus will have an enlarged head due to excess fluid pressing into the skull.

Treatment

Children with hidden spina bifida do not usually need treatment.

If a child suffers from manifest spina bifida, treatment depends on the form of spina bifida or the severity of the case.

A child with meningocele needs immediate attention and is usually recommended for surgery on the first or second day of life. During the first operation, the doctors push the spinal cord to the vertebrae and close the open cavity to prevent infection and to protect the spine.

A child with hydrocephalus will also need brain surgery. This will help lower the pressure in the brain by absorbing excess fluid.

There are also children who will need surgery to correct foot or hip problems.

Knowing the location of the void will help to perform the operation because this way the doctors know exactly which tools to use. Children with a hole at a higher level of the spine and with more extensive paralysis will need a wheelchair to move, while those with a hole located at a lower level will be able to use carriages and their movement is easier to achieve.

Child care with spina bifida

Parents with sick children with spina bifida will be assisted by a medical team, which will include doctors such as neurosurgeons, orthopedic surgeons, recovery specialists and general pediatricians, but also a nurse, a therapist and a social worker.

The purpose is to create for both the child and the parents an environment that is lighter and more common in which the abnormalities of the child do not influence the activities of a normal day of life. This environment will also help to create a psychic environment conducive to the respective family.

Tags Spina bifida Pregnancy fever