myelomeningocele treatment

myelomeningocele




Myelomeningocele, also called meningomyelocele, is a severe form of spina bifida. This leads to a splitting of the spine, emerge through the spinal cord parts.

What is a meningomyelocele?

Myelomeningocele is a congenital spinal cord malformation. It is caused by insufficient occlusion of the neural tube. Meningomyelocele, in addition to meningocele and rachisisis, is an expression of spina bifida aperta.

The skins of the spinal cord (meninges) are located together with the spinal cord outside the vertebral arch. This is recognizable as protrusion (zele) below the skin. The Latin term spina bifida means "open back". This refers to a malformation in the spinal cord and spine region.

Spinal cord and spine emerge from the neural tube during development of the human embryo. Therefore, spina bifida or meningomyelocele is counted among the neural tube defects that are among the most common congenital malformations. In Germany, there are about 500 births each year with a neural tube defect.
causes

A myelomeningocele is caused by a defect in the formation of the neural tube. This causes an inhibition malformation in the central nervous system. As a result of this defect, parts of the spinal cord (myelon) as well as the spinal cord skins pass through the vertebral arches that have not closed, which is why they are exposed unprotected.

Physicians call a spine with open-ended vertebral arches "split spine" (spina bifida). In the case of an unprotected exposure of the spinal cord skins and spinal cord, a spina bifida aperta is mentioned. If spinal cord and spinal cord malformation is hidden under the protective skin, it is a spina bifida occulta.

If the meninges escape through the cleft, resulting in the formation of a bulging sac that is filled with brain water, doctors speak of a meningocele. On the other hand, if the spinal cord and meninges protrude together through the vertebral arches in the posterior direction, a myelomeningocele develops.

What causes a myelomeningocele has not yet been established. In the discussion are the folic acid metabolism as well as genetic factors. Folic acid is understood as meaning a water-soluble vitamin which is one of the B vitamins. In earlier years, physicians believed that neural tube defects were caused by folic acid deficiency.

However, recent studies speak against it. Thus, myelomeningocele also occurred when the folic acid level in the body was normal. Instead, disorders in folate metabolism or the emergence of autoantibodies directed against folic acid receptors are believed to be conceivable.
Symptoms, complaints & signs

Myelomeningocele can cause many different complaints. Partial or complete sensory disturbances or paralyzes often appear on the sacral or lumbar spine. How strong these are depends on the extent of the neural tube defect.

The paralysis in turn cause a dysbalance of the skeletal muscle. As a result, shortening of the muscles and misalignments of the foot, knee and hip joints occur. Other possible complaints of meningomyelocele may include rectal and bladder emptying disorders, which often lead to urinary tract infections.




The onset of cramps such as epilepsy, decubital ulcers on the back and the appearance of a so-called tethered cord (cuffed spinal cord) may also occur. Another typical feature of a myelomeningocele is the expression of a hydrocephalus. Cerebrospinal fluid accumulates due to myelomeningocele, which causes an expansion of the cerebral ventricles.

This in turn leads to harmful pressure on the adjacent brain tissue and to impaired nerve tissue. Not infrequently affected children also suffer from orthopedic complaints such as scoliosis. It usually forms at the transition between the thoracic and lumbar spine or at the border between the lumbar spine and the sacrum. Furthermore, the formation of humps, disorders of respiratory function and lung infections occur.
Diagnosis & disease course

Spina bifida or myelomeningocele can often be detected during pregnancy by ultrasonography (ultrasound examination). So the doctor can usually easily identify the malformation due to the ultrasound. It is also possible to perform an alpha-1-fetoprotein test between the 16th and 18th week of gestation in the mother's blood or amniotic fluid.

Alpha-1 fetoprotein (AFP) is a protein produced by the fetus. If this is in an increased amount, this is considered an important indicator of a neural tube defect. The course of a myelomeningocele depends on its extent. If comprehensive medical care is provided, the affected children can certainly achieve a high life expectancy and a good quality of life.

However, in severe cases there is a risk of complications such as inflammation of the spinal cord and spinal cord skins. A particularly serious concern is an untreated hydrocephalus, which often causes severe disorders.
complications

The myelomeningocele significantly reduces the daily life of the person affected and reduces the quality of life. In most cases, patients suffer from disorders of sensitivity and paralysis. These can occur in different regions of the body and lead to significant restrictions on activities. Movement restrictions can also occur due to myelomeningocele, so that those affected depend on the help of other people in their everyday lives.

Furthermore, muscle shortening occurs and patients suffer from urinary tract and bladder complaints. Various infections can cause pain during urination. Not infrequently, sufferers also suffer from epilepsy and ulcers, which may limit life expectancy. The symptoms of myelomeningocele also continue to cause depression or mental illness.

The lungs may also be affected by this disease, leading to infections or breathing difficulties. As a rule, treatment of myelomeningocele must be carried out immediately after birth. In the process, secondary damage can be restricted in adulthood. Early and successful treatment usually does not cause complications and does not reduce the patient's life expectancy.
Treatment & Therapy

Successful treatment of myelomeningocele requires consistent collaboration of neurosurgeons, neurologists, paediatricians, urologists, orthopedists, occupational therapists, and physiotherapists.

One of the most important therapeutic measures is the closure of the open back. This must be done 24 to 48 hours after birth by surgery to prevent infection of the spinal cord and spinal cord skin. As part of the surgery, the surgeon shifts the spinal cord portions located in the myelomeningocele back to their correct position.


Skin, muscles and muscle sheaths cover the defect. If there is an additional water head, it is necessary to lay a shunt. In this way, the circulatory disturbance of cerebral water, together with increased intracranial pressure, can be compensated again.
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prevention

As a preventive measure against myelomeningocele, the intake of folic acid during pregnancy is recommended. This should reduce the risk of spina bifida by about 50 percent.
You can do that yourself

If a myelomeningocele has been detected, treatment must be initiated promptly. After an operation on the back, the parents of affected children should make sure that the surgical wound heals well and inform the doctor in case of doubt.

In addition, physiotherapeutic and occupational therapy measures must be initiated. Diseased children often show poor posture, which should be corrected immediately. To support the medical treatment, which represents a significant burden on the child, an individual diet can be performed. Animal products such as milk and natural yoghurt reduce back pain, while vegetables such as kale or rocket strengthens bones. Corn salad, spinach, brown rice, eggs and other foods rich in vitamin B and vitamin K should also be on the menu.

If there is also a hydrocephalus, a longer hospital stay is required. The child often needs therapeutic support. In puberty, the external abnormalities can be a major burden, so the parents should act supportive and understanding. Myelomeningocele is a long-standing illness that often needs to be treated for a lifetime. Parents should have the child regularly undergo a medical check-up to ensure close monitoring during adulthood.