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 the 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 which have not closed, which is why they are exposed unprotected.

Doctors call a spine with unclosed vertebral arches "split spine" (spina bifida). In an unprotected exposure of spinal cord and spinal cord is a spina bifida aperta the speech. If the spinal cord and spine malformation is hidden under the protective skin, it is a spina bifida occulta.

If it comes through the gap only to the meninges, which has the formation of a bulging bag, which is filled with brain water, the result, doctors speak of a meningocele. On the other hand, a joint bulging of the spinal cord and meninges through the vertebral arches in the posterior direction produces a myelomeningocele.

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 to be a water-soluble vitamin that 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 appeared when the folic acid level in the body was normal. Instead, disorders in folate metabolism or the emergence of autoantibodies directed against the 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 sacrum or lumbar spine. The severity of these depends on the extent of the neural tube defect.

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

The onset of seizures such as epilepsy, decubitus ulcers on the back and the appearance of a so-called tethered cord (catenary 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 means of ultrasonography (ultrasound examination). Thus, 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 the myelomeningocele, so that those affected depend on the help of other people in their everyday lives.

Furthermore, shortening of the muscles occur and the patients suffer from complaints of the urinary tract and the bladder. 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 health problems.

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.

When should you go to the doctor?

If neurological deficits, paralysis or spasticity occur in the limbs, medical advice is needed. Myelomeningocele is a serious condition that must be promptly medically diagnosed and treated as appropriate. Therefore, first indications of a condition must be medically clarified. Persons who notice a decrease in motor skills or other symptoms that are rapidly getting worse, it's best to talk to the family doctor.

Further treatment is provided by a specialist in internal diseases or by an orthopedist. People who already have back problems should inform the responsible physician if the symptoms mentioned occur. The same applies to people who suffer from myelomeningocele from birth and notice an increase in symptoms. Pregnant, elderly and sick people speak best with a specialist. In addition to the general practitioner and specialist doctors mentioned, the myelomeningocele is also treated by physiotherapists and therapists. The therapy usually takes place in a specialist center for back disorders.

Treatment & Therapy

Successful treatment of a 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 operation, 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.

Outlook & Forecast

How a myelomeningocele affects those affected depends largely on how much the spine and spinal cord are malformed. Nevertheless, children with myelomeningocele can have a fairly long life expectancy and quality of life if they receive the comprehensive medical treatment they need. The cognitive development of the affected children is usually not affected by the sole presence of the disease.

Possible complications of the disease include inflammation of the affected spinal cord and the spinal cord. Renal inflammation and arthrosis can also occur in patients. In the event that the disease leads to a hydrocephalus and this remains untreated, the brain of the affected is exposed in the course of increasing pressure. This can result in very serious disorders and even acute mortal danger for patients.

Affected children with pronounced myelomeningocele often depend on professional medical care throughout their lives. However, it is particularly important to weigh the possible advantages and disadvantages of the different therapies. This is important so that the sick children can live as normally as possible and are also socially best integrated. It is therefore especially advisable that all necessary surgical treatments are completed before the children are enrolled.

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.

aftercare

Affected persons in most cases have only a few and very limited possibilities for direct follow-up in the case of a myelomeningocele. Therefore, the person should ideally seek early on a doctor to prevent further occurrence of complications or other complaints.

There can be no self-healing, so that treatment by a doctor is always necessary. In many cases, those affected by the myelomeningocele in their everyday lives depend on the help and support of their own family. Psychological support can also have a very positive effect on the further course of the disease and prevent depression or other mental upsets.

The disease itself is relieved in most cases by a minor surgery again. After such an intervention, the affected person should rest in any case and protect his body. This should be exempt from efforts or stressful and physical activities.

Likewise, even after the procedure regular checks and examinations by a doctor are very important and necessary. Further follow-up measures are not available to the patient in myelomeningocele. The disease itself does not reduce the life expectancy of the patient.

You can do that yourself

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

In addition, physiotherapeutic and occupational therapy measures must be initiated. Sick children often show bad 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 undergo regular medical examinations and ensure close monitoring during adulthood.

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