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Congenital Brain Abnormalities

DEFINITIONS
Brain abnormalities can occur when the brain is being formed and after the fully formed brain.
Several brain disorders can be known before the baby is born, that is through an ultrasound examination and examination of amniotic fluid.

ANENSEFALUS

Anensefalus is a state where most of the bones of the skull and brain is not formed.

Anensefalus is a neural tube disorder (a disorder that occurs early in fetal development that causes damage to the tissue forming the brain and spinal cord).
Anensefalus occurs when the neural tube fails to close the top, but the exact cause is unknown.
Research shows the possibility anensefalus associated with toxins in the environment are also low levels of folic acid in the blood.

Anensefalus found in the 3.6 to 4.6 in 10,000 newborns.
Anensefalus risk factors are:
- History anensefalus in previous pregnancies
- Low levels of folic acid.

The risk of anensefalus could be reduced by increasing intake of folic acid at least 3 months before pregnancy and during the first month of pregnancy.

Symptoms include:
Mother: polyhydramnios (amniotic fluid in the uterus too much)
Baby
- Do not have the skull bone
- Do not have a brain (cerebral hemispheres and cerebellum)
- Abnormalities in the facial image
- Heart abnormalities.

Inspection is usually done is:
- Levels of fatty acids in the serum of pregnant women
- Amniocentesis (to determine the existence of elevated levels of alpha-fetoprotein)
- Alpha-fetoprotein levels increased (indicating the existence of neural tube defects)
- Levels of estriol in maternal urine
- Ultrasound.

Babies who suffer anensefalus will not last, they were born in a state of dying or will die within a few days after birth.

Microcephaly

Microcephaly is a condition where the size of the head (top of head circumference) smaller than average head size in infants by age and sex.
It said if the size is smaller head circumference less than 42 cm or smaller than three standard deviations below the average.

Microcephaly is often occurred due to failure of brain growth at a normal speed. Various conditions and diseases that affect brain growth can cause Microcephaly.
Microcephaly is often associated with mental retardation.
Microcephaly can occur after an infection that causes damage to the brain in very young infants (eg meningitis and Meningoencephalitis).

The main causes:
Down Syndrome
Cri du chat syndrome
Seckel syndrome
Rubinstein-Taybi syndrome
Trisomy 13
Trisomy 18
Syndrome Smith-Lemli-Opitz
Cornelia de Lange Syndrome

Secondary Cause:
Phenylketonuria in women who are not controlled
Methyl mercury poisoning
Congenital Rubella
Congenital Toxoplasmosis
Congenital cytomegalovirus
Drug abuse by pregnant women
Nutrient deficiency (malnutrition).

Microcephaly Treatment depends on the cause.
Babies who suffer Microcephaly is often able to survive but tend to have mental retardation, seizure disorders and muscle coordination.

ENSEFALOKEL

Ensefalokel is a neural tube disorder characterized by the protrusion meningens (lining of the brain) and brain shaped like a pouch through a hole in the skull.
Ensefalokel caused by failure of neural tube closure during fetal development.

Symptoms include:
- Hydrocephalus
- Paralysis of all four limbs (spastic kuadriplegia)
- Developmental delay
- Microcephaly
- Impaired vision
- Mental and growth retardation
- Ataxia
- Seizures.
Some children have normal intelligence.
Ensefalokel often accompanied by craniofacial abnormalities or other brain disorders.

Usually performed surgery to restore brain tissue protruding into the bone of the skull, remove the bag and repair craniofacial abnormalities that occur.
To hydrocephalus shunt may need to be made a. Other treatment is symptomatic and supportive.

The prognosis depends on the affected brain tissue, the location of the bag and brain abnormalities that accompany it.

PORENSEFALUS

Porensefalus is a condition where the cerebral hemispheres was found a cyst or cavity abnormal.

Porensefalus is the result of brain damage and is usually associated with abnormalities of brain function. But some children who suffer porensefalus have normal intelligence.

HIDRANENSEFALUS

Hidranensefalus is a condition in which the cerebral hemispheres are absent and replaced by sacs containing cerebrospinal fluid.

When born, the baby looked normal. Head size and spontaneous reflexes (eg sucking reflex, swallowing, crying and moving her arms and legs) appear normal. But a few weeks later, usually the baby becomes fussy and muscle tension increases.
After a few months old, seizures and hydrocephalus may occur. Other symptoms are:
- Impaired vision
- Stunted growth
- Deaf
- Blind
- Paralysis (kuadriplegi spastis)
- Disturbance of intelligence.

Porensefalus Hidranensefalus is an extreme form and can be caused by infection or injury of blood vessels that occurs during pregnancy has reached the age of 12 weeks.

Diagnosis may be delayed for several months because early behavior is relatively normal. Some infants show abnormalities at birth, namely in the form of seizures, mioklonus (spasm or twitch muscle or group of muscles) and respiratory disorders.

There is no definitive treatment for hidranensefalus. Treatment is symptomatic and supportive. Hidranensefalus prognosis is bad, babies usually die before age 1 year.
Hydrocephalus can be overcome by making the shunt.

Hydrocephalus

Hydrocephalus is excessive accumulation of cerebrospinal fluid in the brain.

Cerebrospinal fluid is made in the brain and is usually circulated to all parts of the brain, lining of the brain and spinal canal, and then absorbed into the circulatory system.
If there is interference with the circulation and absorption of cerebrospinal fluid, or if too much fluid is formed, then the volume of fluid in the brain are higher than normal. Fluid retention causing pressure on the brain and thus forcing the brain to stimulate the bone of the skull or brain tissue damage.

Symptoms vary, depending on the cause of the blockage of cerebrospinal fluid flow and the extent of brain tissue damage due to hydrocephalus.
In infants, fluid builds up in the central nervous system and lead to the fontanel prominent and enlarged heads. Head can be enlarged because the skull plate is not fully closed. However, if the skull bones have closed (around the age of 5 years), the skull can not expand anymore.

In children, the risk of hydrocephalus was found at:
Congenital abnormalities
Tumors of the central nervous system
Infection in the womb
Central nervous system infections in infants or children (eg meningitis or encephalitis)
Injury to the birth process
Injury before or after birth (eg, subarachnoid hemorrhage).
Mielomeningokel is a disease where there is incomplete closure yan g at the spinal column and is closely related to hydrocephalus.

In children greater, the risk of hydrocephalus include:
History of congenital abnormalities
Space-occupying lesions or brain or spinal cord tumor
Central nervous system infections
Brain hemorrhage
Trauma.

Early symptoms in infants:
- Head enlarge
- The crown stand out with or without enlargement of the head
- Sutura separately.

Symptoms in advanced hydrocephalus:
- Fussy, unable to resist emotion
- Muscle cramps.

Symptoms continued:
- Decreased mental function
- Impaired growth
- Decreased movement
- The movement became slow or obstructed
- Do not want to eat / nurse
- Weakness, sleep continues
- Beser
- Crying with high tone, loud and short
- Disorders of growth.

Symptoms in older infants and children varies, depending on the extent of damage due to the emphasis on the brain. Symptoms similar to symptoms of advanced hydrocephalus in children or can be:
- Headache
- Vomiting
- Impaired vision
- Squint
- Uncontrolled eye movement
- Loss of coordination
- Her steps are not strictly
- Mental disorders (eg psychosis).

Gently knock the skull bone with the tip of your fingers will show abnormal sound due to thinning and separation of skull bones.
The veins in the skull seemed to widen. Enlarged head circumference, or head enlarged only in certain parts (most often in the forehead). The eyes looked depressed (sunset picture, where the whites of the eyes visible above the iris).
Neurological examination showed focal neurological deficits (impaired nerve function are local in character) and abnormal reflexes.

Inspection is usually done:
Transilluminates head may reveal abnormal fluid that accumulated in different parts of the head
CT scan of the head
Lumbar puncture and cerebrospinal fluid examination
X-ray head (show the existence of thinning and separation of skull bones)
Brain scans with radioisotopes may reveal abnormalities in the cerebrospinal fluid path
Arteriografi brain blood vessels.
Ekoensefalogram (ultrasound of the brain, indicate a widening of the ventricles due to hydrocephalus or hemorrhage intraventrikuler).

The aim of treatment is to minimize or prevent brain damage by improving the flow of cerebrospinal fluid.
Surgery is the primary treatment of hydrocephalus. If possible, the source of blockage is removed. If not possible, then made a cerebrospinal fluid shunt so no need to pass through the blocked area.
Shunting into the outer regions of the brain could be done into the right atrium of heart or the peritoneum.

Cautery or removal of part ventricles that produce cerebrospinal fluid could theoretically reduce the amount of cerebrospinal fluid is formed.

If there are signs of infection, given antibiotics immediately.
If there is a severe infection, the possibility of a shunt should be removed.

Further investigation should be conducted regularly throughout life patients to assess the level of child development and to treat any intellectual disorders, neurological and physical.

If untreated, mortality reaches 50-60%. Children who survive will have abnormalities of the intellectual, physical and nervous.
Prognosis for treated hydrocephalus varies, depending on the cause. If the child survived for 1 year, then almost one third have normal intellectual function but remain nervous disorders.
Hydrocephalus is not caused by infection has the best prognosis, whereas if the cause is a tumor then the worst prognosis.

Arnold-Chiari syndrome

Arnold-Chiari syndrome is a disorder in the formation of the lower brain (brain stem).
This syndrome is often associated with hydrocephalus.

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