The brain is an organ that greatly influences a child’s overall growth. Unfortunately, some babies may be born with defects in this crucial part.Hydranencephalyis a type of birth defect that affects babies.
Hydranencephalyincurable. It is not uncommon for babies born with this condition to die in the womb or within a few months of birth.
Definition of Hydranencephaly
Hydranencephalyis a rare birth defect that can cause central nervous system disorders. When this happens, the frontal hemisphere or cerebrum of the baby’s brain is missing. Instead, cerebrospinal fluid that normally cushions the brain fills the space.
The condition affects male and female babies at equal rates. Some babies may be at higher risk if one of their parents carries the gene thought to cause the disorder.
Baby withhydranencephalyeventually develop an enlarged head and other serious symptoms. This is usually fatal before the baby is born or soon after.
Symptoms of Hydranencephaly
Some babies are born withhydranencephalydoes not show any obvious symptoms. In obvious cases, the baby may show the following signs:
- The baby’s head appears larger than usual
- Baby’s movements are limited or jerky
- Failure to grow at normal rate
- Convulsions
- Difficulty seeing
- Excessive muscle reflexes
- Increased muscle tone
- Intellectual disability
- Poor body temperature regulation
- Respiratory and digestive problems
- Stiff arms and legs
- Baby is always fussy
- Loss of appetite
Causes of Hydranencephaly
There are differences of opinion among scientists regarding the cause ofhydranencephalyin infants. Some scientists suspect that this condition is a disorder passed from parent to child.
While others suspecthydranencephalyoccurs due to a blockage of the carotid artery where it enters the head. However, it is not clear how or why the blockage occurs.
Apart from that, there is also another theory which states thathydranencephalyoccurs when the baby’s central nervous system is injured at a very early stage of development, or does not develop properly.
Other possible causes include:
- Uterine infection in early pregnancy
- Maternal exposure to toxic substances
- Blood circulation problems in the fetus
- Other circulation problems with babies
- Infection in the womb that causes the baby’s brain tissue to die
- Lack of oxygen to the baby’s brain
- Genetic syndrome or gene mutation
- Twin-to-twin transfusion syndrome
- Fowler’s syndrome, Trisomy 13 , and other rare syndromes
While the exact causehydranencephalyunknown, it affects male and female babies at equal rates. Increased risk of developinghydranencephalyalso not associated with a particular race or ethnicity.
Frequency of Occurrence
Hydranencephalyis a rare birth defect. Scientists estimate that it occurs in about 1 in 5,000 pregnancies. Some even estimate that it occurs in only about 1 in 10,000 pregnancies.
Quoting fromTexas Children’s Hospital,This condition can occur in 1 in every 250,000 pregnancies in the United States.
Risk Factors
HydranencephalyIt is believed to be the result of impaired blood supply to the fetal brain, affecting the normal development of the central nervous system. Although the exact cause is unknown, factors suspected to trigger this condition include:
- Intrauterine infection
- A blockage in the carotid artery, a blood vessel that carries blood to the brain.
- Twin-twin transfusion syndrome (TTTS), a condition in which identical twins share one placenta and one twin receives too much blood and the other too little
- Other circulation problems
- Exposure to environmental toxins
- Genetics
Diagnosis
Doctors can diagnosehydranencephalyat different times in each baby. Here are some methods that can be done to diagnose this disease:
USG
In some cases,hydranencephalycan be diagnosed while the baby is still in the womb through an ultrasound scan.ultrasounduses sound waves to create images of the developing fetus while it is still in the womb (uterus). This test can show the possibility of a missing hemisphere of the fetus’ brain.
If the ultrasound shows the possibility ofhydranencephalyIn your baby, your healthcare provider may perform an MRI procedure to get more detailed images.
MRI After Baby is Born
Sometimeshydranencephalyundetected during pregnancy. Symptoms may not appear until after the baby is born. It can take weeks or even months to establish a diagnosis because some babies with this condition often look and act normal at first.
A healthcare provider can then make a diagnosis based on the baby’s symptoms and a brain MRI. An MRI can show the difference betweenhydranencephalyand other conditions such as hydrocephalus andholoprosencephaly.
Transillumination
One of the common diagnostic tests is transillumination. Take it easy.Parents , this procedure will not be painful for the baby. During the procedure, the doctor will shine a light on the underside of the child’s head to see if there is fluid under the skin. This test can often reveal whether there is abnormal brain formation.
Angiogram
Your baby’s doctor may also use a special X-ray to take pictures of your baby’s blood vessels using dye. This is called an angiogram. This helps reveal abnormalities with circulation.
CT Scan
A computerized tomography scan may also be performed by your doctor. This X-ray imaging test is called a CT scan. It can give your doctor a detailed, layered view of your brain.
Amniocentesis
Doctors can also take steps to get a diagnosis by doing genetic testing. One that may be recommended is amniocentesis. This is useful for checking for defects in the baby’s chromosomes and to help confirm the diagnosis.
Other Checks
Your pediatrician will also need to perform a thorough examination to get a more detailed picture of your child’s condition. This examination includes:
- Conducting clinical evaluations
- Reviewing the newborn’s medical history
- Identifying the physical characteristics of a baby
How to Treat Hydranencephaly
In fact, until now there is no specific medicine that can cure it.hydranencephaly. The treatment carried out will focus on managing the symptoms that arise related to this condition.
To get the right treatment, parents should follow the treatment plan recommended by their doctor. Medications and therapies should also be discussed with the doctor to avoid the problem of over-medicating the child. Treatment will vary from child to child and will be tailored to manage the individual’s specific symptoms.
For example, to help remove excess cerebrospinal fluid from the brain, a surgeon may placeShunt, or one-way valve, inside the skull.Shuntattached to a catheter, a flexible tube.
The catheter will be fed into the abdominal cavity, where fluid is transferred from the brain. This procedure can help relieve the painful pressure inside your child’s head. It can also extend your child’s life expectancy.
For symptoms such as seizures can be treated with drugs, including anticonvulsants, and other therapies. Diet and calming techniques can sometimes also reduce the intensity of seizures in infants.
Calming and relaxation strategies can also help reduce irritability or a fussy baby. To help treat constipation, you may be recommended a baby-specific laxative .
It is important that treatment for all symptoms and complications is coordinated by your child’s doctor. This will help to avoidovermedicationor unwanted side effects of some medications and procedures.
Your healthcare provider may also recommend the following:
- Nutritional support.
- Physical therapy to improve muscle symptoms.
- Tracheostomy or mechanical ventilation for respiratory failure.
How to Prevent Hydranencephaly
Scientists have not yet discovered the exact cause ofhydranencephalyso they also do not know how to prevent it. However, in some cases that occur in babies, this is often associated with exposure to toxins during pregnancy, such as cocaine, cigarette content, and sodiumvalproate.
To prevent it, pregnant women should avoid these toxins or environments that potentially contain them as much as possible. Pregnant women are also advised not to smoke because it can have a negative impact on the baby in the womb.
If this condition attacks a child, there is nothing that can be done to prevent it. The chances of survival for children withhydranencephalyvery bad.
Most babies die before they are born or before they are one year old. Very rarely, a child withhydranencephalycan survive for years with intensive supportive care.
If you or your closest relative has a child diagnosed withhydranencephalyIt takes extra learning to understand how severe the disorder is. You may also benefit from counseling and support groups to connect with other families facing similar conditions.
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As much as possible, maintain a healthy lifestyle for pregnant mothers for the sake of their own health and that of their baby. Hopefully this article is useful.
Republished with permission from theAsianParent Indonesia
