Dandy Walker Syndromeis a disorder that affects brain development , especially the development of the cerebellum, which is the part of the brain that coordinates movement.
ParentsYou might be wondering what are the signs and symptoms if your little one has this syndrome?
In most individuals with malformationsDandy Walker, signs and symptoms caused by abnormal brain development will appear at birth or develop in the first year of life.
To learnDandy Walker SyndromeFor more details, please continue reading this article, OK?Parents.
What is Dandy Walker Syndrome?
Dandy Walker Syndrome(DWS) is a congenital condition (which occurs before birth) in which the cerebellum does not develop normally. The cerebellum or what is termedcerebellumis an area at the back of the brain that controls movement and balance.
With DWS, parts of the child’s cerebellum may never develop or may be very small. Other parts of the cerebellum may become filled with fluid or even develop cysts.
Health pageNation Wide Childrenreported that, in most cases, the accumulation of fluid inside the skull causes the head to become larger than it should be.
Still citing the same source, DWS can occur by itself, or together with other types of birth defects .
Long-term survival rates for babies with DWS are known to be good. If DWS occurs with other genetic conditions, outcomes can vary. Early treatment to support physical and mental development will increase a child’s chances of having the best quality of life for their condition.
Types of Dandy Walker Syndrome
Disabled
This is the most severe type of DWS . It is usually seen as part of a group of congenital birth defects. With MalformationsDandy Walker, some parts of the child’s cerebellum will be absent.
Other parts of the brain are enlarged with fluid-filled cysts. Most babies have hydrocephalus and macrocephaly and require surgery to placeshunt(a hollow tube) into the brain. This will help drain the extra fluid. These babies are at high risk for long-term neurological and developmental problems.
Variants
This is the mildest form of DWS. This type has a variety of symptoms. Babies with the DWS VariantDandy Walkermay have less fluid buildup in their brains so they will have less damage to the structures in the cerebellum.
Babies with this type of DWS are less likely to need surgery to remove extra fluid from their brains. However, many may still require surgery.
Some children with this type of DWS will continue to have normal intelligence and physical development. Others may require multiple surgeries to drain fluid from the skull. They will also require lifelong physical therapy.
Dandy Walker Syndrome Symptoms
Regarding what are the signs or characteristics of DWS, the pageCleveland Clinicexplains, symptoms in babies with this syndrome include:
- Motor achievements are slower than expected (developmental delay).
- Disproportionately large skull.
- Low muscle tone (hypotonia).
- Stiff muscles (spasticity).
Symptoms in older children include:
- Vomiting, convulsions, and irritability — signs of increased pressure on the brain.
- Uncoordinated movements, unsteadiness or jerky eye movements — signs of conditions with a cerebellum.
Other signs include:
- A bulge or swelling at the back of the skull.
- A condition involving the nerves that control the neck, face, and eyes.
- Unusual breathing rhythm.
- Intellectual disability.
- Symptoms of hydrocephalus.
Nation Wide Childrens adds that sometimes DWS is not discovered until early childhood. In these cases, DWS symptoms can occur suddenly or over time.
Reason
So what exactly causes Dandy-Walker syndrome? The Cleveland Clinic health page explains that Dandy-Walker occurs when there is a problem with the development of the baby’s cerebellum in the womb. In some cases, a genetic mutation (change) can cause the condition.
Some people withDandy Walkerhave a chromosomal condition, such as extra or missing parts of a chromosome. Chromosomes are packages of DNA that carry genes. SyndromeDandy Walkercan also occur as part of a genetic disorder that includes several birth defects (congenital conditions).
Another potential cause ofDandy Walkermay include:
- A particular virus that is transmitted from a birthing parent to the baby during pregnancy.
- Exposure to certain toxins or drugs during pregnancy.
- Parents who give birth suffer from diabetes .
Dandy Walker Syndrome Occurrence Frequency
Medline reports that this condition is estimated to affect 1 in 10,000 to 30,000 newborns.
Diagnosis
HowDandy Walker Syndromediagnosed? Quoting the pageNationwide Children’s, various prenatal (before birth) tests can help your doctor diagnose DWS, including:
1. Ultrasound (USG)
A type of test that can show whether there are any problems with how your baby’s brain is forming.
2. Fetal MRI (Magnetic Resonance Imaging)
A noninvasive imaging test to obtain clearer, more detailed images of the baby’s organs.
3. Other Tests
Some inherited conditions are caused by changes in the DNA, or genetic makeup, of a baby. Doctors may recommend genetic testing to learn more about your baby’s DNA, either during pregnancy or after birth.
The recommended tests may include:
- Cell-Free Fetal DNA Test
A screening test where a sample of your blood is taken to look for copies of the baby’s (fetal) DNA. A positive result means there may be a problem with the baby’s DNA. It must be confirmed with other tests. A normal result does not mean there is no underlying genetic problem. Other tests may be done before or after birth depending on your needs and the needs of your baby.
- Amniocentesis
A medical procedure in which a small amount of amniotic fluid is withdrawn and studied to screen for genetic problems. This test is usually performed in the middle of the second trimester. Amniocentesis can be used to confirm findings on a cell-free fetal DNA test.
- Testing After Birth
After birth, a sample of your baby’s blood is taken to look for chromosome problems. This is called a “karyotype” (a basic chromosome study), or a microarray (a more detailed look at smaller sections of DNA).
Care While the Baby is Still in the Womb
If tests reveal that you are carrying a baby with DWS, you may be referred to see a specialist from a multidisciplinary team including:
- Neonatologists – Doctors who specialize in caring for newborns.
- Neurosurgeon – A doctor who performs brain surgery
- Neurologist – A doctor who specializes in the brain
- Nurse
- Nurse coordinator – Helps answer your questions and concerns, guides you through your pregnancy, and prepares you for what to expect.
Your doctor will monitor your pregnancy closely. If the malformation on your baby’s head is very large, you may need to have a cesarean section.
You will be delivering your baby in a hospital that is set up to care for high-risk babies. You should discuss your birth plan with your obstetrician. Call your doctor right away if you have any concerns.
Postpartum Care
At the maternity hospital, a neonatologist will manage your baby’s care. The neonatologist will assess the baby’s breathing and heart rate. They will also examine the baby for other problems.
PagesNation Wide Childrenexplains some possible post-natal care below:
- Your baby may receive care in the maternity hospital’s neonatal intensive care unit ( NICU ) or be transferred to the main National Children’s NICU for further care.
- An intravenous (IV) line may be started in your baby’s arm, hand, foot, or scalp. The IV delivers fluids, medications, nutrients, and antibiotics to prevent dehydration and infection.
- Your baby may need to have an ultrasound or MRI. This will give the doctor more detailed information about your baby’s brain and other organs.
- A blood sample is drawn from the baby by a doctor to determine whether there are any genetic conditions that may affect the baby’s care and treatment.
- When the baby is able to breastfeed or even allowed to go home, it is still important to follow up with a complete examination or evaluation of the condition with the doctor. Meanwhile, if the baby is not able to immediately breastfeed directly or through a bottle, the doctor will usually advise the mother to pump and store breast milk until the baby is ready to start breastfeeding.
Possible Complications
MalformationDandy Walkerusually only affects the brain, but problems in other systems can cause heart defects, malformations of the urogenital tract, extra fingers or toes (polydactyly) or fingers or toes fused together (syndactyly), or abnormal facial features.
Nation Wide Childrens adds that children with DWS may also have facial or limb defects or problems with vision and hearing.
In rare cases, DWS may not be diagnosed until late childhood or adulthood. DWS can cause delays in motor and mental development.
The severity of symptoms and long-term outcomes vary from child to child. Some children require extensive medical care at birth, but go on to live normal lives with little or no long-term effects.
Other children may lose the ability to move parts of their body (paralysis), have seizures, and problems with thinking and learning.
Dandy Walker Syndrome Prevention
CanDandy Walker Syndromeprevented?
There is no known way to preventDandy Walker Syndrome. Consistent prenatal care provides the best chance for a healthy pregnancy. Follow your doctor’s recommendations for healthy living while you’re pregnant.
Republished with permission from theAsianParent Indonesia
