Contents:
- Medical Video: What is Dandy Walker Syndrome?
- What is Dandy Walker syndrome?
- Symptoms and characteristics of Dandy Walker syndrome
- Causes of Dandy Walker syndrome
- Diagnosis and treatment for Dandy Walker syndrome
Medical Video: What is Dandy Walker Syndrome?
Dandy Walker syndrome was first discovered by Walter Dandy and Arthur Walker in 1914. This abnormality is very rare, estimated to affect only one in 10,000 to 30,000 new births. What are the characteristics and how are they handled? See below.
What is Dandy Walker syndrome?
Dandy Walker Syndrome is a congenital brain disorder that involves the fourth ventricle and cerebellum. This syndrome is characterized by an enlarged fourth ventricle, the absence of cerebellar vermis, the posterior midline area of the cerebellar cortex which is responsible for coordination of body movements.
Various parts of the cerebellum develop abnormally, resulting in defects seen through scans. The middle part of the cerebellum (vermis) is absent or very small and may be in an abnormal position.
The fluid-filled cavity between the brain stem and cerebellum (fourth ventricle) and the skull that contains the cerebellum and brain stem (posterior fossa) is very large. This condition can cause hydrocephalus, or enlargement of fluid in the brain.
Actually, this disorder can be accompanied by hydrocephalus, or not. However, several studies show that 70-90 percent of sufferers experience hydrocephalus, although the symptoms may only appear after birth. This disorder often results in movement disorders, coordination, thought processes,moodand other neurological functions.
Symptoms and characteristics of Dandy Walker syndrome
Some of the symptoms that may be experienced by babies with this syndrome are:
- Motor development disorders in infants such as crawling, walking, and coordinating.
- Hydrocephalus.
- Easy to fuss and hard to calm.
- Nausea and vomiting.
- Seizures.
- Lack of muscle coordination.
- Eye jerks.
- There are abnormalities in the face, limbs, and heart.
Causes of Dandy Walker syndrome
This syndrome has been associated with chromosomal abnormalities, namely the addition of a copy of one chromosome in each cell (trisomy). This condition is most common in people with trisomy 18 (an extra copy of chromosome 18), but can also occur in people with trisomy 13, trisomy 21, or trisomy 9. This condition can also be associated with deletions or copying (duplication) pieces certain chromosomes.
In addition, other studies have shown that Dandy Walker syndrome can be caused by environmental factors that affect early development before birth. For example, exposure of the fetus to substances that cause birth defects (teratogens). In addition, mothers with diabetes are more at risk of having children with Dandy Walker syndrome, compared to healthy mothers.
Diagnosis and treatment for Dandy Walker syndrome
This syndrome can be diagnosed through MRI examination, CT scan, angiography, and ultrasonography (USG).
Hydrocephalus associated with Dandy Walker syndrome can be treated with surgery to insert a tube that aims to direct the fluid that surrounds the brain and helps drain fluid into other parts of the body that can absorb fluids.
Some treatment therapies that can be done include special education, physical therapy and other medical, social or vocational services. Genetic counseling is also recommended for family members who have children with this syndrome.
The life expectancy of a baby with this syndrome depends on the severity of his condition. Because the symptoms or complaints of this disease are very serious, the child with Dandy Walker syndrome may have a shorter life span. This condition can even result in lifelong complications that cause an increased risk of death more quickly.
