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Present by: Zarith Naema

DEFINITION

A rare congenital malformation


that involves the cerebellum and
fourth ventricle. Dandy-walker
malformation is characterized by
agenesis or hypoplasia of the
cerebellar vermis, cystic dilatation
of the fourth ventricle, and
enlargement of the posterior fossa
DEFINITION

The key features of this syndrome are an enlargement of


the fourth ventricle (a small channel that allows fluid to
flow freely between the upper and lower areas of the brain
and spinal cord), a partial or complete absence of the area
of the brain between the two cerebellar hemispheres
(cerebellar vermis), and cyst formation near the lowest part
of the skull.
DEFINITION

The key features of this syndrome are an enlargement of


the fourth ventricle (a small channel that allows fluid to
flow freely between the upper and lower areas of the brain
and spinal cord), a partial or complete absence of the area
of the brain between the two cerebellar hemispheres
(cerebellar vermis), and cyst formation near the lowest part
of the skull. An increase in the size and pressure of the
fluid spaces surrounding the brain (hydrocephalus) may also
be present
FREQUENCY

Dandy-Walker malformation is estimated to


affect 1 in 10,000 to 30,000 newborns.
SYMPTOMS

Slowed motor development


• Kids with Dandy-Walker syndrome often experience delays in
motor skills like crawling, walking, balancing, and other motor
skills that require the coordination of body parts.

Progressive enlargement of the skull and bulging at its base


• Buildup of fluids in the skull can increase the size and
circumference of the skull over time, or cause a large bulge to
develop at its base.
SYMPTOMS

Symptoms of intracranial pressure


• The buildup of fluids can also contribute to increased
intracranial pressure. Although these symptoms are hard to
detect in babies, irritability, bad mood, double vision, and
vomiting may be indicators in older kids.

Jerky, uncoordinated movements, muscle stiffness, and spasms


• The inability to control movements, balance, or perform
coordinated tasks may suggest issues with the development of
the cerebellum.
SYMPTOMS

Seizures
• Approximately 15 to 30 percent people with
Dandy-Walker syndrome experience seizures.
CAUSE / FACTOR

Environment
• Exposure of the fetus to substances that cause
birth defects (teratogens) may be involved in
the development of this condition.
• Such as a mother with diabetes is more likely
than a healthy mother to have a child with
Dandy-Walker malformation.
CAUSE / FACTOR

Environment
• Exposure of the fetus to substances that cause
birth defects (teratogens) may be involved in
the development of this condition.
• Such as a mother with diabetes is more likely
than a healthy mother to have a child with
Dandy-Walker malformation.
TREATMENT

The surgical implantation of a shunt in the skull


• If your child experiences increased intracranial
pressure, doctors may recommend inserting a shunt
(a small tube) in the skull to help alleviate pressure.
This tube will drain fluid from the skull and carry it
to other areas of the body, where it can be safely
absorbed.
TREATMENT

Various therapies
• Special education, occupational therapy, speech
therapy, and physical therapy can help your child
manage the issues linked to Dandy-Walker syndrome.
Talk to your pediatrician about the therapies that
will be most effective for your child.

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