Download Dandy Walker Syndrome

Dandy-Walker Syndrome
Fact Sheet
By Shelly Sumner
Description
Dandy-Walker syndrome is a
congenital disorder involving brain
malformation and is a common
cause of hydrocephalus (increased
fluid in the brain. The malformation
involves the cerebellum an area in
the back of the bran that controls
movement the fluid filled spaces
around it and an enlargement of the
fourth ventricle (a small channel that
allows fluid to flow between the
upper and lower areas of the brain
and spinal cord). It also involves a
partial or complete absence of the
cerebellar vermis (area between the
two cerebellar hemispheres which is
important for coordination and
voluntary muscel movements, and
cyst formation near the base of the
skull. The syndrome has a wide
range of severety. Some people may
not show any symptoms while others
result in severe disabilities or even
death.
Visual System Affected
Common vision problems include:
 nystagmus (involuntary back and
forth movement of the eyes).
 Strabismus: misalignment of the
eye, surgery may improve eye
alignment.
 Cataracts
 Retinal dysgenesis: abnormal
formation of the retina
 Chorid Coloboma: abnormal
formation of the eye between the
sclera (white part of the eye) and
the retina.
 Cortical Visual Impairment
Effects on the condition
of the visual system
Vision problems can vary from mild
visual deterioration to severe vision
loss. Vision problems may also affect
perceptual abilities such as eye hand
coordination. Children with
hydrocephalus (increased fluid
pressure in the brain) may also have
trouble with visual motor skills such
as trouble judging distance and
direction or seeing and organizing a
sequence of movements or have
trouble separating relevant
information from a background which
can affect their ability to interpret
meaning from pictures.
Common Treatments
The primary treatment for DandyWalker syndrome is associated with
hydrocephalus is placing a shunt in
the brain to drain the excess fluid
which can be reabsorbed by the
body. Other treatments include the
use of anti-seizure medication,
OT/PT and speech therapy if
needed, and surgery to correct the
eye alignment.
Congenital or
adventitious and
prossressive or stable
Anticipated Functional
Implications of the
condition:
Dandy-Walker syndrome is
congenital (present at birth). The
occurance is isolated and sporadic
and have little risk of recurrence. It
may be inherited as an autosomal
recessive trait which would imply a
25% chance of recurrence.
The effects of the syndrome vary.
Some children will have normal
cognition while others may have
severe disabilities and a shortened
life span.
References
Dandy Walker Alliance. (2010). DW basics. Retrieved from
http://www.dandy-walker.org/blog/dw-basics/
Elquist, M. & Demchak, M.A. Fact Sheet- Dandy Walker Syndrome. Colorado
Services to Children and Youth with Combined Vision and Hearing Loss
Project. Retreived from
Klein, O., et al. (2001). Dandy-Walker Malformation: Prenatial Diagnosis and
Prognosis. Childs Nervous System. 19 (August 2001), 484-9.
National Institute of Neurological Disorders and Stroke, (May 2010). NINDS
Dandy-Walker Syndrome Information Page, Retrieved from
http://www.ninds.nih.gov/disorders/dandywalker/dandywalker.htm
Polzin, S. J. (2005). Dandy-Walker Syndrome Health Article. Gale Encyclopedia
of Neurological Disorders. Retrieved from
http://www.healthline.com/galecontent/dandy-walker-syndrome/2
Yilmaz, A. M. DR., Real Brain Section. Dandy-Walker Homepage Personal Web
Page on DWS. Retrieved July 2010 from
http://www.dandywalker.net/index.php?option=com_content&task=view&id
=4&Itemid=32