Dandy Walker varient

[Jamil1946]

My grand daughter ( born on 19th December 2006 ) who is 22 months old at present, is having problem in controling her movements. She even can not hold her neck. MRI report dated 17-1-2008 reads as under :-
"There is paucity of white matter with thinning of corpus callosum. Posterior fossa shows large cystic area not communicating with the 4th. ventricle. Ventricular system is normal. No evidence of intra / extra axial hemorrhage. There is no evidence of recent or remote infarct. Pituitary gland and optic chiasm are normal. 7th & 8th nerve complexes are normal & symmetrical bilaterally.
Impression :- MR features described favour white matter hypoplasia with Dandy Walker variant in the posterior fossa. Comparison with the previous study of May 2007 shows no interval change".
She can neither roll over nor holds something. Other audio visual senses are normal.Can You kindly help us in guiding as what to do in treatment of the baby. Thanks.

[Jamil1946]

Photo added with the question has been inadvertantly sent which may kindly be removed. Thanks . JAMIL

[DrNoopurMD]

Hi,

Thanks for writing in.
Variant type of Dandy walker syndrome is usually a mild type of DWS and is characterized by wide ranging symptoms.
http://en.wikipedia.org/wiki/Dandy-Walker_syndrome
I would suggest you to consult an experienced neurologist. The prognosis of these children depends upon the severity of symptoms and treatment is usually focused around treatment of symptoms. These children usually present with hydrocephalus and then a shunt surgery is done which improves some of the symptoms.
Please seek help from a good neurologist in your area. Take care!

[Jamil1946]

Hi Doctor Sahiba
Thanks for your valuable opinion regarding my grand daughter. You might have gone through the MRI report which indicates that paucity of white matter may represent Dandy Walker Syndrome. Although a large cystic area is seen in the posterior fossa communicating with the 4th ventricle, yet no specific syndrome could be determined as yet. No sign of fluid is noticed in the cyst therefore possibility of hydrocephalus is also not there. We had shown the MRI report to the surgeon of the Shifa International Hospital Islamabad who after examining the patient advised that surgical intervention is not required. The condition of the patient is that she neither holds her neck nor can she move her limbs at will. The only improvement after getting physiotherapy for one year from a qualified doctor (at the age of  22months) is that her joints are movable besides some intake betterment. Recurrent chest infection attacks are now decreasing and span of remaining infection free has been increased to some extent. Consultant doctors in the paediatrics have instead of diagnosing specific treatment have advised to “wait and see” along with symptomatic treatment. Will you kindly advise as to whether any medicine can improve the brain functions especially motor functions?
Thanks.

[DrNoopurMD]

Hi,
How are you?

The best way to improve motor functions will be to continue with the physiotherapy. Since she has responded well so far it will benefit her now also.
It is difficult to prescribe a straightforward treatment because there is no medication available to treat dandy walker syndrome. It is a good sign that she does not have hdrocephalus. I would suggest you to seek a second opinion on this as first it is important to make the correct diagnosis and then the course of treatment can be decided upon.
Let us know if you need more information.