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Neurology (Expert Forum)
Son with Dandy Walker
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Son with Dandy Walker
by dedicated mom, Aug 01, 2002 12:00AM
What is the difference between dandy walker and joulbert syndrome? My son has a dandy walker malformation/cyst 4th vent. with hydro. partial agenisis of the vermis. We have followed up with the ped. neuro. and with surgeons, no shunt, we are looking into a procedure called third ventriculosomy. He is turning a year old next week, not crawling but tring and he is very alert and smart, we do PT twice a week and now we will be starting OT once a week. Have you heard of seizures in children with hydro?. I have read Polycystic kidneys are associated with dandy walker and joulbert syndrome, now my son has not been tested for polycystic kidneys yet, but my uncle passed away at the age of 29 because of polycystic kidneys, is this something that is heritary? And what are my chances of having another child with dandy walker? I love my son to dealth and i am taking this all in day by day. I do alot of research and always have questions, my therory is to always be prepared for all possibilities, hopefully they will not be as severe as i have read about. My heart and blessings go out to all parents with children with disabilities.
Doctor's Answer
by CCF-Neuro-M.D.-JT, Aug 06, 2002 12:00AM
1.Joubert syndrome is similar to Dandy Walker in that there is vermal agenesis, but it also includes a characteristic face that docs can recognize, eye movement problems, and difficulty breathing due to problems with the respiratory centers of the brain. These do not appear to be associated with Dandy Walker.
2.It is uncommon but possible to get seizures from hydrocephalus.More common when there is a shunt.
3.As for the polycystic kidneys, there is a big difference between the polycystic kidneys that are associated with syndromes such as Dandy Walker and the more common Adult POlycystic Kidney disease that is seen in the general population. The former is just associated with the congenital syndrome whereas the latter is something different in that it is seen in patients who are apparently normal and is a disease all its own. Adult Polycystic kidney disease is hereditary in what's called an "autosomal dominant" fashion, which means that if you yourself have the disease, your child has a 50% chance of getting it. But if you or your spouse do NOT have the disease, then your child theoretically is safe from adult polycystic kidney disease (but not necessarily polycystic kidneys in association with congenital disease).
Hope that helps, and happy birthday to your son.
2.It is uncommon but possible to get seizures from hydrocephalus.More common when there is a shunt.
3.As for the polycystic kidneys, there is a big difference between the polycystic kidneys that are associated with syndromes such as Dandy Walker and the more common Adult POlycystic Kidney disease that is seen in the general population. The former is just associated with the congenital syndrome whereas the latter is something different in that it is seen in patients who are apparently normal and is a disease all its own. Adult Polycystic kidney disease is hereditary in what's called an "autosomal dominant" fashion, which means that if you yourself have the disease, your child has a 50% chance of getting it. But if you or your spouse do NOT have the disease, then your child theoretically is safe from adult polycystic kidney disease (but not necessarily polycystic kidneys in association with congenital disease).
Hope that helps, and happy birthday to your son.
