Dandy-Walker Syndrome

Dandy-Walker syndrome, also known as both Dandy-Walker malformation and/or Dandy-Walker complex, is a collective set of brain deformations. The disease is named after renowned medical doctors Arthur E. Walker and Walter E. Dandy, who recognized the disease in the early 20th century. Essentially, the brain is composed of four ventricles, that are filled cerebospinal fluid. The first and second ventricles are located within the cerebral hemispheres, and the third and fourth ventricles are located underneath them respectively. With Dandy-Walker syndrome, the following brain abnormalities occur: the fourth ventricle is severely enlarged; there is a lack of development of the cerebellar vermis; and a cyst forms and becomes dilated around the posterior fossa. Additionally, Dandy-Walker syndrome causes a blockage of the channel betwee the Magendie and Luschka, where cerebospinal fluid flows into the brain stem and thus, such a blockage is critical. Since the cerebospinal fluid is not able to circulate throughout the body, an increase in pressure within the ventricles occurs.

Dandy-Walker commonly leads to what is known as hydrocephalus, essentially an increased amount of fluid level within the brain. Though Dandy-Walker syndrome is not necessarily a well-known disease, it is relatively prevalent within newborns, as approximately one in every one-thousand newborns is infected with hydrocephalus, with Dandy-Walker syndrome being a leading cause.

Currently, the precise cause of Dandy-Walker syndrome is unknown within the medical community, though it is hypothesized that it is a developmental disruption that occurs during the embryonic stage, leading to the abnormalities of the syndrome.

Dandy-Walker syndrome is not known to be very hereditary, nor does there seem to be any preventive measures for it, instead, the disease is relatively random. Those infected with Dandy-Walker syndrome often have a large bump/bulge in the back of their skull, and a larger overall head-size. Potential symptoms for infants include: vomiting, irritability, seizures, abnormal breaching patterns, nystagmus, and slowed development. Symptoms more prevalent in older children and adults that are infected with the disease include: lack of coordination, severe headaches, mental retardation, and/or vision problems.

Dandy-Walker syndrome is unique, in that it can be diagnosed in the womb, as early as 12-24 weeks post-conception, though ultrasounds tend to reveal more. The diagnosis of Dandy-Walker syndrome is often confirmed using the ultrasounds, as they are more accurate. Additionally, it is possible for the parents to opt for amniocentesis, which is essentially a fetal chromosome test. Using the given symptoms, it is also possible to diagnosis the disease post-birth using an MRI and CT brain scan.

The treatment for Dandy-Walker syndrome is somewhat rigorous, as there are multiple aspects of the disease. Often, there are a dozen medical doctors in the room performing surgery and assisting on the placement of shunt in a ventricle of the brain, and in the abdominal cavity. This tube essentially regulates the cerebospinal fluid, so that there is not an excess build-up in the peritoneal cavity. At times, neurosurgery may also have to be performed, in which the surgeon inserts an endoscope into the brain, and creates an opening between the third and fourth brain ventricles. Though they don't necessarily address the root of the disease, only the symptoms, anti-seizure medication and other medicine are at times administered. The treatment of Dandy-Walker syndrome is a split success-rate, some children fully recover with the assistance of the shunt, wheras others gain further infection.