Dandy Walker Syndrome Treatment

To understand the possible Dandy Walker Syndrome treatment options, it is first necessary to perform a brief overview of this condition.

In the world of genetic disorders, Dandy-Walker Syndrome is perhaps one of the lesser known afflictions. Statistically speaking, this disorder affects every one in thirty thousand live births. Nonetheless, its symptoms are quite debilitating and the prognosis can often be difficult to ascertain until later in life.

Causes and Symptoms

Dandy-Walker Syndrome is a disease with genetic origins. While the mechanisms behind its occurrence are not widely understood, the main symptoms are a malformation of the cerebellum. This area of the brain is heavily involved in motor control as well as certain cognitive abilities such as attention span and memory. In this condition, parts of the brain are missing; particularly the area between the two halves of the cerebellum (linking the two) known as the cerebellar vermis. In addition, the enlargement of a ventricle allows for the accretion of cerebrospinal fluid to accumulate in these cavities. This can place pressure on the brain and cause further developmental damage.

Symptoms will vary but normally they will begin to manifest themselves during childhood. Poor cognition, hampered motor skills and enlargement of the growing skull are the most common. Should pressure within the skull increase, nausea, vomiting and lack of motor control will also become more prevalent as the child ages. Strange breathing patterns and loss of ocular control are also possible.

Treatment Options

It should be first stated that there is no known cure for this disease, as it is genetically induced. However, there are several Dandy-Walker Syndrome treatment options. The main action will be to introduce a surgical shunt into the back of the head or neck. This shunt will serve to drain the excess of accumulated cerebrospinal fluid. In turn, this will help reduce intracranial pressure; thus alleviating any symptoms caused by this fluid.

Notwithstanding this procedure, palliative treatment options are also quite prevalent. Such therapies can include but are not limited to:

  • Physiotherapy
  • Speech Therapy
  • Occupational Therapy
  • Specialised Educational Programs

More proactive treatments are also available for parents of children affected with Dandy-Walker Syndrome, particularly if they intend to have more children. As our knowledge and understanding of genetic predispositions increases, there are hopes that future alternatives will allow doctors to diagnose this condition while the fetus is still developing. Some scientists predict that genetically targeted drugs may help to reverse this syndrome while the child is still in the womb. However, it should be stressed that there are currently no possibilities to replace the parts of the cerebellum that are normally lacking due to this condition.

Prognosis

The ultimate prognosis will depend on the severity of the condition. As Dandy-Walker Syndrome treatment options are only intended to reduce symptoms, some children will be able to lead relatively normal lives and levels of cognition. Conversely, others may become learning impaired and require specialised attention throughout their adolescent and adult lives. Much of this will depend on the reduction of the fluid contained within the cranial cavity. Of course, a child may have a shorter life span if he or she is born with additional congenital defects. It is hoped that future treatment options may serve to mitigate these chances as medical science continues to progress.