Description, Causes and Risk Factors:
Alternative Names: Dandy-Walker malformation (DWM), Dandy-Walker complex, hydrocephalus, internal, Dandy-Walker type, hydrocephalus, noncommunicating, Dandy-Walker type, and Luschka-Magendie foramina atresia.
Developmental anomaly of the fourth ventricle associated with atresia of the foramina of Luschka and Magendie, results in cerebellar hypoplasia, hydrocephalus, and posterior fossa cyst formation.
Dandy-Walker syndrome is a brain malformation involving the cerebellum and the fluid filled spaces around it. The brain contains four ventricles, which are inner, hollow portions filled with CSF (cerebrospinal fluid). The first and second (lateral) ventricles are inside the cerebral hemispheres, and the third and fourth ventricles are below them, closer to the brainstem. DWS consists of a specific group of brain malformations, including enlargement of the fourth ventricle, complete or partial agenesis of the cerebellar vermis (the middle portion of the cerebellum) and cyst formation and dilation of the posterior fossa (a small, hollow section between the lower cerebellum and skull).
Other characteristic of Dandy-Walker syndrome is blockage or closure of the foramina of Magendie and Luschka, two channels at the base of the brain through which CSF normally flows. When these openings are obstructed, CSF produced in the ventricles has no outlet for normal circulation. This causes fluid pressure to build, and the ventricles to enlarge.
The cause of Dandy-Walker syndrome is largely unknown. Although family (generic) occurrence has been reported, it is believed to be the result of multiple factors. Some predisposing factors might include exposure to rubella, cytomegalovirus, toxoplasmosis, and/or isotretinoin during the first trimester. Genetic factors also have a major role in the etiology of this condition. Dandy-Walker syndrome may occur as part of Mendelian disorders and chromosomal aberrations. Environmental factors including viral infections, alcohol and diabetes have also been suggested to play a role in the genesis of DWS, but the evidence is uncertain.
The disease occurs more frequently in females than males.
Symptoms of Dandy-Walker syndrome:
Some symptoms include
- Slow motor development.
- Vomiting and convulsions.
- Lack of muscle coordination.
- Jerky movements of the eyes.
- Problems with the nerves that control, eyes, face, and neck.
The majority of patients who have the classic Dandy-Walker syndrome are usually diagnosedwithin the first year of life. The symptomstend to be related to hydrocephalusand increased intracranialpressure. Diagnosing of DWS involve the use of imaging. The imaging technology used to diagnose this syndrome include magnetic resonance imaging (MRI), ultrasound and computed tomography (CT scan). Those who are diagnosed with this syndrome are most often diagnosed during their first year of life.
This syndrome is incurable so those with this syndrome will receive treatment that helps them to manage their symptoms and associated problems. Those who experience intracranial pressure, a special tube to reduce intracranial pressure may be placed inside the skull to control swelling. Other treatments include those for the symptoms of hydrocephalus and cerebellar agenesis, such as anti-seizure medications, and OT/PT for neuromuscular problems.
The Dandy-Walker syndrome is surgically treatable. The available treatment requires that a shunt from the interior of the brain or cyst is inserted to allow the continuous drainage of the blocked non flowing CSF. This shunt is a detour or bypass procedure that is made by placing a catheter in the ventricles, in the interior of the brain, or in the posterior fossa cyst. The fluid drainage is accomplished with a pressure-controlled valve, and usually the fluid is drained into the abdominal cavity where it is reabsorbed along the belly wall. Usually, the surgeon will connect both catheters to the same valve. The primary malformation of the brain, however, cannot be corrected, and these patients continue to have problems with motor coordination and balance as well as with learning to walk.
Many research has been going on that explore the complex mechanisms of normal brain development. The knowledge gained from these fundamental studies provides the foundation for understanding abnormal brain development and offers hope for new ways to treat and prevent developmental brain disorders such as DWS.
:The above information is general information (informational purpose only, sometimes may not be accurate). The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.