Dandy-Walker syndrome is a congenital developmental disorder with various courses. Particularly if diagnosed early, the syndrome is currently incurable but symptomatically treatable.
What is dandy-walker syndrome?
Dandy-Walker syndrome is a developmental brain disorder that is congenital. Occasionally, the terms Dandy-Walker complex or Dandy-Walker cyst are also found for Dandy-Walker syndrome. The syndrome is named after the neurosurgeon Dandy and the neurologist Walker, who first described and further defined the condition. Dandy-Walker syndrome is usually characterized by the presence of a malformation of the cerebellum (a part of the brain responsible, among other things, for controlling movements or coordination) in affected individuals:
The connection between the two cerebellar hemispheres that exists in healthy people is either underdeveloped or non-existent. In addition, Dandy-Walker syndrome usually shows an enlargement of the so-called 4th cerebrospinal fluid chamber of the brain. Here there is a possible accumulation of cerebral fluid, which can cause a so-called hydrocephalus in infants and young children. Such hydrocephalus is developed by a large proportion of children affected by Dandy-Walker syndrome.
Causes
Often, the causes that lead to the developmental disorder Dandy-Walker syndrome in individual cases cannot be clearly understood. However, both hereditary and nonhereditary contributing factors are possible. Hereditary (genetic) causes for Dandy-Walker syndrome may be, for example, other hereditary diseases transmitted to a newborn, which cause the malformations observed in Dandy-Walker syndrome. Non-hereditary causes of the presence of Dandy-Walker syndrome in a newborn may include factors such as the presence of rubella infection in the mother during pregnancy or the consumption of alcohol by the mother during the period of pregnancy.
Symptoms, complaints, and signs
Dandy-Walker syndrome is associated with a number of different complaints that can significantly complicate the life and daily routine of the affected individual. First and foremost, the syndrome causes significant disturbances in childhood development, so most patients continue to suffer from symptoms of the syndrome in adulthood. The head of the affected person is significantly enlarged, so that there can be aesthetic limitations, which have a very negative effect on the patient’s self-esteem. Especially in children, this often leads to bullying or teasing. Furthermore, patients also suffer from severe headaches, nausea and vomiting due to the dandy-walker syndrome, whereby these complaints usually occur without a recognizable reason. Disturbances of consciousness may also occur, with the affected person completely losing consciousness and possibly injuring himself in a fall. Furthermore, the syndrome is accompanied by severe visual disturbances, which, however, can be compensated by visual aids. In some cases, patients suffer from cramps that can occur in different parts of the body and in different muscles. The quality of life of the affected person is considerably limited and reduced by the syndrome. Frequently, the relatives or parents also suffer from severe psychological upsets or depression.
Diagnosis and course
Dandy-Walker syndrome can be diagnosed in newborns shortly after birth. Usually, this is done with the help of an ultrasound examination if there is any suspicion. The diagnosis of Dandy-Walker syndrome can be confirmed using methods such as magnetic resonance imaging (MRI) or computer tomography (CT). The course of the developmental disorder Dandy-Walker syndrome depends, among other things, on the severity of the disorder in the individual case. While Dandy-Walker syndrome can take a very severe course, it occasionally goes undetected for long periods of time. Hydrocephalus in the context of Dandy-Walker syndrome can lead to increased intracranial pressure in children at an older age; this manifests itself, for example, in nausea, headaches or visual disturbances. Frequently, in the course of Dandy-Walker syndrome, there are also impairments in mental development. As a rule, the earlier the disease is detected and treated, the better the prognosis for Dandy-Walker syndrome.
When should you see a doctor?
Usually, Dandy-Walker syndrome is a congenital complaint, so additional diagnosis does not need to take place in most cases. However, a doctor should be consulted early on, as early treatment can significantly reduce most complications and symptoms. Parents should see a doctor with their child when there are developmental disruptions due to Dandy-Walker syndrome. High intracranial pressure or constant headaches may also be indicative of the condition and should be investigated. Likewise, the syndrome can cause visual disturbances or nausea and vomiting, so a medical examination is also necessary for these complaints. Most children also suffer from cramps. The diagnosis of Dandy-Walker syndrome is usually made by a general practitioner. Further treatment is carried out by the respective specialists, so that all complaints are limited. The earlier the disease is diagnosed, the higher the chances of a complete cure of Dandy-Walker syndrome.
Treatment and therapy
According to current medical knowledge, Dandy-Walker syndrome cannot be cured. However, several therapeutic methods are available to treat the symptoms that may be associated with Dandy-Walker syndrome. As a rule, children suffering from Dandy-Walker syndrome undergo medical examinations at regular intervals. For example, increased intracranial pressure, which can develop due to the accumulation of cerebrospinal fluid in the 4th cerebral ventricle, can be detected early. If such increased intracranial pressure is detected in a child affected by Dandy-Walker syndrome, the cerebrospinal fluid can be drained with the help of a tube in one treatment step. Such an intervention is performed by a trained specialist. If a person affected by Dandy-Walker syndrome suffers from impaired balance, this can often be positively influenced by specific physiotherapeutic (physiotherapy) measures. Seizures, which in Dandy-Walker syndrome can be the result of increased intracranial pressure, are often treated by the administration of so-called antiepileptic drugs (medications that can resolve acute seizures and prevent new seizures).
Outlook and prognosis
A complete cure cannot be achieved in Dandy-Walker syndrome. Therefore, affected individuals must rely on purely symptomatic treatment to relieve symptoms. If the syndrome is not treated, the affected children suffer from severe developmental disorders and also high intracranial pressure, resulting in headaches. There are visual problems and disorders of consciousness, and convulsions or even epileptic seizures may also occur. The patient’s quality of life is significantly reduced by this syndrome if it is not treated. Due to the symptoms, many patients and their relatives or parents suffer from severe psychological upsets or depression. Treatment always depends on the exact symptoms and is primarily aimed at reducing the intracranial pressure. Surgical intervention is necessary, but this is not associated with any particular complications. The other complaints are alleviated with the help of medication and physiotherapy measures. Although this does not result in a complete cure, the patient’s quality of life is significantly improved. As a rule, life expectancy is not reduced if the syndrome is treated. Early treatment has a very positive effect on the further course of the disease.
Prevention
Dandy-Walker syndrome is difficult to prevent, among other reasons, because the exact causes for the development of the developmental disorder are usually unclear. General measures that women of childbearing potential can consider to prevent Dandy-Walker syndrome in their newborns include early rubella vaccination and abstaining from alcohol consumption during pregnancy. If genetic disorders are known to run in the family, prenatal diagnosis is also possible.
Follow-up
Because Dandy-Walker syndrome is a congenital disease, it cannot be completely cured, so the options for follow-up care are also severely limited.First and foremost, very early detection and treatment of the disease is also important in order to prevent further complications. If a patient with Dandy-Walker syndrome wishes to have children, genetic testing and counseling can also take place to prevent the syndrome from being passed on to the children. In this regard, parents of affected children should contact a doctor at the first signs or symptoms. This can prevent damage to the brain. The treatment of Dandy-Walker syndrome is usually done by surgery. After such an operation, the patient should in any case rest and also take care of his body. In any case, the patient should refrain from exertion or stressful and physical activities. In case of a cramp, an emergency doctor should be called or the patient should go to the hospital. In general, the help and support of one’s family and friends also has a positive effect on the course of the disease. There may also be a reduced life expectancy of the affected person due to dandy walker syndrome.
What you can do yourself
Dandy-Walker syndrome always requires medical diagnosis and treatment. The mostly symptomatic therapy can be supported by some measures. First, however, it is important to have the sick child examined by a doctor regularly so that increased intracranial pressure can be detected early. Independently of this, sports and physiotherapeutic exercises can be used to counteract possible seizures and balance disorders. A healthy and balanced diet can reduce the risk of corresponding symptoms and also improve the general well-being of the sufferer. Later in life, other symptoms may be added, often leading to the exclusion of those affected. Parents of children with the disease can counteract this by promoting the child’s self-confidence at an early stage. Therapeutic measures are a necessary complement to this, through which the affected person can better understand and accept the disease. Affected parents can also find suitable contact persons within the framework of therapy and can also exchange information with other affected persons. Any measures should be worked out together with the pediatrician in charge. Should unusual behavior occur in the child, a specialized clinic for congenital developmental disorders must be consulted in any case.
