Arnold Chiari malformation, also known as a tonsillar hernia or tonsillar ectopy, is an abnormality in which the cerebellar tissue extends into the spinal canal, due to the skull being particularly small or misshapen.
In other words, the condition occurs when the cerebellum (the part of the brain that controls muscle coordination and movements) invades part of the space normally occupied by the spinal cord.
Chiari malformation is classified into three main types (I, II and II), defined according to the anatomy of the brain tissue that moves towards the spinal canal and by the abnormalities that eventually appear in the development of the brain or spine.
Symptoms
Patients with type I Chiari malformation often suffer from headaches, usually severe, mainly after coughing, sneezing, or sudden straining, in addition to other conditions such as:
- Dizziness
- Neck pain
- Balance problems
- Limitations on your fine motor skills
- Numbness and tingling in the hands and feet.
- Difficulty swallowing, nausea, choking, and vomiting
- Hoarseness
The patient may also experience ringing in the ears, feel weak, have a slow heart rate, breathe abnormally, and show a curvature of the spine related to spinal cord dysfunction.
In type II malformation, signs and symptoms may be related to a form of spina bifida (myelomeningocele) that occurs when the spine and spinal canal did not close properly before the person was born.
In the presence of Type II, the patient may experience changes in breathing rate, swallowing difficulties, rapid eye movements, and arm weakness.
Type III is the most serious malformation, because it can cause neurological problems and because, in statistical terms, it presents the highest mortality rate.
Treatment
Chiari malformation is rare, and many people who have it do not have symptoms or need treatment.
Type I malformations develop as the skull and brain grow, and types II and III malformations are congenital. Generally, it cannot be diagnosed until the patient’s later childhood or adolescence.
When the patient presents symptoms, neurosurgeons focus the treatments of the syndrome on alleviating pain (head and neck, for example) and stopping the lesions that present in the brain and spinal cord.
We also usually recommend that the patient reduce activities that make symptoms worse.
But, if the symptoms get worse, we consider the possibility of performing a surgical intervention in order to reduce the pressure exerted on the brain and spinal cord, restore the circulation of the cerebrospinal fluid and prevent subsequent injuries.
Treatment may consist of decompression or implantation of a bypass valve to help drain fluid and relieve pressure on the brain.
In any case, it must be borne in mind that Chari’s malformation has no cure; that the treatment helps to reduce the problems and that, before practicing an operation, we must observe that the intervention can be painful and expose the patient to infections and other complications.
Feel free to ask about Arnold Chiari syndrome and treatments that we provide in Costa Rica. We can schedule an appointment for an assessment.
