Arnold-Chiari syndrome is a rare malformation of congenital origin characterized by displacement of the cerebellum , bulb, and cerebral bridge down through the occipital foramen.
Hydrocephalus may also occur, consisting of cerebrospinal fluid (CSF) accumulation in the brain cavity.
Because of the herniation of the posterior part of the cerebellum and the trunk, the balance between the entry and exit of the blood is altered and causes CSF obstruction.
Classification of Arnold-Chiari syndrome
Arnold-Chiari syndrome type I
Generally, the signs can be observed during the childhood, between these exist:
- A severe pain in the neckthat extends downward along with a pain in the chest
- Difficulty of movement or lack of eye-hand coordination
- Stuttering, dizziness and blurred or double vision
- Tingling and numbness in hands and feet
- Difficulty swallowing that sometimes causes vomiting and suffocation
- Tinnitus in one or both ears
- Loss of bladder control ( incontinence )
- Abnormal curvature of the spine and abnormal breathing
Arnold-Chiari Syndrome Type II
This is one of the types of congenital malformation that develops along with the body.
This health problem is deeply linked to myelomeningocele or spina bifida cystica.
It is a neural tube defect characterized by the inability to develop the spinal cord.
The affected person suffers from:
- Headache that is often confused with migraine
- A feeling of pressure on the back of the neck
- If the doctor does not find the abnormality before delivery, the symptoms can also be confused with those of multiple sclerosis .
Symptoms of Arnold-Chiari syndrome
- Vision problems
Along with brain dislocation the affected individual may have the following symptoms:
Syringomyelia: It is a disease that causes the formation of a cavity inside the spinal cord.
Over time, the cavity increases and can damage the spinal cord.
Syringomyelia causes different spinal problems such as weakness , pain and stiffness .
Column curvature: is the situation where the spinal cord is misshapen and distances itself from its normal curvature.
Ehlers-Danlos Syndrome: A type of connective tissue disease, the consequences of Ehlers-Danlos syndrome are problems in the synthesis of collagen.
Collagen is a protein found in connective tissue.
Marfan syndrome is characterized by :
- Mitral valve insufficiency and prolapse
- Displacement of the eye lens
- Excessive growth of the long bones of the body.
The Marfan syndrome is inherited.
Diagnosis of Arnold-Chiari syndrome
Normally, magnetic resonance imaging (MRI) is used to diagnose the disease. If the displacement of the parts of the brain is greater than 5 mm down, the doctor diagnoses the Arnold-Chiari malformation.
The problem is usually discovered in young people, however, some of its characteristics can be observed even in the prenatal stages.
Surgery for Chiari Malformation
Surgery is the only treatment for this brain disease.
The most indicated type of surgery is ‘decompression’ surgery.
The purpose of the operation is to remove excess spinal fluid.
During the procedure, the neurosurgeon may need to remove the first three cervical vertebrae temporarily from the patient’s body.
It also removes the occipital bone.
This action allows the fluid to flow through a shunt, that is, a surgical rubber or plastic tube that helps drain fluids.
Treatment for Arnold-Chiari Syndrome
Treatment of this disease includes surgery, medications, and regular checks, depending on several factors, including the severity of the disease and the symptoms it causes.
In some cases, treatment is not necessary.
Sometimes the person does not realize that he is suffering from this health problem unless the symptoms become severe.
Surgical treatment is the most commonly used to treat symptomatic Chiari malformation.
The surgery aims to prevent the progression of anatomical changes in the brain and spinal canal.
The surgeon performs a craniectomy of the posterior fossa (decompression of the posterior fossa), a small part of the bone in the back of the skull is removed to create more space for the brain.
Then the surgeon makes an opening in the dura mater (ie the lining of the brain membrane) and a new patch is sewn to increase size and give more space to the brain.
The material used for the patch may be artificial or a tissue removed from the neck or leg.
In addition to alleviating the symptoms of the disease, surgery also reduces pressure on the brain and spinal cord and simultaneously restores the flow of the spinal fluid.
Any surgery in this part of the body causes a number of risks, surgery for Chiari malformation is no exception.
Possible risks in this case are:
The person may also have difficulty swallowing and abnormal eye movements. The chances of infection still persist and therefore the person should perform regular checks.
Average recovery time? The prognosis
The recovery period may vary from individual to individual, based on several factors.
It is necessary to weigh the potential benefits and risks and consult your doctor to see if this is the best alternative.
On average, the intervention takes 2-3 hours and hospitalization (hospitalization) is 2-4 days.