Arnold Chiari Malformations are some kind of a neurological abnormality seen in children and can be associated with a wide range of symptoms. This abnormality can be diagnosed and treated by the most skilled doctors in Turkey.
What is Arnold Chiari Malformations?
Arnold Chiari syndrome was first described by Dr. Hans Chiari in 1890. Later, Dr. Jules Arnold described the syndrome more clearly, That is way it is called the Arnold Chiari malformation.
The deformity is thought to occur during fetal life due to genetic mutations or the mother's lack of nutrients during pregnancy.
The Malformation occurs when a part of the lower brain (cerebellum) descends toward the vertebral canal through the foramen magnum due to the small bony space at the base of the skull, which leads to high pressure of the cerebellum and falls down.
This falling down causes blockage of cerebrospinal fluid drainage, causing Hydrocephalus .
Types of Arnold Chiari Malformations
Chiari malformation is classified into four major types according to the degree of decline, symptoms, and the presence of associated neurological abnormalities.
Type I of Chiari Malformations
The size of the skull is small, which leads to high pressure during the development of the skull while the child is growing. This type is seen in the late childhood stages (adolescence), where the pressure increases and the cerebellum descends downward through the large foramen towards the vertebral canal. This type can occur due to acquired causes that raise pressure (such as infections and drugs) and this is the least dangerous type of chiari Malformations.
Type II of Chiari malformation
In this type, a greater amount of cerebral tissue descends through the foramen magnum towards the vertebral canal. We see the decline of the cerebellum and part of the brainstem in younger children, and this type is usually associated with another neurological malformation affecting the spine that has the name of myelomeningocele, which is a type of spina befida spina bifida spina bifida.
Arnold-Chiari syndrome type III
It is the most severe type where the cerebellum and brainstem descend through the magnum fossa into the vertebral column. This type is rare and has poor prognosis and is diagnosed in newborns or during pregnancy using ultrasound.
Type IV Arnold-Chiari Malformation
This type includes an underdeveloped cerebellum, the cerebellum is in its normal position, but parts of the cerebellum are underdeveloped. This type is the rarest of all types.
Symptoms of Arnold Chiari Malformations
The malformation can be asymptomatic, detected by chance and does not require treatment, and this is often in type 1 Chiari malformation, sometimes it may be associated with many symptoms, especially those related to cerebellar functions. We mention some of the symptoms seen in children with Arnold Chiari syndrome:
- Headache and pain in the neck and at the base of the head, especially when coughing
- Balanced issues
- numbness and tingling
- Poor muscle coordination
- Loss of feeling in the hands or feet
- Some have visual problems
- ringing in the ear
- speech difficulties
- Dysphagia
Other symptoms related to the functions of the cranial nerves located in the brain stem can be seen in the most dangerous patterns, such as the third type, and these signs are an indication of a neurological injury.
Arnold Chiari Diagnosis
The diagnosis of Arnold Chiari Syndrome can be done using modern imaging techniques, foremost of which is the MRI of the head, which is the best in the diagnosis.
A 3D Head CT scan may also be used.
When diagnosing the patient, the need for intervention and disease prognosis can be determined.
How to treat Arnold Chiari syndrome in Turkey
Treatment is mainly aimed at relieving symptoms and improving quality of life. Treatment of Chiari malformation depends on the severity of the condition and symptoms. If there are no symptoms, no treatment is required. In the case of severe symptoms, the intervention must be done as soon as possible.
In mild cases of the disease, symptoms such as headaches can be treated with analgesics only as an symptoms treatment.
The basic treatment is the surgical procedure that is carried out in advanced centers and with a selection of qualified doctorsTurkey One of the best examples.
In fact, the main purpose of the surgical procedure is to relieve pressure and drain the cerebrospinal fluid, and to do this we resort to the following well-known technique:
Decompression Surgery
It is the best surgical procedure, which depends on removing a small part of the base of the skull, and we may suffice with this amount to relieve pressure.
In some cases, the surgeon open the dura and expose the cerebellar tonsils, so that the surgeon can reduces the size of the tonsils by electrocautery, which leads to pressure relief and drainage of cerebrospinal fluid.
The decision to open an epidural depends on several factors related to the condition, including the symptoms seen in the patient, his age, and the presence or absence of an accompanying disease such as syringomyelia.
The surgeon may use real-time Dopplar ultrasound during the procedure to determine if an epidural is needed or not.
Arnold Chiari's surgery (decompression surgery) that involves opening the epidural takes about 3 to 4 hours, while the procedure without opening the epidural takes about two hours.
Surgical techniques for draining CSF
Endoscopic third ventricleostomy (ETV)
One of the advanced surgical techniques, where the surgeon inserts a small camera into the brain through a small hole in the skull and makes a small hole in one of the ventricles, which leads to pressure relief, you can watch the surgery from Here.
Some patients complain of headache and nausea for several days after the procedure.
cerebral shunt
It is a good technique to use for hydrocephalus.
As a result of excessive fluid accumulation, a shunt is placed that draws fluid from the brain and places the other end of the catheter in the peritoneal cavity.
The shunt may be associated with complications such as infection to the brain or mechanical problems such as blockage of the shunt or displacement of the shunt from its place.
risks of Arnold Chiari surgery
In fact, no surgical procedure is completely risk-free. The risks of Chiari malformation surgery include:
- Heavy bleeding as a result of an artery injury and hematoma formation
- CSF leak
- Infection or stroke
- Anesthesia related problems
- cranial nerve injury
- In some cases, symptoms do not improve after surgery
Chiari malformation can be associated with spinal injuries due to compression of the spinal cord in the cerebellum and lower part of the brain. spine surgery.
Preparation before Arnold Chiari surgery
The doctor must be aware of the medications the patient is taking. NSAIDs and anti-inflammatory drugs (aspirin) should be stopped at least one week before the surgery, and alcohol and smoking should be avoided at least one week before the operation.
Avoid eating or drinking the night before the operation to prevent the risk of resorption and inhalation of food through the digestive tract that occurs during anesthesia.
Recovery after Arnold Chiari sugery
The recovery time from the surgery varies according to the patient's health and usually takes from 4 to 6 weeks.
Full recovery from symptoms may take months, and a follow-up MRI of the brain should be done six months or a year after the surgery.
Some patients suffer from post surgery headaches that last for several days and can be treated with analgesics. After the surgery, the patient is prevented from any activity that raises pressure, such as lifting heavy weights and he should avoid bending while lowering the head.
Constipation should be prevented by drinking plenty of water, eating foods rich in fiber, avoid prolonged coughing, and preventing the patient from any strenuous activity such as housework.
The patient after surgery should not drink alcohol because alcohol increases the risk of bleeding and it is preferable to avoid smoking and driving a car.
You should not take NSAIDs or thinners without consulting your doctor.
success rate of Arnold Chiari surgery
In fact, the success rate of decompression surgery according to numerous studies reaches about 85%, which means that the surgery is fairly safe.
85% to 95% of the patients have their symptoms disappear after decompression surgery. The success of the surgery depends on the patient, and if the surgeon opens the epidural or only removes part of the base of the skull.
how common is Arnold Chiari?
Arnold-Chiari type 1 malformation is the most common type of chiari malformations
The incidence of Arnold chiari is estimated at 1 in every 1000 births, and it is often asymptomatic and detected by accident when imaging is performed for other reasons. Females are affected more than males in a simple 1.3:1 ratio.
The second type of Arnold-Chiari syndrome is associated with other neural tube abnormalities and occurs particularly with a congenital abnormality called myelomeningocele.
Arnold-Chiari syndrome type II is almost always associated with myelomeningocele, a type of a spina bifida.
spina bifida is a serious congenital deformity that requires urgent intervention after birth.
Arnold-Chiari Syndrome Type III is the most serious but rare type, while Arnold-Chiari Syndrome Type IV is the rarest.